SURGERY NOTES Adapted from Andre Tan

1

1. TRAUMA & PERIOPERATIVE CARE 1. ADVANCED TRAUMA LIFE SUPPORT 2. ABDOMINAL TRAUMA 3. CARDIOTHORACIC TRAUMA4. NEUROSURGICAL TRAUMA 5. MUSCULOSKELETAL TRAUMA 6. POST-OPERATIVE COMPLICATIONS 7. SHOCK 8. PERIOPERATIVE CARE 2. LUMPS & BUMPS 1. HERNIA 2. SCROTAL SWELLINGS 3. APPROACH TO LUMPS & BUMPS 4. LIPOMA 5. SEBACEOUS CYST 6. GANGLION 7. BASAL CELL CARCINOMA 8. SQUAMOUS CELL CARCINOMA 9. MALIGNANT MELANOMA 10. NEUROFIBROMA 11. DERMOID CYST 12. SEBORRHOEIC KERATOSIS 13. HAEMANGIOMA 14. PYOGENIC GRANULOMA 15. PAPILLOMA 16. KERATOCANTHOMA 17. KELOID (HYPERTROPHIC SCAR) 18. KAPOSI’S SARCOMA 19. FIBROSARCOMA 20. PYODERMA GANGRENOSUM 21. RADIOTHERAPY MARKS 3. SURGICAL INSTRUMENTS & PROCEDURES 1. DRAINS 2. CENTRAL VENOUS PRESSURE LINE 3. NASOGASTRIC TUBE 4. TRACHEOSTOMY 5. SENGSTAKEN-BLAKEMORE TUBE (MINNESOTA

TUBE) 6. URINARY CATHETERISATION 7. CHEST TUBE 4. ABDOMINAL SURGICAL EMERGENCIES 1. APPROACH TO ABDOMINAL PAIN 2. APPROACH TO ABDOMINAL MASSES 3. INTESTINAL OBSTRUCTION (& MECKEL’S

DIVERTICULUM) 4. ISCHAEMIC BOWEL 5. ACUTE APPENDICITIS

5. OESOPHAGEAL DISEASES 1. ANATOMY OF THE OESOPHAGUS 2. PHYSIOLOGY OF SWALLOWING 3. APPROACH TO DYSPHAGIA 4. OESOPHAGEAL CANCER 5. GASTRO-OESOPHAGEAL REFLUX DISEASE 6. BARRETT’S OESOPHAGUS 7. ACHALASIA 6. UPPER GIT 1. APPROACH TO BLEEDING UPPER GIT 2. VARICEAL BLEEDING 3. PEPTIC ULCER DISEASE 4. GASTRIC CANCER 7. LOWER GIT & COLORECTAL DISEASES 1. APPROACH TO BLEEDING LOWER GIT 2. COLORECTAL CANCER 3. STOMAS 4. DIVERTICULAR DISEASE 5. INFLAMMATORY BOWEL DISEASE (CROHN’S

DISEASE, ULCERATIVE COLITIS) 8. ANAL & PERIANAL DISORDERS 1. HAEMORRHOIDS 2. ANAL FISTULA 3. ANAL FISSURES 9. LIVER DISEASES 1. ANATOMY OF THE LIVER 2. CAUSES OF HEPATOMEGALY 3. CAUSES FOR A LIVER NODULE ON IMAGING 4. HEPATOCELLULAR CARCINOMA 5. LIVER METASTASES 6. LIVER HAEMANGIOMA 7. SIMPLE LIVER CYSTS 8. HEPATIC ABSCESS (PYOGENIC / AMOEBIC) 9. ASCITES 10. PANCREATIC DISEASES 1. ACUTE PANCREATITIS 2. CHRONIC PANCREATITIS 3. PANCREATIC CANCER

2

11. BILIARY TRACT DISEASES 1. CAUSES OF JAUNDICE 2. APPROACH TO OBSTRUCTIVE JAUNDICE 3. GALLSTONE DISEASE 4. ACUTE CHOLECYSTITIS 5. GALLBLADDER CANCER 6. CHOLEDOCHOLITHIASIS 7. CHOLANGITIS 8. MIRIZZI’S SYNDROME 9. RECURRENT PYOGENIC CHOLANGITIS 10. CHOLANGIOCARCINOMA 12. BREAST DISEASES 1. ANATOMY OF THE BREAST 2. PRESENTATION OF BREAST DISEASES 3. APPROACH TO A BREAST LUMP 4. APPROACH TO NIPPLE DISCHARGE 5. BREAST CANCER 6. PAGET’S DISEASE OF THE NIPPLE 7. GYNAECOMASTIA 13. NECK MASSES 1. ANATOMY OF THE NECK 2. THYROGLOSSAL CYST 3. DERMOID CYST 4. PLUNGING RANULA 5. BRANCHIAL CYST/FISTULA 6. CHEMODECTOMA 7. PHARYNGEAL POUCH 8. CYSTIC HYGROMA 9. CERVICAL RIB 10. NEUROMA/SCHWANNOMA 11. CERVICAL LYMPHADENOPATHY

14. SALIVARY GLAND SWELLINGS 1. ANATOMY OF THE PAROTID GLAND 2. ANATOMY OF THE SUBMANDIBULAR GLAND 3. ANATOMY OF THE SUBLINGUAL GLAND 4. APPROACH TO SALIVARY GLAND SWELLINGS 5. SIALOLITHIASIS 6. SALIVARY GLAND TUMOURS 15. THYROID DISEASES 1. ANATOMY OF THE THYROID GLAND 2. APPROACH TO THYROID PROBLEMS 3. APPROACH TO THE SOLITARY THYROID NODULE 4. THYROID CANCERS 5. SURGERY IN BENIGN THYROID DISEASE 16. PERIPHERAL ARTERIAL DISEASES 1. ANATOMY OF THE LOWER LIMB ARTERIES 2. ACUTE LIMB ISCHAEMIA 3. CHRONIC LIMB ISCHAEMIA 17. ABDOMINAL AORTIC ANEURYSM 18. PERIPHERAL VENOUS DISEASES 1. ANATOMY OF THE VENOUS SYSTEM OF THE

LOWER LIMB 2. CHRONIC VENOUS INSUFFICIENCY 3. VARICOSE VEINS 4. VENOUS ULCERS 19. UROLOGICAL DISEASES 1. APPROACH TO HAEMATURIA 2. RENAL CELL CARCINOMA 3. BLADDER TRANSITIONAL CELL CARCINOMA 4. UROLITHIASIS 5. APPROACH TO ACUTE RETENTION OF URINE 6. BENIGN PROSTATIC HYPERPLASIA 7. PROSTATIC CANCER

3

1. TRAUMA & PERIOPERATIVE CARE 1. ADVANCED TRAUMA LIFE SUPPORT

Main principles: x Treat greatest threat to life first x Definitive diagnosis is less important x Time is important – the “golden hour” after trauma is

when 30% of trauma deaths occur, and are preventable by ATLS

Approach: x Primary survey and resuscitation with adjuncts x Re-evaluation of the patient x Secondary survey with adjuncts x Post-resuscitation monitoring and re-evaluation x Optimise for transfer and definitive care

PRIMARY SURVEY – ABCDE AIRWAY Assessment of airway patency

x Is patient alert, can patient speak? x Gurgling, stridor x Maxillofacial injuries x Laryngeal injuries x Caution: C-spine injury

Establishing patent airway x Chin-lift / modified jaw thrust (protect C-spine!) x Remove any FBs in the mouth if possible x Oro/nasopharyngeal airway x Definitive airway – endotracheal tube,

cricothyroidotomy, tracheostomy

BREATHING Assessment of breathing x Look, listen, feel: chest rise, breath

sounds – rhythm and equality bilaterally x Rate of respiration x Effort of respiration x Colour of patient x Percuss chest x Look for chest deformities e.g. flail chest

Management of breathing x Supplemental oxygen x Ventilate as required if patient requires

assistance with breathing x Needle thoracotomy for tension pneumothorax,

followed by chest tube x Occlusive dressing for open pneumothorax

CIRCULATION Assessment of organ perfusion x Level of consciousness x Skin colour and temperature (cold, mottled, clammy), capillary refill x Pulse rate and character (weak, thread) – all major pulses x Blood pressure Classes of haemorrhagic shock - gradual transition from one stage to the next

I II III IV Bld loss : Amt (ml) %

<750 <15

750-1500 15-30

1500-2000 30-40

>2000 >40

Ht rate <100 >100 >120 >140 BP Normal Normal/min low Decreased Critical Cap refill Normal Prolonged Prolonged Prolonged Resp rate 14-20 20-30 30-40 >35 Ur output (ml/h) >30 20-30 5-15 Oliguric-anuric

Mental state Sl anxious Mild anxiety Anxious-confused

Confused-lethargic

Fluid replacement Crystalloid Crystalloid Crystalloid + blood Blood

Rapid and shallow respirations due to sympathetic nervous system stimulation and acidosis. Hypothermia due to decreased perfusion and evaporation of sweat. Some people have only minimal symptoms such as confusion and weakness. Those on B-blockers, those who are athletic and in 30% of cases with shock due to intra abdominal bleeding may have a normal or slow heart rate.

4

Management x Sources of bleeding Æ apply direct pressure or pressure on proximal pressure point x Suspect occult bleeding e.g. intraperitoneal, retroperitoneal (pelvic #), soft tissue (long bone #) x Venous access – large bore, proximal veins x Restore circulatory volume with rapid crystalloid infusion – Ringer’s lactate x Blood transfusion if not responsive to fluids or response is transient x Reassess frequently

DISABILITY Glasgow coma scale Eye (E4) Verbal (V5) Motor (M6)

Spontaneous opening 4 Opens to voice 3 Opens to pain 2 No response 1

Oriented speech 5 Confused 4 Inappropriate 3 Incomprehensible 2 No verbal response 1

Obeys 6 Purposeful 5 Withdraws 4 Flexion response 3 Extension response 2 No response 1

GCS: > 13 (minor); 8-13 (moderate); < 8 (severe) AVPU score: Alert, Verbal stimuli (responds to), Pain stimuli, Unresponsive Pupillary reactivity Call for neurosurgical consult as indicated

EXPOSURE Remove all clothes Check everywhere for injuries (log-roll to look at the back) Prevent hypothermia

ADJUNCTS Monitoring x Vital signs – BP, pulse rate, saturation (pulse oximeter) x ECG monitoring x Arterial blood gas Diagnostic tools x Screening X-ray films (trauma series): CXR, AP pelvis, lateral C-spine x Focused abdominal sonography in trauma (FAST) x Diagnostic peritoneal lavage Urinary catheter x Functions: decompress bladder, measurement of urinary output x Caution in urethral injury: blood at urethral meatus, perineal ecchymosis/haematoma, high-riding

prostate Gastric catheter (orogastric or nasogastric) x Function: decompress stomach, look at aspirate (bloody? bilious?) x Caution in base of skull fracture: CSF otorrhoea/rhinorrhoea, periorbital ecchymosis, mid-face

instability (grab the incisors and rock), haemotympanum Æ insert orogastric tube instead of nasogastric

SECONDARY SURVEY When to do secondary survey: Primary survey and resuscitation completed ABCDEs reassessed Vital functions returning to normal i.e. no need for active resuscitation at the moment AMPLE HISTORY Allergy

Medications Past history Last meal Events leading to injury, Environment in which trauma occurred

5

COMPLETE HEAD-TO-TOE EXAMINATION

Head x Complete neurological examination x GCS or AVPU assessment x Comprehensive examination of eyes and ears for base of skull fractures x Caution: unconscious patient; periorbital oedema; occluded auditory canal Maxillofacial x Bony crepitus/deformity, palpable deformity x Comprehensive oral/dental examination x Caution: potential airway obstruction in maxillofacial injury; cribriform plate # with CSF

rhinorrhoea Æ do not insert nasogastric tube Cervical spine x Palpate for tenderness, any step deformity x Complete neurological examination x C-spine imaging x Caution: Injury above clavicles; altered consciousness (cannot assess accurately); other severe,

painful injury (distracts from cervical spine pain) Neck (soft tissues) x Blunt versus penetrating injuries x Airway obstruction, hoarseness x Crepitus (subcutaneous emphysema), haematoma, stridor, bruit x Caution: delayed s/s of airway obstruction that progressively develop; occult injuries Chest x Inspect, palpate, percuss, auscultate x Re-evaluate frequently x Look at CXR x Caution: missed injury; increase in chest tube drainage Abdomen x Inspect, palpate, percuss, auscultate x Abrasions and ecchymosis – “seat-belt sign” x Lower rib fractures Æ liver and spleen injury x Re-evaluate frequently x Special studies: FAST, DPL (diagnostic peritoneal lavage), CT scan x Caution: hollow viscus and retroperitoneal injuries; excessive pelvic manipulation Perineum x Contusions, haematomas, lacerations x Urethral blood x DRE: Sphincter tone, high-riding prostate, pelvic #; rectal wall integrity; blood x Vaginal examination: blood, lacerations Musculoskeletal – extremities x Contusion, deformity x Pain, Perfusion, Neurovascular status x X-rays as appropriate x Caution: potential blood loss is high in certain injuries (e.g. pelvic fracture, femoral shaft

fracture); missed fractures; soft-tissue or ligamentous injuries; examine patient’s back

ADJUNCTS & SPECIAL DX TESTS

As required according to suspicion, but should not delay transfer

FREQUENT RE-EVALUATION

Have a high index of suspicion for injuries to avoid missing them Frequent re-evaluation & continuous monitoring Æ rapidly recognise when pt is deteriorating

PAIN MX Intravenous analgesia as appropriate

6

2. ABDOMINAL TRAUMA TYPES OF INTRA-ABDOMINAL INJURY IN BLUNT TRAUMA x Solid organ injury: spleen, liver – bleeding (may be quite massive) x Hollow viscus injury with rupture x Vascular injury with bleeding INDICATIONS FOR IMMEDIATE LAPAROTOMY 1) Evisceration, stab wounds with implement in-situ, gunshot wounds traversing abdominal cavity 2) Any penetrating injury to the abdomen with haemodynamic instability or peritoneal irritation 3) Obvious or strongly suspected intra-abdominal injury with shock or difficulty in stabilising haemodynamics 4) Obvious signs of peritoneal irritation 5) Rectal exam reveals fresh blood 6) Persistent fresh blood aspirated from nasogastric tube (oropharyngeal injuries excluded as source of bleeding) 7) X-ray evidence of pneumoperitoneum or diaphragmatic rupture INVESTIGATIONS If patient is stable: FAST and/or CT scan If patient is unstable: FAST and/or DPL FOCUSED ABDOMINAL SONOGRAPHY IN TRAUMA (FAST) Ultrasonographic evaluation of 4 windows: Pericardial, R upper quadrant, L upper quadrant, Pelvis

Advantages x Portable x Can be done quickly in <5min x Can be used for serial examination x Does not require contrast, no radiation risk

Disadvantages x Does not image solid parenchymal

damage, retroperitoneum, diaphragmatic defects or bowel injury

x Compromised in uncooperative, agitated patient, obesity, substantial bowel gas, subcutaneous air

x Less sensitive, more operator-dependent, cannot distinguish blood from ascites

x Intermediate results require follow-up attempts or alternative diagnostic tests

CT SCAN Only suitable for stable patient as quite long time involved in imaging with only patient in the room Æ pt can collapse

Advantages x Able to precisely locate intra-abdominal

lesions preoperatively x Able to evaluate retroperitoneum x Able to identify injuries that can be

managed non-operatively x Not invasive

Disadvantages x Expensive x Time required to transport patient x Use of contrast

DIAGNOSTIC PERITONEAL LAVAGE (DPL) Involves making a cut in the infraumbilical region and inserting a catheter into the peritoneal cavity, aspirate, then instillation of saline and re-aspiration

Positive DPL: Frank blood (>5ml) or obvious bowel contents aspirated Lavage fluid seen to exit from chest drain or urinary catheter RBC >100,000 per mm3, WBC >500, Gram stain + for bacteria in effluent

Indications x Any unstable patient with suspicion of

abdominal trauma or where clinical exam is difficult or equivocal

x Unexplained hypotension in multi trauma x Patient requiring immediate surgery for

extra-abdominal injuries

Contraindications x Absolute indication for laparotomy exists x Previous abdominal surgery or infections x Gravid uterus x Morbid obesity x Coagulopathy

Advantages x Can promptly reveal or exclude the

presence of intraperitoneal haemorrhage x Valuable in discovery of potentially lethal

bowel perforation

Disadvantages x Morbidity involved – wound cxs

(haematoma, infx); intraperitoneal injury x False negative rate of 2% when there is

failure to recover lavage fluid, early hollow viscus injury, diaphragmatic injuries, injuries to retroperitoneal structures

7

3. CARDIOTHORACIC TRAUMA There are 5 clinical scenarios in chest trauma where bedside procedures are lifesaving: cardiac tamponade, airway obstruction, flail chest, haemothorax, and pneumothorax. CARDIAC TAMPONADE High index of suspicion required

Clinical features x Chest trauma and hypotension x Beck’s triad (hypotension, muffled heart sounds, distended neck veins) – only in 50% of

cases as hypovolaemia may prevent neck vein distension; muffled heart sounds least reliable x Pulseless electrical activity x Kussmaul’s signs (increased neck distension during inspiration, pulsus paradoxus) Diagnostic clues x Enlarged cardiac shadow in CXR (globular heart – very rarely seen)

x Small ECG voltages, electrical alternans = alternation of QRS complex amplitude or axis

between beats.

x Pericardial fluid demonstrated on FAST or 2D-echo - definitive Management x Aggressive fluid resuscitation – helps maintain cardiac output and buys time x Pericardiocentesis: 2D-echo guided or ECG lead-guided (Stop inserting needle when an abrupt

change in the ECG waveform is noted. If the ECG waveform shows an injury pattern (ST segment elevation), slowly withdraw the needle until the pattern returns to normal, as this change in waveform suggests that the spinal needle is in direct contact with the myocardium)

8

AIRWAY OBSTRUCTION

x Chin lift or jaw thrust x Remove any foreign body manually, suction blood/secretions x Definitive airway – ETT, cricothyroidotomy, tracheostomy

FLAIL CHEST x When 2 or more ribs are fractured at 2 points forming a flail segment that moves paradoxically with breathing.

x Results in hypoxaemia mainly due to underlying pulmonary contusion, contributed to by pain with restricted chest wall movement.

Management: x Ensure adequate oxygenation and ventilation; judicious fluid therapy (avoid fluid overload);

adequate intravenous analgesia x Consider mechanical ventilation in high risk patients: shock, severe head injury, previous

pulmonary disease, fracture of >8 ribs, age > 65, >3 associated injuries HAEMOTHORAX x Chest tube insertion in the triangle of safety (bound by the lateral border of the pectoralis

major medially, a line just anterior to the mid-axillary line laterally, and the upper border of the fifth rib inferiorly)

x Be wary of sudden cessation of chest tube drainage as tube can get blocked by clot x If blood >1500mls Æ massive haemothorax, call urgent cardiothoracic consult

PNEUMOTHORAX (TENSION/ OPEN)

Tension pneumothorax = develops when air is trapped in the pleural cavity under positive pressure, displacing mediastinal structures and compromising cardiopulmonary function x It is a clinical diagnosis (CXR will only delay treatment, and may cause death) – signs of

pneumothorax, hypotension, neck vein distension, severe respiratory distress x Decreased venous return caused by compression of the relatively thin walls of the atria

impairs cardiac function. The inferior vena cava is thought to be the first to kink and restrict blood flow back to the heart. It is most evident in trauma patients who may be hypovolemic with reduced venous blood return to the heart.

x Immediate needle thoracotomy in second intercostal space in mid-clavicular line x Followed by chest tube insertion Open pneumothorax occurs in a large chest wall defect with equilibration between intrathoracic and atmospheric pressure, producing a “sucking chest wound”. x Cover defect with a sterile dressing, taping it down on 3 sides to produce a flutter-valve effect,

letting air out of the pleural cavity but not back in x Insert chest tube (not through the wound)

9

4. NEUROSURGICAL TRAUMA AIM: prevention of secondary brain injury (from hypotension, hypoxaemia, increased ICP etc) since neuronal death is irreversible. PATHOLOGIES Concussion x Physiological dysfunction without anatomical or radiological abnormality (Physiological dysfunction is the first

step towards cell death, but is reversible if no further insult occurs) x Usually recovers in 2-3 hours

Contusion Small haematoma <1cm

Intracranial haemorrhage

Extradural haemorrhage

Lens-shaped haematoma outside the dura (between skull & dura) x Pathology: expanding space-occupying lesion x 20% of patients with EDH are alert and well; underlying brain is minimally

damaged, thus drainage gives good results

Subdural haemorrhage

Crescent shaped haematoma under the dura (between dura & arachnoid) x More severe than EDH (usu due to nature of injury that causes SDH to

occur – associated with higher impact, thus brain has other injuries) x Most often caused by head injury, when rapidly changing velocities within the

skull may stretch and tear small bridging veins x Generally result from shearing injuries due to various rotational or linear

forces (e.g. shaken baby syndrome, in which similar shearing forces classically cause intra- and pre-retinal hemorrhages)

x Pathology: underlying brain damage in addition to expanding SOL x Removal of blood does not solve brain damage Æ poorer results

Traumatic subarachnoid haemorrhage

Star shaped appearance (cisterns) x Usually only small amount of blood Æ conservative tx sufficient

Intraparenchymal haemorrhage

Any shape, size, location x If large haematoma, will require evacuation

Diffuse axonal injury

x Global injury of axons x Arises from injury that causes rotational and shearing forces (high impact injury) – rapid acceleration and

deceleration of brain in the intracranial cavity against relatively fixed points of attachment at the falx and tentorium (e.g. RTA, falls, assaults, shaken baby syndrome)

x Maximal effects at corpus callosum and brainstem x One of the major causes of unconsciousness and persistent vegetative state after head trauma x If severe, will see punctate haemorrhages at the grey-white border

Cerebral oedema (3 types)

Hypoxic / Cytotoxic (cellular) x Decreased blood supply (oxygenation) Æ loss of function of Na-K pump as ATP decreases Æ increased

intracellular sodium Æ cellular swelling x Conventionally thought to be resistant to any known medical treatment

Interstitial x Impaired absorption of CSF Æ increases in transependymal CSF flow Æ acute hydrocephalus x Also not responsive to steroid administration, and its response to osmotherapy is debatable

Vasogenic x Breakdown of blood-brain barrier Æ proteins enter interstitial space Æ oedema x Seen in TBI, neoplasms, and inflammatory conditions x This edema subtype is responsive to both steroid administration and osmotherapy

10

PATHOPHYSIOLOGY Monroe-Kellie doctrine

The CNS & its contents (brain, CSF, blood) are enclosed in a rigid space whose total volume tends to remain constant Æ increase in the volume of one component will elevate pressure and decrease the volume of one of the other elements

Cerebral perfusion pressure = Mean arterial pressure – Intracranial pressure Compensatory mechanisms: x Hyperventilation Æ vasoconstriction of cerebral vessels due to È pCO2 Æ È blood volume x CSF pushed into spinal canal (but limited volume available) Removal of any reversible cause of raised ICP will improve cerebral perfusion

Fixed dilated pupil

x Constrictor fibres to the pupil run in the oculomotor nerve, which exits the brainstem at the upper midbrain – nerve fibres lie just under the tentorium

x Uncus of the temporal lobe sits on the tentorium x In raised ICP, the uncus herniates over the edge of the tentorium, compressing the fibres of the

oculomotor nerve just below x Thus a fixed dilated pupil occurs on the side of the compression due to unopposed sympathetic

supply (dilates the pupil) Cushing’s reflex

A triad of: Widened pulse pressure (HTN) Irregular breathing (Cheyne-Stokes breathing) Bradycardia From Monroe-Kellie doctrine, Ç MAP maintains cerebral perfusion pressure when ICP is raised. Increase in mean arterial pressure achieved by sympathetic overdrive:

a) Increased heart rate b) Increased contractility c) Increased vasoconstriction – increased total peripheral resistance

(a) and (b) increase cardiac output Æ increased BP; (c) increases BP x Baroreceptors detect abnormally raised BP and try to decrease it by triggering a parasympathetic

response via vagus nerve + Direct distortion of vagus nerve due to raised ICP Æ heart rate ÈÈ x Distortion and/or increased pressure of brainstem (controls involuntary breathing) Æ irregular

breathing and/or apnoea MANAGEMENT Assessment 3 important parameters: ABCs, GCS, pupil size Minor head injury (GCS >13)

x Most common x Indications for admission: Persistent headache and/or vomiting CSF leak Neurological deficit Skull fracture History of loss of consciousness Amnesia x In ward: NBM, IV drip (no dextrose saline!), no sedation, monitor GCS x If pt deteriorates Æ CT scan

Exclude metabolic causes (e.g. hypoglycaemia) Do septic workup (exclude sepsis)

Moderate head injury (GCS 8 - 13)

x All will be CT-scanned at ED Æ NES will operate if any indication to do so x In ward: as per mild head injury

11

Severe head injury Must scan to look for reversible causes of raised ICP but stabilise patient first Medical methods to lower ICP x Raise head of bed (improves venous drainage but could È pressure of blood to the head) x Intubate and hyperventilate (the induced constriction of blood vessels limits blood flow to

the brain at a time when the brain may already be ischemic -- hence it is no longer widely used. Furthermore, the brain adjusts to the new level of carbon dioxide after 48 to 72 hours of hyperventilation, which could cause the vessels to rapidly dilate if carbon-dioxide levels were returned to normal too quickly)

x IV mannitol: create a hypertonic solution within the blood to draw water out of the neurons. This helps to reduce the fluid within intracranial space, however prolonged administration may lead to increase in ICP (must catheterise patient also; do not give if patient is unstable)

x Screen for other life-threatening injuries (likely to be multi-trauma patient) Achieve haemodynamic stability x Check for long bone fractures x FAST for bleeding into abdominal cavity x ABG to detect acidosis x Keep monitoring patient and re-investigate where appropriate Operate if reversible cause found x Craniectomy (i.e. bone flap not replaced) or craniotomy (bone flap replaced after blood

evacuated) [Burrhole usually not big enough to drain an acute bleed] x Evacuate clot x Insert endoventricular drain (EVD) if there is hydrocephalus Total sedation after operation, ward in ICU x Prevents patient from struggling which will raise ICP

Depressed skull fracture

x Can leave alone unless depression is greater than the thickness of the skull bone

Compound depressed fracture

x There is through-and-through skin laceration over the fracture x Always explore to ensure underlying dura is intact, and repair if dura is torn (since

meningitis can occur with a torn dura)

12

5. MUSCULOSKELETAL TRAUMA GENERAL POINTS x Extremity trauma tends not to be life-threatening x But occult blood loss can occur in large volumes esp in certain injuries – pelvic # (up to 3L), femoral shaft # (2L) x Need to have high level of suspicion and treat with urgency x Look out for any tachycardia, early signs of shock x Prepare to resuscitate patient ASSESSMENT OF THE EXTREMITY x Perfusion: colour, pulses, skin temperature, capillary refill x Viability of the limb x Neurological assessment x Wounds – open or closed injury; abrasion over a fracture is considered open fracture x Soft tissue assessment x Deformity x Abnormal joint mobility – ligamentous injury around joint; in knee, highly likely that popliteal artery is injured as well THE PULSELESS EXTREMITY

Things to consider x Is pulselessness due to shock? x Arterial or venous compromise? x Is there compartment syndrome (pulselessness is a very late sign) x Any pre-existing vascular disease? Physical examination x Any limb deformity (can result in kinking of vessels)? x Any joint instability (dislocation of a joint can result in intimal tear in the major vessel running

across it, with thrombosis and occlusion)? x Skin colour/temperature x Post-reduction tibial pulse in knee dislocation – if still absent, do an urgent angiogram!

SOFT TISSUE INJURIES

Types x Open: laceration, abrasion x Crushing x Degloving: open or closed x Closed Wound care x Swabs of the wounds for culture and sensitivity x IV antibiotic prophylaxis x Tetanus toxoid cover x Photograph wound (to prevent re-opening of wound by every doctor that comes to see pt) x Betadine (povidone-iodine) dressing x In OT: generous debridement, irrigation (within 4-8 hours, especially in open fractures),

fracture stabilisation (internal or external fixation depending on Gustilo classification) x Leave wound OPEN

MANAGEMENT OF FRACTURES

x Recognise fracture and/or dislocation x Complete neurovascular examination of the limb involved before reduction x Appropriate X-rays (at least 2 planes) x Analgesia x Correction of deformity x Temporary immobilisation – backslab, malleable splint x Neurovascular examination; examine for compartment syndrome x Circulation chart

13

OPEN FRACTURES Definition: communication between the # or fracture haematoma and the external environment Gustilo-Andersen classification

Type I <1cm AND clean Type II >1cm AND no extensive soft tissue damage, avulsions or flaps Type IIIA Extensive soft tissue damage, avulsions or flaps but adequate soft tissue

coverage of bone OR High-energy trauma cause regardless of size of wound Type IIIB Extensive soft tissue loss with periosteal stripping and exposure of bone.

Massive contamination common. Type IIIC Arterial injury requiring repair

Management of open fractures x Stabilise patient first x Pain relief and analgesia x Cover the wound with moist gauze x Temporary immobilisation and splinting x IV broad spectrum antibiotics x Appropriate X-rays x Nil by mouth x Pre-op investigations: FBC, U/E/Cr, PT/PTT, GXM, ECG, CXR x Arrange for emergency operation x Angiogram if needed Surgery involves x Generous debridement of the wound with irrigation to decrease bacterial load x Treat any soft-tissue injuries x Stabilise fracture – usually using external fixator

6. POST OPERATIVE COMPLICATIONS General or specific? Immediate, early or late? Local or systemic? IMMEDIATE (<1 hour post-op)

Local x Damage to surrounding structures x Primary haemorrhage (either starting during surgery or following post-op Ç in BP)

Replace blood loss and may require return to theatre to re-explore wound Systemic x Basal atelectasis (collapse of alveoli):

Ç bronchial secretions post-op & patient does not breathe deeply due to pain Tx: chest physiotherapy, incentive spirometry

x Shock: due to blood loss, or inadequate fluid replacement peri-operatively x AMI, pulmonary embolism

EARLY (first 24-48 hours post-op)

Local x Pain, hemorrhage Systemic x Atelectasis (commonest cause of mild pyrexia in first 48hr) x Shock, AMI, PE x Nausea & vomiting (analgesia/anaesthesia induced) x Acute confusion: dehydration or sepsis (post-op confusion seen in 40%)

14

LATE (between 48 hours to 1 month post-op)

Local x Post-op paralytic ileus x Wound or anastomotic dehiscence (POD 7 – 10)

Serosanguinous exudate Tx: Analgesia, sterile wound dressing, fluid resuscitation, resuture under GA in OT Mortality up to 30%

x Post-op wound infection (usu within 1st week post-op) Pain, redness, swelling, discharge, usually due to skin staphylococci

x Incisional hernia 10-15% of abdominal wounds Usu asymptomatic, but if painful, consider strangulation (uncommon due to wide neck) RF: Obesity, poor muscle tone (old age), (increased intra-abdominal pressure (chronic cough, straining from constipation), wound infection, multiple use of same incision site Tx: Surgical repair of hernia if strangulated or if enlarging

x Bowel obstruction due to adhesions x Fistula formation x Secondary haemorrhage: often as a result of infection (usu 1-2wk post-op) Systemic x Fever:

Wind (atelectasis), Water (UTI), Walking (DVT/PE), Wound (infxn), Wonder drug (drug fever) x Infections (day 3-5): phlebitis, pneumonia, UTI, abscesses (subphrenic, pelvic) x Acute confusion: exclude dehydration & sepsis x DVT & PE (day 5-7)

POST-OP HAEMORRHAGE EARLY x Consumptive coagulopathy – if large volumes of blood transfused

x Pre-op anticoagulation x Unrecognized bleeding diathesis x Damage to blood vessels/vascular organs Management x FBC (plt), PT/PTT, GXM & order blood x Give protamine if heparin was used x Give FFP / platelet concentrates if clotting screen abnormal x Consider surgical re-exploration

LATE Occurs several days after surgery

x Usually due to infection damaging vessels at the operation site Management x Treat infection and consider exploratory surgery

POOR WOUND HEALING

Patient factors Wound factors Surgeon factors

x Poor blood supply x Malnutrition x Vitamin deficiency x Immunosuppressive therapy x Long term steroids x Radiotherapy x Co-morbidities e.g. DM, HIV

x Wound infection x Poor wound care

x Suturing under tension x Did not observe aseptic

technique

15

DVT/PE Affects 25-50% of surgical patients DVT in deep calf veins: 5-10% risk of PE DVT in iliofemoral veins: 50-60% risk of PE Risk Factors Virchow’s Triad 1) Alterations to flow 2) Endothelial injury 3) Hypercoagulable state

x Prolonged bed rest, pregnancy (IVC compression) x Trauma, pelvic/ vascular surgery, intravenous drug use x Congenital: protein C/ protein S deficiency, Factor V Leiden mutation, anti-

phospholipid syndrome, homocysteinuria/homocysteinemia x Acquired: liver disease, cancer (production of pro-thrombotic factors), OCP use

Presentation x Post-operative fever (typically day 5-7) x Lower limb pain & swelling, discolouration: may not be at site of clot x Classic triad of PE: chest pain, dyspnoea, haemoptysis (poor sensitivity/specificity) x Others: seizures, syncope, new onset atrial fibrillation etc

Physical Examination x Mild fever x Calf warmth, tender, swelling, erythema x Pitting oedema x Homan’s sign: passive dorsiflexion of the ankle with knees flexed Æ sharp pain in calf

(DON’T DO: risk of clot propagation/ dislodging)

Investigations x Duplex ultrasound to detect DVT x Chest x-ray: usually normal (classical findings listed below are rarely seen)

x CT pulmonary angiogram (gold standard)

Look for filling defects in pulmonary artery x V/Q perfusion scan: no longer done

Inhale radioactive isotope, do CXR to assess ventilation of lungs IV contrast, do CXR again to assess perfusion of lungs Look for areas of ventilation without perfusion (V/Q mismatch) Correlate with clinical findings, calculate probability of PE

x ECG May have normal ECG with sinus tachycardia Signs of right heart strain Right axis deviation: S waves in lead I Right ventricular hypertrophy: tall R waves in V1, deep S waves in V6 Right atrial hypertrophy: peaked P waves RBBB Classic S1Q3T3: S wave in I, Q wave in III, T inversion in III (rarely seen)

x D-dimer: poor sensitivity & specificity in ruling in/ out DVT or pulmonary embolism

Atelectasis, pleural effusion, raised hemi-diaphragm Westermark sign: dilatation of the pulmonary vessels proximal to the embolism, with collapse of the distal vessels (oligemia) Hampton hump: late sign, wedged shaped infiltrate with apex towards the hilum

16

Prophylaxis/ Treatment Non-pharmacological x Use of inflatable calf pumps intra-operatively in patients undergoing long operations x Early ambulation post surgery x Thromboembolic deterrent (TED) stockings/ intermittent pneumatic leg compression Pharmacological x Unfractionated heparin or LMW heparin – acute setting

Unfractionated heparin Potentiates effect of anti-thrombin III, inactivates thrombin (PTT Ç) Onset: immediate for IV; 30 mins subcutaneously Efficacy monitored using aPTT Antidote: protamine sulphate Side effects: thrombocytopaenia LMW heparin (Clexane) Potentiates effect of anti-factor 10a (not reflected by PTT) Onset: immediate for IV More predictable dose-effect relationship Reversibility by protamine sulphate limited

x Warfarin – long term (8-9 months, or until clot dissolves)

Vitamin K antagonist, inhibits synthesis of Vit K dependent factors (II, VII, IX, X) Onset: 2 to 4 days (start by overlapping with heparin for 4 days) Efficacy monitored using PT/INR (target INR: 2.0 to 3.0) Antidote: vitamin K, FFP SE: haemorrhage, hepatitis, skin necrosis (in patients with protein C/S deficiency)

Surgical x IVC filter (permanent or temporary: remove after 2 weeks of anti-coagulation) x Indications: History of recurrent DVT/PE Free floating thombus seen on duplex scan Patient not suitable for anti-coagulation e.g. up to 7 days post-op Allergy to anti-coagulation Treatment of Pulmonary Embolism x Supplemental oxygen,, intubation, mechanical ventilation x Initiate anti-coagulation x Intra-venous thrombolytics if not contraindicated x Surgical/ catheter embolectomy

Well’s Criteria A set of criteria to determine the pretest probability of DVT

Clinical feature Score Active cancer (treatment ongoing or within the previous 6 months or palliative) 1 Paralysis, paresis, or recent plaster immobilization of the lower extremities 1 Recently bedridden for more than 3 days or major surgery, within 4 weeks 1 Localized tenderness along the distribution of the deep venous system 1 Entire leg swollen 1 Calf swelling by more than 3 cm when compared to the asymptomatic leg (measured below tibial tuberosity)

1

Pitting edema (greater in the symptomatic leg) 1 Collateral superficial veins (nonvaricose) 1 Alternative diagnosis as likely or more likely than that of deep venous thrombosis -2

High Probability: ≥ 3 – treat as susp DVT and perform compression U/S Moderate Probability 1 or 2 – treat as susp DVT and perform compression U/S Low Probability ≤0 – D-dimer test

17

7. SHOCK Shock = inadequate tissue & organ perfusion to meet metabolic demands Æ cellular hypoxia S/S: ÈÈ BP, È urine output, tachycardia, diaphoresis, AMS TYPES OF SHOCK

Types Hypovolaemic Cardiogenic Neurogenic Obstructive Septic Anaphylactic

Causes x Haemorrhage x Burns x Ruptured

ectopic pregnancy

x Severe GE x Acute

pancreatitis

x AMI x Dysrhythmia

x Spinal injury x Tension pneumothorax

x Cardiac tamponade

x Pulmonary embolism

x Infxns

S/S Pallor Cold clammy skin nperi vas :

Pallor Cold clammy skin nperi vas :

Warm skin N/p heart rate Neuro deficit

Fever, rigors Warm skin

Fever, rigors Warm skin

Invxs È Hct (late) Cardiac enzymes ECG

FBC Bld C/S

GENERAL MANAGEMENT AIRWAY Maintain airway – consider intubation if necessary BREATHING 100% O2 via non-rebreather mask CIRCULATION 2 large bore (14-16G) cannulae

r Inotropic support x IV dopamine 5-10Pg/kg/min x IV dobutamine 5-10Pg/kg/min (esp for cardiogenic shock) x IV norepinephrine 5-20Pg/kg/min (esp for septic shock)

MONITORING x Pulse oximetry x ECG x BP x Heart rate x Urine output – catheterize patient

SPECIFIC MANAGEMENT HYPOVOLAEMIC SHOCK

x FBC – Hct (but inaccurate marker of acute blood loss - Ç Hct in alcoholic binge from dieresis) x GXM 6 units x U/E/Cr x Trop T (Cardiac enzymes) x Coagulation profile with DIVC screen (PT/PTT, D-dimer) x ABG – metabolic acidosis, Ç lactate, base deficits = poor prognostic factor x UPT – r/o ectopic pregnancy (ask for LMP) x Examine the abdomen for any pulsatile AAA x Fluid resus: 1L crystalloid fast infusion over 1 hr

Assess response Subsequent colloid or whole blood infusion

x CVP line insertion – to guide fluid resus CARDIOGENIC SHOCK

x ECG, Trop T & cardiac enzymes x Manage accordingly to cause – refer Emed book

18

OBSTRUCTIVE SHOCK

Tension Pneumothorax x Decompression: insert 14G cannula over 2nd intercostals space in mid-clavicular line Cardiac Tamponade x IV fluid bolus 500ml N/S x r IV dopamine infusion 5Pg/kg/min x Prepare for pericardiocentesis Pulmonary Embolism x FBC, GXM x 6 units, U/E/Cr, D-dimer x ABG: È PaO2 & N/È PaCO2, widened alveolo-arterial PO2 gradient (AaPO2 >20mmHg) x ECG x CXR (Exclude Ddx – pneumothorax, pneumonia, heart failure, tumour, rib #, pleural effusion, collapse) x Pulmonary angiogram (gold standard) x Pain relieve – use opioids with caution (resp depression) x Fluid resus & inotropic support if haemodynamically unstable x Anticoagulation:

IV heparin 5000U bolus OR subcut fraxiparine (0.4ml <50kg, 0.5ml 50-65 kg, 0.6ml >65kg) Convert to oral warfarin later

x Thrombolysis: intra pulm arterial urokinase for 12-24hrs x Surgical: complete IV ligation or partial caval interruption

NEUROGENIC SHOCK

x Trauma – site, mechanism, force x Neuro exam, DRE – document initial neurological deficits x Immobilize spine in neutral position x C-spine X-ray (AP & lat) – ensure visualization up to C7/T1 junction

r Swimmer’s view (visualize C7/T1 jn) & open mouth view (visualize C1/2 injury) x Thoracic & lumbar spine X-ray (AP & lat) x r CT scan x r MRI later x Titrate fluid resus with urine output x r vasopressors if BP does not respond to fluid challenge x r IV methyl Prednisolone 30 mg/kg over 15mins, followed by 5.4mg/kg/h for nxt 23 hrs

Indications – non-penetrating spinal cord injury & w/in 8 hrs of injury Contraindications: <13YO

Pregnancy Mild injury of the cauda equina / nerve root Abdominal trauma present Major life-threatening morbidity x Refer Ortho / NeuroSx

SEPTIC SHOCK (SIRS + source of sepsis + shock)

SIRS = t 2 of the following present: Temp >38 or <36oC HR > 90bpm RR > 20 breaths/min OR PaCO2<32mmHg WCC>12000/mm3, <4000/mm3,or >10% immature forms x Identify site of infxn – UTI (indwelling cathether), gallbladder dz, peritonitis, pneumonia,

appendicitis, immunocompromised state x FBC, U/E/Cr, DIVC screen (PT/PTT, fibrinogen, D-dimer), blood C/S, ABG, CXR, ECG,

UFEME and urine C/S) x Rapid infusion 1-2L crystalloids

r CVP line insertion x If no response to fluid Rx Æ inotropic support

Noradrenaline (drug of choice) - 1Pg/kg/min OR Dopamine 5-20Pg/kg/min

19

x Empirical Abx: Immunocompetent w/o obvious source

x 3rd gen cephalosporin (IV ceftriaxone 1g) OR x Quinolones (ciprofloxacin 200mg)

Immunocompromised w/o obvious source

x Anti-pseudomonal ABx (IV ceftazidime 1g) OR x Quinolone x PLUS aminoglycoside (Gentamicin 80mg)

Gram-positive (burns, FB / lines present)

x IV cefazolin 2g x IV vancomycin 1g (if IVDA, indwelling cath, pen allergy)

Anaerobic source (intra-abdo, biliary, female genital tract, aspiration pneumonia)

IV metronidazole 500mg + ceftriazone 1g + IV gentamicin 80mg

ANAPHYLACTIC SHOCK

Definitions x Urticaria – oedematous & pruritic plaques w pale centre & raised edges x Angioedema – oedema of deeper layers of the skin. Non-pruritic. May be a/w numbness & pain x Anaphylaxis – severe systemic allergic rxn to an Ag. Ppt by abrupt release of chemical

mediators in a previously sensitized patient x Anaphylactoid rxn – resembles anaphylactic rxn, but due to direct histamine release from mast

cells w/o need for prior sensitization Common causes x Drugs – penicililns & NSAIDS commonest, aspirin, TCM, sulpha drugs x Food – shellfish, egg white, peanuts x Venoms – bees, wasps, hornets x Environment – dust, pollen x Infections – EBV, HBV, coxsackie virus, parasites Stop Pptant x Stop administration of suspected agent / flick out insect stinger with tongue blade x Gastric lavage & activated charcoal if drug was ingested Airway x Prepare for intubation or cricothyroidectomy – ENT/Anaesthesia consult Fluid Rx x 2L Hartman’s or N/S bolus Drug Rx

Adrenaline x Normotensive – 0.01ml/kg (max 0.5ml) 1:1000 dilution SC/IM x Hypotensive – 0.1ml/kg (max 5ml) 1:10,000 dilution IV over 5 mins

Glucagon x Indications: failure of adrenaline Rx OR if adrenaline is contraindicated e.g. IHD, severe HPT, pregnancy, E-blocker use

x 0.5-1.0mg IV/IM. Can be repeated once after 30mins Antihistamines x Diphenhydramine 25mg IM/IV

x Chlorpheniramine 10mg IM/IV x Promethazine 25mg IM/IV

Cimetidine x For persistent symptoms unresponsive to above Rx x 200-400mg IV bolus

Nebulised bronchodilator

x For persistent bronchospasm x Salbutamol 2:2 q20-30mins

Corticosteroids x Hydrocortisone 200-300mg IV bolus, q 6hr

20

8. PERIOPERATIVE CARE INPUT / OUTPUT

Normal daily intake Normal daily output Water Diet 2300 ml Metbolism 200ml

Urine 1400ml (min obligatory volume = 400ml) Skin loss 500ml (obligatory diffusion & vaporisation)

Sweating in pyrexia / heat can cause several litres extra loss per day Lung loss 500ml (obligatory) Faecal loss 100ml

Sodium Diet 150 mmol/day (range 50 – 300 mmol)

Stool 5 mmol/day Skin transpiration 5 mmol/day (in absence of sweating) Urine 140 mmol/day (can fall to 15 mmol/day)

Potassium Diet 100 mmol/day (range 50 – 200 mmol)

Stool 10 mmol/day (obligatory) Skin < 5 mmol/day Urine 85 mmol/day (rarely falls below 60 mmol/day)

DAILY GASTROINTESTINAL SECRETIONS & ELECTROLYTE COMPOSITION

Secretion Volume (L) Na (mmol/L) K (mmol/L) Cl (mmol/L) HCO3 (mmol/L)

Saliva 1 – 1.5 20 – 80 10 – 20 20 – 40 20 – 160

Gastric juice 1 – 2.5 20 – 100 5 – 10 120 – 160 Nil

Bile Up to 1 150 – 250 5 – 10 40 – 60 20 – 60

Pancreatic juice 1 – 2 120 5 – 10 10 – 60 80 – 120

Small bowel secretions (succus entericus)

2 – 3 140 5 (up to 40 in inflammatory diarrhoea)

Variable Variable

DAILY REQUIREMENTS Fluid 100 ml/kg/day Na 2 mmol/kg/day K 1-2 mmol/kg/day PARKLAND’S FORMULA FOR BURNS

2-4 ml / kg (body wt) / % (BSA of burn) x Divide total volume into 2 halves x Infuse 1st half in 1st 8h x Infuse the rest in next 16h Start time = time of burn injury Fluid of choice = Hartmann’s solution

DAILY CALORIC REQUIREMENTS 2000 – 2500 kcal/day CALORIC CONTENTS x Glucose: 4 kcal/g x Protein 4 kcal/g x Fats: 9 kcal/g x Alcohol: 7 kcal/g

21

FLUIDS / MAINTENANCE Body composition x Total body water = 55% body weight x Extracellular fluid = 20% body weight x Blood volume = 7% body weight Acute blood loss required to produce shock = 25-30% of blood volume

Basal requirements Children have higher water content (higher metabolic rate, greater surface area to body weight ratio) Holliday-Segar method: 1st 10 kg – 100 ml/kg/day (5ml /kg/hr) 2nd 10 kg – 50 ml/kg/day (2ml /kg/hr) > 20 kg – 20 ml/kg/day (1ml /kg/hr)

Deficits Thirsty: 1.5L deficit Tachycardia: 3L deficit Hypotensive: 6L deficit

Ongoing/ anticipated losses x Insensible losses

o Every 100kcal burned = 30ml loss through skin

o Losses Ç 10% for every Ç deg of fever > 37°C

x Sweat x GIT x Urine (normal = 0.5 – 1 ml/kg/hr) x Blood x Respirators

Components of regularly used IV fluids 0.9% NaCl/ Normal saline (NS) 9g Na in 1L / 154 mmol Na & Cl (isotonic) Dextrose 5% (D5W) 50g glucose in 1L (200kcal; 1g glucose = 4kcal) (isotonic) Dextrose 5% in 0.9% NaCl (D5NS) 50g glucose + 9g Na in 1L (hypertonic) Hartmann’s solution / Ringer’s lactate Na 131 mmol/L

Cl 111 mmol/L K 5 mmol/L Ca 2 mmol/L Lactate 29 mmol/L

x Amount of required (KCl) infused in divided doses over 24h x Premixed IV fluids are generally available with 20 or 40 mmol of KCl

per 500ml or 1000ml infusion bags x If concentrations of KCl > 40 mmol in 500ml are required, they

should be given via infusion in the ICU with cardiac monitoring x Added K is not usually required in the 1st 24-48h after surgery

because K is released from damaged cells

HCT From loss of pure plasma (burns/pancreatitis/peritonitis): 1 point Ç = 100ml loss of fluid From loss of isotonic extracellular fluid (GIT): 1 point Ç = 500 ml loss of fluid From loss of pure water (evaporation from lungs): no change CHOICE OF FLUID REPLACEMENT If pt losing blood Æ blood transfusion Diarrhoea / vomiting Æ crystalloids Burns pt (losing protein) Æ colloids

Sample daily IV fluid regimen 1L NS + 20 mmol KCl + 1L D5W + 20 mmol KCl + 1L D5W + 20 mmol KCL (each bag given over 8 h) Total: 154 mmol Na + 60 mmol K

22

CVP MONITORING Swan-Ganz/ pulmonary artery catheter

x Pressure in RA x Pressure in PA x Pulmonary capillary wedge pressure

(indirect estimate of LA pressure) Normal CVP = 5 – 10 mmHg x Useful in evaluating blood volume status when

fluids are administered during hypotensive shock x Administer fluids at a rate that maintains CVP at

12 – 15 mmHg (cardiac output optimal) Ohm’s Law:

𝐶𝑂 = 𝑀𝐴𝑃 − 𝐶𝑉𝑃𝑆𝑉𝑅 × 80

VENTILATION Ventilator settings x Tidal Volume = vol of air in each breath (8-12 cm3 /kg) x Rate = no of breaths delivered per min x FiO2 = amt of O2 delivered (N = 40%; the higher it is , the more O2 damage to the lungs) x PEEP = positive end expiratory pressure (opens alveoli that would otherwise collapse in expiration)

o Normal: 3 – 5 cmH2O (physiologic PEEP) o Therapeutic PEEP can go up to 10 – 35 cmH2O (but too high impedes venous return to the heart)

Atelectasis = V/Q mismatch (shunt)

23

ACID BASE & ELECTROLYTES Metabolic Acidosis pH < 7.35 [HCO3] < 20 mmol/L Respiratory Acidosis pH < 7.35 PCO2 > 45 mmHg Metabolic Alkalosis pH > 7.45 [HCO3] > 24 mmol/L Respiratory Alkalosis pH > 7.45 PCO2 < 35 mmHg Anion gap = [Na] – [Cl] – [HCO3] x Normal = 3-11 mmol/L x Hypoalbuminemia: anion gap È in 2.5 mmol/L for every È 1 g/dL of albumin x Elevated anion gap = HAGMA even in the presence of a normal pH / [HCO3] Compensation Metabolic Acidosis

È [HCO3] 1 mmol/L = È PCO2 1.2 mmHg Expected PCO2 = (1.5 x [HCO3]) + 8 mmHg x PCO2 < expected Æ concurrent respi alkalosis x PCO2 > expected Æ concurrent respi acideosis Calculate change in anion gap (' AG) ' AG + [HCO3] = normal [HCO3] Æ HAGMA ' AG + [HCO3] > normal [HCO3] Æ concurrent metabolic alkalosis ' AG + [HCO3] < normal [HCO3] Æ concurrent NAGMA

Metabolic Alkalosis

Ç [HCO3] 1 mmol/L = Ç PCO2 0.7 mmHg Expected PCO2 = (0.6 x [HCO3 – 24]) + 40 mmHg x PCO2 < expected Æ concurrent respi alkalosis x PCO2 > expected Æ concurrent respi acidosis

Respiratory Acidosis

Acute: Ç PCO2 1 mmHg = Ç [HCO3] 0.1 mmol/L Chronic: Ç PCO2 1 mmHg = Ç [HCO3] 0.4 mmol/L

x Acute: [HCO3] ' 1-2 mmol/L for every ' PCO2 by 10 mmHg

x Chronic: [HCO3] ' 4-5 mmol/L for every ' PCO2 by 10 mmHg Respiratory

Alkalosis Acute: È PCO2 1 mmHg = È [HCO3] 0.2 mmol/L Chronic: È PCO2 1mmHg = È [HCO3] 0.4 mmol/L

If pH normal, check for balanced acid base disorder: [HCO3] < 20 PCO2 < 35 Metabolic acidosis + Respiratory alkalosis [HCO3] > 24 PCO2 > 45 Metabolic alkalosis + Respiratory acidosis [HCO3] & PCO2 normal AG > 11 HAGMA + metabolic alkalosis [HCO3] & PCO2 normal AG normal Normal (unlikely NAGMA + metabolic alkalosis)

24

Hyponatremia Hypernatremia

Signs x Confusion x Seizures x Cardiac failure x Muscle weakness x Nausea, anorexia

x Thirst x Dehydration x Confusion x Coma x Seizures

Causes x Diuretics (esp thiazides) x Water excess x Diarrhoea / vomiting x Intestinal fistula x Cardiac failure / Renal failure x SIADH x Addison’s disease

x Fluid loss w/o water replacement (esp burns) x Saline excess x Diabetes Insipidus x DKA x Conn’s syndrome

Treatment x Rehydration with appropriate sodium-containing IV fluids

x Diuretics for cardiac failure x Fluid restriction to 1L per day

x Encourage oral intake x IV fluids with low sodium content

Hypokalemia Hyperkalemia

Signs x Cardiac arrhythmias x Muscle weakness x Hypotonia (& È GI motility Æ paralytic ileus) x Muscle cramps Æ Tetany

x Cardiac arrhythmias x Sudden death

Causes x Diarrhoea / vomiting x Intestinal fistula x Diuretics x Renal tubular failure x Cushing’s / exogenous steroids / ACTH x Conn’s syndrome x Metabolic alkalosis

x Sampling artefact (haemolysis) x Drugs (ACEI, spironolactone, suxamethonium) x Digoxin poisoning x Massive blood transfusion x KCl excess x Burns x Rhabdomyolysis x Tumour lysis x Renal failure x Addison’s disease x Metabolic acidosis

Treatment x Oral potassium supplements x KCl added to IV fluids

1. Calcium gluconate 10% (50-100ml) 2. Insulin 20 units

+ 250ml 25% dextrose over 4-6 hrs 3. Diuretics 4. E2 adrenergic agonists 5. Sodium bicarbonate (50 – 100ml) 6. Resin (polystyrene sulfonate sodium)

(every g exchanges 3 mmol of Na for K) 7. Hemodialysis (definitive)

25

NUTRITION Nutritional support should be considered for: x BMI < 18.5 x Unintentional weight loss > 10% BW within last 3 – 6 months x BMI < 20 and unintentional weight loss > 5% within last 3 – 6 months x Poor oral intake for > 5 days x Poor absorptive capacity, high nutrient losses, increased catabolic rate REFEEDING SYNDROME x Gastric emptying one of the last aspects of gut function to recover after an operation/major insult x Risk factors: low BMI, duration of reduced intake, degree of weight loss, electrolyte abnormalities x Feeding should be introduced gradually over 48 – 72 hours (Safe to start feeding at 50% of estimated protein and

calorie requirements and build up to full requirements over a 24 – 48 hour period.) ENTERAL FEEDING Start at 20ml/h x 6h, then increase by 20-30ml/h and repeat. Indications:

x Proximal small intestine intact & functional x Stimulation of secretory function does not worsen the condition being treated (e.g. small bowel fistula)

Contraindications:

x Complete small bowel obstruction x Inadequately treated shock states (risk of intestinal ischemia) x Severe diarrhoea (slow rate of feeding) x Proximal small intestinal fistula x Severe pancreatitis

Routes: x Nasogastric tube feeding x Nasojejunal or nasoduodenal feeding (when gastric emptying is a problem) x Tube enterostomy x Percutaneous endoscopic gastrostomy x Complications: infection at PEG site, blockage/extrusion/removal, peritonitis, aspiration pneumonia Complications of enteral feeding: x Fistulation x Wound infection x Peritonitis x Displacement & catheter migration x Blockage of tube x Aspiration pneumonia x Feed intolerance x Diarrhoea Types of Oral Feeds: x Ensure (protein 9g /serving) x Ensure Plus (highly concentrated in calories 1.5 cal/ml & protein 13 g/serving) x Glucerna (for DM pts Æ È carbohydrates, modified fat) x Pulmocare (for COPD pts Æ high calorie, low carb to help È CO2 production) x Novasource Renal (for renal pts Æ low protein & nitrogen content)

26

TOTAL PARENTERAL NUTRITION Provision of all nutritional requirements by the intravenous route alone Due to high osmolality of the mixture, TPN must be given into a central vein TPN orders: x 24 hr feeding volume 2500ml, rate 100ml/hr x 5 days a week: kcal from carbohydrate 2500, kcal from fat 0 x 2 days a week: kcal from carbohydrate 2000, kcal from fat 500

Indications: x Critical illness: where enteral feeding is not established within 5 days x Obstruction of GIT: proximal small bowel obstruction not immediately relieved x Short bowel syndrome:

o Temporary (before adaptation) in < 3m of functional small intestine o Permanent in < 1m of functional small intestine

x Proximal intestinal fistula: facilitate fistula closure x Refractory inflammatory disease of the GIT (e.g. Crohn’s, UC) x Inability to use the GIT: pancreatitis with pseudocysts/abscess where enteral nutrition is not tolerated Complications: x Catheter-related

o Mechanical: blockage, central vein thrombosis, migration, fracture, dislodgement o Infective: exit-site infection, line sepsis, infective endocarditis

x Metabolic o Hyperglycaemia o Deranged liver function: biliary stasis, enzyme induction from amino acid imbalance and excessive calorie

administration, fat deposition in liver o Hypoglycaemia (too rapid cessation of glucose infusion) o Hypertriglyceridemia o Hyperchloraemic acidosis

27

2. LUMPS & BUMPS 1. HERNIA x Hernia = a protrusion of an organ through an opening in the wall of the cavity in which it is normally contained x Lifetime risk = 2-10% x Causes of abdominal wall weakness

- Congenital – normal (due to piercing structures), or patent processus vaginalis - Acquired – trauma, surgical incision or disease

x Consist of 3 parts: - Sac: pouch of peritoneum (neck & body) - Coverings of sac: layers of abdominal wall - Contents

Types:

1) Inguinal (96%) Î indirect (85%), direct (15%) or pantaloon (direct & indirect) 2) Femoral (4%) & Richter’s hernia (knuckle of bowel wall is strangulated but lumen is patent) 3) Incisional, Parastomal 4) Umbilical, Epigastric (herniation of extra-peritoneal fat) 5) Rare types: Spigelian (herniation of linea semilunaris: lat. border of rectus), Obturator, Lumbar, Gluteal, Internal, Sliding

(herniation of posterior peritoneum with underlying retroperitoneal structures: caecum, sigmoid, bladder)

PHYSICAL EXAMINTION (common signs)

- Location: all occur at congenital or acquired weak spots in the abdominal wall - Reducibility: on direct pressure or lying down - Expansile cough impulse

28

INGUINAL HERNIA: indirect or direct; Indirect inguinal hernia: - Most common; 65% - Congenital; patent processus vaginalis + weakened fascia at deep ring - Hernial sac enters the inguinal canal with the spermatic cord via the deep ring, then emerges from the superficial ring &

descends into the scrotum - > , R> L, 20% are bilateral, children>adults Direct inguinal hernia: - Bulges directly ant though a weakened fascia Hesselbach’s triangle, post to the inguinal canal (med to inferior epigastric art)

Hesselbach’s ∆: inf. Epigastric art., rectus abd, inguinal ligament

- Hernia sac is not with the spermatic cord - Rare in , usually occur bilaterally in with

weak abdominal muscles and comorbid conditions causing ↑ intra-abdominal pressure

Clinical differences between indirect and direct inguinal hernia

Indirect Hernia Direct Hernia

Neck lies lateral to inferior epigastric artery, out of Hasselbach’s triangle

Neck lies medial to inferior epigastric artery, within Hasselbach’s triangle

Reduces upwards, laterally and backwards Reduces upwards and straight backwards

Controlled after reduction by pressure over the deep ring Controlled after reduction by pressure over the superficial ring

May descend down the scrotum Does not descend down the scrotum

May cause strangulation at superficial ring (narrow) Rarely causes strangulation due to wide hernia neck

Does not readily reduce on lying down Readily reduces on lying down

More common in young adults and infants More common in old men

29

Anatomy of inguinal canal - 4 to 6 cm long oblique passage above the inguinal ligament, from the deep to superficial rings. - Deep inguinal ring lies in the midpoint of the inguinal LIGAMENT & is formed from a defect in the transversalis fascia. - Superficial ring is a triangular defect in the aponeurosis of the external oblique. Boundaries: x Ant. wall: aponeurosis of the

external oblique (reinforced in lat. third by internal oblique)

x Post. wall: transversalis fascia (reinforced in med. third by conjoint tendon)

x Roof: arching fibres of the int. oblique & transversus abdominis before they merge as conjoint tendon

x Floor: inguinal ligament and lacunar ligament medially

Contents: x Ilioinguinal nerve (L1): supplies

skin over root of penis & upper part of scrotum or skin over mons pubis & labia majora

x : round ligament of the uterus from uterus to labia majora

x : transmits spermatic cord from abdomen to testes

Anatomy of the spermatic cord Coverings: 3 concentric layers of fascia x Internal spermatic fascia:

derived from transversalis fascia x Cremasteric fascia & muscle:

derived from internal oblique x External spermatic fascia:

derived from external oblique Contents: x 3 Arteries:

Testicular artery Artery to the vas deferens Cremasteric artery

x 3 Nerves: Nerve to cremaster Autonomic nerves (T10) Genital br of genitofemoral nerve

x 3 Others: Vas deferens Pampiform venous plexus Lymphatics

30

Complications Reducible → Irreducible / Incarcerated → Obstructed → Strangulated

- Irreducible/ Incarcerated hernia: contents of hernial sac cannot be replaced into abdomen - Obstructed: loop of bowel trapped in hernial sac such that its lumen, but not blood supply, is obstructed (no ischaemia Î

not unduly tender, but with IO) - Stangulated: blood supply to trapped bowel is cut off, bowel is dead or dying; 6hrs to gangrene.

(acutely tender with s/s of IO;; exception: Richter’s hernia: segment of bowel is trapped & ischaemic but lumen is patentÎ no IO)

Management Non-surgical:

x Raised intraabdominal pressure o Weight loss, change jobs, avoid heavy lifting o Treat medical conditions causing chronic cough, chronic constipation

x Truss: for compression of reducible hernia at deep ring (poor pickup rate) x If obstructed/strangulated: NBM, IV drip, NG tube on suction, IV ABx

Surgical:

x Hernioplasty/ Herniorraphy with mesh repair (open or laparoscopic) o Immediately if suspect incarceration to prevent any bowel perforation

x Principles: reduce bowel, ±excise hernia sac, reinforce posterior wall o Lichtenstein tension-free mesh repair (lightweight, polypropylene mesh insertion & suture) o Shouldice repair (non-mesh technique: 2 continuous back & forth sutures with permanent suture material)

x Complications o 2o to GA: AMI, CVA o Immediate to early

1. ARU 2. Bruising , bleeding (testicular, cremasteric, artery to vas deferens, inferior epigastric, femoral arteries) 3. Injury to surrounding structuresÎ pain, parasthesia, impotence: spermatic cord (vas deferens,

testicular artery), round ligament, ilioinguinal nerve, nerve to cremaster o Early

1. Infection of wound/ mesh 2. Haematoma (10%; no cough impulse, non-reducible, hard) 3. Wound dehiscence 4. pain

o Late 1. Chronic post-op pain 2. Recurrence of hernia (<0.5%; incomplete dissection, poor tissue, too early mobilization, uncontrolled co-

morbidities) 3. Ischaemic orchitis & Testicular atrophy from testicular artery damage or from pampiniform plexus

thrombosis

x Post- operation monitoring: o Work leave for 6/52 with avoiding of heavy lifting o Treat any medical conditions to avoid coughing, constipation o Early mobilization from D10 o Keep area clean and wash carefully, but able to bathe immediately o Abx cover???

31

APPROACH TO AN INGUINAL HERNIA 1. Groin vs Inguinoscrotal a) groin Æ cough impulse

b) inguinoscrotal Æ 1. cough impulse (may not be present in a large one) 2. cannot get above lump 3. cannot separate from testis

2. Risk factors:

Increased intra-abdo pressure: chronic cough, BPH, chronic constipation Also 4Cs: cirrhosis, heart failure, cancer, catheter (dialysis)

3. Complications a) Obstructed/ Strangulated b) Spontaneous rupture c) Involved in peritoneal diseases

4. Patient profile

- Indirect: M, young, right, increase intraperitoneal fluid - Direct: M, old, raised intraabdominal pressure

5. Treatment:

a) OPEN hernia repair!!! b) Lap only if 1. Recurrent, 2. Bilateral (2 types of lap: TEP, TAP) c) Conservative only if: small, easily reducible, direct hernia

6. Post-op complications a) Immediate: bruise, wound hematoma, scrotal hematoma, ARU, pain b) Later: mesh infection, recurrence (10% /10years), nerve injury, ischemic orchitis

EXAMINATION OF AN INGUINAL HERNIA

“Please examine this patient’s groin” Don gloves, introduce yourself and explain your intention, then expose the patient STAND patient up, examine both sides - Mr X is a ___ who appears comfortable at rest. - I notice a groin / inguinoscrotal lump. Squat down and examine! - Inspect as per a lump: (if unable to see, ask the patient)

1. Is lump above or below the inguinal ligament? Any scrotal lump? 2. Estimate the dimensions of the lump 3. Any skin changes? Previous scars (look hard)? 4. Any lump on the other side? 5. Abdominal distension / visible abdo mass?

- Sir, could you turn head and cough? Æ Look for Visible cough impulse (seen in large inguinoscrotal hernias) - Sir, is there any pain over the groin area? I am going to feel the lump.

Palpate: 1. Can get above the lump? 2. Can feel testis? 3. Lump: consistency (soft, fluctuant), size, temperature, any tenderness? 4. Sir, could you turn head and cough again? Æ Feel for Palpable cough impulse (bilaterally?)

- Sir, could you reduce the lump for me? o Reducible: The point of reduction is “above and medial to the pubic tubercle” (superficial ring) o Incarcerated: The patient is unable to reduce the lump.

Lay the patient supine. (supposing you’re standing on patient’s LEFT) - Reduce the hernia if patient has not done so. - Locate the Deep inguinal ring: [vice versa for right side]

o Left hand define pt’s pubic tubercle: from umbilicus down Æ pubic symp. Æ to the left Æ 1st bony prominence o Right hand define the ASIS o Left hand to the midpoint of inguinal ligament Æ 2cm above

- Keep pressure on deep ring, ask patient to sit up & support his pelvis, then swing over the bed and stand With patient standing: - Sir, could you turn head and cough?

o if remains reduced – indirect hernia, o if not, direct hernia. (poor accuracy)

- Remove pressure & watch movement of hernia: slide obliquely (indirect) or project forward (direct) - Percuss & ascultate for bowel sounds Examine other side

Offer: 1) Abdo exam: scars, masses, ascites, ARU, constipation, IO 2) DRE for BPH, impacted stools 3) Respiratory exam for COPD 4) Ask patient for history of heavy lifting

Differential diagnosis: - Femoral hernia - Inguinal LN - Hydrocele of the cord (boys), or canal of Nuck (girls) - Saphenous varix: [bluish-tinge, disappears on lying supine, also

has positive cough impulse] - Undescended testes - Lipoma of the cord

32

FEMORAL HERNIA - Uncommon, but more common in females - Rarely put up for exams as it is operated quickly; - Femoral hernia is a marble shaped lump below the inguinal ligament and medial to femoral pulse

o usually irreducible; narrow neck Î risk of strangulation is high o Usually does not have a cough impulse

- DDx: o Skin/ soft tissue: cyst, lipoma o Vascular masses: saphena varix, femoral aneurysm, inguinal LAD o Hernia: inguinal hernia, obturator hernia o Other: psoas bursa, ectopic testis

Differences between an inguinal and a femoral hernia Inguinal Hernia Femoral Hernia

Appear through superficial ring above & medial to pubic tubercle Appears through femoral canal below & lateral to pubic tubercle Usually reducible Usually not reducible

Expansile cough impulse usually present Expansile cough impulse usually absent Low risk of strangulation High risk of strangulation

Anatomy of the femoral canal - The femoral sheath is the downward protrusion of

fascia containing the femoral vessels & lymphatics below inguinal lig.

- The medial compartment of the sheath constitutes the femoral canal, which carries the lymphatics.

- The superior opening of the femoral canal is known as the femoral ring.

Boundaries of the femoral ring:

x Anterior: inguinal ligament x Posterior: iliopectineal ligament and superior

ramus of pubis x Medial: lacunar ligament x Lateral: femoral vein

INCISIONAL HERNIA

- Extrusion of the peritoneum and abdominal contents through a weak scar or wound on the abdominal wall - Physical examination similar, just add:

o lifting head off the bed to exacerbate hernia o palpate to determine the size of defect

- Similar complications as any hernia - Risk factors:

o Pre-op: age, immunocompromised, obese/ distended abdomen, Malignancy, o Intra-Op: poor technique of wound closure, placement of drains o Post-op: wound haematoma, wound infx, early mobilization, post-op coughing

- Treatment options: o Not all should undergo repair due to high risk of wound haematoma, infection, dehiscence and recurrent hernia o Conservative:

- Offer corset or truss - Weight loss and control the risk factors leading to

o Surgical: - Offer if complications of hernia are present - Control CVS & resp. disease; encourage pre-op wt loss - Principles: dissect the sac and close the defect using mesh overlapping

UMBILICAL HERNIA/ PARAUMBILICAL HERNIA

- Uncommon b4 40YO; may grow to large size. - Cause: defect through the linea alba 2o to stretching the fibers

o Pregnancy, ascites, cyst, fibroids, distention - Issues of concern:

o Narrow Neck of hernia sac Î higher risk of strangulation/ infarction o Fistula formation with discharge of contents may occur

- Management: o Conservative: treat medcial co-morbidities. o Surgical: Mayo’s Vest over pants operation

33

2. SCROTAL SWELLINGS ANSWER 4 QUESTIONS: 1. Can you get above the swelling? 2. Can you identify the testis and the epididymis? 3. Is the swelling transilluminable? 4. Is the swelling tender? Cannot get above swelling

x Cough impulse x Reducible x Testis palpable x Opaque

Hernia

x No cough impulse x Not reducible x Testis not palpable x Transilluminable

Infantile hydrocoele

Can get above swelling Testis not definable from

epididymis Opaque

Non tender Chronic haematocoele Gumma Tumour

Tender Torsion Epididymo-orchitis Acute haematocoele

Transilluminable Hydrocoele

Testis definable from epididymis

Opaque

Non-tender swelling of testis

Tumour

Non-tender swelling of epididymis

TB epididymis

Tender Epididymoorchitis

Transilluminable Cyst of epididymis EXAMINATION OF THE SCROTUM

- “ Examine this gentleman’s scrotum:” - Always examine him STANDING!

Inspect - inspect the groin and scrotum: scars and swelling - groin incisions are usually oblique; scrotal incisions are usually in the median raphe

Palpate - ask for any pain; when palpate, look at patient for tenderness - palpate one testes at a time - if palpated any swelling:

Is it tender? Can you get above the swelling? Can you identify the epididymis and testis Î lump is separate or part of them? Is it transilluminable?

Offer to: - Transilluminate the swelling if it is likely at hydrocele - Continue the examine the groin if it is a inguinoscrotal hernia - Examine the abdomen

34

HYDROCELE - Points from examination:

o Very swollen scrotum; uniformly enlarged o Cannot define testis well; no separable from testis o Maybe firm, tense or lax o Maybe transilluminable if acute (less in chronic hydrocele) o Can get above the mass; the superficial ring is distinct

- Definition of hydrocele:

o Excess accumulation of fluid in processus vaginalis (fold of peritoneum as testis descends; usually patent for fluid to accumulate)

- Types of 1o hydrocele:

o Vaginal hydrocele: Only in tunica vaginalis & does not extend into the cord

o Hydrocele of the cord Mass around the cord; attached distally to the testis Difficult to distinguish from irreducible inguinoscrotal hernia May extend up and beyond

o Congenital hydrocele: patent processus vaginalis filled with peritoneal fluid o Infantile hydrocele: hydrocele of cord and congenital hydrocele

- Types of 2o hydrocele o From testicular tumours o From torsion o From trauma o From orchitis (any inflm)

- Treatment options: o Conservative:

Watch & wait or Aspiration [tend to reccumulate] Must exclude a 2o cause

o Surgical: Lord’s plication of the sac Jaboulay’s operation to evert the sac

TESTICULAR TUMOUR

- Points from examination: o Inseparable form the testis; can get above i o Hard, nodular, irregular, non tender o Not transilluminable (but maybe a/w hydrocele)

- DDx: o Chronic infection with scarring o Long standing hydrocele with calcification,

- Classiccation: o Mostly seminomas or teratomas

Seminomas: 30-40YO, normal tumour markers, Rx with RT to paraaortic nodes and CT according to staging

Teratomas: 20-30YO, AFP/ BHCG raised in 90%, Rx with CT o Others: embryonal carcinoma, choriocarcinoma, yolk sac tumour, Leydig cell tumours (10% malignant ; a/w

gynaecomastia), Sertoli cell tumour (a/w gynaecomastia), Lymphoma (look for lymphoma elsewhere; poor prognosis)

- Treatment: o Staging, excision of whole testis with combination chemotherapy

EPIDIDYMAL CYST

- Points from examination: o Small mass separate from testis; can get above it o Firm; maybe loculated; transilluminable if large cyst

- Often multiple in the head of epididymis - May occur as a complication of vasectomy (spermatoceles) - Treatment:

o Conservative [mainstay] o Surgical: if painful, very large or frequent recurrences. Complicated by operative damage and fibrosis of

epididymis Î subfertility

35

VARICOCELE - Best noticed on palpating the standing patient - Points from examination:

o Mass is separate from testis; can get above it o Feels like a bag of worms; +ve cough impulse o Not transilluminable

- Definition: o Dilated, tortuous pampiniform plexus o 98% on Left side: left vein is more vertical, connects to left renal vein, longer than right one, often lacks a

terminal valve - Causes:

o Idiopathic in younger males around puberty o In older men with retroperitoneal disease: RCC

- Treatment options: o Conservative: risk of infertility o Surgical:

Transfemoral radiological embolization with coil or sclerosant Excise the surrounding veins via high retroperitoneum, inguinal or laprascopic approach

TESTICULAR TORSION (Surgical emergency)

- Clinical features: o Children/ teenagers o acute abdomen (T10 inervation) & acute scrotum a/w vomiting o swollen and tender scrotum, testis is high in scrotum; pain worsen by lifting testis up o Previous attacks of self limitng pain; ppt by trauma, cycling, straining, coitus

- DDx: o Epididymitis o Torsion of testicular appendage (pea coloured lump through scrotum) o Strangulated inguinoscrotal hernia

- Cause: o Maldescended testis hanging like a bell clapper within tunica vaginalis

- Treatment: o Emergency exporation if Doppler US –ve for flow or clinical suspicion

Untwisting (lateral) of affected testis and orchidoplexy of both testis to scrotum Warm up with warm pad to see reperfusion or check with doppler after untwisting [4h before ischaemia]

If dead, excise and replace with prosthesis

36

3. APPROACH TO LUMPS & BUMPS Permission: Introduce yourself Position: Ensure patient (and you) are comfortable Exposure: Expose area to be examined fully (Remember: Compare with other side if applicable) Inspection 1. Number: solitary / multiple 2. Site: take reference from bony points 3. Shape / Symmetry:

- Hemispherical, Round, Exophytic 4. Size 5. Scars 6. Colour & skin changes?

a. Sinuses, discharge b. Ulceration c. Erythema / cellulitis

Palpation (Ask patient: Is area painful?) 1. Overlying skin temperature 2. Tenderness 3. Surface:

- Smooth/ Irregular/ Rough 4. Margins clearly defined? 5. Consistency

- Hard > Firm > Tense > Soft 6. Mobility

- Fully mobile in all directions? - Fixed and immobile? - Mobile only in certain directions?

7. Relations to surrounding tissues - Move lump in 2 perpendicular planes

o Attached to skin? muscle / tendon / bone? o If appears to be attached to muscle:

Ask patient to tense muscle; Reassess mobility in the 2 planes x Intramuscular or below the muscle, it will disappear. x Above the muscle it will be more prominent. x Fixed to muscle, it will become less mobile.

8. Fluctuant? (for small / medium lumps) - Paget’s sign: Rest 2 fingers on opposite sides of lump, press down on middle of lumpÎ +ve: Feel fingers moving apart

9. Special tests - Transillumination [only for large lumps; Use pen torch on one side] - Pulsatility (only for some sites, e.g. Neck, abdomen)

- Place finger on opposite sides of lump o Expansile: fingers pushed apart o Transmitted: Fingers pushed in same direction (usually upwards)

- Slip sign – if lipoma is suspected - Tends to slip away from the examining finger on gentle pressure

- Compressibility / reducibility [if AVM, haemangioma, hernia suspected] - Compressible: Disappears on pressure, reappears on release (AVM) - Reducible: Disappears on pressure, reappears with opposing force (hernia)

- Auscultation – only for certain sites / lesions (e.g. neck, abdomen, etc.) Request – “I would like to complete my examination by…” x Examine the draining LNs x If sebaceous cyst / lipoma Æ “Looking for other lumps elsewhere” x Ganglion Æ “Looking for other lumps elsewhere” + “Asking for hand dominance” + “Taking an occupational history”

“Mr. X is a young Chinese gentleman…” “On inspection, there is a (single) (hemispherical) lump on the (dorsum of the forearm)” “It measures <take out ruler> ( x by x cm)” “There are (? scars, sinuses, ulceration, or discharge seen, nor overlying or surrounding skin changes)” “Is the lump tender?” “On palpation, the overlying skin is (not) found to be warm. It is (non-tender)” “The surface is (smooth), with clearly-defined margins” “The consistency is firm, and it is (non-fluctuant)” “The lump is (not) attached to the overlying skin, and it is non-pulsitile.” “It (appears to be attached to underlying muscle, as it is mobile horizontally but not longitudinally)” S: site, size, shape, scars, skin changes, surface

E: edge, expansility/ pulsatility C: colour, consistency, compressibility T: tenderness, temperature, transillumability O: others [fluctuance, fulid thrill] R: reducability, relationship to each other Ulcers: Base: granulation tissue, slough, fascia, muscle, bone Edge: sloping (healing), punched out, undermined, rolled (BCC), everted (SCC) Discharge: serous, sanguinous, haemoserous, purulent

37

Symptoms and signs Diagnostic significance 1. Lump in or on the skin Size, shape and surface features Revealed by inspection-is the lesion smooth-surfaced, irregular, exophytic (i.e. projecting out of the surface)?

Epidermal lesions such as warts usually have a surface abnormality but deeper lesions are usually covered by normal epidermis. A punctum suggests the abnormality arises from an epidermal appendage, e.g. epidermal (sebaceous) cyst

Depth within the skin Superficial and deep attachments. Which tissue is the swellingderived from?

Tends to reflect the layer from which lesion is derived and therefore the range of differential diagnosis (i.e. epidermis, dermis, hypodermis or deeper)

Character of the margin Discreteness, tethering to surrounding tissues, three-dimensional shape

A regular shaped, discrete lesion is most likely cystic or encapsulated (e.g. benign tumour). Deep tethering implies origin from deeper structures (e.g. ganglion). Immobility of overlying epidermis suggests a lesion derived from skin appendage (e.g. epidermal cyst)

Consistency Soft, firm, hard, 'indurated', rubbery

Soft lesions are usually lipomas or fluid-filled cysts. Most cysts are fluctuant unless filled by semi-solid material (e.g.epidermal cysts), or the cyst is tense (e.g. small ganglion). Malignant lesions tend to be hard and irregular ('indurated') with an ill-defined margin due to invasion of surrounding tissue. Bony-hard lesions are either mineralised (e.g. gouty tophi) or consist of bone (e.g. exostoses)

Pulsatility Pulsatility is usually transmitted from an underlying artery which may simply be tortuous or may be abnormal (e.g. aneurysm or arteriovenous fistula)

Emptying and refilling Vascular lesions (e.g. venous malformations or haemangiomas) empty or blanch on pressure and then refill

Transilluminability Lesions filled with clear fluid such as cysts 'light up' when transilluminated Temperature Excessive warmth implies acute inflammation, e.g. pilonidal abscess 2. Pain, tenderness and discomfort These symptoms often indicate acute inflammation. Pain also develops if a non-

inflammatory lesion becomes inflamed or infected (e.g. inflamed epidermal cyst). Malignant lesions are usually painless

38

3. Ulceration (i.e. loss of epidermal integrity with an inflamed base formed by dermis or deeper tissues)

Malignant lesions and keratoacanthomas tend to ulcerate as a result of central necrosis. Surface breakdown also occurs in arterial or venous insufficiency (e.g. ischaemic leg ulcers), chronic infection (e.g. TB or tropical ulcers) or trauma, particularly in an insensate foot

Character of the ulcer margin Benign ulcers-the margin is only slightly raised by inflammatory oedema. The base lies below the level of normal skin Malignant ulcers-these begin as a solid mass of proliferating epidermal cells, the centre of which eventually becomes necrotic and breaks down. The margin is typically elevated 'rolled' and indurated by tumour growth and invasion

Behaviour of the ulcer Malignant ulcers expand inexorably (though often slowly), but may go through cycles of breakdown and healing (often with bleeding)

4. Colour and pigmentation Normal colour If a lesion is covered by normal-coloured skin then the lesion must lie deeply in the skin (e.g.

epidermal cyst) or deep to the skin (e.g. ganglion) Red or purple Redness implies increased arterial vascularity, which is most common in inflammatory conditions like

furuncles. Vascular abnormalities which contain a high proportion of arterial blood such as Campbell de Morgan spots or strawberry naevi are also red, whereas venous disorders such as port-wine stain are darker. Vascular lesions blanch on pressure and must be distinguished from purpura which does not

Deeply pigmented Benign naevi (moles) and their malignant counterpart, malignant melanomas, are nearly always pigmented. Other lesions such as warts, papillomata or seborrhoeic keratoses may become pigmented secondarily. Hairy pigmented moles are almost never malignant. Rarely, malignant melanomas may be non-pigmented (amelanotic). New darkening of a pigmented lesion should be viewed with suspicion as it may indicated malignant change

5. Rapidly developing lesion

Keratoacanthoma, warts and pyogenic granuloma may all develop rapidly and eventually regress spontaneously. When fully developed, these conditions may be difficult to distinguish from malignancy. Spontaneous regression marks the lesion as benign

6. Multiple, recurrent and spreading lesions

In certain rare syndromes, multiple similar lesions develop over a period. Examples include neurofibromatosis and recurrent lipomata in Dercum's disease. Prolonged or intense sun exposure predisposes a large area of skin to malignant change. Viral warts may appear in crops. Malignant melanoma may spread diffusely (superficial spreading melanoma) or produce satellite lesions via dermal lymphatics

7. Site of the lesion Some skin lesions arise much more commonly in certain areas of the body. The reason may be anatomical (e.g. pilonidal sinus, external angular dermoid or multiple pilar cysts of the scalp) or because of exposure to sun (e.g. solar keratoses or basal cell carcinomas of hands and face)

8. Age when lesion noticed Congenital vascular abnormalities such as strawberry naevus or port-wine stain may be present at birth. Benign pigmented naevi (moles) may be detectable at birth, but only begin to enlarge and darken after the age of 2

39

4. LIPOMA Inspection

o Can be single, often multiple o Usually at neck, trunk o Hemispherical – may appear lobulated o Scars Î Implies recurrent lipoma

Palpation

o Smooth or lobulated on firm pressure – bulging between strands of fibrous tissue) o Soft / firm (depending on nature of fat) o Well defined edges (may not be regular; series of curves corresponding to each lobule o Pseudo-fluctuance if large – lipomas are not liquid; but fat maybe more liquid o Mobile in all directions (if subcutaneous) o Positive slip sign; No transilluminance / thrill o Usually in the subcutaneous tissue. [check attachment skin & muscle]

Background Information ¾ Definition: Benign tumour consisting of mature fat cells (distended with fat from over-activity)

o Malignant change does not occur o Liposarcomas arise de novo; occur in older age (deeper tissues – retroperitoneal, deep tissues of thigh, subscapular)

Liposarcoma classification 1. Well-differentiated 2. Myxoid, round cell (poorly differentiated myxoid) 3. Pleomorphic liposarcoma

¾ Clinical features o Can occur at all ages (not common in children) o Slow-growing, never regress o May be multiple: lipomatosis (multiple continuous lipomata)

Occur in buttocks / neck Can cause distortion of subcutaneous tissues.

¾ Treatment o Non-surgical – watch & wait o Surgical – If patient wants it removed (Pain / peripheral neuropathy – Dercum’s disease, Cosmesis)

Can be removed under LA Nuchal lipomas (back of the neck): extremely fibrous septae: difficult to excise If close to joint: LA may not be possible (may communicate with joint)

¾ Variants of lipomas / syndromes associated with lipomas

o Adiposis dolorosa (Dercum’s disease) Multiple painful lipomas in limbs, sometimes trunk Associated with peripheral neuropathy Angiolipomas: prominent vascular component

o Hibernomas: brown fat cells o Cowden’s disease – associated with:

Thyroid cancers Lipomas Palmoplantar keratoses Multiple facial papules Oral papillomatoses

o Bannayan-Zonana syndrome – rare AD dz: lipomas with macrocephaly and haemangiomas, intestinal polyps

“Mr. X is a young Chinese gentleman…” “On inspection, there is a hemispherical lump over the right scapula” “It measures 10 by 8 cm” “There are no scars, punctum, ulceration, or discharge seen, nor any overlying or surrounding skin changes” “On palpation, the overlying skin is not warm, it is non-tender” “The surface is lobulated, and its margins are not well-defined” “The consistency is soft, and it is fluctuant” “The lump is not attached to the overlying skin” “It is mobile in all directions with a positive slip sign” “It is not transilluminable” “I would like to complete my examination by looking for other similar lumps” “My provisional diagnosis is a lipoma” “My differential diagnosis is: large sebaceous cyst”

40

“Mr. X is a young Chinese gentleman…” “On inspection, there is a single hemispherical lump in the middle of the forehead just above the eyebrows” “It measures 1 by 1 cm in diameter” “There is a visible punctum over the lump” “There are no scars, sinuses, ulceration, or discharge seen, nor any overlying or surrounding skin changes” “On palpation, the overlying skin is not warm. It is non-tender.” “The surface is smooth, with clearly-defined margins” “The consistency is firm, and it is non-fluctuant” “The lump is attached to the overlying skin” “It is mobile in all directions” “Slip sign is negative” “I would like to complete my examination by…”

“Looking for other lumps elsewhere” “My provisional diagnosis is a sebaceous cyst” My differentials are: Lipoma / Dermoid cyst

5. SEBACEOUS CYST Inspection x Usually solitary (can be multiple) x Hemispherical x Site: face, trunk, back, neck, scalp, shoulders (none on palms / soles) x Variable size ; few mm to 4-5 cm x May have bluish discolouration x Punctum in apex: in 50% x May exhibit plastic deformation on palpation Palpation x Normal Temperature, non-tender (unless inflamed) x Smooth surface x Well-defined margins (lies in subcutaneous fat) x Tense consistency, may stretch overlying skin ( plastic deformation) x Non fluctuant, not transilluminable x Attached to skin, not attached to deeper structures, mobile in all directions Background Information ¾ Sometimes considered to be similar to epidermoid cyst

o More accurate terminology: pilar / trichilemmal cysts ¾ 2 histological types:

o Epidermal cyst: from infundibular portions of hair follicles o Trichilemmal cyst: from hair follicle epithelium (most common on scalp), frequently multiple (AD inheritance)

¾ Arise from infundibular parts of hair follicles ¾ Definition: Distension of sebaceous glands with sebum from blockage of opening ¾ Clinical features

o Occur in all age groups, rarely present before adolescence o Slow growing,– may appear suddenly at adolescence o May become infected: acutely painful, sudden increase in size o May spontaneously discharge contents through punctum, regress

Point of fixation & discharge along a hair follicle Point gets pulled inwards on enlargement of the mass – creates punctum Sebaceous horn may form from hardening of slow discharge from wide punctum

x Sebum slowly exudes, dries and hardens into conical spike x Sebum usually washed away – horn results only if overlying skin not washed x Can be pulled out of skin x Treatment: excision / curettage along with base + histological assessment

¾ Complications 1. Infection (±discharge) 2. Ulceration 3. Calcification (trichilemmal cyst) (may lead to cyst hardening) 4. Sebaceous horn formation, [hardening of a slow discharge of sebum from a large, central punctum.] 5. Malignant change

¾ Treatment o Non-surgical: leave alone (if small, asymptomatic). o Surgical

Complete excision of cyst and contents under LA. Prevention of recurrence: by removal of elliptical portion of skin containing punctum along the lines of Langers. If at the angle of jaw, be careful of the facial nerve during operation. Damage to zygomatic branch can cause eye ulceration.

¾ If lump is increasing in size, what to exclude? - Malignancies: BCC, Malignant melanoma. ¾ Cock’s peculiar tumour (complication)

o Proliferating trichilemmal cysts that can grow to large size, ulcerate may become infected, open, granulating & edematous Boggy, painful, discharging swelling solitary, 90% occur in scalp

o often mistaken for SCC scalp; Angry, malignant-looking (malignant transformation rare) Heaping up of granulation tissue from the lining of the cyst burst through skin, giving everted appearance regional lymphadenopathy may be present

¾ Gardner’s syndrome (If multiple lumps found)

o Genetic disorder associated with: Multiple osteomata of skull & epidermal cysts Adenomatous polyposis of large bowel & CRCs Desmoid tumours Thyroid cancers

Inflamed Cyst

41

6. GANGLION Inspection ¾ Single; may have ovelying scar [recurrent mass] ¾ Hemispherical, flattened, ¾ Near joint capsules, tendon sheaths (90% on wrist, hand – ventral / dorsal) ¾ Variable (0.5 – 6 cm) Palpation x Normal temperature, non-tender x Smooth surface with Well-defined margins x May be multilocular x Soft & fluctuant if large > firm consistency if small x Weakly transilluminant. (gelatinous material) x Mobility:

o Should assess mobility in 2 perpendicular planes, then with underlying muscles tensed (less mobile when tensed) o Not attached to overlying skin (mobile over it) o Attached to fibrous structures of origin [to joint capsule, tendon sheath, intramuscular septum, fixed when tensed]

x Reducibility: may slip between deep structures when pressed (appears falsely reduce into joint) Request x Other similar lumps x Ask which hand is dominant (may affect management) x Occupation

Background Information ¾ Definition: Cystic myxomatous degeneration related to synovial lined cavity [joint capsule or tendon sheath]

Origin controversial: pockets of synovium communicating with joint, tendon sheath / degeneration of mucoid fibrous tissue ¾ Site:

o Can occur anywhere in body o Common in areas of fibrous tissue (e.g. around joints, esp. Dorsal > Volar wrist @ scapholunate joint)

Most common soft-tissue mass in the hand ¾ Types:

1. Simple 2. Compound – chronic inflammation distends tendon sheath above and below the flexor retinaculum. 3. Occult 4. Interosseous

“Mr. X is a young Chinese gentleman, who is alert and comfortable…” “On inspection, there is a single hemispherical lump on the dorsum of the left wrist” “It measures 3 by 2 cm” “There are no scars, ulcerations, or discharge seen, nor any overlying or surrounding skin changes” “On palpation, the overlying skin is not warm. It is non-tender” “The surface is smooth, with clearly-defined margins” “The consistency is soft, and it is fluctuant” “The lump is not attached to the overlying skin” “It appears to be attached to underlying muscle, as it is mobile horizontally but not longitudinally” “It is transilluminant” “I would like to complete my examination by…”

“Looking for other similar lumps” “Asking Mr. X for his hand dominance” “Taking an occupational history”

“My provisional diagnosis is a ganglion” My differential is a 1. Bursa 2. Cystic protrusions of synovial cavity of arthritic joints

3. Benign giant cell tumours of flexor shealth (These 2 are normally soft in consistency. Ganglion is more tense.)

42

¾ Clinical features o Majority between 20 and 60 years (rare in children) o Grow slowly over months / years o Non painful

¾ Differentials

o Bursae (soft) o Cystic protrusion of synovial cavity in OA (joint will be abnormal) o Benign giant cell tumours of flexor shealth (Pigmented VilloNodular Synovitis) o Lipoma o Sebaceous cyst

¾ Treatment

o Non-surgical Watch & wait, usually may disappear after a few months. Aspiration + 3/52 of immobilisation (successful in 30-50%). High chance of recurrence 6-12/12 later.

o Surgical Complete excision to include neck of ganglion at site of origin. Along the lines of Langers. Complications

x Wound complications: Scar, haematoma, infection x Recurrence <10% x Damage to adjacent neurovascular structures. x Stiffness & Contractures

43

7. BASAL CELL CARCINOMA – “Examine this gentleman’s face” (If the other side of the face, etc. needs to be inspected, ask the patient to turn his head – avoid moving around the patient)

Inspection x Single (often multiple) x Commonly found on the face, (above line drawn from angle of mouth to earlobe) x Hair-bearing, sun-exposed skin (especially around the eye) x Lesions raised above the skin:

o Nodular/ nodulo-ulcerated type (most common): Pearly, rolled edges [smooth, glistening, slightly transparent] May be pigmented, with telangiectasia over the lump May have central ulceration; erode facial structures if advanced

o Cystic: large cystic nodule x Lesions not raised:

o Pigmented: contains melanin; confused with malignant melanoma o Sclerosing: flat or depressed with ill defined edges; maybe ulcerated o Bush-fire / Cicatricial: multiple superficial erythematous lesions with pale atrophic areas o Superficial: erythematous scaly patches

x Base: o May be covered with coat of dried serum & epithelial cells o If deep: may expose deeper tissues (bone, muscle, etc.), covered with poor-quality granulation tissue o On face: may erode deep into facial structures

Palpation (Important to palpate for mobility: fixation and deep local invasion) x Normal temperature, may be painful / itchy x Firm / solid consistency x should be mobile over underlying structures, confined to skin

o If fixed, immobile Î deeper invasion x Regional LAD (metastases are extremely rare, to rule out SCC) + Ask about pre-disposing factors

A = nodular B = pigmented C = sclerosing D = superficial

“Mr. X is an elderly Chinese gentleman…” “On inspection, there is a single hemispherical lump just above alar of the nose” “It measures 2 by 2 cm” “The edges of the lump are well defined, with a pearly, rolled appearance” “There is a small area of pigmentation on the periphery of the lump, and fine telangiectasia are seen over the lump” “There are no visible scars, ulceration, or discharge seen, nor any other overlying or surrounding skin changes” “On palpation, the overlying skin is not warm. It is non-tender.” “The surface is smooth” “The consistency is firm” “The lump arises from the skin” “It is freely mobile over the underlying tissues” “My provisional diagnosis is basal cell carcinoma” “My differentials include SqCC, keratoacanthoma” “I would like to complete my examination by…”

Examining for cervical lymphadenopathy (although very rare in BCC)

44

Background Information ¾ Locally invasive carcinoma of basal layer of the epidermis ¾ Does not metastasize, but can infiltrate adjacent tissues ¾ Common in sun-exposed skin ¾ Pre-disposing factors

o Congenital (rare) Xeroderma pigmentosum (familial, associated with failure of DNA transcription) Gorlin’s syndrome (rare autosomal dominant cancer)

o Acquired Sunlight (especially UV light; UV-B range) Carcinogens (smoking, arsenic) Previous RT Malignant transformation in previous skin lesions (e.g. naevus sebaceous)

¾ Clinical features o In elderly people (incidence increases with age) o Rare in dark-skinned races o Males > females o Grow very slowly; months / years typically o May spread radially – leaving central scar o Persistent nodule / ulcer with central scab (repeatedly falls off, reforms) o May have itch / pain o If neglected: deep infected ulcer

¾ Macroscopic appearances: o Above the skin:

Nodular Nodulo-ulcerative / Deeply eroding ulcer (“rodent ulcer”) Cystic

o Not raised above the skin: Pigmented Geographical / cicatricial / “bush-fire” (advancing edge, healing centre) Sclerosing (flat / depressed tumour, ill-defined edge) Superficial (erythematous scaly patches)

¾ Microscopic features o Most commonly islands and nests of basaloid cells in dermis o High mitotic rates, peripheral palisading o (islands arranged radially with long axes in // alignment)

¾ Origin of various appearances: o Tumour always starts as a nodule o When central epithelium dies, ulcer develops (nodulo-ulcerative)

Edge rolled – raised up and rounded (but not everted) (may be only clue to diagnosis) o If centre of tumour does not necrose / ulcerate: nodule enlarges → cystic appearance

Not really cystic: solid and non-fluctuant o If pigmented brown by excess melanin: pigmented BCC o Geographical appearance:

When nodule first ulcerates, rolled edge is circular Shape becomes irregular as malignant cells spread As ulcer heals: irregular, raised edge around flat white scar – “bush-fire” BCC

¾ Differentials o SqCC

Especially if ulcerated But if rolled edge: more likely BCC

o Keratoacanthoma (adenoma sebaceum, molluscum pseudo-carcinomatosum) But scar will be deep (see below)

o If pigmented: malignant melanoma (rare in Singapore) ¾ Treatment

o Raised above skin: excision with 0.5 cm margin (maximum) o Not raised above skin: wider margin of excision, especially if at inner acanthus of eye, nasolabial fold, nasal floor, ear

Frozen section may be needed to ensure adequate excision o Alternative:

RT o Eyes, ears, nasolabial fold lesions: Moh’s chemosurgery

Staged chemosurgery, histological assessment of margins & electrodessication

45

8. SQUAMOUS CELL CARCINOMA

Inspection x Single (may be multiple) x More common on sun-exposed skin – head, neck, arms, hands, trunk x may be of considerable size (> 1 cm) x Round nodule or Circular ulcer or Irregular/ exophytic/ fungating mass x Well defined edges:

o everted (excessive growth raises it above skin) o dark, red-brown colour (very vascular)

x May have central ulceration, Base: o Necrotic tumour; may be covered with coagulated blood / serum o Granulation tissue: tends to be pale, unhealthy o Deep tissues may be exposed o Depth: variable (may be very deep; especially in soft tissue) o Can be copious, bloody, purulent, foul-smelling discharge

x Surrounding tissue may be oedematous, thickened Palpation x normal temperature, not tender x usually mobile

o If immobile: invasion into deep structures Request for: x Examination of local lymph nodes (5% at time of presentation)

o Often enlarged (but may not contain tumour even if enlarged – can be from infection) x Examination for sites of metastases

o Respiratory: lung (pleural effusion) o Abdominal: liver (hepatomegaly)

x Take a history looking for predisposing factors (see below) Background Information ¾ Carcinoma of the cells of the epidermis forming superficial keratinous squamous layer ¾ Local invasion into epidermis, dermis, adjacent tissues, & lymphatic spread to LNs ¾ Microscopy:

o Tongues of tumours cells spreading in all directions o “Epithelial pearls” – nest of squamous epithelium, cells are arranged in concentric circles surrounding a central focus of

acellular keratin ¾ Clinical features

o Incidence increases with age (usually elderly male) o Predisposition:

Congenital: x Xeroderma pigmentosum (AD, failure of DNA transcription)

“Mr. X is an elderly Chinese gentleman…” “On inspection, there is a single irregular ulcer on the dorsum of the right hand, proximal to the 1st & 2nd MCP joints” “It measures 1.5 by 1.5 cm. The edge of the ulcer is well-defined, red and heaped up.” “The base of the ulcer is shallow and contains red granulation tissue.” “There are no scars, or discharge seen, nor any surrounding skin changes.” “On palpation, the surrounding skin is not warm. It is non-tender” “The edges are firm” “The lump arises from the skin” “It is fixed and immobile” “My provisional diagnosis is squamous cell carcinoma” “My differentials are…”

o Benign: Keratoacanthoma, infected seborrhoeic wart, solar acanthosis, pyogenic granuloma o Malignant: BCC, malignant melanoma, solar keratosis

“I would like to complete my examination by…” “Examining the local lymph nodes for lymphadenopathy” “Taking a history looking for sites of metastases” “Examining the abdomen and lungs for signs of metastases”

46

Acquired x Envn: sunlight, Irradiation, Chemicals x Pre-existing lesions: Solar keratosis, Bowen’s disease x Chronic ulcers: old burns, chronic venous ulcers x Immunosuppresion (post-transplant, HIV)

o Usually has been growing for 1 – 2 months before being noticed Begins as small nodules on skin As enlargement occurs, centre necroses, sloughs Nodule turns into ulcer Ulcer initially circular with prominent everted edges Subsequently enlarges & changes to any shape

x Bleeding (more common with SCC than BCC) x Discharge x Pain (invasion of deep structures) x Lymphadenopathy

¾ Complications o Infection o Bleeding (massive / fatal if erosion into large vessel)

¾ Treatment (depending on site of lesion)

o Wide-excision with 1 cm margin o Radiotherapy (if unresectable, nodal spread) o + Block dissection of regional lymph nodes (if involved) o Eyes, ears, nasolabial fold lesions: Moh’s chemosurgery

Staged chemosurgery, histological assessment of margins & electrodessication Lesions Associated with SqCC Marjolin’s ulcer

¾ SqCC arising in long-standing benign ulcer / scar o Commonest ulcer: venous ulcer o Commonest scar: burns

¾ Very similar in appearance to classic SqCC, but may not be so florid

Bowen’s disease (SqCC in situ)

¾ Very slowly growing, may progress to SqCC ¾ red, scaly irregular plaque on the trunk

o if on the penis, vulva or oral cavity = erythroplasia of Queyrat ¾ Intraepidermal carcinoma

o a/w visceral malignancies in 5-7 yrs time esp if area of skin has not been exposed to the sun

¾ HPV has been found in some lesions ¾ Microscopically

o Epidermis (Atypical keratinocytes) o Basal layer is intact

¾ Treatment: excision (SqCC will grow eventually)

Solar (actinic) keratosis (SqCC in situ)

o Multiple yellow-grey to brown scaly tumour Small, hard, Begin with thickening of skin

o On sun-exposed skin of elderly patients o 25% may undergo change to SqCC o Microscopically: hyperkeratosis, atypical dividing cells in prickle cell layer (irregular

acanthosis), focal parakeratosis, basal layer atypical only (vs atypia in whole epidermis in SqCC)

o Treatment: Non-surgical: cryotherapy, topical chemotherapy (5-FU) Surgical: curettage of affected skin

47

9. MALIGNANT MELANOMA

Inspection x Usually single (may have satellite lesions around primary lesion) x Site: Limbs, head & neck, trunk, subungual, mucocutaneous junction, mouth, anus x Any colour: pale pink, brown, black, purple (rich blood supply) x Clearly defined but irregular x May ulcerate, discharge x May have surrounding halo: brown (pigment), pink (inflammation) x Types:

o Superficial spreading melanoma (70%): Legs of women and backs of men Red, white, blue in colour Irregular edge

o Nodular type melanoma (15-30%): On trunk Polyploidal and raised Smooth surface with irregular edge Frequently ulcerated

o Lentigo maligna melanoma: On face or dorsum of hands & forearms Underlying lesion is flat, brown-black with irregular outline Malignant area is thicker and darker

o Acral lentiginous melanoma: More common in Asians and Blacks On hairless skin: subungual area, palms, soles Irregular area of brown/ balck pigmentation

o Others: amelanotic melanoma, intra-cranial melanoma, retinal melanoma “Beware of the man with the glass eye and hepatomegaly”

Palpation x Normal Temperature, Non-tender x Surface

o If small: smooth epithelium o If ulcerates: covered with crust (blood + serum) o If bleeding, / infected: wet, soft, boggy

x Firm consistency (small satellite nodules feel hard) x Mobile, moves with skin over deeper structures Request x Palpation for regional lymphadenopathy x Palpation for other subcutaneous nodules (lying along course of draining lymphatics)

“Mr. X is a middle aged Chinese gentleman…” “On inspection, there is a single flat-looking lesions over the right foot” “It measures 2 by 4 cm” “It is variegated in appearance, and exhibits red, white, and black discolouration” “The margins are clearly-defined and irregular” “There are no scars, nor any surrounding pigmentation, erythema, or ulceration, bleeding, or discharge” “On palpation, the overlying lesion is palpable. It is not warm. It is non-tender” “The surface is smooth, and its consistency is firm” “The lump is attached to the skin, and moves with it over deeper structures” “My provisional diagnosis is malignant melanoma” “My differential diagnoses are: BCC, pigmented naevus” “I would like to complete my examination by…”

“Examining for regional lymphadenopathy” “Take a history for: cardinal symptoms of malignant change, and any predisposing factors”

48

Background information 4 commonest types of malignant melanoma ¾ Superficial spreading melanoma (70%) ¾ Nodular melanoma (15 - 30%) ¾ Lentigo maligna melanoma ¾ Acral lentigous melanoma Microscopic features ¾ Consists of loose nests of melanocytes in basal cell layer: ¾ Invade epidermis (leading to destruction, ulceration) & deeper into dermis, subcutaneous fat Clinical Features ¾ Very rare before puberty (usually > 20 years old) ¾ Equally in both sexes (but distribution different – see below) ¾ > 25% arise de novo

o Change in surface, size, colour, Halo, satellite nodules o Ulceration, bleeding o Itch, No pain o Lymphadenopathy

¾ Symptoms of distant metastases: LOW, SOB, jaundice o Lymphatogenouly to: regional LN o haematogenously to: Lungs [pleural effusion], Liver [hepatomegaly], Brain [focal neurological signs] & Skin and

subcutaneous tissues Predisposing Factors ¾ Congenital / non-modifiable

o Light skinned race o Xeroderma pigmentosum o Dysplastic naevus syndrome (B-K mole, FAMM syndrome) – 100% risk if 2 family members affected o Large congenital naevi o FHx in 1st degree relatives (1.5X risk)

¾ Acquired / modifiable o Sunlight exposure o Pre-existing skin lesions

Lentigo > 20 benign pigmented naevi (3 x risk)

o Previous melanoma (3.5 x risk)

Features of pigmented skin lesion suspicious of malignancy 1. Asymmetry 2. Bleeding & ulceration (late) 3. Change in: colour, size, shape, surface, number (early)

a. Surface: i. Loss of normal surface markings (e.g. skin creases) around lesion ii. Skin may become rough / scaly iii. Itchy with pale-pink halo (inflammation)

b. Size: i. Growth of newly-formed / long-standing mole ii. Increase in edge, width, thickness

c. Colour: i. Becoming darker ii. Halo of brown discolouration in skin around the lesion iii. Patchy colour change (black, to blue-purple Æ ↑ vascularity) iv. Occasionally colourless: no melanin production

d. Number: i. Satellite nodules of tumour around the lesion ii. Enlarged inguinal, axillary lymph nodes

4. Diameter >6mm 5. Elevation (flat plaque → nodule)

49

Request ¾ Examine draining lymph nodes ¾ Take a history for:

o Cardinal symptoms of malignant change in a mole Rapid increase in size Itching Bleeding Change in colour / shape / thickness

o Predisposing factors Differentials ¾ Benign

o Moles (pigmented naevus – ↑ melanocytes, ↑ melanin) o Freckles (normal number of melanocytes, ↑ melanin from each) o Lentigo (↑ melanocytes, normal amount of melanin from each) o Pigmented seborrhoeic keratoses o Dermatofibroma o Thrombosed haemangioma

¾ Malignant o Pigmented BCC

Staging by depth of invasion Clark’s levels of invasion

Level Extent of Tumour 5-Year Survival I Epidermis only 98% II Invades papillary dermis 96% III Fills papillary dermis 94% IV Invades reticular dermis 78% V Subcutaneous tissue invasion 44%

Breslow’s thickness (different in absolute depth of invasion, LN involvement)

Breslow Thickness 10-Year Survival < 0.76 mm 92%

< 3 mm 50% < 4 mm 30%

LN Involvement < 40% (8 yr) ¾ Also: Beahrs and Myer’s system ¾ Prognosis generally poor – above 3 types of staging, or other indicators of poor prognosis:

a. Elderly b. Male c. Lesions on trunk d. Ulceration e. Depigmentation, amelanotic f. Aneuploidy, high mitotic index

Treatment ¾ Prevention (VERY IMPORTANT):

a. Avoidance of causative factors ¾ Surgical excision with wide margins down to deep fascia

a. Main lesion: i. < 0.76 mm: excise with 1 cm margin ii. 0.76 – 1.0 mm: excise with 2 cm margin iii. > 1.0 mm: excise with 3 cm margin

b. Nodal spread: i. If clinical suspicion, biopsy or FNAC of lymph nodes ii. If palpable: therapeutic block dissection

¾ Palliation / adjuvant for distant metastases a. Intralesional BCG therapy b. Immunotherapy: vaccines (raises anti-melanoma response), monoclonal antibody, cytokine interferon therapy

50

Superficial Spreading Nodular Lentigo Maligna Acral Lentiginous % 70% 15-30% – Rare

Site : Back : Legs Trunk

Face; Dorsum of hand /

forearm Hairless skin (palms, soles,

subungual area)

Colour Red, white, blue (varying pigmentation) Most often black Brown / black Brown / black

Edge Irregular Regular outline Irregular Irregular

Shape Palpable, but thin Thick, polypoid, raised Flat Flat

Surface - Smooth - -

Remarks - • Frequently ulcerated, bleeding

• Arises from patch of Hutchinson’s Lentigo • Malignant area usually thicker, darker • Area seldom ulcerates

• Rare type • Often misdiagnosed as haematoma, paronychia

Pictures

51

10. NEUROFIBROMA

Inspection x Often multiple x Anywhere in skin, subcutaneous tissues, e.g. forearm x Spherical / Pedunculated / Fusiform (long axes lie along length of limb) x Rarely more than few cm x Comment on any café-au-lait spots Palpation x normal temp., Non-tender x Smooth, Well-defined x Soft/ fleshy, rubbery consistency x Non-fluctuant x If in subcutaneous tissue: mobile within it x Move most freely perpendicular to course of nerve Request x Look for other similar lesions & other manifestations of NF-1: café-au-lait spots, axillary freckling, lisch nodules, optic glioma x Measure the BP (HPT 2o to phaeochromocytoma, CoA, RAS) x Examination of cranial nerve VII & VIII (acoustic neuroma) Background Information Sporadic Neurofibroma ¾ Benign tumour containing mixture of elements from peripheral nerves:

o Neural (ectodermal) o Fibrous (mesodermal)

¾ Often multiple ¾ History

o Any age (but usually adult) o Symptoms: usually cause no discomfort, rarely disfiguring o If related to nerve trunk, may be tender

Patient may get tingling sensations in distribution of nerve ¾ Histology

o Schwann cells: appear as bundles of elongated wavy spindle cells o Collagen fibrils, myxoid material o Often not encapsulated (unlike neurilemmomas)

¾ Complications of Neurofibroma o Pressure effects: spinal cord, nerve root compression o Deafness: involvement of VIII o Neurofibrosarcoma (only in NF-1): 5-13 % o Intra-abdominal effects: obstruction, chronic GI bleeding o Skeletal changes: kyphoscoliosis, cystic changes, pseudoarthrosis

¾ Treatment (single neurofibroma) o Non-surgical: leave alone if asymptomatic, patient agreeable o Surgical: indicated only if malignancy suspected

Local re-growth common (cannot be surgically detached from underlying nerve)

“Mr. X is a young Chinese gentleman…” “On inspection, there are two spherical, pedunculated masses on the back” “One measures 2 cm, and the other 0.5 cm in diameter” They are pink in colour, with well-defined margins” “There are no scars, sinuses, ulceration, or discharge seen, nor any surrounding skin changes” “On palpation, they are not warm and non-tender” “Their surfaces are smooth, and are firm, and rubbery” “They are attached to the skin, and are mobile over the deeper tissue layers” “My provisional diagnosis is neurofibromata” “I would like to complete my examination by…”

x "Looking for other similar lesions” x “Looking for manifestations of neurofibromatosis” x “Examining the cranial nerves”

52

Neurofibromatosis I (Von Recklinhausen’s disease) (Refer to paediatrics notes – neurocutaneous syndromes) ¾ AD, neurocutaneous syndrome; chr 17 ¾ Characterised by pigmentary changes, tumours, and skeletal, vascular dysplasias

o Fibroma ≥2 NF or ≥1 plexiform NF o Iiris harmatomas (Lisch Nodules) o Bone: dysplasia, pseudoarthrosis o Relatives o Optic glioma o Macules >6; >15mm post-pubertal o Axillary freckling

¾ Neurofibromata of all sizes (few mm to large subcutaneous nodules), related differently to skin o Within skin o Tethered to skin o Pedunculated

Plexiform Neurofibroma (elephantiasis neurofibromatosis) ¾ Very rare ¾ Excessive overgrowth of neural tissue in subcutaneous layers, giving tissue swollen oedematous appearance

o Often mistaken for lymphoedema, but lymphatic drainage is normal ¾ Can result in severe deformity: diffuse enlargement of peripheral nerve with skin involvement

53

11. DERMOID CYST

Inspection x Usually single x Ovoid / spherical x Site:

o Congenital, 1-2 cm usually Along lines of fusion of ophthalmic & maxillary facial processes Inner & outer ends of upper eyebrow

o Acquired, 0.5-1 cm usually Beneath skin likely to be injured e.g. fingers Scars often present

Palpation x Not warm, maybe tender if infected x Smooth surface, Well-defined margins x Consistency

o Congenital: Soft (not tense / hard) o Acquired: Hard & tense (sometimes stony hard)

x Fluctuant (if large) x Mobile over deeper tissues

o Deep to skin, in subcutaneous tissue i. Congenital: Not attached to skin or underlying structures ii. Acquired: may be tethered to scar

Background Information ¾ A dermoid cyst is a cyst deep to the skin, lined by skin ¾ 2 different methods of formation:

o Congenital: Accident during antenatal development o Acquired: Implantation of skin into subcutaneous tissue by injury

Clinical features ¾ Congenital (suspect if in child, young adult)

o Formed intra-utero, when skin dermatomes fuse o Occur at any point in mid-line, common in neck / face / nose

Particularly along lines of fusion of ophthalmic & maxillary facial processes Also: inner & outer ends of upper eyebrow

o May be seen at birth o Distends a few years later, becomes obvious; few symptoms other than cosmetic problems o Rarely infected

¾ Acquired – Implantation dermoid (suspect if in adult – Browse pg 60) o Develop when piece of skin survives after being forcibly implanted into subcutaneous tissue

Often by injury: cut, stab, etc. o Symptoms

Small, tense lump Painful and tender (in areas subjected to repeated trauma) Local effects (e.g. problems with grip / touch if on finger) Also rarely infected

o Differentials Sebaceous cyst (look for old injury, presence of scar near cyst: more likely dermoid)

Treatment ¾ Congenital

o Surgical treatment; complete excision o Full extent should first be established with X-ray / CT

Midline cysts may communicate with CSF; must exclude bony defect ¾ Acquired

o Complete excision of cyst

“Ms. X is a young Chinese girl…” “On inspection, there is a single ovoid lump just above the lateral edge of the left eyebrow” “It measures 3 by 2 cm” “There are no scars, ulceration, or discharge seen, nor any overlying or surrounding skin changes)” “On palpation, the overlying skin is not warm. It is non-tender.” “The surface is smooth, with clearly-defined margins” “The consistency is firm, and it is fluctuant” “The lump is not attached to the overlying skin nor underlying tissues.” “It is fully mobile in all directions.” “My provisional diagnosis is a congenital dermoid cyst” “My differentials are: sebaceous cyst, lipoma”

54

12. SEBORRHOEIC KERATOSIS (Senile wart / seborrhoeic wart / verruca senilis / basal cell papilloma)

Inspection x Often multiple x Any part of skin; most found on back & face x Round / oval x Light brown → black x “stuck on appearance”;; appears warty x Varying size; Few mm to 2-3 cm x Distinct margins Palpation x No warmth, no tenderness x Rough surface (sometimes papilliferous) x More firm than surrounding skin x Attached to skin x Special tests

o May be picked off gently – reveals patch of pale-pink skin, 1-2 surface capillaries (bleed slightly) (DON’T DO THIS IN EXAM)

Request to look for similar lesions elsewhere Background Information ¾ Benign outgrowth of basal layer of epidermis

o Raised above the level of normal epidermis ¾ Microscopy:

o Hyperkeratosis (thickening of keratin layer) o Acanthosis (thickening of prickle cell layer) o Hyperplasia of variably pigmented basaloid cells

Clinical features ¾ Occur in both sexes ¾ More common in elderly people ¾ Begin as a patch,

o increases in area, size over months / years o May not increase in thickeness o May suddenly fall off: leave pale-pink patch of skin

¾ Complications: o May become disfiguring, catch on clothes o May get infected (may imitate SCC, pyogenic granuloma) o Seldom bleeds (may cause it to change colour to brown)

¾ Leser-Trelat sign: Sudden onset of multiple seborrhoeic keratoses may imply visceral malignancy Treatment ¾ Non-surgical

o Can be left alone as it is benign ¾ Surgical – for cosmetic reasons, etc.

o Superficial shaving (lies above level of normal epidermis) o Cautery

“Mr. X is an elderly Chinese gentleman…” “On inspection, there is a single ovoid lesion lump on the back” “It measures 1 by 2 cm” “The margins are well-defined, and it appears to be slightly raised above the skin” “There are no scars, ulceration, or discharge seen, nor any surrounding skin changes” “On palpation, the overlying skin is not warm. It is non-tender” “The surface is rough and greasy; the consistency is firm” “The lump arises from the skin” “My provisional diagnosis is seborrhoeic keratosis” “My differential diagnosis is: pigmented naevus, melanoma” “I would like to complete my examination by…”

x “Looking for similar lesions elsewhere”

55

13. HAEMANGIOMA Background Information ¾ Vascular malformations

o Types Capillary: ⅔ of cases, include the cutaneous haemangiomata, telangiectasias Predominantly venous: venous angioma

x Deeper levels of subcutaneous tissue, may extend into muscle / joint x May have distended veins over the surface of the mass x Empty with pressure, may have bruit

Predominantly lymphatic: lymphangioma circumscriptum o Features

Develop as abnormal proliferation of embryonic vascular network Hamartomas May ulcerate, induce hyperkeratosis in overlying stratum corneum

¾ Many forms of cutaneous haemangiomata: (see table) o Strawberry naevus (cavernous haemangioma) o Port-wine stain (naevus vinosus) o Spider naevus o Campbell de Morgan spot

Strawberry Naevus Port-Wine Stain Spider Naevi Cambell de Morgan Spot Inspection

Site Head & Neck (can be anywhere)

Lips, face, mucous membranes of mouth, shoulders, neck, buttock

Upper torso, head and neck (drainage of SVC)

Trunk (bilat) – upper > lower Occasionally on limbs

Number May be multiple Usually single Usually multiple Usually multiple Colour Bright red / dark red Purple-red Bright red Dark red / deep purple Size Variable; 1-10 cm Variable Variable; few mm 1-3 mm Edge Well-defined Well-defined – Well-defined

Shape Sessile → pedunculated as they grow larger Variable Variable Circular, may be raised

Skin Changes

May have small areas of ulceration with scabs

May have dilated subcutaneous veins around lesion – –

Palpation

Surface Irregular, covered with smooth, pitted epithelium Smooth – Smooth

Consistency Soft – – – Mobility Mobile – – – Relations Confined to skin – – –

Special Tests

Compressible: pressure squeezes mass, leaves it collapsed: slowly refills

Not pulsatile – Compressible, fade completely –

Background Information Age Infants (congenital) Infants (congenital) – Middle-age → elderly Gender = – – –

Symptoms • Cosmesis • May ulcerate, bleed on trauma

• Cosmesis • Bleeding may occur – • Cosmesis

• Non-tender

Pressure • Collapses on pressure • Diminishes colour but doesn’t revert to normal

• Branches fade when arteriole compressed • Fade slightly

Remarks Regress spontaneously (few months – 3 years)

• Extensive, intradermal • Present from birth, does not change in size • Sturge-Weber syndrome: facial PWS with corresponding haemangioma in brain – contralateral focal fits • Found in limbs when a/w Klippel-Tranaunay Syndrome

• Form of telangiectasia • Dilated skin arteriole feeding small branches (leaving radially) • Increase in number • Associated with pregnancy, chronic liver disease (> 5) – request for abdominal examination

• Formed by collection of dilated capillaries fed by single / small cluster of arterioles • Have appearance of drops of sealing wax • No clinical significance

Pictures

56

Also ¾ Telangiectasias

o Dilatation of normal capillaries o Can be secondary to irradiation o Can be part of hereditary haemorrhagic telangiectasia (Osler-Rendu-Weber syndrome)

Autosomal dominant disease Overt and occult haemorrhage can present as haematuria, haematemesis, melaena, epistaxis, iron-deficiency

anaemia ¾ Vin rosé patch

o Congenital intradermal vascular abnormality o Mild dilatation of vessels in sub-papillary dermal plexus o Can occur anywhere, gives skin pale-pink colour o Associated with other vascular abnormalities (e.g. haemangiomata, AV fistulae, lymphoedema) o Usually not disfiguring

57

14. PYOGENIC GRANULOMA

Inspection x Single; usu < 1 cm, bright red

o May be blood-encrusted or Ulceration x Hemispherical; may be sessile / pedunculated x Likely sites to be injured, e.g. hands, face x Bright red; long-standing lesions may be skin-coloured x May have sinuses, associated serous / purulent discharge, Erythema / cellulitis Palpation – request to palpate: may bleed easily x May be slightly tender

o May bleed easily on palpation x Well-defined edges x Soft, fleshy consistency x Confined to skin x Slightly compressible (vascular origin) Request x Take history for previous injury x Rate of growth of lump? (rapid growth in few days) Background Information – Neither pyogenic nor a granuloma! ¾ Rapidly-growing capillary haemangioma, usually less then 1 cm ¾ Occur commonly after injury:

o Small capillary loops develop in healing wound, form granulation tissue o When capillary loops grow too vigorously, form protruding mass, epithelisation o Mass form called pyogenic granuloma (surface often ulcerated, infected)

Clinical features ¾ Uncommon in children ¾ May have history of minor injury, chronic infection (e.g. paronychia) ¾ Rapidly-growing lump on skin, ¾ Bleeds easily, discharges serous / purulent fluid

o Bleeding, pain stops once lump epithelises ¾ Once nodule is completely covered, begins to shrink (rarely disappears completely) Treatment ¾ Surgical

o Curettage with diathermy of the base o Complete excision biopsy

(if recurrent; malignancy e.g. amelanotic melanoma has to be excluded) ¾ Non-surgical

o Regression is uncommon: surgical treatment best option o Silver nitrate cautery is possible

“Mr. X is a young Chinese gentleman…” “On inspection, there is a single hemispherical lump over the thenar eminence of the right hand” “It measures about 0.8 cm in diameter, with clearly-defined margins” “It is bright red in colour, with surrouding erythema” “There are no scars, sinuses, ulceration, active bleeding or discharge seen” “I would like to proceed on to palpation” “On palpation, it is not warm. It is slightly tender” “The surface is smooth. The consistency is soft and fleshy” “The lump is confined to the skin” “My provisional diagnosis is pyogenic granuloma” “My differential diagnoses are SCC, non-pigmented melanma” “I would like to complete my examination by…”

o “Asking Mr. X for any previous injuries to the hand” o “Asking him how rapidly the lump has been growing”

58

15. PAPILLOMA (skin tags / fibroepithelial polyps)

Inspection x Single / multiple x Variable: from raised plaque to pedunculated polyp x Site: Neck, trunk, face, anus (anywhere on skin) x Variable x Flesh-coloured Palpation x Not warm, non-tender x Variable: smooth to papilliferous x Soft, not compressible x Arises from skin Request x Similar lumps elsewhere x Ask for associated conditions: pregnancy, diabetes, intestinal polyposis Background Information ¾ An overgrowth of all layers of the skin with central vascular core ¾ Not a neoplasm, but a hamartoma (skin tag is a more accurate term) ¾ Increasingly common with age – may be congenital Clinical features ¾ Catches on cloths, rubs against other body parts ¾ May resemble carcinoma if granulation is excessive ¾ Complications:

o May become red, swollen, and ulcerate o May become infarcted if injured o May be infected (contains all skin components – sebaceous glands, etc.)

Treatment ¾ Excision – diathermy, scissors

o Bleeding from central vascular core controlled using single suture / diathermy

“Mr. X is a young Chinese gentleman…” “On inspection, there is a (single) (hemispherical) lump on the (dorsum of the forearm)” “It measures 3 by 2 cm. “The surface is papilliferous – there is no ulceration or discharge seen, nor any surrounding skin changes.” “On palpation, the skin is not warm. It is non-tender. The consistency is soft.” “The lump is attached to the skin” “My provisional diagnosis is: papilloma” “My differential diagnosis is: viral wart” “I would like to complete my examination by…looking for similar lumps, asking for associated conditions”

59

16. KERATOCANTHOMA (adenoma sebaceum, molluscum pseudo-carcinomatosum) Inspection ¾ Often found on face ¾ Usually solitary;1 – 2 cm in diameter ¾ Hemispherical or conical, with central crater ¾ Normal skin colour Palpation ¾ Firm and rubbery (central core is hard) ¾ Confined to skin, freely mobile over subcutaneous tissues Background information ¾ Benign overgrowth of hair follicle cells with a central plug of keratin ¾ Occur in adults ¾ complain of rapidly-growing lump in skin ¾ Not painful, but can be unsightly ¾ Takes 2 – 4 weeks to grow, regresses in 2 – 3 months

o Central slough appears, o surrounding skin retracts to form puckered scar

¾ Cause is unknown (may be self-limiting benign neoplasm or post-viral infection) ¾ Treatment:

o Conservative if asymptomatic o Surgical excision of lesion with histology to r/o SqCC

17. KELOID (HYPERTROPHIC SCAR) ¾ Healing by primary intention – 3 stages:

o Tissue defect filled by blood / fibrin o Replacement by collagen and fibrous tissue o Organisation of fibrous tissue to maximise wound strength

¾ Most surgical scars have thin lines, but tissue response may be excessive: hypertrophic / keloid scar ¾ Wounds prone to hypertrophic / keloid scar

o Infection o Trauma o Burns o Tension o Susceptible areas: across flexion areas, earlobes, chest, neck, shoulder

¾ Hypertrophic scar

o any age – common 8-20 years, =, all races o Excessive amount of fibrous tissue, but confined to scar (between skin edges) o Located across flexor surfaces, skin creases o Common, especially if infection / excessive tension o Only enlarge for 2-3 months, then regress spontaneuosly o Do not recur if excised and causative factor eliminated

¾ Keloid scar o puberty to 30 years, >, black, hispanic more likely o Hypertrophy and overgrowth extend beyond original wound o Located at earlobes, chin, neck, shoulder, chest o Due to local release of fibroblast growth factors o Continue to enlarge 6-12/12 after initial injury o May be tender, unsightly o Will recur unless special measures taken

¾ Treatment (recurrence can be as high as 55%) o Non-surgical: mechanical pressure therapy – topical silicone gel sheets (day and night for 1 year), Intralesional steroid,

LA injections: e.g. triamcinolone with lignocaine o Surgical: revision of scar by direct suturing, skin grafting (avoid excessive tension)

60

18. KAPOSI’S SARCOMA Inspection x Purple papules and plaques x Solitary, but usually multiple x Site: limbs, mouth, tip of nose/ palate or anywhere on the skin or mucosa Request to take a history of previous transplantation or current underlying immunocompromise. Background information x Derived from capillary endothelial cells or from fibrous tisse x Linked to HHV-8 x Types:

o Classic Kaposi’s Sarcoma Confined to skin of lower limbs of elderly Jews Not fatal

o AIDS associated Kaposi’s Sarcoma AIDS defining; Found in 1/3 of AIDS patients 1/3 develops a 2nd malignancy e.g. leukaemia/ lymphoma

o Endemic (African) Kaposi’s Sarcoma Aggressive and invasive fatal tumour Good response to chemotherapy

o Transplation- associated Kaposi’s Sarcoma Following high dose immunosuppressive therapy Often regress when treatment is ended

Treatment x Conservative if asymptomatic. Start anti-retrovirals if HIV +ve x Surgical: local radiotherapy amd chemotherapy (IFN- alpha, doxorubicin, intralesional vinblastine) 19. FIBROSARCOMA Inspection x Single; Usually limbs (but can be anywhere) x Spherical or hemispherical x If large, vascular: may make skin shiny & pink x May have

o Sinuses & Discharge o Ulceration o Erythema / cellulitis

Palpation x Usually feel warmer (abnormal blood supply) x May be tender x Smooth surface (may be bosselated – covered with knobs) x Well-defined margins (indistinct if fast-growing, invasive) x Firm / hard consistency (rarely stony hard; do not ossify) x Usually fixed x May pulsate, have audible bruit, palpable thrill (may be very vascular) Request to test for distal neurological status (for invasion of nerve) Background Information ¾ Fibrosarcoma is one of the commonest mesodermal soft tissue malignant tumours

o Pure benign fibroma is very rare ¾ History

o More common in elderly (but can occur any age) o Common complaints

Growth: disfigurement, interference with ROM Pain Weakness (infiltration of other structures) General debility

¾ Prognosis: generally good

61

20. PYODERMA GANGRENOSUM Inspect

x Ulcer with a necrotis base x Irregular bluish red overhanging edges x a/w surrounding erythematous plaques with pustules

Request to examine for evidence of inflammatory bowel disease, RA Backgound information x more common in males x pyoderma gangrenosum is associated with:

o IBD o RA o Myeloproliferative disorders: PRV, myeloma o Autoimmune hepatitis

x Differential diagnosis: o Autoimmune: rheumatoid vasculitis o Infectious: tertiary syphilis, amoebiasis o Iatrogenic: warfarin necrosis o Others: Behcet’s disease

x Treatment: o Non-surgical: treat underlying condition, saline cleansing, high dose oral or intralesional steroids.

KIV cyclosporine & antibiotics o Surgical: serial allograft followed by autologous skin graft or muscle flap coverage when necessary

62

21. RADIOTHERAPY MARKS Vital points on examination:

x Of the underlying disease: o Cachexia, o masectomy scar/ wide excision scar Î suggest breast cancer o obvious skin cancer, o clubbing & other signs of chest disease Î suggest lung cancer o suprapubic mass Î suggest pelvic tumour o neck swellings with cranial nerve palsies Î head and neck tumour

x of the radiotherapy: o site of radiation o shape: usually well defined borders o features of active RT:

Indian ink marks, skin markings Erythema, desquamation

o Features of previous RT: Telangiectasia, hyperpigmentation

x Complications of radiotherapy: o Depends of site o Look for future cancers:

Haematogenous malignancy Thyroid cancers Breast cancers

Background information

x High energy X-rays interact with tissue to release electrons that cause local damage to DNA in adjacent cells via oxygen dependent mechanism.

o Damage is usually irreparable, and normal cells have greater ability to repopulate than tumour cells in this setting o If reparable, manifests as chromosomal abnormalities

x Radiotherapy affects cells with: o Rapid turnover: Skin (epidermal layers), small intestine, bone marrow stem cells o Limited replicative ability: spinal cord, gonads

x Complications: o Early:

General: malaise, fatigue, LOA, N/V Skin changes & temporary hair loss Bone marrow suppresion, esp. if to long bone and pelvis GI: diarrhea

o Late: Skin changes Heart: IHD Lung: pneumonitis, pulmonary fibrosis Bld vssl: radiation arteritis, esp to carotids Î necrosis, distal ischaemia and vssl rupture CNS: spinal cord myelopathy Uro: bladder fibrosis, Renal impairment (depletion of tubular cells) Abdo: IO 2o to strictures & adhesions, Genital: infertility Endocrine: hypothyroidism Eye: cataracts Increase incidence of future cancers:

x Haematogenous malignancy, e.g. leukemia x Solid tumours: Thyroid cancers x Breast cancers

x Minimalising of side effects of radiotherapy: o Lead shields to eyes, gonads and thyroid o Dose fractionation (to allow recovery of normal cells) o Prior chemotherapy (increase sensitivity of tumour cells) o Regional hypothermia o Radiolabelled antibody to deliver local radiation to tumour

63

3. SURGICAL INSTRUMENTS & PROCEDURES 1. DRAINS FUNCTIONS OF DRAINS Drains are inserted to: Evacuate collections of pus, blood or fluids (e.g. lymph) Drain potential collections Rationale: Drainage of fluid removes further fluid collections Allow early detection of anastomotic leaks/ haemorrhage Leave tract for potential collections to drain after removal

COMPLICATIONS x Infection x Bleeding x Tissue damage- by mechanical pressure or suction x Drain failure - blocked/slipped/kinked x Incisional hernia - occurs when drain inserted through incision

wound site- create a separate incision site for drain!

TYPES OF DRAINS x Drains are often made from inert silastic material x They induce minimal tissue reaction x Red rubber drains induce an intense tissue reaction allowing a tract to form x In some situations this may be useful (e.g. biliary t-tube)

Open Active

x Corrugated drain, Yeates drain, Penrose drain x Drain fluid collects in gauze pad or stoma bag x Easier to drain infected collections

x Active drains require suction. x Jackson-Pratt Drain, Redivac Drain, T-tube x Have expandable chambers to create low-pressure suction x Used when small – mod amts of drainage are expected or when a

passive drainage system won't provide adequate drainage x Tubing of the low-pressure active drainage system is placed

through a separate puncture wound or the tube may exit the edge of the surgical wound

x If the tubing isn't sutured in place, it could become dislodged x If a portion of the tube is pulled outside the skin, an air leak will

cause the chamber to fill with air & it won't drain properly.

Closed Passive

x Consist of tubes draining into a bag or bottle x They include chest and abdominal drains x The risk of infection is reduced

x Passive drains rely on gravity. x Passive drains have no suction, rely on gravity x Works by differential pressure betw body cavities and the exterior x Used when a mod – large amt of drainage is expected

CARE AND PREVENTION OF COMPLICATIONS OF TUBES: x Prevent Infection- maintain meticulous skin care and aseptic technique around the insertion site x Prevent blockage of the drain- do not allow bottles to fill up x Prevent slippage by securing drain carefully to skin; refix as required x Never hold a drainage collection device higher than the tube insertion site to prevent the drainage from flowing backward into the patient x Note amount of drainage daily REMOVAL OF DRAINS x A drain is removed as soon as it is no longer required. The following are general guidelines: x Drains put in to cover perioperative bleeding and haematoma formation, can come out after 24— 48 hours. x Where a drain has been put in to drain an infection (abscess), remove it when fever settles or when there is evidence of complete drainage.

64

2. CENTRAL VENOUS PRESSURE LINE INDICATIONS 1. Vascular access 2. Total parenteral nutrition 3. Infusion of irritant drugs 4. Measurement of CVP 5. Cardiac catheterization 6. Pulmonary artery catheterization 7. Transvenous cardiac pacing.

CONTRAINDICATIONS: 1. Do not insert into an infected area. 2. Avoid infraclavicular approach to

subclavian vein if patient has apical emphysema or bullae.

3. Avoid internal jugular vein if carotid aneurysm present on the same side.

4. Bleeding diatheses 5. Septicaemia 6. Hypercoagulable states

ROUTES FOR CENTRAL VENOUS CANNULATION INCLUDE: 1. Internal jugular vein 2. Subclavian vein 3. Femoral vein 4. External jugular vein

CANNULATION OF THE INTERNAL JUGULAR VEIN The internal jugular vein (IJV) is accessible, so cannulation of this vein is associated with a lower complication rate than with other approaches. Hence, it is the vessel of choice for central venous cannulation. Anatomy of the IJV The vein originates at the jugular foramen and runs down the neck, to terminate behind the sternoclavicular joint, where it joins the subclavian vein. It lies alongside the carotid artery and vagus nerve within the carotid sheath. The vein is initially posterior to, then lateral and then anterolateral to the carotid artery during its descent in the neck. The vein lies most superficially in the upper part of the neck. Relations of the IJV x Anterior: Internal carotid artery and vagus nerve. x Posterior: C1, sympathetic chain, dome of the pleura.

On the left side, the IJV lies anterior to the thoracic duct. x Medial: Carotid arteries, cranial nerves IX-XII Technique of IJV cannulation Place the patient in a supine position, at least 15 degrees head-down to distend the neck veins and to reduce the risk of air embolism. Turn the head away from the venepuncture site. Cleanse the skin and drape the area. Sterile gloves and a gown should be worn to avoid catheter-related sepsis.

Procedure 1. Use local anaesthetic to numb the venepuncture site. 2. Introduce the large calibre needle, attached to an empty 10 ml syringe. 3. Surface mark the internal jugular vein at the centre of the triangle formed by the two lower heads of the sternocleidomastoid muscle and the

clavicle. Palpate the carotid artery and ensure that the needle enters the skin lateral to the artery. 4. Direct the needle caudally, parallel to the sagittal plane, aiming towards the ipsilateral nipple. 5. While needle is advanced, maintain gentle aspiration. 6. When vein is entered, flush of blood appears in the syringe. Now, cannulate the vein via the Seldinger technique (below). 7. Remove syringe, holding needle firmly in place. Occlude needle to prevent air embolism or bleeding. 8. Advance guide wire, J-shaped end first, into the vessel through the needle. 9. Hold guide wire in place and remove needle. Maintain a firm grip on the guide wire at all times. 10. Use a dilator to enlarge the hole in the vein. Remove the dilator. 11. Thread tip of catheter into the vein through the guidewire.

Grasp the catheter near the skin and advance it into the vein with a slight twisting motion. 12. Advance catheter into final indwelling position. Hold catheter and REMOVE GUIDEWIRE. 13. Check lumen placement by aspirating through all the pigtails and flushing with saline next. 14. Suture the catheter to the skin to keep it in place. 15. Apply dressing according to hospital protocol. 16. The catheter tip should lie in the superior vena cava above the pericardial reflection. 17. Perform check chest X-ray to confirm position and exclude pneumothorax. Complications 1. Pneumothorax/haemothorax 2. Air embolism - ensure head-down position. 3. Arrhythmias – This happens if cathether “irritates” the heart. Avoid passing guidewire too far, cardiac monitoring during insertion. 4. Carotid artery puncture/cannulation - palpate artery and ensure needle is lateral to it, or use ultrasound-guided placement, transduce needle

before dilating and passing central line into vessel, or remove syringe from needle and ensure blood is venous. 5. Chylothorax- Avoid cannulating the vein on the left side as the thoracic duct lies there. 6. Catheter-related sepsis

65

CANNULATION OF THE SUBCLAVIAN VEIN The subclavian vein (SVC) may be preferred for central venous access if 1. Patient has a cervical spine injury 2. Line is for long-term use e.g. dialysis, feeding. This site may be more comfortable for the patient. Anatomy of the SCV The SCV is the continuation of the axillary vein and originates at the lateral border of the first rib. The SCV passes over the first rib anterior to the subclavian artery, to join with the internal jugular vein at the medial end of the clavicle. The external jugular vein joins the SCV at the midpoint of the clavicle. Technique 1. Place the patient in a supine position, head-down. 2. Turn the head to the contralateral side

(if C-spine injury excluded). 3. Adopt full asepsis. 4. Introduce a needle attached to a 10 ml syringe. 5. Surface mark the subclavian vein 1 cm below the junction

of the middle and medial thirds of the clavicle. Direct the needle medially, slightly cephalad, and posteriorly behind the clavicle toward the suprasternal notch.

6. Slowly advance needle while gently withdrawing plunger. 7. When a free flow of blood appears, follow the Seldinger

approach, as detailed previously. 8. The catheter tip should lie in the superior vena cava above

the pericardial reflection. 9. Perform check chest X-ray to confirm position and exclude

pneumothorax.

Complications As listed for internal jugular venous cannulation. The risk of pneumothorax is far greater with this technique. Damage to the subclavian artery may occur; direct pressure cannot be applied to prevent bleeding. Ensure that a chest X-ray is ordered, to identify the position of the line and to exclude pneumothorax.

66

3. NASOGASTRIC TUBE INDICATIONS [4]

1. Diagnostic

a) bleeding from the upper gastrointestinal tract, haematemesis b) pentagastrin studies (rarely done now)

2. Decompresssion

Therapeutic a) intestinal obstruction b) pyloric stenosis c) haematemesis, esp in pts at risk of hepatic encephalopathy

Preventive: d) therapeutic and prophylactic decompression after major abdominal surgery e) prevention of further soilage after gastric perforation f) prevention of anastomotic rupture after gastric surgery g) prevention of obstruction of the operative field by air in the stomach

3. Nutrition

a) patients with dysphagia b) comatose or weak patients

4. Lavage

a) poisoning b) gastrointestinal bleeding

CONTRAINDICATIONS 1. Base of skull # 2. Oesophageal tear 3. Severe facial injury

The cuffed endotracheal and tracheostomy tubes should be deflated prior to nasogastric tube insertion. PRE-PROCEDURE 1. Gather equipment. 2. Don non-sterile gloves. 3. Explain the procedure to the patient and show equipment. 4. If possible, sit patient upright with head forward for optimal neck/stomach alignment. Otherwise, prop the patient up at 45 °. 5. Deflate the endotracheal tube or tracheostomy cuff 6. Determine the size of the nasogastric tube required (usually 14 – 16FG). If aspirating, use as large a tube as possible to reduce the risk of

blocking during use or the formation of a false passage during introduction; if feeding, a smaller tube may be used (eg. 8FG) because it is more comfortable in the long term.

PROCEDURE 1. Estimate the length of the tube to be inserted: from the bridge of the nose to the tragus of the ear to the point halfway between the

xiphisternum and the navel. Mark the measured length with a marker or note the distance. 2. Examine nostrils for deformity/obstructions (eg. choanal stenosis) to determine best side for insertion. Select the largest nostril for insertion. 3. Lubricate tube with water. The nose may be lubricated with lignocaine gel. 4. Introduce the tube through the nostril horizontally in, passing the tube along the floor of the nose. Resistance may be felt as tip reaches the

nasopharynx, which is the most uncomfortable part of the procedure. In the operation theatre, when the patient is under general anaesthesia, the McGill’s forceps may be used to guide the tube down.

5. Instruct the patient to swallow (you may offer ice chips/water if not contraindicated) and advance the tube as the patient swallows. Swallowing of small sips of water may enhance passage of tube into esophagus. If patient is uncooperative, bend his head to elicit a swallowing reflex.

6. Continue to advance the tube down the oesophagus. There should not be resistance. If resistance is met, rotate the tube slowly with downward advancement towards the closer ear. Do not force the tube down against resistance as this may form a false passage.

7. Withdraw the tube immediately if changes occur in the patient's respiratory status, if the tube coils in the mouth, or if the patient begins to cough or turns pretty colours.

8. Advance the tube until mark is reached (approximately 40cm). Stop. 9. Check for correct placement by attaching a syringe to the free end of the tube and aspirating a sample of gastric contents to test with litmus,

auscultating the epigastrium while injecting air through the tube, or obtaining an x-ray to verify placement before instilling any feedings/medications or if you have concerns about the placement of the tube.

10. Secure the tube with adhesive tape. 11. Re-inflate the endotracheal tube or tracheostomy cuff if necessary. 12. If for suction, remove the syringe from the free end of the tube; connect to suction; set machine on type of suction and pressure as prescribed. 13. Document the reason for the tube insertion, type & size of tube, the nature and amount of aspirate, the type of suction and pressure setting if

for suction, the nature and amount of drainage, and the effectiveness of the intervention. PROBLEMS AND COMPLICATIONS [5] 1. Technical 2. Gastrointestinal 3. Lung complications 4. Loss of fluids

& electrolytes, especially sodium, potassium, chloride and hydrogen ions.

5. Dry mouth and parotitis due to fluid loss and mouth breathing.

a) insertion into the trachea Æ choking.

b) coiling & reentry into oesophagus (rare).

c) trauma to the nose and the pharynx.

d) dislodgement e) perforation of the

pharynx and oesophagus

a) Gastric erosions b) Pressure necrosis of

pharynx, oesophagus or the external nares.

c) Varices: traumatic haemorrhage

d) GERD e) Oesophageal erosions

Æ strictures

a) decreased ventilation b) aspiration pneumonia

67

4. TRACHEOSTOMY INDICATIONS FOR TRACHEOSTOMY 1. Maintenance of airway patency. 2. Protection of the airway from aspiration. 3. Application of positive pressure to the airway. 4. Delivery of high oxygen concentrations. 5. Facilitation of secretion clearance. RELATIVE CONTRAINDICATIONS 1. Evidence of infection in the soft tissues of the neck at the prospective surgical site. 2. Medically uncorrectable bleeding diatheses. 3. Gross distortion of the neck anatomy due to hematoma, tumour, thyromegaly, high innominate artery or scarring from previous

neck surgery. 4. Documented or clinically suspected tracheomalacia. 5. Need for positive end-expiratory pressure (PEEP) of more than 15 cm of water. 6. Patient obesity with short neck that obscures neck landmarks. 7. Patient age younger than 15 years. TYPES OF TRACHEOTOMY 1. Temporary: Portex (cuffed). 2. Permanent: Consist of inner and outer tubes made of stainless steel. Tracheostomy is more useful in the elective setting compared to endotracheal intubation because: 1. Better tolerated. 2. Avoids risk of laryngeal stenosis 3. Avoids risk of endotracheal obstruction. PROCEDURE 1. Position the patient. Place rolled towel under the patient’s neck to hyperextend the neck for better exposure. 2. Clean and drape. Clean the skin of the neck from the chin to the suprasternal notch and laterally to the base of the neck and

clavicles. Drape field. 3. Identify anatomical landmarks (thyroid cartilage, cricoid cartilage). 4. Administer local anaethesia. 5. Incise skin. In the emergency setting, make a vertical incision 3cm from cricoid cartilage downwards. In the elective setting,

make a tranverse incision 4cm wide, 3cm above the suprasternal notch. 6. Dissect through the subcutaneous layers and platysma. 7. Identify the communicating branch of the anterior jugular vein, clamp and ligate the artery (ignore this in an emergency). 8. Visualise the thyroid isthmus and retract isthmus. 9. Retract cricoid cartilage upwards wth cricoid hook. 10. Incise the trachea between the 2nd and 3rd tracheal rings, making an inverted U-flap incision. 11. Insert tracheal dilator through the tracheostoma and remove the cricoid hooks. 12. Suction of blood and secretions in the lumen. 13. Insert the tracheostomy tube. 14. Remove the obturator and insert the inner cannula. 15. Dress wound and secure to the neck using sutures and adhesive tape.

68

COMPLICATIONS During Procedure 1. Bleeding if damage to the innominate or inferior thyroid artery. 2. Damage to surrounding structures, eg esophagus, recurrent laryngeal nerve, brachiocephalic vein. 3. Pneumothorax. Pneumomediastinum. Immediate post-op 1. Surgical emphysema (refers to the condition that causes air to be trapped under the skin). Subcutaneous emphysema. 2. Obstruction, eg clot, mucus. 3. Bleeding. 4. Dislodgment. Late post-op 1. Infection . 2. Obstruction, eg dislodgment of tube, crust formation from secretions. 3. Tracheal stenosis. 4. Tracheomalacia. 5. Tracheo-esophageal fistula. Wound breakdown 6. Scarring. POST-OP CARE 1. Position patient in a propped up position. 2. Prevent obstruction by suction, saline irrigation, mucolytic agents (mucomyst, guaifenesin) and humidified air. 3. Change Portex tube every 3rd day and remove the inner tube for cleaning everyday. 4. Unlock the metal tube every night so that the patient can cough it out if it becomes obstructed.

69

5. SENGSTAKEN-BLAKEMORE TUBE (MINNESOTA TUBE) INDICATIONS Oesophageal varices CONTRAINDICATIONS 1. Base of skull fracture 2. Oesophageal tear 3. Severe facial injury PROCEDURE - Keep SBT in fridge to make it stiff. 1. Measure the length of the tube. Test balloons. Test patency of the tube. 2. Sit the patient upright or at 45 degrees. 3. Apply local anaesthesia (lignocaine nasal spray). 4. Lubricate and insert the tube through the nose, asking the patient to swallow or drink water to aid in smoother passage of the

tube through the pharynx and oesophagus. 5. Inflate the gastric balloon slowly with 100-150ml water. 6. Check that the tube is in the stomach by:

(i) aspirating fluid and testing it with litmus, (ii) auscultating the epigastrium while injecting air, or (iii) doing an X-ray.

7. Traction. 8. Inflate the oesophageal balloon to 35 – 45mmHg (above portal HTN pressure): use the Y-connector piece with one arm to the

BP set and the other to the syringe to pump in air. 9. Aspirate fluid from the oesophagus through the Ryle’s tube, or if using the Minnesota tube, use the additional lumen provided

(with the additional lumen for aspirating fluid in the oesophagus, the Minnesota tube decreases the likelihood of aspiration pneumonia occurring).

10. Check the oesophageal balloon pressure hourly and release 5mins hourly. 11. Release oeophageal balloon after 24hrs. 12. Release gastric balloon after 48hrs. 13. The tube should not be used for more than 72hrs. COMPLICATIONS 1. Aspiration pneumonia 2. Respiratory obstruction 3. Oesophageal ulceration and rupture 4. Rebleeding 5. Gastric varices not controlled

70

6. URINARY CATHETERISATION INDICATIONS FOR SHORT-TERM CATHETERISATION 1. Relief of acute retention of urine, e.g. benign prostatic hypertrophy, bladder outflow obstruction. 2. Bladder washout, e.g. blood clots causing acute retention of urine. 3. Cystourethrogram. 4. Administration of intra-vesical drugs. 5. As an adjunctive measure pre/post-operatively

a) Pre-operatively: (i) to drain the bladder so as to improve access to the pelvis in urologic or pelvic surgery. (ii) to allow accurate measurement of urine output in major surgery.

b) Post-operatively: (i) to relieve acute urinary retention because post –op pain results in failure of the sphincter to relax.

6. Urinary output monitoring, e.g. in patient with hypovolaemic shock or the critically ill. INDICATIONS FOR LONG-TERM INDWELLING CATHETERIZATION 1. Refractory bladder outlet obstruction. 2. Chronic retention of urine, eg. neurogenic bladder. 3. Incontinence, e.g. in palliative care of terminally ill or patient’s preference.

CONTRAINDICATIONS 1. Presence of urethral injury, as manifested by:

a) blood from the meatus, b) scrotal haematoma, c) pelvic fracture, or d) high-riding prostate, elicited from a genital and digital rectal examination. (alternative: suprapubic drainage)

2. Urinary tract infection, as an indwelling catheter causes difficulty in treatment. PROCEDURE 1. Gather equipment. 2. Explain procedure to the patient. Maximize patient’s privacy. Have a chaperone if performing the procedure on a member of the opposite sex. 3. Assist patient into supine position with legs spread and feet together. 4. Open the catheterization kit and catheter. 5. Prepare sterile field. Don the sterile gloves from the kit. 6. Test the balloon at the tip of the catheter. 7. Generously coat the distal portion (2 - 5cm) of the catheter with lubricant. 8. Using the non-dominant hand to come in contact with the patient and the dominant hand to use items from the kit (recall that once your hand

comes in contact with the patient, it is no longer sterile and cannot be used to obtain items from the kit), cleanse the peri-urethral mucosa with antiseptic-drenched swabs held by forceps. Cleanse anterior to posterior, inner to outer, one swipe per swab, discard away from sterile field. a) Male: Hold the penis and retract the foreskin. Swab the penis and surrounding area, making sure to cleanse beneath the foreskin. b) Female: Retract the labia majora. Swab the perineum.

9. Apply sterile drape. 10. Installation of local anaesthesia.

a) Male: (i) Smear lignocaine gel around the meatus and apply the gel gently into urethra. (ii) Massage gel carefully down the urethra to sphincter, squeezing the meatus shut (iii) Wait for for 5 minutes (alternatively, with less anaesthetic effect, smear gel over the catheter tip).

b) Female: (i) Apply lignocaine gel to urethra or catheter tip.

11. In the male, lift the penis to a position perpendicular to patient's body and apply light upward traction (with non-dominant hand); in the female, expose the external urethral orifice.

12. Gently insert tip of catheter into the meatus using forceps until 1 to 2 inches beyond where urine is noted to drain into kidney dish. If no urine appears although the catheter seems to be in the right place, flush with sterile saline as the lumen may be blocked with gel. If this is still unsuccessful, withdraw and reinsert.

13. Inflate balloon, using correct amount of sterile saline (usually 20 – 30mls but check actual balloon size). This process should be painless. If patient feels pain, deflate balloon immediately and reposition catheter.

14. Gently pull catheter until inflation balloon is snug against bladder neck. 15. Connect catheter to drainage system. 16. Secure catheter to abdomen or thigh, without tension on tubing. 17. Place drainage bag below level of bladder. 18. Evaluate catheter function and amount, color, odour and quality of urine. 19. Remove gloves. Dispose equipment appropriately. Wash hands. 20. Document size of catheter inserted, amount of water in balloon, patient's response to procedure and assessment of urine. COMPLICATIONS 1. Infection, which may lead to stone formation. 2. Stricture formation due either to faulty technique or an irritant material used in the catheter. 3. Creation of a false passage due to wrong technique of insertion. 4. Occasionally, irritation of the bladder may cause severe bladder spasms.

71

7. CHEST TUBE Chest tubes are inserted to drain blood, fluid, or air and allow full expansion of the lungs. The tube is placed between the ribs and into the space pleural space. The area where the tube will be inserted is anesthetized locally. The patient may also be sedated. The chest tube is inserted through an incision between the ribs into the chest and is connected to a bottle or canister that contains sterile water (underwater seal). Suction is attached to the system to encourage drainage. A suture and adhesive tape is used to keep the tube in place. The chest tube usually remains in place until the X-rays show that all the blood, fluid, or air has drained from the chest and the lung has fully re-expanded. When the chest tube is no longer needed, it can be easily removed, usually without the need for medications to sedate or numb the patient. Antibiotics may be used to prevent or treat infection. INDICATIONS 1. Pneumothorax. 2. Hemothorax. 3. Drainage of pleural effusion. 4. Chylothorax 5. Drainage of empyema/lung abcesses 6. Prophylactic placement of chest tubes in a patient with suspected chest trauma before transport to specialized trauma center CONTRAINDICATIONS 1. Infection over insertion site 2. Uncontrolled bleeding diathesis/coagulopathy MATERIALS 1. Iodine & alcohol swabs for skin prep 2. Sterile drapes & gloves 3. Scalpel blade & handle 4. Clamp 5. Silk suture 6. Needle holder 7. Petrolatum-impregnated gauze 8. Sterile gauze 9. Tape 10. Suction apparatus (Pleuravac)/underwater seal apparatus 11. Chest tube (size 32 to 40 Fr, depending on clinical setting) 12. 1% lignocaine with epinephrine, 10 cc syringe, 25- & 22-g needles PRE-PROCEDURE PATIENT EDUCATION 1. Obtain informed consent 2. Inform the patient of the possibility of major complications and their treatment 3. Explain the major steps of the procedure, and necessity for repeated chest radiographs PROCEDURE 1. Determine the site of insertion. Locate the triangle of safety; bounded by the lateral border of the pectoris major, 5th or 6th

intercostal space, imaginary vertical line between the anterior and mid axillary lines. 2. Surgically prepare and drape the chest at the predetermined site of the tube insertion. 3. Locally anaesthetized the skin and rib periosteum. 4. Make a 2-3cm transverse incision at the predetermined site and bluntly dissect through the subcutaneous tissues, just over the

top of the rib. 5. Puncture the parietal pleura with the tip of a clamp and put a gloved finger into the incision to avoid injury to other organs and to

clear any adhesions, clots, etc. 6. Clamp the proximal end of the chest tube and advance the tube into the pleural space to the desired length. 7. Look for fogging of the chest tube with expiration or listen to air movement. 8. Connect the end of the chest tube to an underwater seal apparatus. 9. Suture the tube in place. 10. Apply a dressing and tape the tube to the chest. 11. Do a chest X ray 12. Obtain arterial blood gas values and/or institute pulse oximetry monitoring as necessary.

72

COMPLICATIONS Damage to structures: 1. Laceration or puncture of the intrathoracic and/or abdominal organs, all of which can be prevented by using the finger technique

before inserting the chest tube. 2. Damage to the intercostals nerve, artery or vein. 3. Subcutaneous emphysema, usually at tube site. Equipment: 4. Incorrect intrathoracic or extrathoracic tube position. 5. Chest tube kinking, clogging or dislodging from the chest wall or disconnection from the underwater seal apparatus. 6. Anaphylactic or allergic reaction to surgical preparation or anaesthesia. Failure: 7. Introduction of pleural infection. 8. Persistent pneumothorax 9. Recurrence of pneumothorax upon removal of the chest tube. 10. Lungs fail to expand due to plugged bronchus; bronchoscopy required. Recovery from the chest tube insertion and removal is usually complete, with only a small scar. The patient will stay in the hospital until the chest tube is removed. While the chest tube is in place, the nursing staff will carefully check for possible air leaks, breathing difficulties, and need for additional oxygen. Frequent deep breathing and coughing is necessary to help re-expand the lung, assist with drainage, and prevent normal fluids from collecting in the lungs.

73

4. ABDOMINAL SURGICAL EMERGENCIES 1. APPROACH TO ABDOMINAL PAIN

RHC

Epigastric

LHC

Thoracic Pneumonia Pleural effusion Biliary Cholangitis Cholecystitis Gallstone disease

Hepatic Hepatitis (viral,

autoimm etc) Hepatomegaly Abscess Others Subphrenic

abscess Pancreatitis PUD Appendicitis

Thoracic MI Pericarditis Aortic aneurysm Gastrointestinal Oesophagitis GERD PUD Gastric outlet

obstructn CA stomach

Others Pancreatitis

Thoracic Pneumonia Pleural effusion MI Gastrointestinal PUD Diverticulitis Mesenteric

ischaemia

Others Subphrenic

abscess Splenomegaly Pancreatitis

Rt Loin

Periumbilical Lt Loin

Biliary (see RUQ) Urological Infection Pyelonephritis Abscess Others PKD Renal cyst Angiomyolipoma Infarction

Obstruction Hydronephrosis Nephrolithiasis Ureteral

obstruction CA RCC TCC renal pelvis Bladder ca

(ureteral obstructn) Others Appendicitis

Gastrointestinal Appendicitis

(early) I/O Mesenteric

ischaemia Colitis IBD

Others Aortic Aneurysm Pancreatitis

Splenic disease Urological (see Rt Loin)

RIF

Hypogastric LIF

Gastrointestinal Appendicitis Terminal ileitis Meckel’s

diverticulitis Mesenteric

ischaemia Mesenteric

adenitis IBD Colitis Colorectal CA Hernia

O&G Ovarian cyst Ovarian torsion Ectopic pregnancy PID Orthopaedics (See LIF)

Gastrointestinal Colorectal CA Urological ARU Bladder calculi Cystitis / UTI

O&G Ectopic

pregnancy Abortion PID Uterine rupture Fibroid

complications Adenomyosis Endometriosis

Orthopaedics Infection Septic hip arthritis TB hip Degeneration OA hip Inflammation RA hip Ankylosing spondylitis Reiter’s syndrome Inflitration 1o bone tumour (hip) Metastasis to hip Destruction # - NOF, pubic rami Radiation Back pathologies (referred pain) Paediatric ortho conditions Transient

synovitis Perthes’ dz SCFE

Gastrointestinal Diverticulitis IBD Colitis Colorectal CA Hernia O&G (see RLQ)

74

2. APPROACH TO ABDOMINAL MASSES

RHC

Epigastrium

LHC

Liver Massive Cancer: HCC

Metastases Myeloprolftve dz Alcoholic liver dz Rt ht failure/

tricuspid regurg Moderate Above causes Lymphoprolftve dz Haemochromatosis Amyloidosis Mild Above causes Infxns:

Viral – Hep, IMS Bacterial – abscess Parasitic – hydatid cyst, amoebic abscess

Biliary obstruction Cirrhosis

Gallbladder Pancreatic/periamp

ullary ca Acute cholecystitis Hydrops Empyema Mirizzi syndrome Ascending colon Cancer Diverticular

mass/abscess Faeces Right adrenal gland Right kidney (see Rt lumbar)

Liver (see RHC) Pancreas Pseudocyst Tumour Transverse colon Cancer Diverticular

mass/abscess Faeces

Stomach Cancer Distension (GOO) Aorta Aortic aneurysm Retroperitoneal lNpathy Lymphoma Teratoma Other malignancies

Spleen Massive Infxns CML Myelofibrosis Moderate Above causes Portal hypt Lymphoprolftve dz

(lymphoma, CLL) H’lytic anaemia

(thal, HS) Storage dz

(Gaucher’s) Mild Above causes Infxns:

Viral hep, IMS, Endocarditis

Autoimmune – SLE, RA, PAN

Myeloprolftve dz – PRV, essential thrombocytopaenia

Infiltratn – sarcoid, amyloid

Stomach Descending colon Cancer Diverticular

mass/abscess Faeces Left kidney (see Rt lumbar) Left adrenal gland

Right Lumbar

Umbilical Left Lumbar

Right Kidney Hydro/pyonephrosis Cancer – RCC Polycystic dz Single cyst Amyloidosis Tuberous sclerosis,

VHL

Right adrenal gland Liver (see RHC) Ascending colon mass Cancer Diverticular

mass/abscess Faeces

Liver (see RHC) Stomach(see Epigastrium) Small intestine Obstruction Mesenteric cyst

Pancreas (see Epigastrium) Aorta Aortic Aneurysm Retroperitoneal lNpathy Lymphoma Teratoma Other malignancies

Spleen (see LHC) Left kidney (see right lumbar)

Descending colon Cancer Diverticular

mass/abscess Faeces

RIF

Hypogastrium LIF

Gastrointestinal Appendiceal

mass/abscess TB gut Ca caecum Distended caecum

(due to distal obstruction)

Crohn’s dz (terminal ileitis)

Orthopaedics Chondroma/sarcom

a of ilium Bony metastasis

O&G Ovarian cyst/tumour Fibroids Urogenital: Transplanted kidney Bladder

diverticulum Ectopic or

undescended testis Vascular: Iliac artery

aneurysm Iliac lymphadenitis Skin & Msk: Psoas abscess

Bladder Acute retention of

urine Chronic retention of

urine Anal/rectal mass Cancer

Uterus Gravid uterus Fibroids Tumour Ovary Cyst Tumour

Gastrointestinal Diverticular

mass/abscess Ca colon/sigmoid Crohn’s dz (terminal

ileitis) Faeces Similar causes as RIF mass

75

3. INTESTINAL OBSTRUCTION (& MECKEL’S DIVERTICULUM) Causes of IO 1. Mechanical

Intraluminal Mural Extraluminal x Impacted stool x Gallstone ‘ileus’ x Foreign body x Bezoars (phyto-, tricho-)

x Tumours x Diverticular stricture x Intussusception x Crohn’s strictures x RT strictures

x Adhesions (post-op, Crohn’s) x Herniae x Volvulus x Lymph node compression x Superior mesenteric artery syndrome

2. Functional (give rise to megacolon)

- Paralytic ileus = hypomobility without obstruction Æ accumulate gas/fluids DDx mechanical/pseudo: hypo/absent BS as opposed to highpitch tinkling

1. Post-op (most common): Physiologic ileus spontaneously resolves within 2-3 days, after sigmoid motility returns to normal. (Normal resumption of bowel activity after abdominal surgery follows a predictable pattern: SB - within hours; stomach - 1-2 days; colon - 3-5 days) Ileus that persists for more than 3 days following surgery is termed postoperative adynamic ileus or paralytic ileus. Lap shorter duration of ileus than open. Complications: Cx of immobility, catabolism due to poor nutrition

2. Sepsis 3. ischaemic (eg bowel, MI) 4. metabolic (hypoK/ hypoMg/ hypoNa, uraemia) 5. hypothyroidism, 6. opiate-induced, antacids, TCA 7. intraabd inflammation/peritonitis, biliary/renal colic, retroperitoneal hematoma 8. trauma (# rib/spine/HI)

- Pseudoobstruction = acute marked LB distension without obstruction. Clinical picture similar to mechanical obs. eg. Ogilvie syndrome: in severely ill patients (large bowel peristalsis disappears in ass with retroperitoneal pathology) 50% mortality if patient progresses to necrosis and perf.

- Hirschsprung’s disease: absent ganglia in Auerbach’s plexus

Ogilvie pseudo-obstruction in a septic elderly patient. Note the massive dilatation of the colon, especially the right colon and cecum

Others: intestinal atresia

Small bowel IO 1) Adhesions 2) Herniae 3) Ileocecal tumour 4) Intussussception

Large bowel IO 1) Cancer 2) Post-diverticulitis stricuture 3) Sigmoid volvulus Rarely adhesions (<1%)

76

HISTORY Consider:

x Is there I/O? x Is it functional or mechanical? x Is the bowel strangulated? Æ local peritoneal inflammation x SB or LB obstruction?

4 cardinal symptoms

1. Pain: - Usually colicky (central griping pain interspersed with periods of no/little pain), 4-5 days duration - SB: every 2-20min, LB: every 30min or more - Complete obstruction: constant, sharp pain - Volvulus: sudden, severe pain

2. Vomiting: - Pyloric: watery and acid, projectile - High SB: greenish blue, bile stained - Lower SB: brown and increasingly foul smelling (feculent = thick brown foul) - LB: uncommon esp if competent ileocaecal valve, usually late symptom

3. Abdominal distension - Usu not seen in high obs , esp if pt vomiting - Closed loop: RIF bulge that is hyperresonant - SB: distension in centre of abdomen

4. Constipation - Ask about normal BO frequency - Early in low obs, late in high obs - Complete obstipation: cannot even pass flatus

Other pertinent history

- Symptoms of GIT bleed, infection - Previous surgeries!!! - Underlying GIT disorders - Recent change in bowel habit - Risk factors for ischaemic bowel: atherosclerotic RF, heart disease, previous stroke (see below) - Suspicion of malignancy: LOW, LOA, previous Ca, FH of Ca

PHYSICAL EXAMINATION Vitals: Is patient stable? Any fever? (sepsis) Hypotension, tachycardia? (dehydration) General inspection: Abdominal distension? Cachexia? Confusion? Pale? Jaundice? Peripheries: Look for signs of dehydration e.g. capillary refill, dry tongue Palpate lymph nodes – Virchow, Sister joseph Abdomen:

Dx I/O Any distension? Visible peristalsis? – severe obstruction (see browse) Bowel sounds:

Mechanical: Initially hyperactive/loud Æ resonant/ high-pitch Æ sluggish or absent Tinkling BS: small bowel obstruction Ileus: hypo/absent BS

Succussion splash + epigastric tenderness: gastric outlet obstruction Causes of I/O SCARS from previous abdominal surgery? Any masses Any HERNIAE – inguinal + femoral (more likely strangulated) DRE: any masses felt, any impacted stools?

Cx of I/O Any signs of peritonitis: guarding, rebound tenderness, rigid

77

ACUTE MANAGEMENT o Resuscitate if necessary

1. Maintain airway 2. Give supplementary O2 (high flow if susp ischemia)

o Monitor vitals - Urinary catheter: monitor hourly urine output

o Drip and suck 1. NBM: rest bowel (alone does not give adequate rest as intestine produce up to 9L of fluids per day) 2. NG tube:aspirate stomach contents and then put on constant suction 3. IV drip: rehydrate and correct elec imbalance

o Start broad spectrum ABx [IO affects normal translocation of bacterial flora] – IV Ceftriaxone 1g + Metronidazole 500mg o Correct acidosis (ischemia), replace K+ as guided by investigations Rule out surgical emergencies (history/physical examination/investigations) Æ if suspected, immediate consult with GS Reg

1. Obstructed and strangulated abdominal hernia 2. Volvulus (sigmoid, caecal, stomach) 3. Closed-loop obstruction (competent ileocecal valve) 4. Ischaemic bowel with bowel necrosis 5. Perforation/peritonitis

INVESTIGATIONS Bloods

Assess complications x FBC: any infection, anemia x UECr :

Any dehydration due to: - Intra-luminal 3rd space loss (damaged enterocytes unable to reabsorb) - Vomiting (also assess K+ loss: can perpetuate paralytic ileus)

Acute renal failure from dehydration x ABG

- Acidosis from bowel ischaemia - Alkalosis due to vomiting (more for pyloric stenosis in children)

x ECG (+ CE X2 sets) in elderly

Pre-operative x GXM 4 pints of blood x PT/PTT

Imaging

Assess complications x Erect CXR :

1. air under diaphragm 2. aspiration pneumonia

x KUB/AXR: 1. Rigler’s Sign = double wall sign (obvious bowel wall due to extra-luminal air)

Blue arrows point to falciform ligament, made visible by a large amount of free air in the peritoneal cavity. The red arrows demonstrate both sides of the wall of the stomach (Rigler's sign), a sign of free air. The yellow arrow points to a skin fold.

2. Bowel ischaemia with necrosis? Gas in bowel wall (pneumatosis intestinalis) due to gas gangrene

78

Diagnostic x KUB (not AXR)

Dilated = 3cm SB, 5cm LB, 9cm caecum (increase risk of perf) - Erect: air fluid levels (>6) - Supine (better): look for small or large bowel dilatation on radiograph

Duodenum: C-shaped Jejunum/proximal ileum: centrally located, “stack of coins” (plicae circulares aka valvulae conniventes) Distal ileum: plicae circulares disappears, lead-pipe appearance Colon: more peripheral, incomplete bands (haustrations due to presence of teniae coli) Gas present in rectum? No: complete obstruction (may require KUB film)

- Sigmoid volvulus? Coffee bean sign:

There is an air-fluid level (black arrows) in each segment of dilated bowel. Note also the central cleft (white arrow) of the coffee bean.

- Caecal volvulus

- Closed loop obstruction? Distal lesion with v. distended cecum (thinnest wall; up to 12cm), ileum not dilated x Barium enema

- Gastrografin preferable if risk of perforation; risk of barium peritonitis (100% mortality!) - Upper GI barium studies contraindicated

x Colonoscopy done without bowel prep (not necessary, and also contraindicated, in IO) x CT colonography

DEFINITIVE MANAGEMENT (Depends on cause) 1) Obstructed abdominal hernia

Herniorrhapy 2) Sigmoid volvulus (refer below)

Non surgical/ conservative 1. Ryle’s tube decompression:

- Gown up - Put end of Ryle’s tube in a bottle submerged in water - Insert lubricated Ryle’s tube into anus

2. Flexible sigmoidoscopic decompression (recur in as much ast 50%. Elective laparoscopic sigmoid resection and right hemicolectomy following endoscopic decompression is increasingly being described and performed to treat patients with volvulus. The suggested interval between endoscopic decompression and definitive surgical intervention is 48-72 hours)

Surgical a. Sigmoid colectomy with primary anastomosis: Primary anastomosis is performed if the divided bowel ends are viable,

peritoneal contamination is not evident, and the patient is hemodynamically stable b. Sigmoid colectomy with Hartman’s procedure (may be reversed in 3-6/12 – depends on overall clinical condition and

ability to withstand another major surgery) c. Sigmoid colectomy & formation of double barrel colostomy (Paul-Mikulicz procedure) with future re-anastamosis

d. Sigmoidopexy: fix sigmoid to posterior abdominal wall (rarely done as high risk of recurrence) Other volvulus Caecal volvulus: right hemicolectomy with primary ileocolic anastomosis is the surgical procedure of choice (not

endoscopic decompression as only successful in 15-20%. rare to create ileostomy) Gastric volvulus: gastropexy

Parrot’s beak sign in sigmoid volvulus

79

3) Closed loop obstruction (if due to distal tumour)

Resect bowel + primary anastamosis with proximal defunctioning colostomy Resect bowel + Hartmann’s/Paul-Mikulicz procedure: if bowel is too inflamed/oedematous to anastamose (high risk of

leakage) Palliative: colonoscopy with stenting

4) Ischaemic bowel

Supportive management e.g. NBM, drip & suck, antibiotics while waiting for collaterals to re-supply bowel, re-establish peristalsis

Surgical intervention if bowel is non-viable e.g. gangrenous or necrotic o Will have relook laparotomy in 2-3/7 to ensure good resection of bowel margins??

5) Perforation (usually in caecum as it is thin walled)

Occurs due to ischaemia of stretched out bowel wall; >12cm dilated Emergency laparotomy: resect lesion & perforated bowel with generous peritoneal lavage If lesion e.g. tumour is proximal enough near to the cecum, can do extended right hemi-colectomy If lesion is distal, may require total-colectomy with ileo-rectal anastamosis Continue antibiotics following surgery

6) Intussusception

Children: usually due to hypertrophic Peyer’s patches. Administer barium enema: watch intussception reduce on fluoroscopy

Elderly: usually leading point present (polyp, Ca). Barium enema unlikely to work, or if works recurrence rate is high, therefore surgery is 1st line treatment

7) Post-op paralytic ileus (>3 days post-op)

a. Supportive management: drip & suck, wait for peristalsis to restart b. Oral gastrograffin: hyperosmotic, causes intraluminal osmosis , can re-establish peristalsis c. Prokinetic agents: erythromycin, metaclopromide, cisapride

80

MECKEL’S DIVERTICULUM RULE OF 2s

- Like the appendix, Meckel’s diverticulum is a true congenital diverticulum, a vestigial remnant of the vitelline duct - 2 inches in length, 2cm wide, 2 feet from ileocaecal valve - Occurs in 2% of the population, > in the ratio of 2:1 - Usually asymptomatic but if often presents at age 2 if symptomatic - 2 types of ectopic tissue

Pancreatic (6%) Gastric (60%) – gastric acid secretion can produce inflammation, peptic ulceration & bleeding, strictures with

subsequent IO May have both types of tissue or other rarer types (jejunal, colonic, rectal, hepatobiliary, etc)

PRESENTATION

- Usually asymptomatic, found incidentally during barium study or open surgery - Symptomatic:

1) IO (most common presentation): strictures, intussusception, volvulus, omphalomesenteric band 2) Hematochezia (most common in children): usually massive & painless, due to peptic ulceration 3) Diverticulitis: may present exactly like acute appendicitis i.e. periumbilical pain radiating to RIF. Less prone to

inflammation as most Meckel’s have wide base, little lymphoid tissue & are self emptying 4) Others: umbilical fistula, tumour, perforation etc

INVESTIGATION Blood: As per IO or lower BGIT Imaging:

- Technetium-99m pertechnetate scan (investigation of choice): isotope given IV, taken up by gastric mucosa, detected by gamma scan of the abdomen

- Barium studies: small bowel enteroclysis - CT NOT helpful as hard to distinguish Meckel’s diverticulum from small bowel loops

MANAGEMENT

- NBM, IV drip, correct electrolyte imbalance - IO: NG tube on constant suction, supine AXR - PR bleed: PCT if necessary, gastric lavage to exclude UBGIT, OGD & colonoscopy - Definitive treatment: surgery (open or laparoscopic)

Wide base: wedge ileal resection with anastamosis Narrow base: resection of the diverticulum

81

4. ISCHAEMIC BOWEL RISK FACTORS Occlusive mesenteric ischemia

a. Embolic (most lodge in SMA) – becoming less common i. Recent MI ii. Dysarrhythmia (esp AF Æ embolization) iii. Valvular heart disease

b. Thrombosis (Virchow’s triad) – becoming more common i. Age >50 ii. CCF iii. PVD iv. Hypovolemia v. Prothrombotic states – more in venous thrombosis (young)

Non-occlusive (no arterial or venous abn, due to vasospasm and constriction) – 20-30% of acute ischemia

a. Poor perfusion states i. Hypotension ii. Hypovolemia iii. CCF iv. MI v. Renal failure

b. Compression from ext sources (eg. intraabdominal tumor) c. Vasculitis d. Radiotherapy e. Vasoconstriction

i. digitalis ii. strangulation (from volvulus/hernia)

Tissue injury can result from one of 2 mechanisms: (1) ischemic injury to the bowel or (2) reperfusion injury 2 Watershed areas: a) Marginal artery of Drummond / Griffith’s point: SMA and IMA b) Sudeck’s point: Left colic artery and Superior rectal artery Three progressive phases of ischemic colitis have been described: a) A hyperactive phase occurs first, in which the primary symptoms are severe abdominal pain and the passage of bloody stools. Many patients get better and do not progress beyond this phase. b) A paralytic phase can follow if ischemia continues; in this phase, the abdominal pain becomes more widespread, the belly becomes more tender to the touch, and bowel motility decreases, resulting in abdominal bloating, no further bloody stools, and absent bowel sounds. c) Finally, a shock phase can develop as fluids start to leak through the damaged colon lining. This can result in shock and metabolic acidosis with dehydration, hypotension, tachycardia and confusion. Patients who progress to this phase are often critically ill and require intensive care. Acute mesenteric ischemia (classical triad) 1. Acute severe abdo pain 2. No physical signs 3. Rapid hypovolemia Æ shock HISTORY (typical but vague) – high index of suspicion!! Chronic

x Gut claudication: post prandial colicky pain (10min to 3hr after a meal) Æ food fear Æ LOW and malnutrition x Blood in stools (as bowel begins to die) – maroon or bright red

Acute x Severe abdominal pain: out of proportion with examination findings: tends to be constant and central, or RIF x Blood in stools x Paralytic ileus, usu occurring in the splenic flexure (watershed area receiving blood from the most distal arterial branches) x Diarrhoea/constipation x Positive FOBT (sloughing of dead ischemia bowel) in 75%

82

PHYSICAL EXAMINATION Initial

x Thin, writhing in pain x No peritoneal sign x Auscultation may reveal a bruit x Distension a late feature, often first sign of impending bowel

infarction

Bowel compromise x Fever, Tachycardia, hypotension x Peritonism – rebound, guarding, rigid x Nausea/vomiting x Malaena/hematemesis x Massive abdominal distension x Back pain x Shock

INVESTIGATIONS Bloods 1. FBC: high Hb/ Hct (due to plasma loss/ hemoconcentration), high TW >15 in 75% 2. ABG: Metabolic acidosis (persistant) – 50% 3. UECr: renal failure, hypovolemia 4. PT/PTT: hypercoagulable states (if present, can add Protein C/S, AT III) 5. Raised amylase / LDH Imaging - AXR 1. Submucosal hemorrhage and edema in the colon produce a characteristic thumbprinting 2. Dilated bowel from ileus 3. Intramural air/ air in portal venous system (ischemia) 4. Free air in abdomen (perf) - CTAP:

x Exclude other causes of abdominal pain x Ischemic colitis: SMA or superior mesenteric vein thrombosis, intestinal pneumatosis, portal

venous gas, lack of bowel wall enhancement in contrast CT, thumbprinting and ischemia of other organs.

x Non specific findings: distended bowel, an absence of intestinal gas, a thickened bowel wall, mesenteric vascular engagement, ascites and air-fluid levels

- MRI as sensitive as CT - Barium enema: stricture secondary to ischemia - Arteriography (limited role in diagnosis):

x confirm the presence and extent of occlusive disease. x AP views demonstrate collateral pathways, lateral projections show the origins of visceral

branches. x Features: dilated arteries, prominent accumulation of contrast material in the bowel

parenchymal, and dense and early venous filling x Can ppt acute ischemia: ensure that the patient is well hydrated. x Thrombosis - complete lack of visualization of the SMA and its branches (as thrombosis

usually starts at the prox end of SMA), Embolism to the SMA - filling of the proximal SMA only with a sharp cutoff of the artery

Others

1. ECG: AF, STEMI 2. Colonoscopy: procedure of choice if the diagnosis remains unclear.

a. Ischemic colitis has a distinctive endoscopic appearance; b. Rule out alternate diagnoses eg infx/IBD c. Biopsies can be taken d. Visible light spectroscopy (analyze capillary oxygen levels), performed using catheters placed through the 5 mm channel of

the endoscope, is diagnostic MANAGEMENT Acute

1. ABC a. maintain airway and give high-flow O2 b. establish at least 1 large bore IV line and infuse fluids at maintenance rate (unless patient in shock)

2. Monitoring (as patients are prone to extensive peritonitis, shock, metab acidosis) a. Urinary catheter to monitor urine output

3. Consider NGT 4. Antibiotics: IV Cephalosporin? and metronidazole

Definitive

83

5. ACUTE APPENDICITIS HISTORY

- Classic: LOA with periumbilical pain which radiates to the RIF, followed by nausea & vomiting (in ½) - N/V almost always occurs after pain, if it precedes pain, exclude IO - May have diarrhoea or constipation - Inflamed appendix near bladder/ureter may cause irritative voiding symptoms & haematuria - Fever (late): >39deg uncommon in first 24h but common after rupture

PHYSICAL EXAMINATION

- Most specific physical findings: RIF (McBurney’s point) tenderness on percussion, rebound tenderness, rigidity & guarding - RIF mass may be palpable - LIF tenderness in patients with situs inversus or lengthy appendix extending to the LIF

- Cough sign: RIF pain on coughing (localized peritonitis) - Rovsing sign: RIF pain with palpation of the LIF - While this maneuver stretches the entire peritoneal lining, it only causes

pain in any location where the peritoneum is irritating the muscle. - Obturator/ Cope sign: RIF pain with internal rotation of a flexed right hip - Psoas sign: RIF pain with hyperextension of the right hip - the inflamed appendix is retrocaecal in orientation Atypical ppt: - Infants/children may present with inflamed hemiscrotum due to migration of pus through patent processus vaginalis – often

mistaken for acute testicular torsion - Retrocaecal Æ right flank pain - Pregnancy pushes appendix up Æ right upper quadrant pain - Pelvic appendix irritates bladder and rectum Æ suprapubic pain, pain on urination, feeling of need to defaecate

CAUSES

- Obstruction of appendiceal lumen Faecaliths: calcium salts & faecal debris collect around nidus of faecel material within appendix Lymphoid hyperplasia: a/w inflammatory (Crohn’s) & infective dz (GE, URTI, IMS, measles) Less common causes: parasites, TB, tumour, FB

DIFFERENTIAL DIAGNOSES

1) Mesenteric adenitis: self limiting inflammation of mesenteric LNs in RIF, a/w viral infections 2) Meckel’s diverticulitis: inflammation of Meckel’s diverticulum (see below) 3) Terminal ileitis: usually due to Crohn’s disease 4) Typhilitis/caecitis 5) Ischaemic colitis 6) Colon cancer 7) Appendiceal stump appendicitis 8) Psoas abscess

Most frequent misdiagnoses in Females: PID, followed by gastroenteritis and urinary tract infection / in Children: gastroenteritis, followed by upper respiratory infection and lower respiratory infection. INVESTIGATIONS Blood: FBC, UECr, CRP, Blood culture, PT/PTT, GXM Urine: UFEME (may have pyuria & haematuria) Imaging: Erect CXR, CTAP - greater sensitivity/ accuracy/ NPV, abdominal U/S AXR (limited use): fecalith, appendiceal gas, localized paralytic ileus, blurred right psoas, and free air

84

MANTRELS Score

Characteristic Score M = Migration of pain to the RLQ 1 A = Anorexia 1 N = Nausea and vomiting 1 T = Tenderness in RLQ 2 R = Rebound pain 1 E = Elevated temperature 1 L = Leukocytosis 2 S = Shift of WBCs to the left 1 Total 10 0-3 could be discharged without imaging, 4-6 undergo CT evaluation, > 7 receive surgical consultation MANAGEMENT

- NBM, IV drip, correct electrolyte imbalance - IV antibiotics: Preoperative antibiotics have demonstrated efficacy in decreasing postoperative wound infection rates - Symptomatic relief: anti-emetics & analgesia - Definitive treatment: appendicectomy (open or laparoscopic) - Urgent vs Emergent:

appendectomy within 12-24 hours of presentation is not associated with an increase in hospital length of stay, operative time, advanced stages of appendicitis, or complications compared with appendectomy performed within 12 hours of presentation

- Perforated appendix (emergent vs interval): mild symptoms and localized abscess or phlegmon on abdominopelvic computed tomography (CT) scans Æ initially treated with IV antibiotics and percutaneous or transrectal drainage of any localized abscess. If the patient's symptoms, WBC count, and fever satisfactorily resolve, Æ oral antibiotics. Delayed (interval) appendectomy can be performed 4-8 weeks later.

- Conservative treatment for appendiceal mass: Oschner Sherren regime Omentum naturally wraps around inflamed appendix, containing inflammatory process Symptomatic treatment as well as antibiotics, monitor patient as well as size of mass When mass has reduced in size, patient is stable, do surgery (less inflammation: easier) Exceptions: very young or very old patients, or patients with suspected appendicular abscess (require incision &

drainage of pus) Delayed diagnosis and treatment account for much of the mortality and morbidity associated with appendicitis. The rate of perforation varies from 16% to 40%, with a higher frequency occurring in younger age groups (40-57%) and in patients older than 50 years (55-70%), in whom misdiagnosis and delayed diagnosis are common. Complications occur in 1-5% of patients with appendicitis, and postoperative wound infections account for almost one third of the associated morbidity.

85

5. OESOPHAGEAL DISEASES 1. ANATOMY OF THE OESOPHAGUS

- Oesophagus is a muscular tube that is 25cm (10 inches) long - Starts at the cricoid cartilage (C6 vertebra) from the

oropharynx and continues into the stomach at the level of T10 - Upper oesophageal sphincter = cricopharyngeus muscle - Lower sphincter is not an anatomical sphincter, but

physiological: (i) Increased tone of the muscularis propria at the lower

oesophageal sphincter (ii) Fibres of the right diaphragmatic crus loop around the

cardio-oesophageal junction and contract during coughing, sneezing etc when intra-abdominal pressure increases, thus preventing reflux

(iii) Angle of His where the oesophagus joins the stomach – acts as a valve

(iv) Intra-abdominal pressure being higher than intra-thoracic pressure

- 3 narrow points along the course of the oesophagus

(i) Cricopharyngeus muscle (15cm from incisor teeth) (ii) Carina where the left bronchus crosses the

oesophagus (27cm from incisors) (iii) Where the oesophagus passes through the

diaphragm (40cm from incisors)

- Structure: mucosa, submucosa, muscularis propria, adventitia (no peritoneal lining except for a short segment of intra-

abdominal oesophagus) Æ very distensible Muscularis propria is composed of striated muscle in the upper one-third, striated and smooth muscle in the middle third, and smooth muscle in the lower third

- Blood supply (roughly divided into thirds): Inferior thyroid artery to upper third, oesophageal branches of the aorta to the middle

third, oesophageal branches of left gastric artery to lower third - Venous return also divided into thirds: Brachiocephalic veins (upper), azygos veins (middle), left gastric vein (lower) --- a

portosystemic anastomosis exists at the lower oesophagus thus leading to formation of varices in portal hypertension

2. PHYSIOLOGY OF SWALLOWING

- Process of mastication forms a food bolus on the dorsum of the tongue - The tongue then contracts upwards and backwards pushing the food bolus against the hard palate - Soft palate elevates (contraction of palatoglossus) to close off nasopharynx - Further elevation of tongue pushes food bolus into oropharynx - As the base of the tongue is elevated posterior, the epiglottis falls back; at the same time, the pharyngeal muscles contract to

bring the posterior surface of the larynx upwards to make the laryngeal inlet smaller Æ closed off by the epiglottis - Pharyngeal muscles contract to propel food bolus past the relaxed cricopharyngeus into the oesophagus - Once in the oesophagus, involuntary contractions of the muscularis propria form peristaltic waves to propel food bolus into

stomach

86

3. APPROACH TO DYSPHAGIA

CAUSES OF DYSPHAGIA - Dysphagia can be divided into oropharyngeal and oesophageal dysphagia - In each anatomic region the dysphagia can be caused by neuromuscular dysfunction (impaired physiology of swallowing) or

mechanical obstruction to the lumen

Oropharyngeal Oesophageal Functional (Neuromuscular diseases)

Stroke Parkinson’s disease Brain stem tumours Degenerative conditions e.g. ALS, MS Peripheral neuropathy Myasthaenia gravis Myopathies e.g. myotonic dystrophy

Functional (Neuromuscular diseases) Achalasia Spastic motor disorders

Diffuse oesophageal spasm Hypertensive lower oesophageal sphincter Nutcracker oesophagus

Scleroderma

Mechanical (Obstructive lesions) Tumours Inflammatory masses e.g. abscess Oesophageal webs

Pharyngeal pouch (Zenker’s divert)

Anterior mediastinal mass

Mechanical (Obstructive lesions) Intrinsic structural lesions

Tumours Strictures: Peptic (reflux oesophagitis)

(MRCP) Radiation Chemical (caustic ingestion) Medication

Lower oesophageal rings (Schatzki’s ring)

Oesophageal webs (Plummer-Vinson) Foreign bodies

Extrinsic structural lesions

Mediastinium – retrosternal thyroid, lymphadenopathy, Vascular compression (enlarged aorta or left atrium)

Thoracic inlet mass Kyphoscoliosis

Others Oesophagitis: Reflux Infectious (candida, herpes) Radiation-induced Medication-induced Chemical-induced (alcohol)

Plummer Vinson syndrome / Paterson Brown Kelly syndrome

- Triad of dysphagia (oes web), glossitis, angular stomatitis (Fe def anemia) - post menopausal women - genetic and nutritional factors - premalignant Æ oes/hypopharynx/oral SCC - Tx: Fe supplement, Oes dilatation

87

HISTORY I. Dysphagia: a) Difficulty initiating / Food stuck

b) Solids / Liquids initially c) Progressive (CA, stricture) / Intermittent (webs, rings, oes spasm, nutcracker)

II. Associated symptoms: a) Odynophagia b) Choke, cough, nasal regurgitation c) Regurgitation of undigested food d) Nasal voice

III. Complications IV. RF

1. Differentiating oropharyngeal from oesophageal dypshagia

(i) Oropharyngeal

- Presenting complaint is usually of difficulty in initiating swallowing - May be associated with choking, coughing, nasal regurgitation - Voice may sound nasal (bulbar palsy) - Cause of oropharyngeal dysphagia is usu neuromuscular rather than mechanical; stroke is the most common cause

(ii) Oesophageal - Presenting complaint is that of food “getting stuck” in the throat or chest - Patient’s localisation of the symptom often does not correspond to actual site of pathology - Can be due to either neuromuscular dysfunction or mechanical obstruction

2. Differentiating mechanical obstruction from neuromuscular dysfunction

(i) Mechanical

1. Patient complains of more difficulty swallowing solids than fluids 2. May have regurgitation of undigested food 3. Recent onset dysphagia that is progressively worsening, with loss of weight Æ high suspicion of oesophageal cancer

- Intermittent symptoms are suggestive of webs, rings (ii) Neuromuscular

1. Patient complains of more trouble swallowing fluids than solids 2. Dysphagia more long-standing, slowly progressive 3. Intermittent symptoms suggestive of diffuse oesophageal spasm, nutcracker oesophagus 4. May have history of stroke, neuromuscular disease

3. Is there odynophagia (pain associated with difficulty swallowing)?

(a) Oesophagitis: infectious (candida, herpes), post-radiation, chemical-induced (usually alcohol), reflux oesophagitis (b) Oesophageal spasm (c) Scleroderma (d) Achalasia (occurs late) (e) Oesophageal cancer (occurs late) (f) Oesophageal rupture/stretching

4. Complications (3)

- Symptoms of anaemia (bleeding from tumour, or as part of Plummer-Vinson syndrome) - Symptoms of aspiration pneumonia – fever, cough, shortness of breath esp at night - Locally advanced tumour:

o Hoarseness (recurrent laryngeal nerve) o Thoracic spine pain (spread posteriorly to thoracic spine) o SOB (pleural effusion sec to lymphatic blockage by tumor infiltration) o Fever, cough and haemoptysis (tracheo-oesophageal fistula) o Haematemesis (invasion into aorta Æ massive bleed) – rare Always differentiate hematemesis from hemoptysis.

- Mets: Neck lump (LN), jaundice, bone pain

5. History of predisposing conditions (RF) - Reflux symptoms e.g. retrosternal burning pain (heartburn), sour fluid reflux into mouth (acid brash), excessive salivation (water brash),

postural aggravation on lying down - Caustic chemical ingestion in the past - Medication history: Antibiotics (eg, doxycycline, tetracycline, clindamycin, trimethoprim-sulfamethoxazole), potassium chloride,

nonsteroidal anti-inflammatory drugs (NSAIDs), quinidine, emperonium bromide, and alendronate (Fosamax) - Loss of weight occurs in cancer and achalasia (much later onset in achalasia compared to cancer) - Smoking, chronic alcohol intake - Radiation to the chest - Symptoms of systemic disease e.g. stroke (focal neurological deficits), scleroderma (CREST: telangiectasia, sclerodactyly, calcinosis,

Raynaud’s), Parkinson’s

88

PHYSICAL EXAMINATION 1. General condition

- Vitals: the patient may be hypovolaemic from vomiting/decreased intake o Nutrition: presence of cachexia o Dehydration (mucous membranes, skin turgor, etc)

- Conjunctival pallor: bleeding from tumour, oesophagitis ulcerations, or associated with P-V syndrome - Scleral icterus: metastases to liver

2. Disease/Causes

- Presence of cervical lymph nodes (esp Virchow’s node) - Scars/marks over the chest and abdomen suggesting previous radiation - Palpable mass in abdomen (not likely) - Neuro examination (esp CN, PD features, myopathy) - PR examination for malaena - Bedside swallowing test?

3. Complications of disease

- Signs of pneumonia: patient febrile, may be toxic, lung crepitations, decreased air entry usually over right lower lobe - Signs of mets: Hepatomegaly, Ascites

4. Treatment

- Tube feeding through NG tube, gastrostomy/jejunostomy – if aspirates seen, what is the colour? - Total parenteral nutrition - Surgical scars

MANAGEMENT 1. Stabilise patient

- Resuscitate if patient is haemodynamically unstable - IV fluids (correct fluid deficits and also any electrolyte derangements) - Feeding

o Consider feeding with fluids if patient can tolerate it (only having problems with solid food) otherwise consider tube feeding or TPN Æ need to correct patient’s nutritionally debilitated state

o Keep NBM if patient cannot tolerate even fluids - Treat any aspiration pneumonia – NBM, IV antibiotics

2. Investigate for underlying cause and treat it INVESTIGATIONS Diagnostic 1. Barium swallow

- Advantage of barium swallow is that it is less invasive than OGD, especially when suspecting webs, diverticula in the oesophagus

where OGD may cause perforation; - CI in patients with difficulty initiating swallowing Æ high risk of aspiration (therefore, give slowly and carefully)

- Visualisation of obstructive lesions: o Shouldering of a stricture (benign strictures form a smoother contour whereas malignant strictures form a more right-angled

contour) o Bird’s beak sign of achalasia

Achalasia Benign stricture Carcinoma Cockscrew

- Visualisation of pharyngeal pouch or oesophageal diverticulum - Diffuse oesophageal spasm gives a corkscrew appearance

89

2. Oesophagogastroduodenoscopy (OGD) - Advantage is direct visualisation of the lesion and ability to take tissue biopsy (especially useful in malignancy) - May also be therapeutic (stopping bleeding from a tumour, stenting the lumen, etc)

3. Manometry

- Gold standard for diagnosing achalasia:

(i) Absence of peristalsis (ii) Very high pressures at the lower oesophageal sphincter (iii) Absence of relaxation at the LES on swallowing food

4. Videofluroscopic examination of swallowing (VFES) – using barium

Flexible-endoscopic examination of swallowing (FEES) - Used to assess oropharyngeal dysphagia (neuromuscular causes) by looking for penetration and aspiration of various consistencies of

food during swallowing - VFES limited to cervical oesophagus (unable to exclude distal oes lesions)

Supportive 1. Blood investigations:

- Full blood count – Low Hb (anaemia from chronic blood loss) High TW (aspiration pneumonia) - Urea, electrolytes, creatinine – electrolyte disturbances from vomiting, poor intake; raised creat and urea in dehydration (creat will be

raised more than urea if patient has prerenal failure from dehydration) - Liver function tests – low albumin with nutritional deprivation

2. CXR

- Consolidation (aspiration pneumonia)

3. 24-hour pH probe monitoring

- If patient complains of reflux symptoms and no signs are seen on OGD (see later section on Gastro-oesophageal reflux disease)

90

4. OESOPHAGEAL CANCER

EPIDEMIOLOGY - Third most common gastrointestinal tract cancer in Singapore (1st: CRC, 2nd: gastric) - M > F, Adenocarcinoma more common in West whereas SCC more in East - Increasing incidence with age - Decreasing incidence overall, increasing in distal oes RISK FACTORS

SCC (70%) Adenocarcinoma (30%) Modifiable 1. Smoking (100X)

2. Alcohol (2X) 3. Caustic injury [middle 1/3] 4. Diet: Hot drinks, preserved foods (nitrosamines), betel nuts;

vitamin / mineral def (selenium, vit E, beta-carotene)

1. Smoking (10X) 2. Obesity Æ GERD Æ Barrett’s

Non-modifiable

1. Achalasia (2-8%) 2. PV synd (10%) [upper 1/3] 3. Tylosis (AD disorder with keratosis of palms and soles) 4. Oes. diverticulum 5. Irradiation

Barrett’s (30-40X, 1% per year) [distal 1/3]

PATHOLOGY - 70% squamous cell carcinoma, 30% adenocarcinoma - SCC can arise anywhere in the oesophagus while adenocarcinoma occurs in lower third and gastro-oesophageal junction

(related to reflux and Barrett’s oesophagus) - Overall: 10% of cancers occur in the upper third, 60% in the middle third, 30% in the lower third - Three growth patterns:

Fungating (60%) Ulcerative (25%) Infiltrative (15%)

- Tumour spread: direct extension into surrounding structures, vascular invasion, lymphatic spread - Common sites of metastases: liver, lung, bone STAGING

AJCC 7th edition 2010 x SCC and adenocarcinoma are staged separately x Subclassification of nodal (N) status according to the number of regional nodes containing metastases x Reclassification of tumors at the gastroesophageal junction (GEJ) and/ or the proximal 5 cm of the stomach that extend

into the GEJ or esophagus x Reassignment of stage groupings using T, N, M categories as well as histologic grade of differentiation (G), and for SCCs,

tumor location x Redefining of Tis (carcinoma in situ) as high-grade dysplasia x Subclassification of T4 disease based upon potential resectability of adjacent involved organs or structures

T Tis

T1a T1b T2 T3 T4a T4b

High-grade dysplasia Tumour invading lamina propria or muscularis mucosa Tumour invading submucosa but does not breach submucosa Tumour invades the muscularis propria Tumour invades adventitia Resectable tumor invading pleura, pericardium, or diaphragm Unresectable tumor invading other adjacent structures

N N1 N2 N3

1-2 Regional node involvement 3-6 7 or more

M M1 Distant metastases

Stage T N M Grade Tumor location 0 is 0 0 1, X Any I (A/B) 1-3 0 0 1-3 Any/ lower (Ib) II (A/B) 2 / 3 0-1 0 2/3 Upp mid/ lower III (A-C) 1-4a 0-3 0 Any Any IV any any 1 Any Any

Histologic grade (G) GX Grade cannot be assessed—stage

grouping as G1 G1/2/3/4 Well/Mod/Poorly/Un differentiated

91

PRESENTATION Usually of insidious onset, with earliest symptoms being non-specific e.g. retrosternal discomfort, “indigestion”, and most patients already have advanced disease when they are diagnosed – 75% have lymph node involvement at time of diagnosis. – 50% of patients have unresectable cancer on presentation Asymptomatic from screening of Barrett’s oes patients (early detection) 1. Dysphagia

- Present in 80% of patients – most common presentation - Lack of serosa Æ oes is distensible Æ > 60% occlusion is needed before dysphagia occurs (Late presentation – T3/4)

2. LOW (classically withOUT LOA – patient still feels hungry) 3. Regurgitation Æ1+2+3 = CA oes until proven otherwise Pain develops late and is usually due to extra-oesophageal involvement Complications 4. Anaemia (with or without malaena/frank haematemesis – bleeding is usually occult) 5. Vocal cord paralysis (left - due to its longer course through the aortopulmonary window > right) 6. Aspiration pneumonia 7. Tracheo-oesophageal or broncho-oesophageal fistula INVESTIGATIONS – 4 OBJECTIVES (A) Diagnosis 1. Oesophagogastroduodenoscopy (Gold standard)

1. Allows biopsy of the lesion Æ confirmatory histological diagnosis 2. Estimate distance from incisor (facilitates op planning) 3. Determine extent of tumor 4. Look for synchronous lesions 5. Detect complications: Bleeding (can stop the bleed!), Obstruction/Stenosis 6. Adjunct EUS (more sensitive for depth and nodal status) [Same purposes/advantages as colonoscopy in CRC]

2. Barium swallow

- 92% accuracy in showing mucosal irregularity & annular constrictions but not able to dx malignancy with confidence - Useful if OGD cannot pass through

(B) Staging (Look very hard for CI of surgery due to high morbidity and mortality) i. Local 1. Endoscopic ultrasound (ideal in CA oes, rectal, stomach as structures are enclosed)

- If endoscope can pass around the lesion, good for T staging (T) – depth of invasion - Also to identify enlarged regional lymph nodes (N) – FNA to confirm mets

Suspect LN mets if round, hypoechoic, well-defined borders 2. CT scan or MRI

- Can be used for T, N, and M staging - CT T: local invasion, regional LN,

CT AP: liver/ adrenals mets, distant LN, CT Neck: cervical LN

- Upper oes: CT NTAP Lower oes: CT TAP

3. Bronchoscopy a. Exclude direct tracheal involvement especially in tumours involving upper two-thirds of oesophagus b. Laryngoscope : vocal cord paralysis

Look very hard for CI of surgery due to high morbidity and mortality.

92

ii. Distant 1. Chest X-ray

- Mets o Presence of any lung metastases o Pleural and/or pericardial effusion

- Cx o Aspiration pneumonia o Tracheal deviation or extrinsic compression of tracheobronchial system o Widened superior mediastinum in an upper oesophagus tumour o Raised hemidiaphragm with phrenic nerve involvement

2. CT scan (as above) 3. PET scan / CT PET

- Distant N, M. - Expensive ($2000!) - Used for patients deemed fit for operation.

4. Bone scan for bony metastases Staging laparoscopy not done! (C)Complications 1. Full blood count – Low Hb (anaemia from chronic blood loss) High TW (aspiration pneumonia) 2. Urea, electrolytes, creatinine

a. electrolyte disturbances from vomiting, poor intake; b. raised Cr & urea in dehydration (Cr will be raised more than urea if patient has prerenal failure from dehydration)

3. Liver function tests – low albumin with nutritional deprivation, liver mets (rare)

4. CXR (as above) Predictors of poor outcome: Sig LOW, Low albumin. (D) Pre-op (operation requires thoracotomy) 1. Lung function test eg. Spirometry

a. In view of single lung ventilation during operation b. Risk of COPD – Smoking is impt RF for CA oes (esp SCC)

FEV1??, FVC > 1.25L?? 2. 2DE (in view of major op) – ideal EF>40% 3. PT/PTT, GXM 4. Usual: FBC, UECr

93

TREATMENT Principles - Three modalities available – surgery, chemotherapy, radiotherapy – used singly or in combination - Aims of treatment: Curative or palliative (50%) - Surgical treatment is usually performed with curative intention, but can also achieve good long-term palliation of symptoms - Choice of treatment depends on several patient factors: age, co-morbidities, nutritional state, life expectancy, and prognosis Surgery - Curative in early lesions and part of multimodal therapy in more advanced stages - Resection should not be done in patients with distant metastases or contraindications to surgery 1. Endoluminal surgery – for early lesions (Tis, T1a); no attempt to remove any LNs (usually no LN involvement)

i. Endoscopic mucosal resection (EMR) – Cx: bleeding, perf (may be prevented with sufficient saline injection to raise the mucosa containing the lesion), stricture

ii. Mucosal ablation using photodynamic therapy , Nd-YAG laser, or argon plasma coagulation 2. Oesophagectomy

(i) Ivor-Lewis / Lewis Tanner

Two-stage procedure involving gastric mobilisation (first stage, done through upper midline abdominal incision), oesophagectomy and gastro-oesophageal anastomosis in the chest (second stage, through right thoracotomy incision)

(ii) Trans-hiatal

Done via two incisions – one in the abdomen and one in the neck Blunt oesophagectomy, gastric mobilisation, and gastro-oesophageal anastomosis in the neck Less morbidity than Ivor-Lewis as the chest is not opened, but controversial

(iii) Tri-incisional (McKeown approach)

Three incisions – abdominal, chest, and also left neck incision for gastro-oesophageal anastomosis in the neck Performed with two-field lymphadenectomy (upper abdominal and mediastinal) No difference in survival between trans-hiatal and I-L modalities; the stage of the cancer when the operation is performed is

a greater factor influencing survival Radical en-bloc dissections not shown to improve survival Oesophagectomies have high mortality (5-10%) and morbidity (25%) rates, thus patients have to be carefully selected in

order to maximise survival benefit from surgery Complications

Dependent on extent of surgery and incisions used - Intraoperatively, injury to lung, thoracic duct, RLN can occur

- Respiratory complications higher in thoracotomies – atelectasis, pneumonia - Anastomotic leak and resultant mediastinitis (for chest anastomosis) most feared - Anastomotic stricturing can also occur - Reflux can result in the long term due to loss of the LES

3. Palliative debulking for obstructive symptoms Radiotherapy - Usually given in combination with chemotherapy - Primary treatment for poor-risk patients

Palliation for unresectable lesions with obstructive symptoms, pain and bleeding - SCCs are radiosensitive - Modalities: External beam radiation or brachytherapy - Obstructive symptoms may worsen temporarily after radiotherapy due to oedema - Complications: tracheo-oesophageal fistula, stricture

94

Chemotherapy - Current regimen: 5-Fluorouracil and cisplatin - Addition of chemotherapy to external beam radiation for unresectable cancers shown to have improved survival compared to

EBRT alone - Chemotherapy given preoperatively and postoperatively improves survival

Î Overall curative treatment Preoperative neoadjuvant chemoradiotherapy (increases rates of complete resection), oesophagectomy, and postoperative adjuvant chemoradiotherapy for responsive tumours Palliative treatment 1. Ablation

o Endoscopic laser fulguration (high frequency electric current) o Photodynamic therapy is a new treatment option - use of drugs that are absorbed by cancer cells; when exposed to a

special light, the drugs become active and destroy the cancer cells o Radiofrequency o Cryotherapy

2. Surgical debulking 3. Bypass surgery rarely done nowadays 4. Stenting to maintain lumen patency and occlude fistulas – endoscopic / radiological?

o Cx: metallic Æ Perf (friable tumor) Bleeding Risk of GERD, aspiration Æ lifelong PPI

Feeding in oesophageal obstruction - Feeding via oropharyngeal route is preferred unless the passage is obstructed or it is unsafe for the patient to feed via that

route (i.e. risk of aspiration) o If still able to pass NG tube around tumour Æ feed via NG (but also consider complications with long-term NG

placement e.g. erosions around nasal area, sinusitis); o consider PEG placement for long-term feeding if able to get scope around tumour

- If unable to pass tube or scope around tumour, o consider open gastrostomy o Total parenteral nutrition is another option but has more complications, more costly o Relief of obstruction via various techniques as listed above help to enable oral feeding, but most techniques are not

long-lasting and dysphagia will return with tumour growth PROGNOSIS - 80% mortality at 1 year, overall 5-year survival <10%

95

5. GASTRO-OESOPHAGEAL REFLUX DISEASE (GORD / GERD)

EPIDEMIOLOGY Incidence in Singapore not known Increasing prevalence, more common in males than females Singapore Chinese > other races PATHOPHYSIOLOGY - Lower oesophageal sphincter is a physiological sphincter with various mechanisms that help to prevent reflux - Some physiological reflux occurs that is rapidly cleared by peristaltic movements in the oesophagus - GORD results from various pathophysiological factors (loss of the normal protective mechanisms, or the mechanisms are

overwhelmed) singly or in combination: I. Motor failure of oesophagus with loss of peristalsis

II. Loss of LES function – decreased tone, hiatal hernia, iatrogenic injury III. Increased intra-abdominal pressure – obesity, tight garments, large meal

IV. Delayed gastric emptying

- Acid incites inflammation in lower oesophagus – extent of inflammation increases with increasing duration of contact with acid - Chronic inflammation results in complications of GORD: oesophagitis, stricture, Barrett’s oesophagus CAUSES/RISK FACTORS - Motility disorder of oesophagus e.g. scleroderma i Malfunction of LES i Hiatal hernia (loss of normal LES mechanisms) i Drugs that cause smooth muscle relaxation e.g. calcium channel blockers, sedatives, beta agonists, anticholinergics, etc.

Coffee and smoking also cause LES relaxation. - Chronically increased intra-abdominal pressure – pregnancy, chronic cough, obesity, constipation, etc - Eating habits – lying down after a heavy meal i Any cause of decreased gastric emptying PRESENTATION - Heartburn: retrosternal pyrosis (=burning sensation in upp abdo) - Acid brash/Regurgitation: reflux of sour gastric juices into back of mouth i.e. regurgitation

o These symptoms occur usu after food, particularly a heavy meal, and are aggravated by lying flat (posturally related) - Other symptoms: globus (feeling of a lump at the throat), water brash (hypersalivation in response to reflux)

Chest pain (can mimic anginal pain with radiation to neck, jaw, arm), nausea Æ high false positive - Complications

o Long-standing disease can lead to dysphagia due to stricture formation; dysphagia can also result from an underlying oesophageal motility disorder; odynophagia suggests oesophagitis with ulceration

o Reflux can also lead to pulmonary symptoms: chronic cough, chest infections (aspiration)

96

COMPLICATIONS 1. Pain and spasm 2. Stricture 3. Shortening of oesophagus 4. Dysmotility 5. Haemorrhage (occult > frank) 6. Ulceration 7. Barrett’s oesophagus (see below) 8. Schatzki’s ring (constrictive ring at the squamocolumnar junction composed of mucosa and submucosa) 9. Malignancy (adenocarcinoma arising from Barrett’s oesophagus) DIAGNOSIS 1. History!! is important as most patients with reflux are seen in the primary setting with no facilities for detailed investigation

- Exclude cardiac cause of chest pain, and exclude malignant cause of dysphagia - GerdQ questionnaire

2. Oesophageal pH probe

- Confirmatory test for reflux is the ambulatory 24hr oesophageal pH probe especially if oesophagitis is not seen on OGD - Antimony probe (24hr) most commonly used; alternative is the Bravo capsule (a wireless capsule that is temporarily attached to the

oesophageal wall, 48hr) - The probe is placed 5cm above the manometrically-determined upper limit of the LES (for the wired probe), or 6cm above the

endoscopically-determined squamocolumnar junction (for the wireless capsule) - Diagnosis based on the percentage of time in 24hrs the pH reading is below 4 (positive if >5.5%) - Can have false negative Æ consider repeat test. Either one positive is diagnostic.

3. Oesophagogastroduodenoscopy

- Cannot actually diagnose reflux (poor correlation btw oesophagitis and reflux). Therefore, selective usage. - Uses:

o Dx: Rule out gastric malignancy/ other conditions mimicking symptoms (in areas with high prevalence of gastric CA) o Cx: Can visualise and grade oesophagitis if present, and take biopsy specimens for confirmation (see below) o Ax: May see a hiatal hernia which is associated with reflux (though not all patients with hiatus hernia will have reflux)

4. Barium swallow and follow-through

- Not of much value in diagnosing reflux - Ax: Can detect motility disorders that cause reflux, - Cx: oesophageal ulceration and stricturing resulting from reflux - Can sometimes see reflux of barium contrast into oesophagus

5. Manometry

- No value in reflux except for detecting motility disorder (tro achalasia! – will be much aggravated by surgical Tx of reflux) GRADING OF OESOPHAGITIS 1. Savary-Miller classification

Grade I: One or more supravestibular non-confluent reddish spots, with or without exudates Grade II: Erosive and exudative lesions, may be confluent but not circumferential Grade III: Circumferential erosions covered by haemorrhagic and pseudomembranous exudate Grade IV: Presence of chronic complications such as deep ulcers, stenosis, stricture Grade V: Barrett’s metaplasia

2. Los Angeles classification

- Relevance of classification schemes: subjective and dependent on assessment by the endoscopist; also, due to the multitude of

classification schemes available, just mentioning a grade may not have any meaning if the actual abnormalities are not described - heartburn severity or the symptoms do not reliably predict the severity of erosive esophagitis; older patients experience less symptoms

97

TREATMENT Lifestyle 1. Diet and eating habits

Avoid coffee, chocolate, fatty foods, or anything that worsens symptoms Do not eat 2 hours prior to sleeping Walk after eating Avoid excessive eating; eat small meals

2. Avoid drugs that relax LES e.g. anticholinergics, muscle relaxants, etc. 3. Weight reduction if obese 4. Elevate head of bed 5. Smoking and alcohol intake cessation Medication 1. Acid suppression therapy: PPI (best! switch ard if patient doesn’t respond to one, rather than increasing dosing frequency) or

H2-receptor antagonists May require LT tx. If patient stops PPI and symptoms recur, restart PPI first, no need to reinvestigate.

2. Prokinetics to increase LES pressure e.g. domperidone (anti-dopaminergic), metoclopramide (anti-dopaminergic + anti-

serotoninergic) Surgical - Indications: Failure of medical therapy (or incomplete resolution of symptoms) Oesophagitis with frank ulceration or stricture Complications of reflux oesophagitis – respiratory complications, Barrett’s oesophagus Severe symptoms or progressive disease Compliance problems - patient does not want to be on medication for life (despite good results)

- Goal of surgery: Increase pressure at the gastro-oesophageal junction but not so much that it prevents food from entering the stomach (too

tight Æ dysphagia) - Surgery versus conservative treatment Surgery has higher rates of cure and better long-term results?? / results not longstanding? No need to adhere to strict lifestyle and diet change as well as long-term medication Disadvantage of surgery is the associated morbidity and mortality

98

- Fundoplication (mainstay of surgical therapy) Can be done via open surgery or laparoscopic surgery (most laparoscopic now) a. Nissen fundoplication is the most commonly done – a 360 degree (total) wrap of the fundus around the gastro-

oesophageal junction b. Partial fundoplications can also be done in patients where oesophageal motility is poor or the oesophagus is

foreshortened; anterior 90 degrees, anterior 180 deg, and posterior 270 deg fundoplications are various options available - Complications of surgery

1. Perforation – most feared complication, may result in mediastinitis if not promptly detected and repaired intraoperatively

2. Excessively tight wrap resulting in dysphagia

3. Excessively loose or short wrap – reflux recurs (failure of treatment)

4. “Slipped-Nissen” body of the stomach intussuscepts through the fundoplication, creating an hourglass deformity whereby

the stomach resides both above and below the wrap; usually due to a foreshortened oesophagus unrecognised in the first operation. Causes severe reflux as pouch above the wrap traps food and serves as reservoir.

5. “Gas bloat syndrome” – patient experiences difficulty burping gas that is swallowed (gas accumulation)

- Outcome of surgery 80-90% Excellent to good (no symptoms, no medications and lifestyle changes required) 10-15% Satisfactory (some residual symptoms) <5% Unsatisfactory <1% Mortality 5-40% need for acid suppression therapy at 5 years due to symptoms

- Management of stricture Rule out malignant cause of stricture by taking biopsy

1. Dilatation (variety of means available – balloon, dilators, etc) 2. Treatment of underlying reflux 3. If resistant to dilatation Æ resection and reconstruction Others - Nerve stimulation? of LES to increase tone (still under research)

99

6. BARRETT’S OESOPHAGUS

FEATURES - Intestinal metaplasia of the oesophageal epithelial lining

(stratified squamous epithelium Æ mucus-secreting columnar epithelium with goblet cells) - Associated with long-term reflux – an adaptation mechanism where intestinal epithelium withstands exposure to acidic reflux

better than oesophageal epithelium - Diagnosis:

Endoscopy and histology: The squamocolumnar junction (or Z line) is visible on endoscopy as gastric and intestinal type epithelium is pink and

granular in appearance, but stratified squamous epithelium is smooth and pale The gastro-oesophageal junction is defined as the point where the gastric folds begin If the squamocolumnar junction is above the gastro-oesophageal junction (i.e. they do not align) and biopsy of the junction

shows intestinal metaplasia, the patient is diagnosed to have Barrett’s oesophagus - Complications:

Short segment Barrett’s is defined as the squamocolumnar junction being <3cm above the gastro-oesophageal junction Long segment Barrett’s the distance between the two junctions is >3cm: associated with more severe reflux, as well as higher risk of dysplasia and subsequent adenocarcinoma development than short segment Barrett’s

Risk of development of adenocarcinoma is about 10-15% in 10 years

MANAGEMENT 1. Treatment of underlying reflux

- Lifestyle changes, acid suppression (PPI!), surgery etc - Not shown to decrease risk of cancer Æ still requires surveillance??

2. Endoscopic surveillance

- Not certain regarding benefit for surveillance if patient has Barrett’s but no dysplasia Æ if 2 scopes in a year reveal no dysplasia, repeat OGD once every 3 years (half-yearly Æ 3 yearly)

- Main purpose of surveillance is to pick up dysplasia - If patient has high grade dysplasia, it should be treated (see below), otherwise to undergo intensive surveillance (3 mthly

for at least one year) to detect cancer development 3. Treatment of high-grade dysplasia

1. Endoscopic therapies to ablate the dysplastic tissue e.g. photodynamic therapy, laser therapy, argon plasma coagulation Æ will not remove all dysplastic cells thus potential for malignancy still remains

2. Oesophagectomy is the only definitive treatment to remove all dysplasia, but is associated with high morbidity and mortality (worth it?)

3. Possibility of endoscopic mucosal resection as a treatment modality (research still undergoing) – shown to be as good as oesophagectomy with less morbidity and mortality

100

7. ACHALASIA

FEATURES - Abnormal peristalsis secondary to absence or destruction of Auerbach’s (myenteric) plexus and failure of the LES to relax;;

affects body and distal oesophagus - Lacks nonadrenergic, noncholinergic, inhibitory ganglion cells, causing an imbalance in excitatory and inhibitory

neurotransmission. The result is a hypertensive nonrelaxed esophageal sphincter - Aetiology unknown - Adults, M:F = 1:1 - Patients present with dysphagia, regurgitation, weight loss, retrosternal chest pain, and recurrent pulmonary infections - Barium swallow demonstrates “bird’s beak” narrowing of distal oesophagus with proximal dilatation - Manometric studies (required for diagnosis) show abnormally high pressures at the LES, with incomplete LES relaxation on

swallowing, and lack of progressive peristalsis (often aperistaltic) - 1-10% of patients develop SCC after 15-25 years of disease TREATMENT - Mainly palliative in nature - Non surgical treatment:

Intrasphincteric injection of botulinum toxin to block the release of acetylcholine at the level of the LES (problem is that it is

not long lasting and only used in patients not fit for surgery) Pneumatic balloon dilatation (A balloon is inflated at the level of the gastroesophageal junction to blindly rupture the muscle

fibers while leaving the mucosa intact): about 65% of patients improve, 40% response rate at 5 years) Calcium channel blockers and nitrates (both decrease LES pressure but do not improve LES relaxation. Used in elderly who

are not suitable for surgery/dilatation) - Surgical treatment (primary Tx):

Laparoscopic Heller cardiomyotomy (much like Ramstedt pyloromyotomy for pyloric stenosis) – good results with 85%

symptom-free after 5 years; there is a 3% chance of developing reflux Æ addition of fundoplication (partial wrap) helps prevent this

Esophagectomy was the standard treatment in patients with achalasia and a markedly dilated or sigmoid-shaped esophagus (controversial)

101

6. UPPER GIT 1. APPROACH TO BLEEDING UPPER GIT

CAUSES Variceal vs Non-variceal! 1. Peptic ulcer disease (bleeding peptic ulcer) 2. Gastritis, gastric erosions, duodenitis 3. Gastric malignancy 4. Stress ulcers from burns/ instrumentation 5. Gastro-oesophageal varices 6. Mallory-Weiss tear 7. Rare causes: AV malformation (Dieulafoy lesion) – most will stop bleeding spontaneously, aortoenteric fistula 8. Bleeding from other sources: Haemoptysis, nasopharyngeal bleeding 9. Systemic: coagulopathies, renal failure (uraemic gastritis) HISTORY (if patient is stable) 1. Nature of bleeding

Haematemesis - [oes] Can be fresh red blood as in variceal bleeding, Mallory-Weiss tear, AV malformation - [gastric] Coffee grounds vomitus is altered blood (due to gastric acid) and can come from gastric ulcer, gastritis/erosions, or variceal

blood that has entered the stomach [oes] Malaena - Altered blood; malaena indicates bleeding from the upper GIT i.e. above the ligament of Treitz - Different types of malaena:

(a) Fresh malaena – jet black with sheen, tarry, non-particulate (almost liquid in consistency), foul smelling, floats on water – maroon when you spread it out on gloves

(b) Stale malaena – black-grey, dull, mixed with normal stool, occasionally particulate (c) Iron stool – greenish hue on rubbing between gloved fingers, particulate.

- If gloved finger is stirred in a cup of water, malaena will “dissolve” completely with no sedimentation and turn the water black, but iron stool will sedimentate and turn the water green

Frank PR bleeding - Very brisk upper GI bleed can present as frank PR bleeding as blood passes down so fast it doesn’t get altered

2. Amount of blood - If patient is having haematemesis, ask how much blood Æ Cup? Bowl? - Hematemesis: easily 1.5L of blood in stomach before vomiting out (bleeding more than pylorus can empty)

3. Aetiological clues / RF

Gastric ulcer/gastritis/erosions - Any history of dyspepsia, gastric ulcer (any OGD done in the past showing these problems? On any “gastric” medications?) - Any drugs that may predispose – NSAIDs, antiplatelets, steroids, anticoagulants, TCM Varices - Any history of chronic liver disease - Previous variceal bleeds Mallory-Weiss tear - Binge-drinking with subsequent severe retching and vomiting leading to haemetemesis Malignancy - Recent constitutional symptoms e.g. LOA, LOW, malaise - Early satiety - Dyspepsia

4. Complications - Symptoms of anaemia: postural giddiness, shortness of breath, lethargy, È effort tolerance, palpitations, chest pain - AMI Æ esp. if it’s an old patient with previous history of IHD

5. Comorbidities - Elderly patient (>60) Æ high risk (Rockall score) - Other comorbidities: liver disease, renal disease, IHD Æ high risk (Rockall score)

102

PHYSICAL EXAMINATION 1. Vitals!

- Blood pressure, heart rate stable? Any postural hypotension? (Tachycardia is an early sign of shock) Narrowing pulse pressure (ind systolic hypotension) - check if patient is on BB!!

- RR>20 - Patient’s conscious level – confused? (shock) - Compare current vitals with vitals in ambulance, ED – is there a worsening trend? - Urine output: renal perfusion indicates cardiac output

2. General inspection - Pallor - Cold clammy peripheries Æ impending shock - Stigmata of chronic liver disease

3. Peripheries - Cold clammy peripheries Æ impending shock - LN (mets)

4. Abdomen - Any tenderness (not very helpful), epig mass - Distension (++ blood in stomach)

5. Digital rectal examination - Malaena or frank blood - tumor deposits / rectal mass

IMMEDIATE MANAGEMENT 1. Resuscitation

- ABC: Protect airway, supplemental oxygen, 2 large-bore IV cannula in antecubital fossa

Bloods: 1. FBC (Hb will not drop in first 24hrs, low plt - may not stop bleeding), 2. U/E/Cr (dehydration - raised Ur more than Cr, elec abn from vomiting) 3. PT/PTT (check and correct for coagulopathy), 4. LFT (Child's score - ind etiology and outcome) - do in alcoholic hx or liver disease 5. GXM: order in active bleed 4 pints PCT, FFP, Plt

ECG to detect any acute myocardial ischaemia/infarction, PFO CXR: PFO, aspiration, perforation if pt complains of pain

- Infusion - 1 pint N/S over half to one hour if patient is in shock, followed by more fluids if necessary (be wary in pts with renal & heart failure) - Packed cells if Hb is less than 10, to keep Hb above 10g/dL - May consider platelets if patient is on antiplatelet meds (qualitative defect in platelets - even if plt is normal, they are dysfunctional) - FFP if patient is on anticoagulants or PT/PTT prolonged (+ vitamin K)

2. Adjuncts

- NG tube if patient is having haematemesis – prevents aspiration, allows gastric lavage prior to OGD (DO NOT insert if suspect varices) - Catheterisation – monitor I/O balance esp in elderly or when large amount of fluid resuscitation required, or anticipating surgery - Intubate if: 1) massive uncontrolled active hematemesis

2) signs of decompensation eg. obtunded

3. Early medications - IV omeprazole 80mg bolus --> 8mg/hr infusion X 3/7

Ç stomach pH and stabilises clot formation regimen to prevent rebleed (normal Tx dose 40mg bd)

- If suspecting varices – IV somatostatin/octreotide, IV antibiotics - Stop antiplatelets, anticoagulants, NSAIDS

4. Close monitoring - Monitor for: SHOCK (Ç HR, È BP, È urine output, Ç confusion & lethargy) - HD with hourly para if Elderly with many comorbidities Hypotension

5. Emergency oesophagogastroduodenoscopy

103

Alternatively, scope the next available OGD (usually following day) - 3 Indications:

1. Shock / hemodynamic instability (ensure BP is stable before OGD - requires sedation) 2. Active BGIT (esp hematemesis, also fresh malaena) 3. Suspected variceal bleed

Not just low Hb as emergency scope causes more stress. - Ensure bloods are running before OGD! - Role of endoscopy Diagnostic:

confirm UBGIT & identify source of bleeding, Biopsy (+ CLO test if ulcer)

Therapeutic: 1. Varices: ligation/sclerotherapy, glue 2. Non-variceal: Clip, Coag, Injection

- CI: Perforation - air sufflation during OGD will cause abdominal compartment syndrome --> decrease venous return --> patient may DIE splinting of the diaphragm

- Repeat OGD with greater detail if it is normal the 1st time. Exclude haemoptysis & bleeding from nasopharynx Look for Mallory weiss tear and posterior wall D1 bleeding ulcer (gastroduodenal artery)

Obscure overt bleed: OGD normal Æ Colonoscopy normal Æ Small bowel imaging:

a) Capsule endoscopy b) Tagged RBC c) CT enterography CT mesenteric angio anytime when patient becomes unstable.

104

2. VARICEAL BLEEDING

PATHOPHYSIOLOGY A result of portal hypertension (i.e. portal venous pressure >20 cmH2O or >12 mmHg – normal = 7-14 cmH2O / 5-10 mmHg)

WHEN TO SUSPECT VARICEAL SOURCE IN UBGIT - Previous history of variceal bleed - Chronic alcohol intake - Jaundice or stigmata of chronic liver disease - Profuse hematemesis

MANAGEMENT OF VARICES can be divided into three categories: 1. Acute bleeding 2. Prophylaxis 3. Chronic management I. ACUTE BLEEDING – MANAGEMENT 1. Resuscitate

- Airway, breathing, circulation - If patient appears well, look for early signs of shock – tachycardia, postural hypotension - Look at hydration status

2. Assess mental state

- If patient has altered mental state (encephalopathy) Æ need to protect airway (may require intubation) 3. Vascular access, fluids/blood resuscitation, and blood investigations

- 2 large-bore IV cannula in proximal veins (cubital, EJV, IJV) - Send bloods – GXM 4 pints, FBC, U/E/Cr, LFT, PT/PTT - Infuse fluids - Under-resuscitate in variceal bleed (cf ulcer bleed) to keep Hb around 9, enthusiastic transfusion can Ç portal pressure

4. Management of severe bleeding: Sengstaken-Blakemore tube

- If hypotensive and bleeding is ongoing, may require use of Sengstaken-Blakemore tube for up to 48hr (if unable to ligate) - Protect airway before inserting tube. - Inflate gastric balloon and pull upwards against cardioesophageal junction (balloon will press on perforator veins entering oesophagus

from stomach, and thus decrease oesophageal variceal bleeding); oesophageal balloon is not inflated nowadays 5. IV somatostatin or S/C Octreotide (Not given in ulcer bleed)

- Mode of action: splanchnic vasoconstrictor which È portal blood flow and hence portal pressures Æ È variceal bleeding - Also acts indirectly to inhibit secretion of gut hormones that increase portal blood flow

6. Acid suppression

- Increasing intragastric pH increases clot stability, aids haemostasis - Agents available: omeprazole (Losec), esomeprazole (Nexium), pantoprazole, etc.

7. Antibiotics

- Not given in ulcer bleed - Risk of sepsis in patients with CLD and bleeding - Studies have shown that cover with broad spectrum Abx (Gram neg cover) È infectious cx, mortality, and also risk of recurrent bleed - Ceftriaxone, Ciproflox 1g/day - Preferably started before endoscopy (procedures increase bacteraemia)

8. Emergency endoscopy

- Purpose: confirm diagnosis and institute management - Needs to be done emergently (on admission) as soon as patient is stabilised since bleeding can be torrential and life-threatening - Intervene if ESRF present or active bleed (not electively) - Banding/Ligation is the best modality for oesophageal variceal bleeding (sclerotherapy a/w higher morbidity e.g. mucosal ulceration) - Gastric varices are usually too large to be banded, sclerotherapy/ Glue (Histoacryl) used instead – risk of embolism from histoacryl

9. Observation

- Keep patient in ICU! - NBM 2-3/7 - Continue antibiotics and omeprazole - Continue somatostatin up to the point where haemostasis is achieved or 5 days (exact ideal duration not well studied) - Antiencephalopathy Tx: Lactulose + Cipro (clear gut to prevent absorption of NH3) - Anticipate complications:

(a) encephalopathy – fleet and lactulose, treat hypokalaemia from vomiting (b) aspiration – protect airway; ?benefit of gastric decompression using NG tube (c) risks of procedure – OGD-related risks

10. Management of possible precipitants

- NSAIDs; Hepatic vein thrombosis

105

ÎIf bleeding stops, home in around 1/52 Æ Rescope before home! (ligate if rebleed) ÎIf bleeding is not remediable by endoscopic intervention:

A. Insert Sengstaken-Blakemore tube up to 48hr (only temporary) Æ repeat endoscopy 10-12 hours later If rebleed after 48hr of SBT: consider surgery

B. TIPSS: Radiologically guided insertion of transjugular intrahepatic porto-systemic shunt

- If patient in good Child’s score, to avoid as it ppt. encephalopathy - Constant monitoring of patient. - Cx: encephalopathy (increased risk with bigger shunt), bleeding, thrombosis, failure (blood flow in opposite direction) - usually temporising procedure

C. Shunt surgery Portocaval shunts (portal vein to IVC)

– side-to-side, end-to-side Mesocaval shunts (sup mesenteric vein to IVC) Proximal splenorenal shunt (splenectomy with end-to-

side anastomosis of portal side of splenic vein to left renal vein)

Distal splenorenal shunt (Warren-Zeppa shunt – splenic vein divided, splenic side anastomosed end-to-side to left renal vein)

A. Normal liver with normal blood flow. B. End to side spelnorenal shunt.

C. End to side portacaval shunt. D. Mesocaval H-graft shunt

D. Sugiura procedure: last resort x Most effective non-shunt operation x A key point is that the left gastric (coronary) vein and the paraesophageal collateral veins are preserved to permit portoazygous

collateralization, which inhibits future varix formation x Japan cited a 5.2% operative mortality and a 6.3% rate of recurrent hemorrhage. In United States, operative mortality with this

procedure has exceeded 20%, with bleeding recurring in 35% to 55% of patients.

x Splenectomy x Proximal gastric devascularisation x Selective vagotomy x Pyloroplasty x Oesophageal devascularisation x Oesophageal transection

106

II. PROPHYLAXIS OF VARICEAL BLEEDING SECONDARY = Band ligation + Non-selective beta-blockers

- Best option is combination propranolol unless CI: decrease size and prevent rebleeding 3 weekly ligation until completely obliterated

PRIMARY = Non-selective beta-blockers (oesophageal varices with no previous history of variceal hemorrhage) In pts with small varices with no risk of bleeding, the use of propranolol is of questionable benefit – repeat OGD to monitor varices. - È cardiac output by β1 blockade and splanchnic blood flow by blocking vasodilating β2 receptors at splanchnic vasculature - The use of beta-blockers decreases the risk of initial variceal bleeding by approximately 45%, to be continued indefinitely - The dose: determined by a 20% decrease in baseline resting HR / decrease in HR to 55 bpm / dev adverse effects - If contraindications to using beta-blockers exist, long-acting nitrates (eg, isosorbide 5-mononitrate) are alternatives - Band ligation/ Sclerotherapy: not used as primary prevention. as effective as treatment with propranolol Predictors of variceal haemorrhage: [SRCPS] 1 Site: varices at the GE junction have the thinnest coat of supporting tissue and are at highest risk of rupture and bleeding 2 Size: F1: Small straight varices F2: Enlarged tortuous varices that occupy less than one-third of the lumen F3: Large coil-shaped varices that occupy more than one-third of the lumen 3 Child’s score – patients with higher Child’s score have higher risk 4 Red signs: Red wale marks (longitudinal red streaks) (ESRH) Cherry red spots (flat discrete spots) Haematocystic spots (raised discrete spots – resemble “blood blisters”) Diffuse erythema 5 Previous variceal haemorrhage: 70% of patients will have further variceal bleeds after an index bleed (risk highest in first 48 hours after first bleed) 30% rebleed within 6 weeks; 30% rebleed after 6 weeks III. CHRONIC MANAGEMENT - Start patient on an ablation regimen (endoscopy with initial ligation/sclerotherapy and subsequent endoscopic monitoring and

repeated ligation/sclerotherapy as required to completely ablate varices) - If patient bleeds again Æ failed ablation Æ consider surgery (as above – shunts, or Sugiura) - LT propanolol + PPI - Refer to Hepato for liver disease http://www.heart-intl.net/HEART/011507/PortalHypertension.htm

107

3. PEPTIC ULCER DISEASE

EPIDEMIOLOGY - Incidence about 100 per 100,000 per year - 68% of patients are over 60 years of age - Overall mortality 7-10%, unchanged for last 20yrs – mostly due to ulcer bleeding esp in elderly with significant comorbidities MAIN AETIOLOGICAL FACTORS H. pylori - 60% of population are positive for H. pylori by age 21 - About 10-20% of infected patients develop an ulcer - Accounts for 90-95% of duodenal ulcers, and 50% of gastric ulcers NSAIDs - Accounts for most of the rest of ulcer disease not caused by H. pylori - 30% of patients on NSAIDs will get an ulcer, of which 20% (6% overall) will have a clinically significant ulcer i.e. symptomatic, bleeding Other factors - Cigarette smoking - Alcohol - Steroids and anticoagulants do not increase the risk of ulcer formation, but increase the risk of bleeding in an existent ulcer PATHOGENESIS - An imbalance between mucosal protective mechanisms against acid, and aggressive forces that damage the gastric mucosa - 2 Aggressive forces: gastric activity and pepsin activity - 5 Protective mechanisms: mucus secretion, bicarbonate secretion into mucus, robust mucosal blood flow to remove protons, epithelial

regenerative capacity, prostaglandin secretion by mucosa to maintain blood flow - H. pylori causes a local inflammatory reaction and secretes enzymes that break down the gastric mucosal barrier, and also enhances gastric

acid secretion and decreases bicarbonate production - NSAIDs impair mucosal prostaglandin production (through non-selective COX inhibition) Æ prostaglandins are important for mucosal

bicarbonate and mucin production and inhibiting gastric acid secretion, as well as maintaining mucosal blood flow PRESENTATION 1. Incidentally detected on OGD

2. Symptoms of dyspepsia

(a) Ulcer-like dyspepsia: pain in the upper abdomen is the predominant symptom (b) Dysmotility-like dyspepsia: non-painful discomfort in upper abdo, a/w upper abdo fullness, early satiety, bloating, belching, nausea (c) Unspecified dyspepsia - Pain is usually worse with food in a gastric ulcer, while it is relieved by food in a duodenal ulcer

3. Bleed

- As above, presenting with haematemesis (coffee-grounds vomitus) or malaena 4. Perforation

- Patient presents with sudden generalised abdominal pain that is aggravated by even the slightest movements - Board-like rigidity, guarding will be present on examination (signs of peritonism) - Erect CXR will show air under diaphragm

ENDOSCOPY (OGD) - The most important and valuable investigation - Roles of endoscopy:

(a) Diagnosis

Confirmation of ulcer disease Location of ulcer Biopsy to rule out malignancy – esp gastric (usually 6 bites) Biopsy of antral tissue for CLO (Campylobacter-like organism) test / rapid urease test for H. pylori: biopsy tissue is placed into a

medium containing urea and an indicator such as phenol red. The urease produced by H. pylori hydrolyzes urea to ammonia, which raises the pH of the medium, and changes the color of the specimen from yellow (NEGATIVE) to red (POSITIVE).

108

(b) Prognostication of bleeding risk (in UBGIT) Forrest classification (or endoscopic stigmata of recent haemorrhage – ESRH)

Forrest grade Bleeding risk 1a Spurting (arterial) 90% 1b Non-spurting, ooze (venous) 20% 2a Non-bleeding ulcer with visible vessel 40% 2b Non-bleeding ulcer with adherent clot 20% 2c Ulcer with haematin-covered base (flat spot) 10% 3 Ulcer with clean base 5% Adherent clot Æ must flush and remove clot --> grade ulcer again

Classical bleeding BV: posterior-D1 (gastroduodenal art) Tx: Only pigmented spot can leave alone, the rest must intervene!

(c) Endotherapy (in UBGIT) 1. Injection with adrenaline (1:10,000 dilute to 10ml - continue with N/S if need more) or absolute alcohol – tamponade effect is the most

effective. Also vasoconstriction for adrenaline. Inject around ulcer (4 quadrants). Cx: perforation, bleeding, necrosis, arrhythmia.

2. Coagulation (Heater probe = thermal / Argon plasma) 3. Haemostatic clipping (endoclip) Principle: Dual modality better than single (as less risk of recurrent bleed and mortality) --> Usually Injection + Clip/Heater probe No study proves superiority of either therapy. Failed OGD hemostasis (after 1hr)

a) Surgery is mainstay b) Transcatheter embolisation

a. ind: failed surgery, rebleed post-Sx (usually GDA), not fit for Sx b. identify bleeding vessel and embolise with

i. Mechanical (non-absorbable coil eg. Titanium) ii. Chemical (gel foam): can recannulise again in 2/52

c. Sandwich technique: embolise both prox and distal ends of vessel as bleeding can occur both ways. Post-endotherapy:

- Monitor for rebleed = drop in Hb (not malaena / vomit blood clots as these are expected. Changes in vitals may be too late.) - Diet individualised: usually NBM 1-2/7, 3/7 if worrisome, <1/7 if straight forward ulcer - Re-scope the following day if you’re worried about the outcome from the first scope (notroutine) - Oralise PPI after 3/7 infusion (if worrisome, can give IV bolus PPI BD) - TCU 3-4/52 at clinic

Re-scope in 6 weeks to document ulcer healing If ulcer still present, biopsy ulcer again

1. Histo: exclude malignancy for gastric ulcer 2. Gastric biopsy urease test (to confirm eradication of H. pylori) - not accurate if patient on triple tx

Rescope and biopsy every 6/52 until ulcer is healed Æ resect if persistent Confirm eradication of H Pylori: Urease breath test, Re-biopsy

109

CONSERVATIVE MANAGEMENT 1. Gastroprotection

(a) Standard dose proton pump inhibitor

20mg OM Promotes ulcer healing even with ongoing NSAID use

(b) Double dose famotidine 40mg BD Inferior to omeprazole as famotidine only promotes ulcer healing if NSAIDs are stopped; ulcers will not heal with ongoing

NSAID therapy

2. H. pylori eradication First line triple therapy: omeprazole 40mg BD (for 6 weeks), amoxicillin 1g BD, clarithromycin 500mg BD for 7 days In penicillin-allergic patients, substitute amoxicillin with metronidazole 400mg BD Document eradication by endoscopy with CLO test, urea breath test or stool serology testing Treatment failure occurs in up to 20%

- treat with quadruple therapy: colloidal bismuth subcitrate 120mg QDS, tetracycline 500mg QDS, metronidazole 400mg BD, omeprazole 20mg BD for 7 days

SURGICAL MANAGEMENT Indications for surgery for acute PUD: 1. Bleed: Mainstay of Tx for failed OGD hemostasis (after 1hr) 2. Perforation Indications for surgery for chronic PUD: 1. Recurrent bleeding 2. Splitting BV difficult to clip 3. Prev episode of shock DUODENAL ULCER Indications for surgery: ‘Patient has to BUY the op’ (more complications + duod ulcer will heal provided the high acid secretion of stomach is abolished) 1. Persistent bleeding (e.g. erosion of a post duodenal ulcer into GDA) 2. Perforation 3. Gastric outlet obstruction (patient presents with vomiting of undigested food not long after meal, succussion splash, air-fluid levels on

AXR; characteristic electrolyte abn of hypokalaemic hypochloraemic metabolic alkalosis with paradoxical aciduria) 4. Failure of medical management (ulcer does not heal) Surgery: 1. Bleed =

a. Duodenotomy + Oversewing/Under-running the bleeding vessel b. Antiulcer surgery (if patient stable and known case of rebleed/perf)

2. Perforation = a. Omental patch repair + Bx (small <2cm ulcer / patient unstable) b. Antiulcer Sx (>2cm or suspect malignancy)

(parietal peritoneum, jejunum serosa, stomach local flap) 3. Definitiveantiulcer surgery

= Duodenectomy + Vagotomy with gastric drainage procedures - Usually done in elective setting for chronic duodenal ulcers - Rationale for vagotomy is to eliminate direct cholinergic stimulation to gastric secretion; parietal cells also become less

responsive to histamine and gastrin, and vagal stimulus for gastrin release is abolished - Vagotomy can be truncal, selective, or highly selective (selecting only those branches that appear to supply peptic cells. does

not require drainage as the Nerves of latarjet that supply the pyloric sphincter are not affected) - Drainage procedures Æ pyloroplasty (first) or gastrojejunostomy

Usually done with vagotomy as gastric emptying is decreased with denervation. Pyloroplasty = incise pylorus longitudinally through mucosal layer then suture the incision transversely

Perforated ulcer: IV fluids, IV antibiotics, PPI, surgery (patch repair)

110

GASTRIC ULCER Indications for surgery ‘SELL the operation to the patient’ (risk of malignancy if >2years) 1. Failure to heal after 3 months of conservative therapy 2. Dysplasia or carcinoma 3. Recurrence 4. Perforation, persistent bleeding Surgery 1. Bleed =

a. Ulcerectomy (if patient unstable, old, unfit) b. Antiulcer surgery (if patient stable, can tolerate)

2. Perforation = a. Omental patch repair + Bx (small <2cm ulcer / patient unstable) b. Antiulcer Sx (>2cm or suspect malignancy)

(parietal peritoneum, jejunum serosa, stomach local flap) 3. Definitive antiulcer surgery = Gastrectomy

a. Partial gastrec if distal ulcer b. Proximal ½ ulcers may require total gastrec

Can be used in emergency bleed if patient stable, but considered antiulcer Sx as parietal cell mass and antrum (contains gastrin containing cells) are removed. Bancroft’s closure (for severe inflammation/scarring that prevents dissection around pylorus. Remove all antral mucosa to prevent marginal ulcers)

If prepyloric ulcer, can treat similar to duodenal ulcer

111

4. GASTRIC CANCER

EPIDEMIOLOGY - Fourth most common cancer in males, sixth most common in females in Singapore - Female to male ratio 2:1 - Incidence 10-18 per 100,000 per year - Incidence increases steeply after 50 years old RISK FACTORS 1. Environmental

- Diet: preserved foods (nitrosamines), smoked foods, polycyclic hydrocarbons - Smoking - Alcohol - Occupational exposure: asbestos, heavy metals, rubber - Low socioeconomic status

2. Genetic

- Blood type A - HNPCC – Lynch syndrome II - P53 mutation - Germline mutation of e-cadherin - Family history of gastric cancer

PRECURSOR CONDITIONS 1. Partial gastrectomy for benign disease with Bilroth II reconstruction

- Usually occurs >15 years after surgery - Due to chronic exposure of gastric mucosa to biliary, pancreatic and intestinal secretions at the anastomotic zone

2. Gastric polyps

- Highest risk in inflammatory polyps: 75-90% - 10-20% risk in adenomatous polyps especially in large polyps (>2cm) or those with villous histology - Also increased risk of adenocarcinoma elsewhere in the stomach

3. Chronic atrophic gastritis

- Hypertrophic gastritis (Menetrier’s disease) – inflammatory disease of gastric epithelium, up to 10% risk of malignant change - Pernicious anaemia – autoantibodies to parietal cells with achlorhydria, 2-10% risk of gastric cancer

4. Peptic ulcer disease

- <1% risk of malignant change 5. H. pylori infection

- 3-6X increased risk of gastric cancer HISTOLOGY a) Adenocarcinomas - Make up 90-95% of stomach tumours - Lauren classification:

(a) Intestinal type (most common overall) – occurs in high risk population, distal third of the stomach, in older men; associated with erbB2 and erbB3 receptor stimulation

(b) Diffuse type – occurs in low risk population, proximal third and cardio-oesophageal junction, in younger and female patients; more aggressive, present later, worse prognosis; associated with K-sam oncogene

- Early gastric cancer Confined to mucosa and submucosa Good survival and prognosis regardless of size, lymph node status, histological grade

b) Non-adenocarcinoma - Make up less than 10% of stomach tumours - Types: SCC, neuroendocrine tumour, leiomyosarcoma, GIST (gastrointestinal stromal tumor), primary gastric non-Hodgkin’s lymphoma

(MALT, linitis plastica) – NHL in submucosa Æ adynamic stomach Æ N/V more than anemia

112

MORPHOLOGY Borrmann’s classification:

x Type I (3%): Nonulcerated, polypoid, growing intraluminally x Type II (18%): Ulcerated, circumscribed with sharp margins x Type III (16%): Ulcerated, margin not sharply circumscribed x Type IV (63%): Diffuse, infiltrating, may be ulcerated; may diffuse entire

stomach (linitis plastica)

LOCATION - 37% in cardia - 30% in pyloric channel or antrum - 20% in body - 12% in entire stomach SPREAD - Direct extension to neighbouring organs - Lymphatic spread

(a) Regional nodes (b) Supraclavicular nodes (Virchow’s node) (c) Umbilical (Sister Joseph’s node)

- Haematogenous spread – liver, lung, bone, brain - Peritoneal seeding to omentum, parietal peritoneum, ovaries (Krukenberg’s tumour), or cul-de-sac / pouch of douglas

(Blumer’s shelf = shelf-like tumor of ant rectal wall DDx tuberculous peritonitis) PRESENTATION Very non-specific symptoms and signs: - Abdominal pain (usu late)60% - Weight loss 50% - Nausea/vomiting 40% - Anaemia 40% - Palpable mass 30% - Haematemesis/malaena 25% - Early satiety 17% - Metastatic symptoms late (bony tenderness, neurological deficits, etc) Î New onset dyspepsia at age>35 years old should cause concern COMPLICATIONS - Bleeding - Gastric outlet obstruction Æ vomiting (dehydration, hypokalaemic metabolic alkalosis, aspiration ± pneumonia) - Perforation - Malnutrition

1. Asymptomatic: from histology of ulcer biopsy 2. Symptomatic: non specific abdominal pain, early

satiety, N/V, LOW, LOA 3. Complications: s/s of anaemia, s/s of UBGIT, s/s of

GOO, s/s of metastasis, peritonism (perforation)

113

INVESTIGATIONS Diagnosis

x OGD –visualisation and biopsy (ulcer with heaped-up edges) Staging investigations 1. CXR/ CT thorax– lung mets 2. Endoscopic ultrasound – gold standard for T staging and good for N staging 3. CT abdo pelvis – good for T, N & M staging 4. Staging laparoscopy prior to operation – picks up small peritoneal metastases that are occult on CT scanning (up to 1/5 of

patients) Æ upstage of disease Complications 5. FBC – low Hb 6. U/E/Cr – if vomiting, low potassium, low chloride, alkalosis 7. LFTs – albumin as a marker of nutritional status (alb<35 is poor); liver mets

STAGING

Tis T1 T2 T3 T4

Carcinoma in situ Tumour limited to mucosa and submucosa Tumour invades muscularis mucosa Tumour penetrates serosa Tumour invades adjacent structures

N0 N1 N2 N3

No regional LN 1-6 regional LN involved 7-15 regional LN involved >15 regional LN involved

CURATIVE TREATMENT SURGERY Principles of surgery: - Wide resection of the tumour to negative margins (at least 6cm margins) - En-bloc excision of regional lymph nodes - Choice between total gastrectomy and subtotal gastrectomy Subtotal gastrectomy leaves a small portion of proximal stomach – easier to anastomose to jejunum than oesophagus since

oesophagus does not have serosa (higher risk of leak) Subtotal gastrectomy is associated with less morbidity, better functional outcome (some residual reservoir fx preserved) Total gastrectomy is the resection of choice for proximal tumours (fundus, cardia, body) as well as diffuse-type tumours and

cardio-oesophageal junction tumours - Reconstruction Bilroth I (end-to-end gastroduodenostomy) – rarely done (diff to mobilise duodenum to anastomose with residual stomach) Bilroth II/ Poly-A (gastrojejunostomy) – no protection against biliary reflux into stomach Roux-en-Y – to prevent biliary reflux; but involves 2 anastomoses, higher chance of leak Oesophagojejunostomy (after total gastrectomy)

114

Complications of gastrectomy: Early 1. Bleeding 2. Infection 3. Anastomotic leak 4. Injury to surrounding structures eg. pancreas

Late

1. Early satiety 2. Retained antrum syndrome

a. Not enough antrum removed Æ Ç acidity in residual stomach, with formation of marginal ulcers on the jejunal side of the anastomosis

3. Intestinal hurry a. Inadequate reservoir function leads to poor digestion Æ may have phytobezoar (veg fibre bezoar) formation

4. Dumping syndromes

a. Early dumping syndrome (1/2-1hr after meal): due to increased osmotic load from stomach into small intestine Æ flushing, palpitations, dizziness, nausea, diarrhoea, hypovolemia (due to rapid entry of water into small intestine) Treat by eating small frequent meals with low carbo and high protein/fat.

b. Late dumping syndrome (1-3hr later): reactive hyperinsulinaemia Æ alimentary hypoglycaemia (weak, sweat, dizzy). Treat by eating more carbohydrates.

5. Biliary/intestinal reflux into stomach

a. Leads to symptoms of dyspepsia

6. Afferent limb syndrome a. Occurs in Bilroth II/Polya reconstruction b. Mechanical obstruction of the afferent jejunal loop due to kinking, anastomotic narrowing, or adhesions Æ postprandial epigastric pain

with nausea, non-bilious vomiting c. one of the main causes of duodenal stump blowout in the early postoperative period and is also an etiology for postoperative obstructive

jaundice, ascending cholangitis, and pancreatitis due to transmission of high pressures back to the biliopancreatic ductal system d. Can be decreased by doing Roux-en-Y surgery (but may still occur)

7. Nutritional deficiency

a. Iron deficiency – mixed picture i. Loss of intrinsic factor Æ B12 deficiency ii. Decreased conversion of iron from Fe3+ to Fe2+ by gastric acid Æ decreased iron absorption in terminal ileum

b. Need to supplement with B12 injections and iron supplements Post-gastrectomy weight loss

x 10-33% of pre-op weight x due to LOA (less frequent meals) + decreased reservoir capacity of stomach

Follow-up

- 2/52-1/12: repeat OGD - 6/12: detect local recurrence (esp if margin involved), check for B12 deficiency - 1-2 yr: yearly OGD

CHEMOTHERAPY/RADIOTHERAPY Adjuvant therapy 5-fluorouracil with chemotherapy 5-fluorouracil with epirubicin for advanced disease Neoadjuvant therapy - 5-FU and cisplatin can be used to downstage unresectable, locally advanced disease with a significant increase in resectability (61% Æ 79%) - For resectable disease: preoperative 5-FU, cisplatin, doxorubicin, methotrexate, followed by intraperitoneal 5-FU Æ improved resection rates, response rates, median survival

PALLIATIVE THERAPY - For palliation of symptoms such as pain, bleeding, obstruction 1. Endoscopic laser ablation for obstruction 2. Embolisation for bleeding 3. Surgical options: subtotal gastrectomy (6-15% mortality), total gastrectomy (20-40%), gastrojejunostomy for obstruction 4. External beam radiotherapy for pain, low-level ongoing bleeding (not for heavy bleeding as it takes weeks to cause fibrosis) PROGNOSIS

- Stage I 90% 5-year survival - Stage II 70% - Stage III 40% - Stage IV 0%

115

7. LOWER GIT & COLORECTAL DISEASES 1. APPROACH TO BLEEDING LOWER GIT

1) Colon Bleeding diverticulosis Angiodysplasia Colorectal carcinoma, polyps Colitis

- Infective (gastroenteritis, diverticulitis, colorectal TB) - Inflammatory (UC & Crohn’s) - Ischaemic

2) Ileum: Meckel’s diverticulum - usually dark red blood 3) Anorectal junction: hemorrhoids 4) Anus: anal fissure 5) Massive Upper GIT bleeding, e.g. bleeding DU

HISTORY (if patient is stable) 1) Nature of bleeding

x Haematochezia - Mixed with or coating stool - Torrential or drops, any clots? – Brisk upper GI bleed can present as haematochezia - Bright red (lower) or darker red (higher) - Any mucous

x Malaena - Altered blood, indicates bleeding from upper GIT above ligament of Treitz (duodeno-jejunal junction) - Ask for history as per UBGIT

2) Aetiological clues

x Exclude upper GIT cause - Any malaena, hematemesis, coffee grounds vomitus - History of PUD, gastritis, varices, Ca stomach, Mallory-Weiss tear, risk factors for each

x Bleeding diverticulosis/angiodysplasia - Usually torrential bleeding that stops spontaneously; altered clots - History of previous bleeding episodes

x Colorectal carcinoma - Constitutional symptoms: LOW, LOA, fatigue - Change in bowel habits, tenesmus - Symptoms of anaemia (chronic occult bleed) - Previous history/family history of GIT or ovarian cancer

x Colitis - Infective: any fever/chills/rigors, night sweats, N/V, diarrhoea, pain, recent travel/contact history, eating

seafood, previous TB exposure or infection, BCG vaccination status - Inflammatory: ask about history of UC or Crohn’s, joint, liver, eye & skin manifestations - Ischaemic: ask about atherosclerotic risk factors, previous AMI, stroke

x Hemorrhoids - bleeding to passing motion, blood coating stool, pain - Any mass noticed at anus - History of constipation, hard stools, low fibre diet, chronic straining, recent pregnancy

x Coagulopathy - Any history of bleeding disorders, easily bleeding, petechiae

3) Complications

x Symptoms of anemia (may be only presentation!): SOB, postural giddiness, palpitations, chest pain, ↓ effort tolerance, fatigue, syncope

x Symptoms of dehydration & shock: extreme thirst, confusion, pallor, ↓ urine output x May have complication of AMI if old patient with history of IHD

116

PHYSICAL EXAMINATION

1. Vitals: - HR, supine & postural BP –stable? urine output (if catheter in-situ), - Any fever?

2. General inspection: - pallor, - confusion?

3. Peripheries: - signs of dehydration e.g. capillary refill, dry tongue. - Any supraclavicular lymph nodes? - Any skin manifestations of inflammatory bowel disease? - Stigmata of CLD?

4. Abdomen: - Scars? Any palpable masses

5. DRE: - any anal fissures or prolapsed hemorrhoids seen, - any masses felt, - any fresh blood or malaena

6. Offer proctoscopy to look for internal haemorrhoids ACUTE MANAGEMENT Resuscitation

o Supplemental oxygen, insert 2 large-bore IV cannula with fast infusion of crystalloids (N/S 500ml over ½hr) o Infuse colloids if ongoing blood loss while waiting for whole blood (GXM) o Catheterise and monitor urine output, insert NGT on suction to detect UBGIT o Continuous vital signs monitoring & I/O charting ± CVP & stool chart o ECG + cardiac enzymes to detect possible AMI o Take blood for investigations

- FBC: low Hb level (repeat FBC 2-3 hours later or when necessary) – packed cell transfusion 4 pint PCT: give FFP to prevent over-dilution of clotting factors

- UECr: hydration status, any acute renal failure from shock, electrolyte imbalance – replace - PT/PTT: must correct coagulopathy before trying to stop bleeding – fresh frozen plasma - LFT: exclude bleeding varices - ABG: may have metabolic acidosis in shock due to organ ischaemia – IV bicarb, dialysis if severe - GXM 4 pints

Identify source & stop bleeding

o Arrange urgent colonoscopy (patient must be stabilised before procedure) - Diagnostic: identify cancer, diverticulosis, angiodysplasia, areas of inflammation & bleeding - Therapeutic: clip, diathermize, or inject adrenaline/ sclerosant into bleeding vessel

o Double contrast barium enema: good for picking up diverticulum which may be missed on scope o Mesenteric angiogram or CT mesenteric angiogram

- More precise; can cannulate all 3 main trunks & their branches - Diagnostic: shows blush in active bleeding, shows ↓ perfusion of ischaemic bowel - Therapeutic (not for CT): embolisation/sclerotherapy for angiodysplasia, beware of causing bowel ischaemia

(use foam medium) o Capsule Endoscopy:

- for stable patients with suspected SI bleed & unable to swallow - takes 36hrs to read the results, but unable to localise the site & intervene

o double balloon enteroscope: - only for suspected proximal SI - able to do therapeutic manoeuvres

o Labelled RBC isotope scan under gamma camera - For intermittent, occult bleeds not detectable on mesenteric angiogram - Not for urgent setting; can take up to 24 hours!

o Laparotomy with saline lavage if massive on going bleed, recurrent bleeding - On table scope to transilluminate bowel & identify bleeding source - On table angiogram - Oversewing of bleeding vessel, partial/total colectomy as last resort

117

2. COLORECTAL CANCER

EPIDEMIOLOGY COMMONEST CANCER IN SINGAPORE x Prevalence in Singapore: 6% (Commonest cancer in M, number 2 cancer in F) x Peak incidence at 60-70 years of age (increase with age) x Increasing incidence through the years. x Chinese more prevalent. PATHOLOGY - Almost all tumours are adenocarcinomas - 90% of tumours are sporadic - 8% arise in association with hereditary non-polyposis colon carcinoma (HNPCC) and 1% with familial adenomatous polyposis (APC) - 1% arise in association with long-standing ulcerative colitis (>10 years)

PATHOGENESIS There are 2 pathways for cancer development in the colorectal mucosa: 1. APC pathway (adenoma-carcinoma sequence)

- Accounts for 80% of sporadic colorectal carcinomas - Characterised by chromosomal instability - Stepwise accumulation of mutations in a series of oncogenes and tumour suppressor genes:

Loss of the APC suppressor gene on 5q21 (congenitally absent in patients with familial adenomatous polyposis – APC) is the

earliest event in adenoma formation Æ detected using Protein truncation test APC is required to break down beta-catenin; with the loss of APC, beta-catenin accumulates and activates various genes in the

nucleus (such as MYC and cyclin D1) which promote cell proliferation K-RAS (12p12) mutation follows the loss of APC – an activating mutation that causes the RAS to keep delivering mitotic signals

and prevent apoptosis Loss of tumour suppressor gene at 18q21 Loss of p53 late in carcinogenesis

- The molecular evolution of colon cancer through this pathway occurs through a series of morphologically identifiable stages: localised

epithelial proliferation Æ small adenoma Æ large, more dysplastic adenoma Æ carcinoma in-situ Æ invasive cancer 2. Defects in DNA mismatch repair

- Involved in 10-15% of sporadic cases - Like the APC pathway, there is accumulation of mutations, but due to a different mechanism, and without clearly identifiable

morphologic correlates i.e. no adenomas - Due to mutations in one of the five DNA repair genes (MSH2, MSH6, MLH1, PMS1, PMS2) of which MSH2 and MLH1 are the most

commonly involved in sporadic colorectal carcinomas - Loss of DNA mismatch repair results in microsatellite instability which affects coding or promoter regions of genes involved in cell

growth such as the BAX gene and the type II TGF-β receptor - Tumours that arise from this pathway have a better prognosis than tumours that arise from the APC pathway

SITE Rectosigmoid Æ Caecum + Ascending colon Æ the rest - 25% in caecum and ascending colon, - 25% in transverse colon, - 25% in descending colon and proximal sigmoid, - 25% in distal sigmoid and rectum Most are left-sided though there is an increasing incidence of right-sided tumours

CRC

Sporadic (90%)

APC pathway (80%)

DNA mismatch repair (15%)

Inherited (10%)

HNPCC (8%) FAP (1%) UC > 10yrs (1%)

118

MORPHOLOGY 1. Scirrhous / annular “apple-core” lesions (most common) - more common in the left colon 2. Polypoid – more common in the right colon as there is more space to grow 3. Ulcerated 4. Nodular SPREAD 1. Intramural –Transmural (along bowel wall. Seldom >2cm spread) or Intraluminal 2. Direct extension into surrounding tissues e.g. small bowel, ovary, ureters, bladder 3. Lymphatic 4. Haematogenous – to liver, lungs, bone, brain 5. Transcoelomic – direct seeding: through entire thickness Æ peritoneal cavity Æ spread thru peritoneal fluid. Late stage > T4. RISK FACTORS Non-modifiable (4) 1. Age >50 years 2. Genetic predisposition

(a) Personal history of CRCs x in the first 5 years after resection of a primary CRC,

i. Metachronous CRC: occurs at a rate of 3-5%, ii. Metachronous adenomas: rate of 25-40%

(Metachronous = primary tumors occurring more than 6 months apart) (b) Family history of CRCs

x 1 first-degree relative with CRC: increases risk of CRC 1.7X, x 2 1st-degree relatives with CRC, or if the relative had CRC <55YO: risk > 1.7X x FHx of colonic adenoma same significance as CRC??

(c) Polyposis syndromes x near 100% risk of cancer formation:

i. FAP and its variants ii. Gardner’s syndrome: multiple osteomas in mandible/skull/ long bones, sebaceous cyst, desmoid tumor iii. Turcot’s syndrome: glioblastoma multiforme

x other polyposis syndromes such as Peutz-Jegher’s, Cronkhite-Canada have a small increased malignant potential (d) HNPCC (AD) – more common than FAP, accounting for 8% of cancers.

3. Ulcerative colitis

- Increased risk after 10 years of disease if the patient has pancolitis; after 15-20 years if the patient has disease limited to the left colon

4. Adenomatous polyps - Increased risk if there is a personal history of large (>1cm) adenomatous polyps, and polyps with tubulovillous or villous histology,

particularly if multiple (3-6X increased risk) - Small (<1cm) tubular polyps do not have increased risk

Modifiable 5. Environmental factors

- Diet:

i. high in red meat, preserved foods (nitrosamines) ii. high in refined sugar/fat Æ potentially toxic oxidative byproducts by bac degradation iii. low in fibre Æ decrease stool bulk Æ high fecal retention Æ toxic products in contact with colonic mucosa for longer periods iv. low in vitamins ACE, minerals Ælack antioxidants

- Alcohol, smoking - NSAIDs may be protective against CRC

119

HISTORY A) Presentation

- Asymptomatic: detection during screening (e.g. Ulcerative colitis) - Symptomatic:

Local: Right sided (OCCULT Æ present late)

1. Iron deficiency / MCHC anemia – less Fe stores in the body cf folate & B12, so Fe depleted first 2. Right sided abdo mass 3. Abdo pain NO obstruction as stools more liquid and colon more spacious.

Left sided (due to MASS EFFECT)

1. Change in bowel habits (Progressive constipation, alternating constipation and diarrhoea) 2. Spurious diarrhoea (due to bacteria degradation of prox stools Æ gassy explosive diarrhoea) 3. Decrease in stool calibre 4. PR bleed (Mixed in stool? / Separate from stool?) 5. I/O 6. Tenesmus = constant intense desire to defaecate. may be painful. defaecation Æ nothing/ small amt of

mucus and loose faeces (due to SOL in lumen/wall of rectum) 7. Mucoid stools (suggests polypoid masses)

Consitutional: LOW, LOA, malaise Complications:

1. Perforation : Symptoms of peritonism (rigid, guarding, rebound) 2. Intestinal obstruction (4): abdominal distension, abdominal colick pain, vomiting, absolute constipation 3. Haemorrhage 4. Symptoms of fistula, e.g. faecuria/ pneumaturia/ recurrent UTI (colovesical fistula) 5. Involvement of surrounding structures: eg. Rectosigmoid CA: pain, bladder, ureter Æ hydroureter/hydronephrosis, GU

organs (but usually no S/S as uterus wall very thick) 6. Symptoms of infection: abscesses, peritonitis 7. Iron deficiency anaemia (6): fatigue, SOB, CP, palpitations, decreased ET, postural giddiness

Metastasis: bone pain/ #, LL weakness with loss of sensation, bowel and urinary continence RHC discomfort (parenchymal involvement most common), jaundice SOB (pleural effusion most common), decrease ET, orthopnoea morning headache

B) Strong-risk factors

1. Positive Family history - 1 or more 1st deg relative with CC at age <40 - 2 or more 1st/2nd deg same side relative with CC at any age - Personal or family history of breast, ovarian cancer 2. FAP history / FHx - Diagnosis: Colonoscopy - Can also do Protein truncation test for APC gene mutation 3. HNPCC: Amsterdam II Criteria – [3, 2, 2, 1] - ≥3 family members from the same side with HNPCC related cancer,

one of whom is a 1st deg relative of the other 2 (At least 2 of which must be 1st-degree relatives)

HNPCC related: Colorectal carcinoma, Endometrial carcinoma, other related cancers: small bowel, TCC of the upper urinary tract, stomach, ovarian, brain (Turcot syndrome) and

sebaceous gland adenomas or keratoacanthomas (Muir-Torre syndrome). - 2 successive generations - ≥1 of the HNPCC related cancers must occur prior to age 50 - FAP is excluded 4. History of adenomatous polyps Æ Family history alone = high-risk; FAP / HNPCC = very high-risk

Other risk factors

- Low fibre diet, Red meat - Obesity - Ulcerative colitis >10 years

Stool Changes - Bloody diarrhoea – ddx: IBD Infective e.g. amoeba, TB, hookworm,

Antibiotic Associated Colitis (C. difficile) - Haematochezia = passage of FRESH blood

per anus, usually in or with stools – ddx: Diverticulitis Angiodysplasia (AVM, common in old) Hemorrhoids Massive upper GI

It takes about 14 hours for blood to be broken down within the intestinal lumen; therefore if transit time is less than 14 hours the patient will have hematochezia, and if greater than 14 hours the patient will exhibit melena. One often-stated rule of thumb is that melena only occurs if the source of bleeding is above the ligament of Treitz.

120

SCREENING

- Screening has been shown to improve survival! - Why CRC is suitable for screening

1. Prevalent and lethal disease 2. Precursor can be detected early (long asymptomatic period) 3. Early detection makes a difference (can institute treatment) 4. Safe, effective, feasible test available

- 2 categories i. CA prevention test = detect POLYPS and cancer Æ superior to (ii) as POLYPECTOMY PROVEN TO PREVENT CANCER Colonoscopy is the only test.

ii. CA detection test = detect cancer only Faecal immunochemical testing (more sensitive than FOBT) shown to be the best test.

- Colonoscopy normal Æ Repeat in <10 year intervals

PHYSICAL EXAM 1. Abdominal mass 2. Mass on DRE: (rectal mass)

a. hard, non-tender, polypoid, irregular, contact bleeding b. distance from anal verge >7cm (internal anal sphincter)

3. Complications, Metastases - Cachexia - Pallor at nail beds and conjunctiva (anaemia) - Jaundice, hepatomegaly (irregular surface & nodular edge) - Cervical lymphadenopathy – Virchow’s node - Bone tenderness, consolidation or effusion, AMS

121

INVESTIGATIONS Aims: Diagnose, Stage the cancer & Investigate for complications of cancer I. DIAGNOSIS x Colonoscopy – first-line investigation(diagnostic & therapeutic)

- Ensure good bowel preparation - Visualise lesion: size and location of lesion - Take biopsies of the lesion - Enables detection of synchronous lesions (primary tumors occurring less than 6 months apart) – synchronous polyps in 30%,

synchronous cancer in 3-5%.Therefore, do not do a sigmoidoscopy only. - Enables therapeutic procedures e.g. polypectomy, stenting of obstructed colon

x CT colonography a.k.a virtual colonoscopy - Next best to colonoscopy: needs IV contrast, air and contrast enema - Detects >/- 1cm tumors

x Double-contrast barium enema (barium + air) - Classically can see an apple core lesion with barium enema - Not adequate for diagnostic purposes, may miss small lesions & distal lesions

- Need to insert a rigid sigmoidoscope 10-15cm to instil air & contrast II. STAGING All CRCs (a) CT scan of the abdomen and pelvis for colon tumours

- Local T staging & invasion into bladder, ureter, uterus - Staging of regional and no-regional lymph node involvement - Metastases to the liver, obvious peritoneal seeding, omental kinking, - Look for ascites, hydroureter/ hydronephrosis, intestinal obstruction

(b) CXR + CT scan of the chest (c) Bone scan if symptomatic (more for Ca breast, lung, prostate, thyroid) (d) CT brain if symptomatic Rectal tumors (below peritoneal reflection): (e) Endoscopic ultrasound / transrectal ultrasound (for rectal tumour)

- Very good for T staging to determine depth of involvement: locally advanced CA (≥T3) requires neoadjuvant chemoRT - Can also assess local lymph node status

(f) MRI of the tumour - Superior to CT for delineating fat planes in T staging especially in rectal cancer - Circumferential resection margin (CRM) refers to the fascia propria - High risk tumors for recurrence: CRM < 2mm, T3/4 (+/- bulky T2) Æ Neoadjuvant ChemoRT

III. COMPLICATIONS Basic laboratory investigations: (a) FBC for low Hb, together with iron studies (b) UECr: fluid and electrolyte abnormalities from vomiting (eg. in IO), or 3rd space losses (intraluminal); creatinine may be elevated (prerenal

failure) and hence risk of contrast nephropathy (c) LFT for derangements caused by metastasis (occur late) – raised bilirubin, ALP (first to be raised) (d) PT/PTT, GXM for surgery (e) Carcinoembryonic antigen level (CEA)

- A tumour marker for colorectal carcinoma (glycoprotein synthesised by foetal intestinal tissue) - >90% of CRC produce CEA, Raised levels: 70% CRC, 10-20% lung/ BrCA - Measured pre-operatively as a baseline level – if the CEA is raised pre-op and falls to within normal range post-op, it is likely tumour

has been totally removed. Usually, normal values 6wks post-op?

- Follow-up after surgery with CEA levels to detect tumour recurrence - Causes of false positive raised CEA: smoking, pregnancy, bronchitis, cholangitis and cancers of the stomach, lung, breast, pancreas,

cervix, bladder and kidney

Imaging (f) Erect and supine AXR to look for intestinal obstruction (usually large bowel closed loop obstruction) and caecal distension; near perforation (g) Erect CXR in perforated tumour to detect air under diaphragm (h) ECG :anaesthesia assessment

122

TNM STAGING

DUKE’S STAGING (SURGICAL AND HISTOLOGICAL FINDINGS)

Stg Description 5yr surv A Tumor confined to bowel wall with no extension into extrarectal/ extracolic tissue, no LN mets 75% B Invades past muscularis propria into extrarectal/ extracolic tissue, no LN 55% C LN mets present

C1: only nearby nodes involved (paracolic LNs) C2: continuous string of LN involved up to proximal resection (LN at base of mesentery)

C1:40% C2:20%

D Distant mets/ extensive local mets such that surgically incurable Poor unless mets are resectable

123

TREATMENT SURGERY Aim: eradicate tumor with clear margins and LN drainage Pre-operative measures

- Bowel prep Modification of diet – 3 days low residue diet (reduce frequence and volume of stools – low fibre, reduce food that increase

bowel activity), NBM day before operation Bowel clearance with polyethylene glycol. CI obstruction?

- Prophylactic antibiotics (max at first incision) ampi/ genta/ metronidazole at induction of anesthesia

Principles of surgery for colonic carcinomas - En-bloc resection of tumour with adequate margins

For colonic tumours, a margin of 5 cm proximally and distally is adequate

i While segmental resection is sufficient for primary tumour removal, a wider resection is often required to achieve sufficient lymphadenectomy

Adequate clearance of the draining lymphatics involves excision of the vascular arcades supplying the segment of involved colon back to their origin (from the SMA or IMA) as lymphatics follow the arteries generally

- Obstructed left sided carcinoma

No difference shown for doing a staged procedure (i.e. tumour removed with proximal end of colon brought out as a

colostomy) as compared to creating a primary anastomosis On-table bowel decompression ( irrigation) for clearance of faecal material Segmental colectomy for the tumour with intraoperative decompression is comparable to subtotal/total colectomy without

decompression with regard to bowel function and rates of complications - Site of anastomosis: SB more reliable blood supply so SB-LB >>> LB-LB - Site of surgery for colonic carcinoma

Marginal artery of Drummond = Artery formed by the anastomosis between the ileocolic artery, middle colic artery, right and left colic arteries and sigmoidal artery. It is a continuous vessel running along the inner perimeter of the large intestine in the mesentery as part of the vascular arcade that connects the SMA and IMA, from the ileocolic junction to the rectum. This artery is almost always present and its absence should be considered a variant.

124

Tumour Site Surgery Structures Involved (Excision of structures + Division of vessels) Caecum / Ascending colon

Right hemicolectomy

Excision of caecum + ascending colon x Ileocolic artery x Right colic artery x Right branch of the middle colic artery

Hepatic flexure / transverse colon near hepatic flexure

Extended right hemicolectomy (B)

Excision of caecum, ascending colon and proximal transverse colon x Ileocolic artery x Right colic artery x Middle colic artery (at its origin)

Mid-transverse colon Transverse colectomy (C)

Excision of transverse colon x Middle colic artery

Transverse colon near splenic flexure

Left segmental colectomy (D)

Excision of distal transverse colon and proximal descending colon x Left branch of middle colic artery x Left colic artery

Descending colon Left hemicolectomy

Excision of descending colon x Left colic artery x Left branch of middle colic artery x Inferior mesenteric vessels

Sigmoid colon Sigmoid colectomy = High anterior resection

Excision of sigmoid colon with variable length of upper rectum & distal descending colon x Inferior mesenteric vessels

125

Anterior resection = Resection of IMA at its pedicle (with parts of the colon where it supplies – Sigmoid and Rectum) Not descending colon as the LMCA can extend its supply to it. I/O cases: SB-LB anastomosis better than LB-LB anastomosis - Because SB has more abundant and predictable blood supply - Dilated LB prox to obstruction will be edematous Æ not suitable for anastomosis - Înever do a left hemicolectomy (do right hemicolectomy/extended hemicolectomy) Principles of surgery for rectal carcinomas - En-bloc resection with adequate margins

For rectal tumours a margin of 5 cm proximally and 2 cm distally is adequate (as it has been found that lymphatic spread of rectal tumours

is predominantly in the proximal direction) Radial margins are also important as there is a zone of downward spread within the mesorectum (peritoneal investment of the upper

rectum; just anterior to the sacrum)

- Sphincter-sparing versus loss of sphincter The anal sphincter can be spared if the distal margin is >1-2cm above the level of the sphincter complex, usually taken to be at the level of

the dentate line (which is 5cm above the anal verge) i.e. distal margin of the tumour must be >7cm from the anal verge Sphincter-sparing Î low anterior resection (below the peritoneal reflection); ultra low AR if just above the anal sphincter Sphincter-sacrificing surgeryÎ abdominoperineal resection (entire anus & sphincter complex is dissected, creation of an end colostomy)

- Reconstruction Formation of a straight coloanal anastomosis in anterior resections is a/w poor function due to the lack of reservoir function 1. Creation of a colonic J-pouch using the proximal end of

colon (the end of the colon is folded back on itself to form a J, and the two limbs opened and stitched together to form a pouch, the apex of the J being anastomosed to the anus) is associated with improved post-operative function

2. Coloplasty is another alternative that is equivalent to

colonic J-pouch (the distal colon is cut longitudinally but sewn transversely, widening the diameter at that segment to form a small pouch), done when there is difficulty creating the colonic J-pouch

- Mesorectal excision

Proximal rectal tumours – 5cm distal margin of mesorectal excision Mid-rectal tumours – wide mesorectal excision of at least 4cm distal to the tumour Lower rectum tumours – total mesorectal excision required (complete excision of the intact visceral mesorectal tissue to the level of the

levators)

16cm

12cm: usually where peritoneal reflection sits

8cm

4cm

Anal canal: 2-4cm

Lower 1/3

Middle 1/3

Upper 1/3

High AR: margin above peritoneal reflection

Low AR: margin below peritoneal reflection Ultra low AR: anastomosis to the upper end of the anal canal

126

- Extended resections For locally advanced, adherent tumours (T4), multivisceral resection of organs involved (pelvic exenteration) is associated with improved

local control and overall survival compared with standard resection, though high morbidity of 25-50% is associated Consider neoadjuvant chemoradiotherapy prior to surgery to downstage disease

- Stoma creation

A defunctioning loop ileostomy (or loop colostomy) is usually created during an low AR as the manipulation of the colon deep within the

pelvic cavity causes increased risk of an anastomotic leak & also poorer blood supply to anastomosis A defunctioning stoma does not protect against anastomotic leak, but mitigates against disastrous complications of faecal peritonitis

should a leak occur Closed in 2-6/12 after check with gastrograffin reveals no leak

- Neoadjuvant chemoradiotherapy for rectal tumours

Neoadjuvant RT with 5-fluorouracil can downstage tumour significantly Æ ability to preserve sphincter, ability to resect previously

unresectable tumour, reduce local recurrence Good evidence for ≥T3 tumours, CRM<2mm: benefits > risk of RT (esp. that pt becomes too ill to be operated on) Not possible for colon carcinomas due to risk of small bowel radiation if given above peritoneal reflection Done before Sx as ChemoRT requires blood supply to work (disrupted during Sx)

- Surgical treatment according to stage

Stage of disease Treatment T1 Involvement of submucosa, but no penetration

through muscularis propria Local excision (AR or APR)

T2 Invasion into, but not penetration through, muscularis propria

a) Local excision + adjuvant Chemo/RT OR

b) radical resection T3 Penetration through muscularis propria into

subserosa (if present), or pericolic fat, but not into peritoneal cavity or other organs

Neoadjuvant chemo / RT before radical resection

T4 Invasion of other organs or involvement of free peritoneal cavity

Operative complications

Immediate (<24h) Damage to other organs e.g. ureters

Early (<30 days) Wound infection Bleeding Abscess Anastomosis breakdown/leak Î faecal peritonitis Early stoma complications

Late (>30 days) Diarrhoea Impotence (damage of pelvic nerves) Adhesions (I/O) Anastomotic stricture Late stoma complications

Surgery for palliation - Resection of primary for palliation of symptoms such as bleeding, perforation, obstruction or pain - Resection of asymptomatic primary is controversial, but may confer survival benefit in a select group of patients where metastatic tumour

burden is restricted to one side? and liver involvement is not extensive Surgery for metastases - Isolated liver metastases (synchronous or metachronous) may be resected with survival benefit; neoadjuvant chemotherapy can be given to

downstage the metastases if they are initially resectable - Lung metastases usually indicate systemic dissemination of disease, but in the rare setting that there is an isolated lung secondary, resection

can provide survival benefit Surgery for recurrence - Loco-regional recurrence, if detected early with adequate resection, can confer survival benefit

127

RADIOTHERAPY - Role as neoadjuvant therapy in rectal cancer to downstage tumour (in T3/T4) - Post-operative adjuvant therapy in stage II or III rectal cancer CHEMOTHERAPY Adjuvant therapy - Colon cancer: 5-FU + folinic acid (leucovorin) for 6 months, or 5-FU + levamisole for 12 months in Duke’s C cancer (node

positive);; not recommended in Duke’s B or less - Rectal cancer: post-operative adjuvant therapy in combination with radiotherapy in stage II or III disease (5-FU based regimen

used) Palliative therapy - 5-FU in combination with folinic acid is first-line therapy - Alternatives for first-line therapy: Raltitrexed when 5-FU is not tolerated; capecitabine or UFT (uracil combined with tegafur)

plus folinic acid FOLLOW UP - Follow-up visits 3-monthly for the first 2 years, then 6-monthly for the next three years, and subsequently yearly, measure CEA

at each visit - Yearly colonoscopy - CXR and liver ultrasound to detect metastases (recommended frequency not known)

128

ASSOCIATED CONDITIONS I. Familial adenomatous polyposis (FAP) - 1 in 10,000, autosomal dominant inheritance - Germline mutation of APC gene on 5q21 - >100 adenomatous polyps all over colon; polyps take 5-6 yrs to turn malignant - 50% patients will have polyps by 16yrs; 90% will have colorectal CA by 45yrs - Other sites for polyps: stomach, duodenum - Extraintestinal manifestations Skin: Epidermoid cysts, Lipoma Bone: Osteoma of skull/ mandible, Dental abnormalities Eye: Congenital hypertrophy of retinal pigment epithelium (CHRPE) Other tumours:

Desmoid tumours (intra-abdominal tumours, treated with CT, RT or HT.) Thyroid cancer (follicular or papillary type) Periampullary CA

- Diagnosis Colonoscopy showing >100 polyps Genetic testing

- Surveillance Yearly colonoscopy for at-risk family members from 12y onwards 5 yearly OGD for surveillance of Periampullary Cancer. Genetic testing of at-risk family members Affected members should undergo prophylactic proctocolectomy with ileal pouch anal anastomosis (IPAA – involves folding

loops of ileum back on themselves and stitching or stapling them together to form a reservoir pouch which is them anastomosed to the anus) at ~ 20 YO

Subtotal colectomy is an option if the rectum is relatively spared of polyps

II. Hereditary Non-Polyposis Colorectal CA (HNPCC) - Divided into Lynch syndrome I or Lynch syndrome II based on clinical features - Tumours usually proximal to splenic flexure (~70% proximal to splenic flexure) - Tumours tend to arise from polyps which are commonly flat, with villous histology - Resultant tumour is often poorly differentiated - Lynch syndrome type II is associated with increased risk of cancer elsewhere, most commonly endometrial cancer, and also

ovarian, gastric, small bowel, hepatobiliary, and renal pelvis/ureter cancers o Offer a THBSO with total colectomy if CRC detected

- Diagnosis is based on the Amsterdam criteria – see above - Surveillance – 1-3yrly colonoscopy starting at 20 years old III. Ulcerative colitis - Screening – yearly colonoscopy starting after 10 years of UC

129

3. STOMAS

Stoma: opening of a luminal organ into the external environment Indications 1. For input: feeding (Percutaneous endoscopic gastrostomy) 2. For output: decompression/ lavage, defunctioning/ diversion, draining/ exteriorization (urine, faeces) Nursing intervention - Stoma nurse to perform counselling & discuss best site for stoma placement Stoma siting - Over the rectus sheath Î decreases the risk of prolapse, - Away from the surgical incision Î risk of wound contamination and infection - Away from skin creases or bony prominencesÎ stoma wafer can be flush with the skin (any gaps between skin and wafer Æ

leakage of fluidÎ skin excoriation & infx) - Away from old surgical scars Î risk of hernia - Sited for easy accessibilty i.e. not under a large fold of abdominal fat - Intra-operatively, avoid tension over the stoma to marked site Î causes È vascularity of the stoma Î stoma necrosis Types of stomas Permanent (end colostomy) - When patient or surgeon factors are against reversal of stoma (relative) - When no distal bowel remaining (absolute) After abdomino-perineal resection for Low rectal/ anal tumor After Panproctocolectomy without ileal pouch anal anastomosis

Temporary - Decompression – relief of bowel obstruction causing proximal dilatation - Defunctioning – to reduce effects of anastomotic leak Esp. after low anterior resection, where risk of anastomotic leakage is high 2o to poor blood supply to anastomotic site Usually loop ileostomies or colostomies with 2 openings (ileostomies usually on the right side, colostomies in the

epigastric/hypochondriac [transverse colostomy] or left side) - To rest an inflamed distal portion e.g. acute Crohn’s Colostomy (vs ileostomy)

x ?usually flushed with skin, (vs protrudes 3cm ‘spout’, as ileal contents are corrosive, to prevent contact with skin) x firm brown faceal output (vs watery greenish ilieal output) x larger diameter of stoma (vs smaller diameter in ileostomy)

Stoma Complications Early - Necrosis of terminal bowel (stoma appears dusky (grey Æ black); check by intubating with a glass tube into the stoma to look

at colour of mucosa) o refashion stoma - Obstruction (fecal impaction o explore with finger, enema / secondary to adhesion – more in ileostomy) - Leakage Î skin erosion, parastomal infection o resite - Bleeding - Stoma diarrhoea (high output) Æ correct water & electrolyte imbalance (hypoK), add anti-motility agent to thicken output

(loperamide +/- codeine) http://www.wjgnet.com/1007-9327/7/741.asp

Late - Prolapse of bowel o refashion/refresh - Parastomal hernia (+ve cough impulse)o refashion - Stenosis (unable to pass finger through)o refashion - Retraction o refashion - Fistulae - Skin excoriation - Psychological problems

130

End colostomy

This procedure is most commonly performed to manage carcinoma of the lower rectum or anus, diverticular disease, and rare cases of faecal incontinence that do not respond to medical mx. For example, a very low rectal cancer will require resection of the rectum and anus (abdominoperineal excision of rectum). The remaining descending and sigmoid colon is mobilised and the cut end brought to the abdominal surface at an opening about 2 cm across. This is usually sited in the left iliac fossa.

Abdominoperineal excision of the rectum

If the anus, rectum, and a portion of the lower colon have not been removed, as in Hartmann's procedure, two outcomes are possible. In the first, the distal, non-functioning part of the colon and the rectum can be stapled or sewn closed and left inside the abdomen as a rectal stump. The proximal colon is then taken out as an end colostomy. Because the rectum has not been removed, the urge to have a bowel movement may occur. Mucus and some old stool, if present, will be passed. If the colostomy is temporary, a second operation is needed to reconnect the two ends.

Hartmann's procedure

Less commonly, two separate stomas may be created. One stoma is the exit of the functioning part of the colon through which stool and gas pass. The second stoma opens into the non-functioning portion of the colon and rectum and is called a mucous fistula. The second stoma is usually small, flat, pink-red in colour, and moist, and it produces only mucus.

Loop colostomy

A loop colostomy was traditionally created to defunction an inflamed sigmoid in diverticular disease or to defunction a distal anastomosis.It has largely been replaced by loop ileostomy. A loop of colon is brought to the surface of the body and may be supported on a rod, which is removed after about five days. The bowel wall is partially cut to produce two openings—of an afferent limb and an efferent limb. The opening of the afferent limb leads to the functioning part of the colon, through which stool and gas pass out. The opening of the efferent limb leads into the non-functioning part of the colon. The stoma site was usually high on the abdomen above the waistline because the transverse colon was commonly used. Currently, loop colostomies are more often fashioned from the sigmoid colon to defunction the rectum (for example, in cancer) or anus (for example, in incontinence). A loop colostomy may be temporary or permanent.

End ileostomy

When the entire colon, rectum, and anus must be removed (panproctocolectomy) an end ileostomy must be employed. This occurs most commonly in severe ulcerative colitis but also in familial polyposis and some cases of colorectal cancer (for example, hereditary non-polyposis colorectal cancer). The ileum is resected just short of its junction with the caecum, and 6-7 cm of the small bowel is brought through the abdominal wall, usually in the right iliac fossa. It is everted to form a spout and then sutured to the bowel wall to protect the skin from the irritating content of the ileal fluid. After a panproctocolectomy the ileostomy is permanent. Temporary end ileostomy is often used after an emergency subtotal colectomy, which leaves part of the sigmoid colon and rectum left in place; for acute ulcerative colitis; acute ischaemic bowel; or neoplastic obstruction of the sigmoid .

Loop ileostomy This type of stoma allows for defunctioning of an obstructed colon (in cancer), defunctioning of a distal anastomosis (after resection and primary anastomosis either as an emergency or after radiotherapy), or defunctioning of the anus (in incontinence or perineal involvement in Crohn's disease). Loop ileostomy has largely replaced loop colostomy because it is easier to site, less bulky, and easier to surgically close. A loop ileostomy has two openings, and most are temporary.

131

End-loop ileostomy

This less commonly performed procedure is used when an end ileostomy cannot be fashioned safely because the patient is obese or because of unfavourable mesenteric anatomy. The formation of this stoma is similar to a loop ileostomy, but the efferent limb is short and blind ended. On inspection at the bedside this type of stoma is indistiguishable from a loop ileostomy.

Double barrel stoma When the caecum is removed, the surgeon might create a double barrel stoma. In essence, this is an end ileostomy (small bowel) and a mucous fistula (the remaining colon) sited beside each other. On examination this will look almost identical to a loop ileostomy, however, closer inspection will show two separate stomas.

Urostomy

This is a general term for the surgical diversion of the urinary tract. The main reasons for a urostomy are cancer of the bladder, neuropathic bladder, and resistant urinary incontinence. The bladder is usually removed, but this may depend on the underlying condition. Formation of an ileal conduit is the most common procedure, which constitutes isolation of a segment of ileum. One end of the ileum is closed and the two ureters are anastomosed to it. Finally, the open end of ileum is brought out onto the skin as an everted spout and will look similar to an end ileostomy. Urine drains almost constantly from the kidneys through the ureters and ileal conduit into a bag.

Stoma bags Stoma bags are of two main types. x Single piece systems stick straight on to the patient's skin.

x Two piece systems have a separate base (a flange) that sticks to the skin, and the bag attaches to this. This enables the bag to be changed wo removing the flange.

Some bags have a second opening at the bottom to allow emptying. These are most useful in the period immediately after operation and in patients who have had ileostomy, who need to drain their bag regularly. Closed bags are used when the faeces are well formed and are usually only changed once or twice a day. Most patients with a stoma will use an opaque bag, but in the period immediately after operation a transparent bag is used to observe the new stoma for complications such as persistent oedema or necrosis. Modern stoma bags are fitted with a carbon or charcoal flatus filter that allows gas to escape to prevent the bag from ballooning or detaching and neutralises odour. Complications Functional problems, such as skin excoriation and stoma noises, are the most common complications and are usually managed by the stoma nurse. Patients with stoma admitted to hospital with increased or decreased output should be appropriately managed to exclude any abdominal emergency, with particular emphasis on careful history taking to establish the normal bowel pattern, and attention to fluid balance. Most structural problems, such as stoma prolapse, retraction, and parastomal hernia formation can be managed conservatively with modified bags and specialised belts. Only about 10% of patients with these complications will require further surgery. Patients should be alert to any change in colour of their stoma. Stomal oedema is normal for several days after surgery, but if the mucosa becomes dusky or necrotic the surgeon should be contacted promptly.

132

Question Answer Stoma

Where is the stoma? Left iliac fossa Most likely a colostomy

Right iliac fossa Most likely an ileostomy

How does the bowel lie in relation to the external skin?

Flush with skin Most likely a colostomy

Raised spout Ileostomy; less commonly a urostomy

How many lumens are present? One End colostomy; end ileostomy; urostomy

Two (adjacent)—efferent limb may be difficult to see

Loop colostomy; loop ileostomy; end-loop ileostomy

Two (separate stomas) Most likely end colostomy with a mucous fistula; double barrel stoma; rarely bowel stoma and urostomy

What are the contents of the stoma bag (don't be afraid to feel it)?

Fully formed stool Colostomy

Semisolid or liquid stool Most likely ileostomy; colostomy

Urine Urostomy

Mucus Mucous fistula

Examining a patient with an abdominal stoma and bag

133

4. DIVERTICULAR DISEASE

PATHOLOGY – acquired herniation of colonic mucosa through muscular wall, with a covering of colonic serosa TERMS - Diverticulosis coli – presence of acquired pseudodiverticula - Diverticular disease – symptomatic diverticulosis coli - Diverticulitis – inflammation of diverticula EPIDEMIOLOGY - Increases with age; up to 25% in >70YO (2-5% are <40YO, more in obese M) - Majority are asymptomatic; 10-30% are symptomatic - Risk factors – dietary fibre & genetics - Site

o majority are in the sigmoid colon, right sided are thought to be genetic; o Asians: 40% right, 60% left, Caucasians: 20% right, 80% left o not in rectum as taeni coli has fused

PATHOGENESIS 1. Increased intraluminal pressure

- Associated with lack of dietary fibre 2. Degenerative changes in colonic wall

- Usually at point of entry of terminal arterial branches where serosa is weakest - Associated with weakening of collagen structure with age

PRESENTATIONS - depends on location of the affected diverticulum, the severity of the inflammatory process, and presence of complications 1. Acute diverticulitis

- Symptoms: LLQ pain, N/V, Constipation / diarrhoea, flatulence/bloating - Signs: Low grade fever, Tender palpable mass - Investigation: n WBC

2. Chronic diverticulitis

- Recurrent LIF pain - Irregular bowel habit - Passage of mucus PR

3. Complicated diverticulitis (dangerous)

a. Perforation b. Paracolic abscess / inflammatory mass – 2o to localized perforation c. Bowel obstruction – 2o to stricture or adherence to a diverticular mass d. LGIT haemorrhage – ulcerated vessel @ neck of diverticulum; torrential e. Fistula formation (commonest: colovesical fistula) – 2o to pericolic abscess discharging, operation or drainage of pericolic abscess. May

present with urinary symptoms. Others – colo-cutaneous, colo-uterine, colo-enteric, colo-vaginal Elderly and patients on steroids may have no sign even in severe diverticulitis. There is an increased rate of free perforation (43% vs. 14% in immunocompetent patients), increased need for surgery (58% vs. 33%), and increased postoperative mortality (39% vs. 2%). STAGING - Hinchey classification of complicated acute diverticulitis – need for surgery is reflected by degree of infective complications

Stage 1 Pericolonic / Mesenteric abscess (small)

- ABx, NBM, IV fluids - Consider 1 stage surgery after acute episode – resection of affected bowel

segment with primary anastomosis Stage 2 Pelvic / retroperitoneal

abscess (large) - Percutaneous drainage - Elective 1 stage surgery

Stage 3 Purulent peritonitis (fm perf diverticulitis)

- 2 stage operation – Hartmann’s procedure (partial colectomy + diverting end colostomy & rectal stump formation) + secondary re-anastomosis 3 months later

Stage 4 Faecal peritonitis (fm ruptured diverticula)

Severity staging by CT scanning may allow not only the selection of patients most likely to respond to conservative treatment, but may also predict the risk of failure of medical therapy and of secondary complications after initial conservative treatment. Note: current controversy of management for stage 3: hartmann’s vs segmental resection with primary anastomosis with or without defunctioning ileostomy In the absence of complications and systemic signs and symptoms, patients with mild abdominal tenderness may be treated conservatively. Conservative treatment typically includes dietary modification and oral or IV antibiotics. This has been shown to be successful in 70% to 100% of patients.

134

Presentation Clinical features Investigations Differentials Management Acute Diverticulitis - LIF pain – colicky,

progressing to constant, relieved by defecation

- LIF tenderness - Palpable LIF mass

- Nausea - Urinary symptoms

- Pyrexia - Increase pulse rate

- FBC – leucocytosis, ↑ ESR - Erect CXR to rule out perforation - AXR – ileus, air-fluid level w/in an

abscess - CT scan w triple contrast (choice)

Severity, complication, clinical staging

Water soluble contrast: IV for vascular lesions, oral for small bowels, enema for large bowels

Features – diverticula elsewhere, confirm colitis (mesenteric fat infiltration, concentric bowel thickening) but only suggest diverticulitis, pelvic abscess, free gas, extravasated contrast

Cannot tell if inflm is due to diverticula

Better than intraluminal examinations as bulk of inflm is extraluminal

- Laparoscopy – if diagnosis is in doubt - Barium enema – CI as barium may

leak out into abdo cavity - Avoid colonoscopy as risk of

perforation is high (by air insufflations or instruments) + worsens diverticulitis

Can dev anywhere in GIT. See Ddx to RIF/LIF pain - GI: colitis, GE, IBS, IBD,

mesenteric ischaemia/ adenitis, Ca colorectal

- Right colon/redundant (extra bend in descending colon) sigmoid colon: appendicitis

- Transverse colon: PUD, pancreatitis, cholecystitis

- Inflm adj to UT: UTI, stones, cancer, cyst/ Angiomyolipoma, hydronephrosis

- Lower quad pain in F (Obgyn): PID, torsion of cyst/ ovary, endometriosis, ectopic pregnancy

- Retroperitoneal perf (leg pain): thigh abscess, leg emphysema

- #, infx, inflm,

Conservative - Bed rest - NBM, IV fluid - Broad-spectrum antibiotics –

augmentin or metronidazole or ciprofloxacin

- Antispasmodics After acute phase settled (4-6 wks) - Colonoscopy – confirm dx &

exclude CA colon and/ or - Barium enema is inferior to

colonoscopy in terms of image quality and is usually only performed if the patient has strictures or an excessively tortuous sigmoid colon where colonoscopy is difficult or dangerous

Role of surgery: see below

Chronic Diverticulitis

Not a common clinical entity

- Recurrent LIF pain - Irregular bowel habits –

constipation & bouts of diarrhoea

- Passage of mucus PR - Ruled out cancer, IBD,

ureteric colic, Msk pain etc.

- Rigid sigmoidoscopy – oedematous mucosa & rigidity of rectosigmoid jx

- Flexible sigmoidoscopy – diverticular orifices

- Barium enema – ‘saw-tooth’ appearance, diverticula, strictures

- Colonoscopy – exclude differentials (i.e. Ca colon)

- CA colon – may coexist. Hard to differentiate – therefore, ALWAYS exclude CA colon e.g. histology after bowel resection

- Ischaemic colitis - Radiation colitis - Colonic endometriosis

Conservative – see above Surgical Indications: - Severe / recurrent attacks - Possible CA colon Stage: Segmental resection of affected colon + anastomosis

Generalised peritonitis / perforation

- Acute onset abdominal pain – severe & continuous

- Abdominal guarding & rigidity

- Vomiting - Tachycardia - Pyrexia

- FBC – ↑ TW, ↑Hb (dehydration) - U/E/Cr – dehydration & ARF - CXR – free gas under diaphragm

- Other causes of peritonitis – perforated PUD/ appendicitis/ bowel/ ectopic pregnancy, ishaemic bowel, acute pancreatitis, ruptured AA/ hepatoma, torsion of testis/ ovary, pyonephrosis

Mgmt as for acute abdomen - Resuscitate - Surgical Peritoneal toilet Hartmann’s procedure

(Resection of affected segment + End sigmoid colostomy)

Pericolic abscess - May follow acute diverticulitis

- LIF tenderness & guarding - LIF mass – may be

detected on DRE - Swinging fever

- FBC – ↑ TW - CT – differentiate between

inflammatory phlegmon (spreading diffuse inflammation with pus/purulent exudates) & pericolic abscess

- CT/ US guided percutaneous aspiration

- Surgery – evacuation of pus ± resection of affected segment

Persistent inflammatory mass

- LIF pain, tenderness & palpable mass

- Fever - Malaise

Small bowel I/O - Usually temporary, due to attachment of enteric loop against area of acute diverticulitis

- Surgery if does not resolve Large bowel I/O - PHx of recurrent acute

diverticulitis or irregular bowel habit

- Colicky abdominal pain, constipation & abdo distension

- AXR – dilated bowels prox to stenosis - Water soluble contrast enema

- CA colon - NBM, Drip & suck - Surgery – Resection ± primary

anastomosis

Hemorrhage - Usually in the elderly who have higher density of sigmoid diverticula

- Massive bleed (altered blood ± clots; not melena) usually right-sided

- Colicky pain as blood is irritative & causes spasm

- Invx as for LGIT bleed – resus, investigations + colonoscopy & angiography (both diagnostic AND therapeutic value)

- ± on-table enteroscopy if required - ± tagged RBC scan (not as sensitive

compared to angiogram): RBC tagged with radioisotope

- Anorectal bleed - Angiodysplasia - Ischaemic colitis - Colorectal CA - Colitis (inflm or infx) - UBGIT - Coagulopathy

- Resuscitate & correct coagulopathy

- Colonoscopic management: adrenaline injection, endoclips on bleeding vessel, heat coagulation

- Radiologic embolisation of site of bleeding with temp foam material via angiography

- Surgery – segmental resection; total colectomy if unable to localise bleed

Vesicocolic fistula - PHx of chronic diverticulitis & UTI

- Hx of dysuria, freq, haematuria, pneumaturia, faecaluria

- UFEME & urine c/s: confirm UTI and organisms (polymicrobial as opposed to sterile pyuria in ‘UTI from adj diverticulitis’)

- Cystoscopy – cystitis - KUB – air in bladder - Barium enema – È diverticular bowel

segment - Sigmoidoscopy – usually normal

- Other causes of fistula – CA colon, CA bladder, Crohn’s disease, post-irradiation necrosis

- Surgery – Resection of affected colon + anastomosis + closure of bladder fistula opening

135

Outcomes: well or deteriorate requiring surgery, recurrent episodes, stricture & subacute IO (offer surgery) Indications for emergency operation

1. Sepsis from abscess or faecal peritonitis 2. Perforation 3. Diverticulitis not responding to conservative management 4. Obstruction with pending perforation – need to rule out cancer at the same time 5. Emergency bleed (controversialÎ clamping both side & look for active bleed into

segmentÎ segmental resection) a. Haemodynamically unstable with failure of embolization b. Need > 4 units of PCT c. Previous bleed

Indications for elective operation

1. Stricture 2. Fistula 3. Recurrent attacks – occurs in 30% of patients after 1st episode. a/w higher mortality &

complication rates 4. Young patientss <40YO – high recurrence rates 5. Immunocompromised patients (e.g. renal transplant) – may not show S/S of acute attack or

complications

Advice to patients: - 70% of patients will not have recurrence after first attack (after first attack, 1/3 will have second. out of whom 1/3 will have 3rd) - Advise high fibre diet (prevents recurrence in >70%) & to drink lots of fluid, weight reduction and exercise

Recurrent diverticulitis after surgical treatment - incidence ranging from 1% to 10%. - In general, the progression of diverticular disease in the remaining colon is approximately 15%. - Important factors to be considered in terms of surgery are the adequacy of resection, meaning the degree of proximal resection and level of distal anastomosis. The use of the rectum as the distal margin decreases the rate of recurrence. - Care also must be taken to exclude other components of differential diagnosis, especially irritable bowel syndrome, inflammatory bowel disease, and ischemic colitis.

136

5. INFLAMMATORY BOWEL DISEASE (CROHN’S DISEASE, ULCERATIVE COLITIS)

Crohn’s Ulcerative colitis

Bowel involvement

Can affect entire GIT anywhere from mouth to anus, but usually affecting the terminal ileum

Usually begins in the rectum and can extend proximally to affect entire colon

Location x 40% terminal ileum x 30% small intestine x 30% colon x 3% anorectal (usu. spared)

x 40% rectum alone x 40% left colon x 20% total colitis x Backwash ileitis (10%) – 30cm of ileum affected

due to incompetent ileocecal valve causing reflux of noxious inflammatory mediators

Continuity Not continuous, with skip lesions Longitudinal mucosal continuity

Complications Strictures, fistulae, sinuses, malnutrition (SB involve), SB obstruction, toxic dilatation, CRC

These complications absent. Massive PR bleed, toxic megacolon, venous thrombosis, CRC ++

Histopathology Macroscopic: bowel is thick-walled & nodular (cobblestone appearance) with creeping fat, mesenteric thickening & deep linear ulcers Microscopic: transmural involvement, non-caseating granulomas (pathognomonic, but only present in 2/3)

Macroscopic: granular appearance of mucosa , loss of vascular markings, pseudopolyps interspersed with areas of shallow ulceration Microscopic: only mucosal & submucosal involvement, ulcers, crypt abscesses - Muscularis propria & serosa may be affected in fulminant disease

Risk of carcinoma

Slight increased risk of colorectal Ca, increased risk of small bowel lymphoma

Substantially higher risk of CRC x 2% at 10 years x 8% at 20 years (15% if pan colitis) x 18% at 30 years

Much higher risk with concomitant PSC

Associated Ab ASCA: anti-saccharomyces cerevisiae antibodies p-ANCA: perinuclear antineutrophil cytoplastic ab

Severity Harvey Bradshaw severity index Modified Truelove & Witts severity index (Mild, moderate, severe, fulminant)

Epidemiology

x Bimodal distribution: affects young in the 2nd & 3rd decades of life, with second onset in the 5th & 6th decades of life

x Equal gender predominance x Higher prevalence amongst Ashkenazi Jews & in cooler

climates e.g. Scandinavia, UK, Germany, northern USA x Genetic association

History x GIT: abdominal pain, diarrhea, N/V, bloating, distension,

bloody stools with mucous & pus x Crohn’s fistula: colovesical fistula or coloovarian fistula:

faeces in urine/ pneumaturia, faeces per vaginal/ PID x Non-specific systemic: LOW, LOA, fever, fatigue, symptoms

of anemia, chronic malnutrition x Specific extra-intestinal manifestations

Physical examination: usually normal +/- extra-intestinal manifestations Acute severe: fever, tachycardia, tender/distended abdomen. Nutritional deficits. Extraintestinal: clubbing, oral ulcers, erythema nodosum, pyoderma gangrenosum, conjunctivitis/episcleritis/iritis, arthritis/sacroilitis/AS, fatty liver.

Differential diagnoses 1) Gastroenteritis: exclude with stool microscopy & culture + C.diff toxin

- Bacterial: E. coli, Campylobacter, Salmonella, Shigella, C. difficile - Parasitic: E. histolytica - Viral: rotavirus

2) Other infective causes: diverticulitis, colorectal TB 3) Ischaemic colitis 4) Bleeding diverticulosis 5) Colorectal carcinoma

137

CROHN’S DISEASE P/E: RIF mass, perianal enlarged skin tags/ fistula/ abscesses, anal stricture Investigations: supportive (see UC) & diagnostic Diagnostic (mainly at terminal ileum)

x Contrast radiographic studies: assess location & extent of disease, look for strictures & fistulae o Barium studies: small bowel series & enema (cobblestone) o CT scan with oral & IV contrast

x Endoscopy: look for typical features o Colonoscopy with tissue biopsy (non-caseating granulomas) o OGD: upper GIT involvement o Endoanal U/S (EUS): identify fistula tracts

Management x Medical (principle treatment)

o Nutritional support e.g. TPN (may also aid closure of fistulae) o Pharmacological

Corticosteroids for acute exacerbation of disease, but not for long term use 1st line: 5-ASA or 5-aminosalicylic acid (sulfasalazine, mesalazine) induce & maintain remission of disease:

oral, rectal suppository or enema (up to splenic flexure) 2nd line: immunosuppressive agents e.g. azathioprine, 6-MP, MTX, Tacrolimus & cyclosporine when 1st line

agents ineffective o Cyclosporin and Tacrolimus are nephrotoxic Æ avoid LT

Biologics = created by biologic processes, rather than being chemically synthesized. - Ind: for steroid resistant - Remicade (infliximab) = TNF blocker - ACT 1 and ACT 2 (Acute ulcerative Colitis Treatment) trials showed that 44-45% of patients treated

with infliximab for a year maintained a response to the medication, cf 21% on placebo - Pretreatment: rule out TB, hepatitis first (as tx can cause reactivation) - Require close surveillance for SE (blood disorders, infections, lymphoma & solid tissue cancers,

hepatotoxicity, drug-induced lupus, demyelinating disorders) x Surgical

o Principles Avoid surgery until absolutely necessary (80% require surgery within 20 years of onset) & when indicated

perform bowel preserving surgery as repeated bowel resections can lead to short gut syndrome o Indications :

o Procedure (laparoscopic or open) CT abdomen for pre-operative percutaneous drainage of abscesses (↓ inflammation & risk of sepsis) Small bowel: (bowel preservation is key)

- Short segment disease: stricturoplasty - Long segment disease: long stricturoplasty or resection - Fistula: resect diseased bowel & repair involved organ

Large bowel: - Total colectomy + ileorectal anastamosis (if rectum spared) or proctocolectomy + end ileostomy (if

rectum affected) - Segmental colectomy only in selected cases as high recurrence rate

Perianal disease: - Setons, fistulotomy, proctectomy in severe disease

Disease refractory to medical therapy (common) Serious complications of medical therapy Severe bleeding, perforation Intestinal obstruction due to strictures

Fistulae Abscesses Toxic megacolon Malignancy

138

ULCERATIVE COLITIS Extra-intestinal manifestations (20% of patients)

x Joints (most common) o Transient asymmetrical polyarthritis: mirrors course of colitis & is cured by colectomy o Ankylosing spondylitis o Isolated sacroiliitis

x Liver o PSC (5%): fibrous structuring of entire biliary tree: ↑ ALP, MRCP, ERCP, liver biopsy. Tx: stenting, transplant o Hepatic failure over 5-10 years independent of course of colitis o ↑ risk of cholangiocarcinoma & ↑↑↑ risk of colorectal carcinoma

x Eye o Iritis, episcleritis, anterior uvetitis. Tx: topical and/or oral corticosteroids

x Skin o Pyoderma gangrenosum: deep ulceration with violaceous border that overhangs ulcer bed, usually affecting LL o Erythema nodosum: poorly defined, tender, erythematous nodules tt do not ulcerate/ suppurate, usu on the shin o Tx: corticosteroids, will improve slowly following colectomy

Investigations Supportive (looking for complications & assessing severity of disease)

x Blood tests o FBC: anemia, leukocytosis & thrombocytosis indicate more severe disease o UECr: hypokalemia & dehydration in prolonged diarrhoea o LFT: hypoalbuminemia due to poor nutritional intake o CRP, ESR: markers of severity o Autoantibody assay: p-ANCA ↑ in UC, ASCA ↑ in CD

x Radiological o AXR to evaluate colonic caliber (>6 cm is abnormal) o CXR to rule out perforation (risk of perforation in acute disease)

Diagnostic (mainly at rectum) x Endoscopy: look for typical features

o Flexible sigmoidoscopy with tissue biopsy: bleeding may occur with contact with scope. Never do barium enema during a severe acute attack or as a diagnostic test?? Management x Medical: similar to Crohn’s disease

o Mild: PO pred + Mesalazine. If distal dz: Pred retention enema/ steroid foam. If no improve after 2/52 Æ mod.

o Mod: PO pred (40mg/d x1/52 Æ 30mg/d x 1/52 Æ 20mg/d x 4/52) + 5-ASA + steroid enema BD. If no improve after 2/52 Æ severe.

o Severe: IV Hydrocort400mg/d + rectal steroid. If improve in 5/7 Æ PO pred + 5-ASA. If CRP >45/ >6 stools per day Æ ciclosporin/infliximab/Sx.

x Surgical (25% eventually require surgery) o Indications :

1. Disease refractory to medical therapy with severe & extensive colitis (most common) 2. Serious complications of medical therapy 3. Severe bleeding 4. Perforation 5. Toxic megacolon (colon > 6cm): most common cause of death - Involvement of the muscularis propria in the most

severe cases can lead to damage to the nerve plexus, resulting in colonic dysmotility, dilation, and eventual infarction and gangrene

6. Malignancy o Procedure (laparoscopic or open)

Acute setting (uncontrolled bleeding, perf, toxic megacolon, fulminant attack): total colectomy with end ileostomy: diseased rectum left in-situ with resection & IPAA at later date when patient has regained health & steroids have been withdrawn (as rectum is extraperitoneum organ and dissection/resection takes a long time). Foley catheter used to decompress rectum for 3-4 days

IPAA (ileo-pouch anal anastamosis): standard of care for patients with UC who ultimately require colectomy. Avoid necessity for long term stoma. Crohn’s disease remains an absolute contraindication as overall failure rates approach 50%

Alternative: Total proctocolectomy with end ileostomy o Complications:

o Mortality 2-7%, 50% if perforation o Pouchitis: Tx with Abx (metronidazole + ciproflox X 2/52) + immunosupp.

139

8. ANAL & PERIANAL DISORDERS 1. HAEMORRHOIDS

Anal cushions.They are clusters of vascular tissue (eg, arterioles, venules, arteriolar-venular connections), smooth muscle (eg, Treitz muscle), and connective tissue lined by the normal epithelium of the anal canal. External hemorrhoids develop from ectoderm Æ covered by squamous epithelium Internal hemorrhoids develop from endoderm Æ columnar epithelium of anal mucosa. External hemorrhoids are innervated by cutaneous nerves that supply the perianal area. These nerves include the pudendal nerve and the sacral plexus. Internal hemorrhoids are not supplied by somatic sensory nerves and therefore cannot cause pain - internal hemorrhoids can produce perianal pain by prolapsing and causing spasm of the sphincter complex around the hemorrhoids. Internal hemorrhoids can also cause acute pain when incarcerated and strangulated

Internal hemorrhoids drain through the superior rectal vein into the portal system. External hemorrhoids drain through the inferior rectal vein into the inferior vena cava. Rich anastomoses exist between these 2 and the middle rectal vein, connecting the portal and systemic circulations. Causes:

1. Decreased venous return / increase intra abd pressure: preg, constipation (low fibre diet) Æ straining 2. Portal hypertension and anorectal varices 3. Increase rectal vein pressure: obesity, prolonged sitting

The most common presentation of hemorrhoids is rectal bleeding, pain, pruritus, or prolapse. Painless fresh PR bleeding after defecation: coating / dripping, not mixed with stools Most symptoms arise from enlarged internal hemorrhoids. x Abnormal swelling of the anal cushions causes dilatation and engorgement of the arteriovenous plexuses. This leads to

stretching of the suspensory muscles and eventual prolapse of rectal tissue through the anal canal. The engorged anal mucosa is easily traumatized, leading to rectal bleeding that is typically bright red due to high blood oxygen content within the arteriovenous anastomoses.

x Prolapse leads to soiling and mucus discharge (triggering pruritus) and predisposes to incarceration and strangulation. External hemorrhoids cause symptoms in 2 ways. x Acute thrombosis: usually related to a specific event, such as physical exertion, straining with constipation, a bout of diarrhea,

or a change in diet. Pain results from rapid distention of innervated skin by the clot and surrounding edema. The pain lasts 7-14 days and resolves with resolution of the thrombosis

x Occasionally erode the overlying skin and cause bleeding

x External hemorrhoids: not true hemorrhoids, but rather painful thrombosed veins arising distal to the dentate line x Internal hemorrhoids: those arising proximal to the dentate line, usually painless unless prolapsed & strangulated

Complications: thrombosis, infection, prolapse

140

Classification & Treatment Grade 1 Hemorrhoid protrudes into the anal canal but does not prolapse outside the anus, hyperaemia of mucosa

Conservative: x Lifestyle modifications x Meds (Daflon) – improves venous tone

Grade 2 Hemorrhoid protrudes through the anus during straining or evacuation but returns spontaneously

Surgery: x Rubber band ligation x Injection sclerotherapy (phenol emoilent oil)

Grade 3 Hemorrhoid protrudes through the anus during straining or evacuation but needs to be manually returned to position

Surgery: Haemorrhoidectomy x Staple x Excision

Grade 4 Hemorrhoid remains prolapsed outside the anus

141

2. ANAL FISTULA Anal fistulae are abnormal communications, hollow tracts lined with granulation tissue connecting the primary opening inside the anal canal to a secondary opening in the perineal skin. They are usually associated with anorectal abscesses (obstruction of ducts Æ infection). Conditions associated with multiple anal fistulas: 1. Crohn’s disease 2. TB 3. Actinomycosis 4. Hydra-adenitis suppurativa GOODSALL’S LAW

Posterior ½: (All fistula tracts with external openings within 3 cm of the anal verge and posterior to a line drawn through the ischial spines) x Curvilinear x Internal opening in posterior midline (at level of

dentate line) Anterior ½: x Straight tracts Tracts closer to anal verge = simpler, shorter Tracts further away = transphincteric, long, high tracts

INVESTIGATIONS x Endoanal U/S (H2O2 aided for hyperechoic effect) – to view course of fistula tract x MRI – able to visualise entire pelvis, beyond the sphincter complex x CT/fistulography (in emergency situation) – for complex fistulas / unusual anatomy

142

CRYPTOGLANDULAR THEORY OF PARKS / PARKS CLASSIFICATION

Type Diagram Management

Intersphincteric x The common course is via internal

sphincter to the intersphincteric space and then to the perineum.

x 70% of all anal fistulae x Other possible tracts include no perineal

opening, high blind tract, and high tract to lower rectum or pelvis.

Fistulotomy x Division of the internal sphincter

alone usu does not compromise fetal continence

x Base of the wound is curetted and left open to heal by secondary intention

Transsphincteric x The common course is low via internal

& external sphincters into the ischiorectal fossa and then to the perineum.

x 25% of all anal fistulae x Other possible tracts include high tract

with perineal opening and high blind tract

Low: Fistulotomy x On pts who have good pre-op anal

sphincter function

High / anterior fistulae in women: (Higher risk for post-fistulotomy incontinence) Conservative approach 1. Cutting seton x Reactive suture / elastic placed

through the fistula tract x Tightening of seton tie sequentially

until it cuts through the fistula tract 2. Partial fistulectomy & endoanal flap 3. Injection of fibrin glue

Suprasphincteric x The common course is via

intersphincteric space superiorly to above puborectalis muscle into ischiorectal fossa and then to perineum.

x 5% of all anal fistulae x Other possible tracts include high blind

tract (ie, palpable through rectal wall above dentate line).

x a/w drainage of ischiorectal abscess

Cutting setons Endorectal advancement flap Sphincter reconstruction

Extrasphincteric x The common course is from perianal

skin through levator ani muscles to the rectal wall completely outside the sphincter mechanism.

x 1% of all anal fistulae x Not related to sphincter complex x a/w Crohn’s, CA, recurrent fistulas

Endorectal advancement flap Laparotomy & resection of involved intestinal segment and curettage of fistula tract (for those fistulae from more proximal sections of the colon)

Fistulotomy (for simple, short tracts) – cut & lay open tract Fistulectomy – core along tract & remove tract entirely Seton – for complex, long, high tracts

143

4. ANAL FISSURES

An anal fissure is a painful linear tear or crack in the distal anal canal, which, in the short term, usually involves only the epithelium and, in the long term, involves the full thickness of the anal mucosa. x Most will heal because of good blood supply (within 1 day / 2 days) x If they don’t heal: usu due to spasm of internal sphincter muscle Features of a chronic anal fissure: 1. Boat shaped 2. Punched out 3. Exposing internal sphincter 4. Sentinel skin tag 5. Hypertrophic anal papilla MANAGEMENT Definitive for chronic anal fissure Æ Internal anal sphincterotomy (but do not cut through muscle – irreversible) Medical sphincterotomy (reversible): GTN paste, botulinum toxoid injections

144

9. LIVER DISEASES 1. ANATOMY OF THE LIVER

x The liver is divided into two lobes, right and left x The anatomical division of the liver lobes is demarcated

by the falciform ligament x The functional division (more practical in surgery) is

demarcated by the plane of the gallbladder and inferior vena cava (also by the plane in which the middle hepatic vein runs) Æ impt for reading CT scans and in surgery

The liver can be further divided into 8 functional segments (Couinaud segments) that each have their own vascular inflow, outflow, and biliary drainage, independent of the other segments Segments divided by 1 transverse plane and 3 sagittal planes The transverse plane is at the level of the main branches of the portal vein (divides liver into upper half and lower half) The sagittal planes are formed by the three main hepatic veins (right, middle and left) Segment I is the caudate lobe Segments II to VIII are named clockwise, while facing the patient, starting from the upper right corner (i.e. the upper left segment of liver) Segment IV can be further divided into IVa and IVb, where IVa is the superior and IVb is the inferior subsegment The liver has two blood supplies – portal vein (formed from the joining of the splenic vein and superior mesenteric vein) and hepatic artery (a branch of the coeliac trunk) Drainage is via the 3 hepatic veins into the inferior vena cava

2. CAUSES OF HEPATOMEGALY x Vascular: RHF, TR, Budd-Chiari syndrome x Infective: viral (hepatitis viruses, EBV, CMV, HIV), bacteria (pygenic abscess, TB), parasite( amoebiasis, hydatid cyst, malaria) x Metabolic: fatty liver, haemochromatosis, amyloidosis, wilson’s disease x Tumour: see below

3. CAUSES FOR A LIVER NODULE ON IMAGING

Benign Cyst - Single - Multiple – familial (polycystic) or non-familial

Haemangioma - Small - Big

Adenoma Fibronodular hyperplasia

Malignant Secondary Colorectal, stomach, pancreas, breast, urogenital tract, lung Primary Hepatocellular carcinoma (or hepatoblastoma in children)

Cholangiocarcinoma (only 10% intrahepatic) – presents like HCC except no background of cirrhosis

145

4. HEPATOCELLULAR CARCINOMA

EPIDEMIOLOGY

- Annual incidence in Singapore is 18/100,000 in males, and 4.6/100,000 in females - 3rd most cancer among males, overall 4th most common cancer - Men: female = 3:1 - Peak age of onset: 30-40 years old - 1o liver cancers are mainly HCCs (85%), with a small proportion of intrahepatic cholangiocarcinoma (6%) AETIOLOGY AND RISK FACTORS 1. Hepatitis B infection (high HBV DNA load, HBeAg positivity increase the risk) – 100X normal population 2. Hepatitis C infection 3. Cirrhosis (of which cirrhosis resulting from hep B, hep C and haemochromatosis are associated with the highest risk): 20-30years following

initial insult - Others: Aflatoxin ingestion, α-1- antitrypsin deficiency, haemochromatosis, PBC, anabolic steroids, schistosomiasis

Non-cirrhotic HCC – 5% in west, 40% in Asians PATHOLOGY - Pathogenesis involves a chronic inflammatory process or ongoing hepatocellular damage with high cellular regeneration, which leads to Ç

rates of genetic mutation in the cells and accumulation of these mutations leading to carcinoma formation - Two histological subtypes: Nonfibrolamellar – associated with HBV and cirrhosis Fibrolamellar – associated with younger patients, more common in females, no association with hep B or cirrhosis, AFP usually normal,

70% resectable, good prognosis - Metastasises to lymph nodes, bones, lungs and adrenals (more common in intrahepatic lesions >5cm) PRESENTATION

HCC is frequently diagnosed late in its course because of the absence of pathognomonic symptoms and the liver's large functional reserve. 1. Asymptomatic

- During screening (ultrasound) for chronic hepatitis B carrier - Investigations for liver cirrhosis (esp with rising AFP levels) - Incidentally found on imaging of the abdomen

2. Local signs & symptoms

1. Upper abdominal pain - 2o to a. hemorrhage internally Æ capsular distension or externally b. necrosis c. infection

2. Early satiety/ vomiting (likely 2o to compression) 3. Pyrexia (central tumour necrosis) 4. Hepatomegaly 5. Constitutional: LOW, LOA, malaise 6. Budd-Chiari syndrome: occlusion of hepatic, intrahepatic or portal vein causing portal HTN & congestive hepatopathy 7. Jaundice (5-10%)

a. Cholestatic (conjugated): invasion/ compression of intrahepatic ducts or extrahepatic compression by metastatic LN b. Hepatic (unconjugated): a/w pre-existing cirrhosis or acute flair of chronic hepatitis

3. Tumour rupture (<3%)

- Severe abdominal pain (peritonism), pallor with shock [ddx: ruptured AAA] 50% mortality; Rx with transarterial embolization (TAE); reports of peritoneal seeding in survivors

- US +ve for peritoneal fluid, DPL +ve for blood

4. Liver decompensation (on top of underlying cirrhosis) - Worsening liver function (encephalopathy, jaundice, pruritis, coagulopathy, oedema) Î suspect HCC when there is decompensation

of liver cirrhosis - Worsening portal hypertension: Ascites, LL oedema, haematemesis & melena 2o to bleeding varices (COJ & gastric) - Hepatorenal syndrome in late stages of liver failure

Stages of Hepatic Encephalopathy Stage 1 Sleep-wake cycle inversion Stage 2 Lethargy, personality change, asterixis, ataxia Stage 3 Confusion, acalculia, paranoia, agitation, Babinski, clonus Stage 4 Coma

146

5. Metastases (low incidence in HCC, mortality rarely from mets) - Bone pain, Dyspnoea

6. Paraneoplastic syndromes

1. Hypoglycaemia (due to high metabolic demands of tumour, <5% secrete IGF Æ symptomatic hypogly), 2. Erythrocytosis (tumour produces erythropoietin, but most pts are anemic), 3. Hypercalcaemia (osteolytic lesions, tumor secretes PTH-rel protein) 4. Watery diarrhoea (related to secretion of peptides that cause intestinal secretion eg. gastrin. can be severe & intractable) 5. Cutaneous features (eg dermatomyositis, pemphigus foliaceus)

Complications from hepatocellular carcinoma are those of hepatic failure; death occurs from cachexia, variceal bleeding, or (rarely) tumor rupture and bleeding into the peritoneum. INVESTIGATIONS

x Basic laboratory investigation:

o FBC (low Hb from BGIT, raised TW in SBP, low Plt) o UECr (dehydration, 3rd spacing of fluids, use of diuretics for ascites, r/o nephropathy for contrast imaging) o LFT (low albumin, high bilirubin, raised ALP, nonspecific transaminitis) o AFP (Normal <5; WHO criteria >400; diagnostic & trending; note false +ve: pregnancy, infants, cirrhosis, hepatitis, teratoma,

other CA esp gastric) - Serum levels of AFP do not correlate well with other clinical features of HCC, such as size, stage, or prognosis. Can be normal in up to 40% of small HCC. Alternatives: des-gamma-carboxy prothrombin, tumor associated enzymes of GGT etc.

o Hepatitis markers (look for carrier status or chronic infection) o PT/PTT (raised PT)

x Imaging: o Triphasic CT scan (see below)

DIAGNOSIS Biopsy is usu not performed due to risk of tumour seeding (1-2% risk) along the needle track – may render the patient unsuitable for transplant! Diagnosis is based on clinical, biochemical and radiological tests

WHO criteria for HCC: (a) Risk factors for HCC e.g. hep B/C carrier (b) Characteristic CT findings (of a hypervascular lesion) (c) Raised AFP (>400)

1. Alpha-foetoprotein (AFP): elevated in 80% 2. Triphasic CT scan [gold standard investigation]

- CT liver scan at three different times after IV contrast:

(a) Arterial phase –contrast fills arteries: aorta (& HCC, bleeds) lights up, IVC and portal vein are dark (b) Portal venous phase (most CT scans taken at this phase) – contrast enters portal system so portal vein is as bright as the aorta (c) Delayed phase – contrast drains out, so none of the vessels in the liver are lighted up

- Characteristic feature of HCC is enhancement in the arterial phase (have a rich hepatic arterial supply) with rapid contrast washout in the portal venous phase (hypodense) Haemangioma: capsular enhancement in delayed phase Metastasis: enchancement in portal venous phase

- In a patient with hepatitis B/C and raised AFP, a liver lesion on imaging should be considered HCC until proven otherwise - CT can also look for nodal involvement, and metastases to the adrenals

3. Dynamic MRI scan of the liver

- Adjunct investigation done when CT findings are equivocal - Images liver in greater detail without need for contrast, can be used to exclude benign conditions

4. Hepatic angiogram with lipiodol and post-lipiodol CT scan

- Lipiodol will be retained in HCC even after many days as the HCC does not contain Kupffer cells to ingest lipiodol - Hepatic angiogram may reveal abnormal blood vessels within the HCC - CT scan of the liver weeks after lipiodol ingestion will pick up the areas of tumour (where the pre-lipiodol CT may not have

demonstrated the tumour clearly) - Not commonly used given the need for intraarterial injection

5. If indicated, investigations to look for GI primary

- CEA, CA 19-9, endoscopy

147

STAGING (looking for metastases) 1. CXR, CT thorax: lung 2. CT abdopelvis: liver and adrenals, peritoneum and LNs 3. Bone scan if clinically indicated: bone

Different staging systems: - TNM - Okuda - Barcelona-clinic-liver-cancer (BCLC)

In BCLC, staging depends on extent of liver disease, symptoms, liver function, vascular invasion and extrahepatic spread.

1. Very early HCC is currently very difficult to diagnose confidently prior to surgical ablation. In these lesions the absence of microvascular invasion and dissemination offers the highest likelihood of cure and thus, in Child–Pugh A, patients may theoretically achieve a 5-year survival of almost 100%.

2. Early stage disease includes patients with preserved liver function (Child–Pugh A and B) with solitary HCC or up to 3 nodules </=3 cm in size. These patients can be effectively treated by resection, liver transplantation or percutaneous ablation with possibility of long term cure, with 5-year survival figures ranging from 50% to 75%.

3. The intermediate stage consists of Child–Pugh A and B patients with large/multifocal HCC who do not have cancer related symptoms and do not have macrovascular invasion or extrahepatic spread. Their survival at 3 years without therapy may reach 50%. These are the optimal candidates for transarterial chemoembolization.

4. Patients who present with cancer symptoms and/or with vascular invasion or extrahepatic spread comprise the advanced stage. They have a shorter life expectancy (50% survival at 1 year) and are candidates to enter therapeutic trials with new agents

5. Patients with extensive tumor involvement leading to severe deterioration of their physical capacity [WHO performance status>2] and/or major impairment of liver function (Child–Pugh C), are considered end stage. Their median survival is less than 3 months.

ECOG performance status grades:

148

TREATMENT SURGERY (ACHIEVE TUMOUR REMOVAL WITH GOOD LIVER FUNCTION) - Only about 10-20% of patients with HCC will have disease amenable to surgery - The only curative treatment for HCC is surgical removal of the tumour - 2 surgical possibilities:

(a) Resection of tumour (partial hepatectomy) (b) Liver transplantation

Factors affecting resectability: 1. Stage of disease

- Metastatic disease is not suitable for resection - Multicentric disease affecting both lobes is a contraindication to hepatectomy

2. General fitness for operation 3. Liver function pre-operatively

- Cirrhotic patients have higher risk of post-operative mortality (4-14%) compared to non-cirrhotic patients (0-4%) due to

complications such as liver failure, bleeding and infection - Use of indocyanin green (ICG) – the percentage of ICG remaining in the liver after 15 minutes indicates the level of liver

function. If >15% remains after 15 minutes, the patient cannot tolerate major liver resection (>3 segments removed) - CT volumetry: residual liver function calculated with a CT liver scan via a computer program - If patient has cirrhosis, assess the Child’s status Æ only Child’s A and good Child’s B can be considered for resection

Child-Pugh classification of CLD/ cirrhosis

1) Prognosis 2) Strength of

medical treatment 3) Necessity of liver

transplant

4. Residual liver function post-operatively (at least 20%) - Dependent on tumour size and how much of the liver it takes up, because tumour is non-functional - A large tumour taking up most of the liver segments being resected translates to smaller amount of functional liver tissue

being resected, while a small tumour means that more functional tissue is removed with the same resection margins 5. Degree of portal hypertension (= hepatic vein pressure grad >10mmHg)

- Resection of the liver results in worsened portal hypertension since the effective portal venous capillary bed has

decreased Æ increased resistance to flow - Detected during hepatic vein catheterization. Not necessary if clinically sig portal hpt: varices, ascites requiring diuretic

therapy, plt<100000 a/w splenomeg. - Studies have shown that a normal bilirubin concentration, and the absence of clinically significant portal hypertension are

the best predictors of excellent outcomes after surgery, with almost no risk for postoperative liver failure. 6. Location of tumour

- Has to be located in a suitable location for resection

Points 1 2 3 Albumin (g/L) >35 28-35 <28 Bilirubin (μmoles/L) < 35 35-50 >50 Coagulopathy = PT <1.70 1.70- 2.20 >2.20 Distension (ascites) None Slight - mod Severe/refractory Encephalopathy None Grade I – II Grade III-IV

Child’s A(5-6) B(7-9) C( 10-15) 1 year survival % 100 80 45 2 year survival % 85 60 35

Disease status Well compensated disease

Significant functional compromise

Decompensated liver dz, consider transplant

Treatment in concomitant HCC

Resection of up to 4 segments

Maximum resection of 2 segments

Consider transplant

149

a) Hepatectomy - Problem in patients with cirrhosis is that there is already a “field-change” effect in the liver, thus a new tumour can still

develop in the remnant liver. 75% of HCC are multifocal on diagnosis. - Requires a fine balance between adequate resection margins and preservation of sufficient functional liver to prevent

liver failure - Good immediate and short-term results, but not long term (<30% 5-year survival) - >70% tumor recurrence at 5 years due to dissemination and de novo tumors (eg occurrence of new 1° in the cirrhotic liver) - Most powerful predictors of recurrence: Microvascular invasion and/or more than one tumors (This suggests that the majority

of recurrences are due to dissemination from the 1° tumor and not metachronous tumors developing in a liver with cirrhosis) - No effective adjuvant therapy to reduce recurrence rate. - Solitary recurrence might benefit from repeat resection, but in most patients recurrence after primary resection will be

multifocal because of intra-hepatic dissemination from the primary tumor - only those patients in whom recurrence is due to de novo oncogenesis can be expected to benefit from salvage

transplantation or repeated resection b) Transplantation - Milan criteria for transplantation (>75% 5-year survival if followed)

(a) Single tumour 5cm or smaller, or 3 or less tumours none larger than 3cm (b) No evidence of gross vascular invasion (c) No regional nodal or distant metastasis

- Problems with availability of donor organ – the disease might have progressed past being suitable for transplant by the time

donor organ is available Possibility of “bridging therapy” (radiofrequency ablation) to shrink disease & slow progression until donor liver available

- In hepatitis B carriers, there is a risk for reinfection of the donor liver (high risk factors are HBeAg positivity, high HBV DNA levels) – can be aggressively treated with anti-viral drugs 2 months before transplant and anti-HBV immunoglobulin long-term after transplant

PALLIATIVE THERAPY 1. Loco-regional ablation (a) Radiofrequency ablation (RFA) – best results for locoregional strategies (b) Percutaneous ethanol injection – ethanol kills HCC cells but more painful (c) Cryotherapy 2. Intra-arterial therapy (a) Transarterial chemoembolisation (TACE) (b) Transarterial embolisation (TAE) (c) Selective Intrahepatic Radiation – Yttrium-90 radioactive beads 3. Systemic therapy – limited results; Sorafenib imporves median survival by 3/12

150

SCREENING FOR CHRONIC HEPATITIS CARRIERS - Target pop: Hep B / C carriers, Cirrhosis, FHx of HCC, Patients on transplant waiting list (transplant priority if HCC dev, detect

HCC tt exceeds transplant guidelines) - Combination of 6/12 to yearly Ultrasound + AFP levels

- US is operator dependent & may miss certain areas of the liver where imaging is difficult, but it is not associated with radiation exposure. (sensitivity 65-80%, specificity 90%) Features suggestive of HCC include poorly-defined margins and coarse, irregular internal echoes. Small tumors are often hypoechoic. As the tumor grows, the echo pattern tends to become isoechoic or hyperechoic, indistinguishable from surrounding liver. Added benefit of assessing patency of the hepatic blood supply and the presence of vascular invasion by the tumor.

- AFP is also not a perfect screening test as 20% of HCC will not have raised AFP - Thus the combination of ultrasound and AFP can increase the sensitivity and specificity of screening

- Frequency of screening is controversial, but should be increased in patients at increased risk – HbeAg positivity, high HBV

DNA levels Frequency determined by tumor growth rate, not by the degree of risk.

- Important as it detects smaller and resectable HCCs Î increasing survival from 26 to 88/52 - Screen family for chronic hepatitis B carrier status especially if there is a family history! – e.g. mother had hep B/HCC, sibling

has hep B, etc - Alternatives: des gamma carboxy prothrombin (DGCP aka PIVKA II) – potential marker of portal vein invasion by tumor

151

5. LIVER METASTASES

- Still more common than primary liver tumour for malignancy occurring in the liver - Primaries: Colorectal, gastric, pancreatic, urogenital, breast, lung PRESENTATION DEPENDS ON SITE OF METASTASIS

Mets to liver parenchyma Mets to porta hepatis LN Hx - Incidentally found on follow up (for

cancer) - Hard mass - Heaviness - Pain from rupture

- Symptoms of obstructive jaundice Yellow sclera Tea-coloured urine Pale stools

P/E - Hard, irregular nodular hepatomeg - Jaundice is a late sign

- Jaundice early, progressive - Hepatomegaly may not be present

Invx - Both obstructive and transaminitis picture

- Obstructive picture in early stages

TRIPHASIC CT - Hypodense on arterial phase (as metastases are usu hypovascular compared to hypervascular HCC; spread via portal vein) - Increasing contrast uptake on portal venous and delayed phases ROLE OF SURGERY - Promising results with colorectal and neuroendocrine metastases if isolated resectable metastatic disease – 5-yr survival >50% - Increasing role in urogenital, breast mets - Poor results for stomach, oesophageal mets - Palliation for symptoms in neuroendocrine metastases

6. LIVER HAEMANGIOMA

EPIDEMIOLOGY - Prevalence 0.4-20% - Female to male ratio 3:1 PATHOGENESIS - Vascular malformation that enlarges by ectasia, congenital in origin PRESENTATION 1. Usually small and asymptomatic, found incidentally 2. Mass effects compressing on surrounding organs 3. Pain from liver capsule stretch 4. Rupture (<1%) 5. Kassabach-Merritt syndrome for large haemangiomas – consumptive coagulopathy, thrombocytopaenia 6. Heart failure from large arteriovenous shunt DIAGNOSIS - Radiological

Characteristic features on triphasic CT – slow enhancement of the rims on arterial and portal venous phase, brightest in the delayed phase

- DO NOT BIOPSY TREATMENT - Only for symptomatic or complicated lesions - Possible role for prophylactic surgery in large, lateral, inferior lesions since there is higher risk for rupture

152

7. SIMPLE LIVER CYSTS

EPIDEMIOLOGY - 50% of cysts are single - Prevalence 1-3% - 9:1 female predominance for symptomatic cysts PATHOGENESIS - Congenital malformation when an aberrant bile duct loses communication with the rest of the biliary tree and becomes progressively dilated

(fluid within the cyst is not bilious) - No solid component and not septated (mixed cysts with septations are suggestive of malignancy) - [Cysts that communicate with the biliary system are called choledochal cysts] PRESENTATION – WITH COMPLICATIONS - Torsion - Bleeding & Rupture - Mass effect; Compression of IVC; Cholestasis due to compression of CBD; Portal hypertension - Fistulation into duodenum - Malignant change (rare) TREATMENT (IF SYMPTOMATIC) - Aspiration - Ethanol sclerotherapy (painful) - Fenestration (open or laparoscopic) - Excision/resection

8. HEPATIC ABSCESS (PYOGENIC / AMOEBIC)

PYOGENIC ABSCESS - More common than amoebic abscess locally - Causative organisms: Klebsiella pneumoniae, Enterococcus, Enterobacter, E. coli, Staph aureus - Routes of infection:

(a) Ascending infection from biliary system (ascending cholangitis) (b) Intra-abdominal source through portal vein – acute appendicitis, diverticulitis, IBD, pancreatitis, pelvic abscess (c) Contiguous spread – from gallbladder empyema (d) Haematogenous spread in sepsis e.g. infective endocarditis (e) External inoculation – iatrogenic, traumatic

- Presentation: RHC pain (capsular stretch) with Spiking fever with chills, rigors. 50% of patients have jaundice, and one-third have

hepatomegaly - Investigations:

Laboratory: FBC, U/E/Cr, hepatitis markers Blood cultures, Melioidosis & amoebic serology/ PCR, Stool ova, cysts and parasites Tumour markers: AFP, CA 19-9, CEA (may resemble infected tumour on imaging) If any aspiration done, aspirates for cytology, stains & c/s Imaging: US HBS or CT scan (to exclude liver tumour, KIV endoscopy to rule out GI malignancy) Findings: Irregular lesion with central area of necrosis, air-fluid levels, may be multiloculated. Rim-enhancing appearance on triphasic CT

scan. - Treatment

1. Resuscitate if necessary 2. Close monitoring of vitals with strict IO charting 3. Antibiotics via PICC

Empirical antibiotics – Ampicillin/Gentamicin/Metronidazole Change to definitive antibiotics when blood c/s results return Total duration of 6 /52 – 1st 2/52 IV, next 4/52 PO

4. Drainage

Drainage if >3cm – open drainage or percutaneous aspiration

153

Percutaneous aspiration Open drainage - Minimally invasive, performed under

radiologic guidance - Can be done under LA - Longer stay for patient as drainage tube

stays in patient for a longer time - May require multiple attempts if unable to

completely drain pus - Contraindications: Ascites (pus can leak into peritoneal

cavity) Uncorrected coagulopathy Proximity to vital structures

- Invasive procedure, done under GA - Shorter hospital stay - Single procedure - Not dependent on location - Indications: Concomitant pathology requiring surgery

e.g. gall stones Multiple abscesses or multiloculated

abscess Immunocompromised patient Failed percutaneous drainage (tube

blocked, or pt not getting better) Ascites Ruptured abscess

AMOEBIC ABSCESS - Causative organism: Entamoeba histolytica (infects the gut, forming ulcers in the colon, then spreads to the liver through the portal vein) - Transmission is faecal-oral - Presentation Usually single abscess No sepsis, jaundice Hepatomegaly often present Complications: rupture into pleural/peritoneal spaces

- Treatment: Metronidazole (very responsive) Aspiration if amoebic serology inconclusive; pregnancy (metronidazole contraindicated); suspicion of secondary infection; severe

symptoms from distension or fever; impending rupture 9. ASCITES Vital signs on examination:

o Abdominal distension o Flank dullness Î shifting dullness Î fluid thrill o Peripheral stigmata of chronic liver disease and portal HPT o Other signs of fluid overload: LL, sacral oedema, bibasal crepitations o Signs of malignancy

Background information x Causes of ascites: Transudate (<30g/L protein) Exudative (>30g/L) Cardiac: CCF, RHF, TR, constrictive pericarditis Cirrhosis Abdo: CLD Malignancy Renal: ESRF, nephrotic syndrome Infective causes: TB GIT: protein losing enteropathy Chylous ascites x Role of peritoneal tap:

o diagnostic and therapeutic Send fluid for FEME, protein, microbiology, cytology Relieve of discomfort & diaphragm splinting from distension

o Indications: Failed medical treatment symptomatic

o perform under aseptic technique, LA, US guidance may insert a pigtail catheter via seldinger technique open drain into stoma bag

x Treatment of ascites: o Conservative: low salt diet, diuresis o Peritoneal tap o Surgical: shunt sx (TIPSS, peritoneovenous shunt [silastic catheter], Denver shunt when with a subcutaneous pump]

154

10. PANCREATIC DISEASES 1. ACUTE PANCREATITIS

DEFINITION An acute inflammatory process of the pancreas with variable involvement of regional tissues or remote organ systems EPIDEMIOLOGY - Incidence is difficult to measure accurately (not all diagnosed). - ~ 7-10% presenting with abdominal painÎ acute pancreatitis CAUSES (I GET SMASHED)

1. Idiopathic (up to 10%) 2. Gallstones 3. Ethanol 4. Trauma – penetrating > blunt trauma 5. Steroids 6. Mumps and other infx (VZV, Coxsackie, EBV, measles, echovirus, ascaris,

mycoplasma, salmonella, campylobacter, MTB) 7. Autoimmune – SLE, PAN 8. Scorpion toxin 9. Hypercalcaemia, HyperTG (>1000 mg/dL, T1 and TV Æ Severe), hypothermia 10. ERCP (4%) - aggressive preintervention intravenous hydration to prevent 11. Drug-induced usually mild (SAND: Sulphonamides/ azathioprine/ NSAIDs/ diuretics, HCTZ, metronidazole, nitrofurantoin,

procainamide, chemo (esp asparaginase, cisplatin), cimetidine, diphenoxylate, estrogen) 12. Rare causes: Cystic fibrosis, cancer of the head of the pancreas, pancreas divisum (failure of the dorsal and ventral pancreatic

ducts to fuse during embryogenesis), Sphincter of Oddi dysfunction Gallstone: usu very small stones due to edema at the ampulla as stone passes into duod Æ increases pancreatic duct pressure (no stone in 80% suggesting stone passage) The disease develops in pts whose alcohol ingestion is habitual over 5-15 years. Alcoholics are usually admitted with an acute exacerbation of chronic pancreatitis. Occasionally, however, pancreatitis can develop in a patient with a weekend binging habit. PATHOPHYSIOLOGY - The initiating event may be anything that injures the acinar cell and impairs the secretion of zymogen granules

Gallstones: obstruction of the pancreatic duct causing interstitial oedema which impairs blood flow to the pancreatic cells Æ ischaemic cellular injury Æ predisposition to enzyme activation

It is believed alcohol itself results in injury to pancreatic cells through generation of free radicals during its metabolism, and may sensitise the pancreas to injury by other agents

- The final common pathway Æ inappropriate activation of proenzymes stored within zymogen granules in the pancreatic

cell (trypsin activates most of the proenzymes secreted by the pancreas when they are secreted into the duodenum)

- The activated lytic enzymes destroy the pancreatic acinar cells Æ release of potent cytokines that attract neutrophils and macrophages Æ themselves secrete pro-inflammatory cytokines

- The cytokine cascade amplifies the local inflammatory response and also results in a systemic inflammatory response [2 of 4: T>38 / <36, HR> 100, RR>20 or TW>15], may progress to sepsis (if source of infection is found) Î severe sepsis with 1 organ dysfunction Î septic shock Î MODS

Trauma: blunt trauma, ERCP, gall stones Infx: mumps, VZV Drugs: Alc, Steriods, SAND Metabolic: hyperlipidemia, hypercalcemia Autoimmune: SLE, PAN Neoplastic: Ca head of pancreas Congenital: CFs, Pancreas divisum

most common: >90% combined

155

PRESENTATION - Symptoms (generally non-specific):

Abdominal pain (most consistent, in >90% of patients) – constant epigastric pain, classically radiating to the back

(in 50%), maximal intensity within several hours of onset; usually occurs after a heavy meal; alleviated by sitting up & leaning forward, worse on movement

Nausea, vomiting, Anorexia

Also rule out other causes: Gastric causes: PUD Perforated viscus (mainly on examination: look for peritonism) AMI, DKA or even a lower lobe pneumonia Hepatitis or GB / CBD disease

Ask for causes of pancreatitis: Gallstone disease: biliary colic, symptoms of cholecytitis/ CBD stone/ cholangitis Recent alcohol abuse or chronic alcohol abuse Recent blunt trauma or ERCP done PHx or hyperlipidemia, hypercalcemia, autoimmune disease (SLE, PAN) Recent symptoms of mumps (viral fever + bilateral jaw pain/swelling) Recent drug history: steroids, NSAIDs, diuretics

- Signs (also non-specific) Tachycardia, low grade fever, low BP, toxic looking, jaundiced? Epigastric tenderness, signs of peritonism (<1/3 of patients) May have a palpable mass (pseudocyst, pancreatic phlegmon) Abdominal distension ± ascites with diminished or no bowel sounds (ileus) Ecchymoses (usually not present): flank (Grey-Turner’s sign) or umbilical (Cullen’s) suggest severe haemorrhagic

pancreatitis associated with profound fluid loss (third-spacing)

Organ failure

Ask for urine output Offer to auscultate the lungs for any effusion or ARDS (creps, reduced air entry)

MANAGEMENT STRATEGY

Diagnosis

Severity stratification

Assess for aetiology

Supportive treatment

Monitor for

complications

Treat aetiology

(reverse / control)

Manage complications

Prevent future

recurrence

156

INVESTIGATIONS DIAGNOSTIC (CLINICAL + BIOCHEMICAL DIAGNOSIS) 1. Serum amylase

- raised within 12 hours of onset, usually more than 1000 or 3 times normal - High sensitivity and moderate specificity (specificity increased when cut-off taken at 3 times normal upper limit) - Returns to normal level 48 to 72 hours after onset – not useful in late diagnosis

2. Serum lipase

- Rises within 8 hours of onset of symptoms and returns to normal after 7-10 days, also 3 times normal - Thus more useful in late diagnosis of acute pancreatitis

3. Urinary diastase

- Similar function to serum lipase, used when serum amylase is equivocally raised or normal - elevated for even longer period after onset

Other causes of elevated serum amylase: GI sources - PUD, IO, perforation, Ischaemic bowel (amylase in the thousands), Cholecystitis, cholangitis Non-GI sources - Ectopic pregnancy

- Impaired clearance due to chronic renal failure - Kidney stone - Salivary gland injury or inflammation (rare) - Macroamylasaemia

PROGNOSTIC / SUPPORTIVE / LOOKING FOR AETIOLOGY Imaging

4. Ultrasound [1st investigation]

- Preferred over CT scan - Good for visualising biliary tree and picking up gallstones (can intervene!)

Dilated CBD Æ ERCP to remove gallstones/Bx tumor Not dilated Æ MRCP (susp parenchymal disease)

- Pancreas may be diffusely enlarged and hypoechoeic – often difficult to visualise due to overlying bowel gas

5. Erect CXR & AXR - Erect CXR may show no air under the diaphragm, pleural

effusion, elevated hemidiaphragm, pulmonary infiltrates; complete whiteout (ARDS)

- AXR may show the “sentinel loop sign” (dilated proximal jejunal loop near the pancreas) or “colon cut-off sign” (distended colon from ascending to mid-transverse with no air distally) due to functional spasm of the bowel around the pancreas resulting from inflammation

- presence of calcifications within the pancreas may indicate chronic pancreatitis

6. CT AbdoPelvis

1. Only if considering other pathologies eg. CA in elderly (CT may worsen pancreatitis) 2. At ~72hrs of known case of pancreatitis: detect fluid collections; necrosis (IV contrast needs to be given ) Balthazar for severity

Laboratory Severity/ Cx: 1. FBC (TW for Ranson, Glasgow; haematocrit for Ranson)

2. U/E/Cr (urea for Ranson and Glasgow; electrolyte imbalances, dehydration) 3. Glucose (for Ranson and Glasgow) 4. LFTs (AST for Ranson and Glasgow; albumin for Glasgow; obstructive picture in gallstone pancreatitis) 5. CRP 6. Lactate dehydrogenase (for Ranson and Glasgow) 7. ABG (PaO2 for Ranson and Glasgow; base excess for Ranson)

Etiologies: 8. Ca/Mg/PO4 with albumin (hypercalcaemia – aetiology) 9. Fasting lipids (hyperlipidaemia – aetiology) 10. ECG & cardiac enzymes (rule out AMI as a cause of epigastric pain)

157

SEVERITY STRATIFICATION (RANSON, GLASGOW, APACHE II) I. Danger signs in the first few hours - Encephalopathy - Hypoxaemia - Tachycardia >130/min, Hypotension <90mmHg, Haematocrit >50 - Presence of Gray Turner’s/Cullen’s sign - Oliguria <50mls/hr, Azotemia (serum urea) II. Ranson criteria

Present at admission Within 48 hours of admission 1. Age >55 yrs 2. White cell count >16x109/dL 3. Fasting bld gluc >11.2mmol/L 4. LDH >600U/L 5. AST >120U/L

1. Fall in Hct >10% 2. Rise in urea >0.9mmol/L 3. Calcium <2mmol/L 4. PaO2 <60mmHg 5. Base excess >4mmol/L 6. Neg fluid balance >6L

- Ranson’s criteria prognosticates mortality according to score - Any patient with a score of 3 and above is considered to have severe pancreatitis - Mortality: <3 0.9% 3-4 15% 5-6 50% >6 90% - Shortfalls of Ranson’s: validated for alcoholic pancreatitis only Îrevised Ranson’s score was created for gallstone pancreatitis, difficult to tell aetiology in acute setting & cumbersome to wait for 48 hours, and difficult to assess for negative fluid balance

III. Glasgow/ Imrie score- PANCREAS

1. PaO2 <60mmHg 2. Age >55yrs 3. Neutrophil/WBC >15x109/dL 4. Calcium <2mmol/L 5. Renal (urea) >16mmol/L 6. Enzymes LDH >600U/L AST >100/L 7. Albumin <32g/L 8. Sugar (Glucose ) >10mmol/L

>3 criteria Æ severe

- Preferred over Ranson’s scoring in certain centres - combine with CRP IV. C-reactive peptide - As a single prognostic marker; <100 is unlikely severe - If CRP is >210mg/dL at 48 hours, the pancreatitis is more likely to be severe - No relevance >3/7 of onset as other confounding factors come into the picture - Combination of Glasgow score and CRP improves overall prognostic value V. Balthazar’s CT severity index (consider a CT AP @ ~ 3/7 from onset) - Grades severity of disease according to CT findingsÎ detects h’ge & necrosis - Not very useful as CT is not usually done in the 1/52 in local context, and disease is still evolving (CT findings lag behind) in the early stages 1. A - Normal 2. B - Enlargement 3. C - Peripancreatic inflammation 4. D - Single fluid collection 5. E - Multiple fluid collections: 50% chance of developing an infection and a 15% chance of dying

ATLANTA CLASSIFICATION (FOR SEVERITY) Mild acute pancreatitis

- Interstitial oedema - Minimal organ

dysfunction - Uneventful recovery

Severe acute pancreatitis (15-25%) - Pancreatic or peripancreatic necrosis - A/w organ failure or local Cx - Any 1 of the following

Ranson’s 3 or more APACHE II 8 or more (within 1st 48hr) Organ failure (RT, CVS, Renal, BGIT) Local Cx (pancreatic necrosis, abscess, pseudocyst)

158

COURSE OF DISEASE - 75%: mild course of disease; recover unless comorbidities cause deterioration

-------Deterioration from mild to severe very rapid!------ - 20-25%: severe outcome (Acute necrotizing pancreatitis 15%) Æ 1/3 of these patients will ultimately die - Overall mortality rate <10%. Death is bimodally distributed:

(a) Early Within 1/52; due to severe organ failure, SIRS Very little can be done in terms of treatment

(b) Late Most common cause is INFECTION with resultant sepsis Multi-organ failure can be the course of death

SUPPORTIVE TREATMENT 1. Resuscitate if needed 2. Monitoring

- In general ward if mild pancreatitis; HD/ICU monitoring if severe (≥3) - Monitor vitals [SpO2, BP, HR, Temp.], urine output & CVP

3. NBM (bowel rest) and IV fluid replacement

- Fast patients for at least 2/7 until more stable - Fluid resuscitate with crystalloids - May include gastric decompression with NGT if there is persistent vomiting, significant gastroparesis, or intestinal

obstruction (ileus) - May start oral feeding early with fluids in mild pancreatitis if tolerated

Prolonged NBM results in poorer recovery due to nutritional debilitation – think about NJ feeding, or open jejunostomy creation early in patients with severe pancreatitis; if not tolerable, then consider TPN

- Acid suppression does not change course of disease, but protects against stress ulcer formation; octetride has no benefit (thought to reduce pancreatic secretions)

4. Analgesia

- Do not give NSAIDs as they can worsen pancreatitis & cause renal failure (since there is already decreased renal

perfusion in acute pancreatitis) - Use opioid analgesics (tramadol, pethidine)

NOT morphine (causes increased tone of sphincter of Oddi) 5. Treatment of fluid and electrolyte abnormalitiesÎ hypocalcaemia, hypoglycemia 6. Antibiotics

- Either prophylactic or therapeutic

Not shown to have any benefit in mild pancreatitis a) Prophylactic in severe acute pancreatitis to prevent infection of necrosis infection will occur in 40-70% of patients with necrosis and increases the mortality rate from 12 to 33% Carbapenem (only imipenem has been shown to prevent sepsis, imipenem and quinolones have better penetration

into pancreatic tissues) b)Therapeutic in cholangitis (coexisting with Gallstone disease or as complication of pancreatitis) & infection of pancreatic necrosis/ pseudocyst

- Duration: 14-28 days

7. Support for organ failure o Ventilate with PEEP if hypoxemic (e.g. ARDS) o Dialysis & CVP monitoring if in ARF o Fluid resuscitation & inotropes if Hypotensive

159

MONITORING FOR COMPLICATIONS AND TREATING Local complications: - Acute fluid collections Due to increased vascular permeability; 70-80% resolve spontaneously

- Pseudocyst Persistent fluid collection (enzymes, blood, necrotic tissue) walled off by fibrosis and not an epithelium-lined surface (after 4

weeks) Presents as 1. Gastric outlet obstruction, 2. infx, 3. peritonitis, 4. h’ge (erosion of splenic vessels), Persistently raised

amylase despite resolution of pancreatitis 50% resolve spontaneously

- Abscess Infection of fluid collection (not necrosis)

- Pancreatic necrosis Areas of no contrast uptake on CT with intravenous contrast

- Infected necrosis Gas bubbles on CT scan Positive bacterial culture on CT, U/S guided FNA accts for much of the morbidity and mortality - Infections occurring early (<3 weeks) in the course of the illness appear to be

associated with a higher mortality rate Often polymicrobial and involve both aerobic and anaerobic bacteria – GIT org: Escherichia coli, Klebsiella spp,

Enterobacter spp, Proteus spp, Pseudomonas aeruginosa, Bacteroides spp, and Clostridium spp, and the enterococci - Chronic pancreatitis, exocrine insufficiency & endocrine insufficiency Systemic complications o Peritoneal sepsis o Pancreatic ascites (massive accumulation of pancreatic fluid in peritoneum) o Intra-abdominal haemorrhage (erosion of splenic vessels) o Multiple organ failure (ARDS, acute renal failure, hypovolaemic shock, DIVC) o Hypocalcaemia, hyper/ hypoglycaemia Intervention for local complications

- ERCP

- No benefit in mild biliary pancreatitis - Indications: Severe pancreatitis Evidence of ductal stones Cholangitis No response to treatment within 48 hours

- ERCP should be done within first 48-72 hours for maximum benefit - CT-guided aspiration of pancreatic necrosis Can help differentiate between sterile and infected necrosis Consider surgery if patient doing poorly

- Necrosectomy for infected necrosis Some kind of lavage and drainage procedure is done after necrosectomy to decrease infective load

- Role of surgery

x Infected necrotic pancreas (mortality 100% without operation) x Sterile necrotic pancreas (necrosectomy)

x Delay surgery till as late as possible for demarcation of necrotic areas (repeated surgeries required) x Diagnostic uncertainty x Complications e.g. intra-abdominal haemorrhage

- Pseudocyst Operate if larger than 6cm and persisting for more than 6 weeks as the chance of spontaneous resolution is low and risk

of complications (infection, haemorrhage, rupture) is high Surgery can be open, laparoscopic, endoscopic or percutaneous (radiologically guided)

Endoscopic – internal drainage via a cystogastrostomy, cystoduodenostomy or cystojejunostomy

160

MANAGEMENT OF AETIOLOGY & PREVENTION OF RECURRENCE Avoid alcohol, stop all offending medication & control hyperlipidemia Cholecystectomy for biliary pancreatitis - 18-21% of patients with biliary pancreatitis will have another episode - Among these, 25-65% will develop the 2nd episode within 30 days of the initial one - done in the same admission for pts with mild pancreatitis - In patients with severe pancreatitis, there is reluctance to do the surgery early, as the patient may develop complications that

require surgical intervention – better to do all surgery in the same operation instead of opening the patient twice 13. CHRONIC PANCREATITIS

Chronic, irreversible architectural disruption of the pancreas resulting in chronic epigastric pain and pancreatic insufficiency Causes: NOT GALLSTONES!

- Alcholism – 80% - strictures (congenital, acquired), - trauma, - genetic (North Indians) - Smoking - CRF - Hypercalcemia

Presentation:

a) Pain (85%) – mostly episodic, some have persistent relentless pain b) Diarrhoea (15%) c) LOW (sec to anorexia, malabsorption) d) Newly diagnosed DM (30%) e) Steatorrhea (late)

Complications: x Local complications: persistent pseudocyst, fistulae, ascites x Chronic pain x Pancreatic insufficiency

o Type I DM o Steatorrhoea, vitamin deficiency, malnutrition

x Pancreatic cancer INVESTIGATIONS

a) Dx: a. MRCP (choice!): chain of lakes appearance of pancreatic duct (strictures and cystic dilatations) b. AXR: calcification c. EUS

b) Baseline function a. Secretin injection Æ check for pure pancreatic juice (gold std but invasive) b. Faecal pancreatic elastase

c) Complications: MRCP (pseudocyst, duodenal stenosis, portal vein thrombosis)

Outline of management x Treat underlying cause (stop alcohol, relieve ductal stricture) x Control blood sugar with insulin x Pancreatic enzyme supplements x Pain relief

Role of surgery

x Persistent local complications: pseudocyst, fistulae, local obstruction secondary to fibrosis (CBD, duodenum) x Pain relief (pancreatectomy, coeliac plexus block, thoracic splanchnicectomy) x Relief of pancreatic duct obstruction

o Puestow procedure: side to side pancreatico-jejunostomy

161

14. PANCREATIC CANCER

EPIDEMIOLOGY - Incidence about 3-5 per 100,000 per year in each gender - Eighth cause of cancer death in Singapore - 1.7:1 male to female ratio, increase with age - Very poor prognosis – median survival for unresectable disease is 6 months (80% of patients have unresectable disease at

presentation); overall 5-year survival <3% ASSOCIATIONS - Cigarette smoking (most clearly established – 2-5X increased risk) - Industrial carcinogens – benzidine, betanaphthylamine (dye) - Diabetes mellitus - Chronic pancreatitis - Genetic factors (mutations in K-ras gene, p16 gene) - Familial cancer syndromes e.g. Peutz-Jeghers (Hereditary intestinal polyposis syndrome, AD, hyperpigmented macules in lips

and oral mucoas), HNPCC, MEN, FAMMM (familial atypical mole multiple melanoma syndrome) - Lower socioeconomic class PATHOLOGY - Most common histology is ductal adenocarcinoma (90% of tumours) - Anatomic distribution: 75% in the head, 20% in the body, 5% in the tail - Distinct category of tumours collectively called periampullary tumour (30%):

Malignant cells arise from one of a few cells:

(a) Duodenal epithelium (best outcome out of all three) (b) Biliary ductular epithelium (c) Ampullary ductular epithelium

The periampullary tumours have better tumour biology than pancreatic adenoca

smaller size, better diff, low nodal stage, margins neg, less vascular invasion Different TNM staging 90% resectability, median survival after Sx is 38mth

Prognosis is also better as they present earlier with obstructive jaundice

PRESENTATION May be asymptomatic, picked up on imaging for some other purpose

Pancreatic head or periampullary Pancreatic body/tail - OBSTRUCTIVE JAUNDICE

Painless obstructive jaundice with palpable GB (Courvoisier’s sign) + Cholangitis

- Duodenal obstruction (vomit) - Bleeding upper GIT (haematemesis

and/or malaena) - Malaise, LOW!, anorexia, nausea - Endocrine: Diabetes mellitus

Late presentation - Coeliac and mesenteric plexus invasion – dull constant (incessant and

boring) PAIN in the epigastrium radiating to the back, relieves with bending fwd

- Malaise, weight loss, anorexia, nausea - Exocrine insufficiency with duct obstruction Æ steatorrhoea, malabsorption

- Metastatic symptoms: ascites, bone pain, CNS symptoms, dyspnoea - Paraneoplastic syndromes – migratory thrombophlebitis in 6%

Also - Recurrent venous thrombosis - Acute pancreatitis - Depression

162

IMMEDIATE MANAGEMENT - Treat any life-threatening complications such as cholangitis, pancreatitis, bleeding INVESTIGATIONS DIAGNOSTIC 1. CA 19-9

- Not a screening test for pancreatic cancer as it can be false positive - Can act as a prognostic marker: high CA 19-9 levels usu associated with unresectable disease with poorer prognosis - Can be used as a marker for tumour recurrence during post-op follow-up

2. CT scan (MAIN)

- Better sensitivity (85-90%) and equal specificity (90-95%) in diagnosing pancreatic cancer - 1. Mass lesion within pancreas,

2. Both bile & pancreatic duct dilatation in head of pancreas tumours (double duct sign) Æ 3. GB distension 4. Staging: Extra-pancreatic spread (Involvement of regional LNs, liver metastases, ascites)

3. MRI pancreas with MRCP – MRI pancreas is not superior to CT scan (Diagnosis and staging)

MRCP is useful in delineating biliary system anatomy especially if the system is not obstructed and there are no therapeutic indications for ERCP (since there are considerable risks with ERCP)

4. ERCP with stenting to relieve obstruction (in cholangitis) 5. Endoscopic ultrasound + FNA biopsy

- Dx: FNA with EUS guidance preferred to transcutaneous biopsy (less risk of tumour seeding) - Staging: tumour and nodal involvement

The most difficult clinical situation in which to diagnose pancreatic carcinoma is in the patient with underlying chronic pancreatitis. All of the above imaging studies may show abnormalities that may not help to differentiate between pancreatic carcinoma and chronic pancreatitis. Even tumor markers can be elevated in patients with chronic pancreatitis. Æ Often combine multiple imaging modalities, close clinical follow-up, serial imaging studies, and, occasionally, empiric resection, to diagnose an underlying pancreatic carcinoma

localized areas of reticulated erythema and hyperpigmentation due to chronic and repeated exposure to heat: regular application of heat to the same painful part over a period of at least a few weeks suggests that the pain is severe and consistent.

163

STAGING [LIVER, LUNGS, PERITONEUM, BONE] 1. CT/MRI of the abdomen – T, N stage; metastasis to the liver 2. Endoscopic ultrasound – T, N stage 3. Lungs – CXR + CT thorax 4. Bones – bone scan when suspicion is high 5. Staging laparoscopy – for peritoneal metastases, just before definitive operation for a resectable tumour (since CT/MRI may

miss small peritoneal deposits in ard 25%) Æ if no peritoneal disease found, continue with surgery, otherwise, close up and abort surgery

TREATMENT SURGERY Curative resection - Improves chances of survival - Only about 15-20% of patients will have resectable disease at presentation – usu in periampullary / head of pancreas tumours - However, recurrence rates after surgery are high – 5 year survival only 10 to 30% - Recent trials: improved survival with adjuvant chemo (5-FU + Folinic acid or gemcitabine) - Resectable disease: No metastases (lung, liver, bone, peritoneum) Patent superior mesenteric vein and portal vein – no longer absolute CI Definable tissue plane between tumour and superior mesenteric artery as well as coeliac axis

Whipple’s operation Pancreaticoduodenectomy for head of pancreas or periampullary tumour Usually preceded by a staging laparoscopy to confirm absence of peritoneal metastases Removal of the head of the pancreas, duodenum, proximal 15cm of jejunum, common bile duct, gallbladder, distal part of

the stomach and LN removal (impt prog factor) Common hepatic duct and pancreas are then anastomosed to the jejunum, 45-60cm proximal to the gastrojejunostomy

164

- Complications of Whipple’s operation Mortality rate is 2-7%, with a morbidity rate of up to 20-30% (mostly mild complications) Intraoperative/early complications

General: (a) Injury to other organs – liver, kidney, bowel (b) Bleeding (c) Infection Æ abscess, sepsis Specific: (d) Pancreatitis (e) Pancreatic anastomotic leak (5-20%) (f) Biliary anastomotic breakdown (g) Fistulation, pseudocyst formation may occur due to anastomotic leaks

Late Surgical: (a) Gastric stasis with or without pylorus-preserving Whipple’s (25%) – NG decompression, longer hosp stay (b) Diarrhoea resulting from autonomic nerve injury during lymph node dissection Æ LOW (c) Dumping syndrome: increased gastric emptying (from antrectomy) causing Nutritional deficiency Functional: (d) Long-term exocrine insufficiency resulting in malabsorption and steatorrhoea (e) Endocrine insufficiency Æ DM

Pylorus-preserving pancreaticoduodenectomy (PPPD)

- comparable operation time, blood loss, hospital stay, mortality, morbidity, and incidence of delayed gastric emptying. - The overall long-term and disease-free survival was comparable in both groups. - Both surgical procedures are equally effective for the treatment of pancreatic and periampullary carcinoma

Palliative surgery Surgical bypass of obstruction - Triple bypass involving anastomosis between [all to jejunum]

(a) Stomach and jejunum (gastrojejunostomy) (b) Biliary system and jejunum (choledocho-/hepatico-/cholecysto-jejunostomy) (c) Jejunum and jejunum, to prevent reflux of food into biliary tree – essentially a Roux-en-Y loop (jejunojejunostomy)

NON-SURGICAL PALLIATIVE MEASURES 1. Endoscopic stenting

- Stenting of obstructed biliary duct - Stenting of obstructed duodenum

2. Coeliac plexus block for pain 3. Palliative chemotherapy/radiotherapy/chemoradiotherapy

- Not shown to provide good outcomes

165

11. BILIARY TRACT DISEASES 1. CAUSES OF JAUNDICE

Pre-hepatic Hepatic Cholestatic

o Urine/stools normal o May have symptoms of anemia o Unconjugated hyperbilirubinemia o ↑ LDH, ↓ haptoglobin o PBF o Direct Coomb’s test for autoimmune

hemolytic anemia o Stool OCP (ova, cysts, parasites) malaria Hemolytic anemias: 1) Inherited

x Thalassemia x G6DP x Spherocytosis x Sickle-cell anemia

2) Acquired x Infective: malaria x Autoimmune: SLE, Evan’s

syndrome x Hemolytic uremic syndrome

(hemolytic anemia, ARF & thrombocytopaenia)

o Mixed signs; urine may be dark with normal stools

o Variable liver biochemistry o ↑ ALT AST disproportionate to ALP GGT o ↓ Albumin, prolonged INR (cirrhosis) o AFP: exclude complication of malignancy o Viral hepatitis serology

x Acute: Anti-HBc IgM, Anti-HAV IgM x Chronic: HBsAg, anti-HCV, HCV RNA

o Autoimmune screen x ANA, anti-dsDNA, AMA

o Metabolic screen x Caeruloplasmin, 24hr urine copper

o Abdo U/S: surface nodularity & ↑ echogenicity in cirrhosis, signs of portal HTN (splenomegaly, ascites)

1) Infective

x Acute viral hepatitis (HBV, HAV) x EBV, CMV x TB

2) Liver cirrhosis/chronic liver disease x Alcoholic liver disease x Chronic viral hepatitis (HBV, HCV) x Metabolic (Wilson’s,

hematochromatosis) x Infiltrative (Sarcoidosis,

amyloidosis) 3) Hepatotoxic drugs

x Alcohol, paracetamol, TCM, isoniazid, augmentin, MTX, phenytoin, corticosteroids

4) Autoimmune hepatitis x SLE

5) Inherited ↓/absent activity of UGT (unconjugated hyperbilirubinemia) x Gilbert’s syndrome x Crigler Najjar 1 & 2

6) Inherited impaired biliary excretion (conjugated hyperbilirubinemia)

x Dubin-Johnson syndrome x Rotor syndrome

o Tea-colored urine, pale stools, pruritis o Conjugated hyperbilirubinemia o ↑ ALP, GGT disproportionate to ALT, AST o Do abdominal U/S

x If ducts dilated (>8mm), do ERCP/MRCP/PTC

x If ducts not dilated, further serologic testing: AMA (M2-IgG most specific for PBC), p-ANCA (PSC), ANA & anti-SMA. Consider ERCP, liver biopsy

1) Intraluminal x Gallstones (painful) x Parasites: Ascaris lumbricoides,

schistosomiasis 2) Mural

x Biliary strictures post ERCP x Biliary strictures from gallstones,

chronic pancreatitis x PBC (intrahepatic bile ducts):

middle aged x PSC (intra & extrahepatic):

with IBD esp. UC x Cholangitis (Charcot’s triad) x Choledochal cyst (type I-V) x Distal cholangiocarcinoma

3) Extraluminal x Ca head of pancreas (painless, w

distended GB) x Other peri-ampullary Ca

(cholangio, dudeno, ampullary) x Mirrizi’s syndrome (chronic

cholecystitis) 4) Others:

x Biliary atresia x Drug-induced: paracetamol,

penicillins, corticosteroids

166

2. APPROACH TO OBSTRUCTIVE JAUNDICE

CAUSES (POSTHEPATIC JAUNDICE)

Intraluminal Benign - Gallstones - Parasitic infections (recurrent pyogenic cholangitis)

Mural

Benign - Post-instrumentation strictures (ERCP, operation) - Strictures from other causes (gallstones, chronic pancreatitis) - Benign papillary fibrosis: prev gallstone passage (can give post cholecystectomy pain, acute pancreatitis) - Primary sclerosing cholangitis - Choledochal cyst Malignant - Cholangiocarcinoma (distal)

Extramural

Benign - Mirizzi syndrome - Pancreatitis: secondary bile duct compression from edema Malignant - Head of pancreas cancer - Periampullary cancer - Metastases to the porta hepatis

Obstructive jaundice ÆPainless: CA HOP, Cholangiocarcinoma, Periamp CA Æ Painful: Stone, Stricture, Hepatic causes

Commonest causes of jaundice 1. Gallstone 2. Tumor 3. Hepatitis

HISTORY 1. Confirm obstructive jaundice

- Confirm jaundice – pt’s sclera are yellow (carotenemia does not result in scleral icterus or elevation of the bilirubin level) - Establish obstructive jaundice – tea-coloured urine, pale stools - Exclude pre-hepatic and hepatic jaundice (more importantly hepatic jaundice since it can also cause tea-coloured urine) Symptoms suggestive of viral hepatitis: prodrome of fever, malaise, arthralgia, myalgia, nausea/vomiting, etc. Risk factors for viral hepatitis: travel history, ingestion of seafood, family history of hepatitis (esp mother, siblings), blood transfusions,

drug abuse/needle sharing, needlestick injuries, sexual contact Alcohol intake: shown to affect bile acid uptake and secretion, resulting in cholestasis. Chronic alcohol use may result in fatty liver

(steatosis), hepatitis, and cirrhosis Drug history: any TCM intake recently, any new medications taken History of chronic liver disease

2. Aetiology – benign or malignant

- Recurrent spikes of similar jaundice that resolve on their own with time suggest benign obstruction e.g. stones, strictures - A young patient with painful jaundice Æ usually benign cause - Previous history of gallstone disease or biliary colic symptoms - Previous history of surgery to the biliary tract or ERCP - Malignancy is suggested if the patient is old, jaundice is of new onset and progressively worsening, and there is no associated pain (i.e.

painless progressive jaundice) - Constitutional symptoms: loss of appetite, loss of weight, malaise - Metastatic symptoms: bone pain, neck lump, dyspnoea, etc - Pain is a late symptom of pancreatic CA & tends to be constant and relentless compared to biliary colic which subsides after few hours

3. Complications

- Symptoms of cholangitis: fever, chills, rigors with RHC pain and jaundice - Pancreatitis (gallstone as cause): abdominal pain radiating to the back with N/V - Pruritus as a result of bile salt retention - Decompensation: encephalopathy, hepatic fetor, worsening ascites - Fat malabsorption: steatorrhoea, fat-soluble vitamin deficiency (A, D, E, K) – especially coagulopathy (very unlikely in acute setting)

167

PHYSICAL EXAMINATION 1. Vitals: Is patient haemodynamically stable? Any fever? 2. General inspection: Jaundice. Pallor? Any abdominal distension, leg swelling? 3. Peripheries: Stigmata of chronic liver disease? Scratch marks? Conjunctival pallor? 4. Abdomen

- Any scars of abdominal surgery? - Generalised distension? Ascites could be due to:

1. malnutrition (low albumin) 2. peritoneal malignancy 3. obstruction of portal vein by cancer

- Hepatomegaly? (Could be due to metastatic disease, or primary liver pathology) - Enlarged gallbladder? (Courvoisier’s law – if the gallbladder is palpable with painless obstructive jaundice, cause is unlikely stones) - Splenomegaly? (Portal hypertension – think prehepatic, hepatic, posthepatic)

5. DRE: Pale stools? 6. Cervical and supraclavicular lymph nodes 7. Bony tenderness 8. Respiratory examination INVESTIGATIONS (guided by clinical suspicion after Hx and P/E) Bloods Firstly to confirm obstructive jaundice: 1. LFT – classical obstructive picture = bilirubin raised (+/- direct>indirect; Normal 3:7) + raised ALP (and GGT - only ordered if Dx is ambiguous,

not routine) more than AST and ALT **Classical picture more often seen in CA than gallstone disease. Secondly to confirm inflammation/infection: 2. FBC – any infection (impt as some pts haven mounted ferbrile response), anaemia (correct before ERCP) Followed by the rest: 3. U/E/Cr: dehydration, Cr level for suitability of CT imaging!! 4. Amylase: concomitant pancreatitis 5. PT/PTT – any prolonged PT from vitamin K malabsorption, liver dysfunction (to be CORRECTED before procedures like ERCP can be done) 6. Tumour markers – CA 19-9, CEA (cholangioca and pancreatic ca) 7. Blood c/s if febrile and jaundiced (TRO HBS sepsis) 8. GXM – in case of op (eg. cholecystectomy) Imaging (Ultrasound versus CT - Both useful in demonstrating dilated biliary system as well as site & cause of obstruction)

1. U/S HBS: best for imaging stones a. US findings:

i. Choledocholithiasis: Duct dilation>8mm (impt to identify dilated INTRAhepatic ducts as it indicates that (a) obstruction is more severe (b) PTC is a possible option if ERCP fails)

ii. Gallstone disease / Cholecystitis: GB stones or sludge, thickened GB wall, pericholecystic fluid, fat stranding iii. Complications of gallstones: iv. Liver consistency (fatty or cirrhotic)

b. Disadv: i. Unable to detect malignancy well ii. Unable to detect distal CBD stones well iii. Sensitivity reduced with fat patient habitus iv. Operator dependent

2. CTAP: a. Indications

i. Suspected perf oratedGB (view air ard GB in lung window) ii. Rule out malignant etiology

b. Adv i. Preferred if there is a suspicion of malignancy (Ca pancreas or periampulary cancer) Î define the tumour (T)

better & stage at the same time (N & M) ii. Logistics (can be done earlier esp cholangitic patient to plan for early intervention)

3. CXR a. ARDS in cholangitis b. Pleural effusion

MANAGEMENT The patient is managed as for the causative aetiology (see relevant sections)

168

3. GALLSTONE DISEASE

DEFINITION Gallstone is a generic term for any kind of stone (cholesterol, pigment) in any part of the biliary system (gallbladder, cystic duct, hepatic duct, common bile duct, etc) EPIDEMIOLOGY - Exact incidence in Singapore not known - In the West: overall 10-15%; 20% in women and 10% in men - Consistent 2:1 female to male ratio, 1:1 in elderly - Typical picture (the 5 F’s): Fat, female, forty, fertile, flatulent – refers to cholesterol stones but no longer applies. NORMAL PHYSIOLOGY OF BILE - Normal bile contains bile salts (primary and secondary), phospholipids, cholesterol, protein, and bilirubin - Bile salts and phospholipids are amphiphatic and help to solubilise cholesterol (in micelles, vesicles) - a/w simple refined sugar in diet STONE COMPOSITION AND PATHOPHYSIOLOGY 1. Cholesterol: cholesterol - hard and faceted 2. Black: mostly calcium salts and bilirubin - soft stones and crumble easily 3. Brown: calcium salts, bilirubin, and more cholesterol than black pigment stones Cholesterol stones - More common in older (peak at 40-50 years) but increasing in younger - Developed countries – 22% of SG population (18% with signs and symptoms) - M=F - From disruption in the solubility equilibrium of bile.

1. Increased cholesterol secretion in bile old Obesity, rapid weight loss Hyperlipidaemia Increased oestrogens: female, pregnancy, exogenous administration 2. Decreased emptying of the gallbladder Gallbladder malignancy is an important cause to exclude Gallbladder stasis: Truncal vagotomy, Spinal cord injury Pregnancy Fasting, TPN

Pigment stones - bilirubin - More common in younger patients, developing countries

a) Black pigment stones: a. increased secretion of bilirubin into bile (e.g. chronic HAEMOLYSIS, - most commonly G6PD-def, CIRRHOSIS,

chronic liver disease, TPN), b. decreased bilirubin solubilisers, and gallbladder stasis

b) Brown stones: = RPC!!! Form in the biliary ducts due to infection with bacterial degradation of biliary lipids (hydrolyse conjugate bilirubin to free form), the degradation products of which then precipitate as calcium bilirubinate. Increasing in incidence due to influx of foreign workers.

Mixed - Majority Biliary sludge - Microlithiasis suspended in bile; a milieu that predisposes to stone formation - Can be visualised on the ultrasound scan as layering in the biliary tree - Sludge is a pre-stone condition, but not all sludge becomes stones - 20% of biliary sludge will disappear, 60% recur, and 10% form stones

169

CLINICAL COURSE Asymptomatic Æ Leave alone

- 80-95% of patients - Risk of symptom occurrence = 1 to 2% per year

greatest risk in first 5 years of diagnosis – 10% at 5 yrs, 15% at 10 yrs, 18% at 15-20 yrs 7-10% mod, 3-5% severe; the rest minor symptoms

Î majority do not require removal of the stones or the gallbladder = expectant mx (Risks of Sx > Risks of doing nth) - Role of surgery: [3]

(a) Suspicious / high risk of MALIGNANCY (causing gallbladder stasis Æ stones) eg. susp gallbladder mass, gallbladder polyp, porcelain gallbladder Æ prophylactic surgery

(b) IMMUNOCOMPROMISED Î presentation is abnormal & difficult to detect (c) Patients with CHRONIC HAEMOLYTIC DISEASE (e.g. sickle cell anaemia, thalassaemia) – as high as 50-60% will

develop symptomatic disease in their lifetime Symptomatic sequlae 1. Biliary colic (15%)

- Location: epigastric (70%) or RHC pain - Radiation: inferior angle of the right scapula, tip of right shoulder or interscapular - Character: NOT a true colic: Waxing-waning in character but rarely have any pain-free intervals between waves of pain (Basal

pain is due to inflm of ductal epithelium & proximal distension) - CBD is not muscular, so absence of biliary colic, but a constant pain with OJ 2o to obstruction

- Duration: minutes to 2 hours, often resolves spontaneously (if >6hrs, susp complication eg. cholecystitis)

- Trigger: meals – binge-eating, fried oily foods, dehydration

- Associated: N/V (patient gets better after vomiting), bloating, abdominal distension Biliary colic is a “herald” symptom that indicates risk of further sequelae.

2. Infection

i. Acute / Chronic cholecystitis ii. Cholangitis iii. Pancreatitis

3. Gallstone dyspepsia: (non-ulcer dyspepsia) fatty food intolerance, dyspepsia and flatulence not due to other causes Complications

4. Choledocholithiasis with obstructive jaundice 5. Mirizzi syndrome with obstructive jaundice (see below) 6. Mucocoele of the gallbladder / Hydrops / Empyema of Gallbladder (see below) 7. Fistulation and gallstone ileus (see below) 8. Porcelain gallbladder: due to calcium salts ppt in GB wall. Calcium secreted into lumen of hydropic GB Æ ‘limey’ bile. 9. Gallbladder CA INVESTIGATIONS FOR GALLSTONE DISEASE

Plain abdominal X-ray - Pickup rate for gallstones is less than 10% since most stones are radiolucent

1. Ultrasound of the hepatobiliary system

- Investigation of choice for gallstones - >92% sensitivity, 99% specificity - Even more sensitive than CT scan for stones since CT may miss small stones due to the spacing of the cuts taken - Features of stone on ultrasound: strong echogeneic rim around the stone, with posterior acoustic shadowing - Bile should appear as black patch in gallbladder; if not homogeneous Æ sludge

170

2. CT scan - Usually not done to diagnose stones (as mentioned above)

a) DDx: Usually done in symptomatic patient where it is uncertain what is the cause of symptoms Æ looking for other possible causes as well (liver/pancreatic)

b) Cx: detect complications of gallstones 3. Magnetic resonance cholangiopancreatography (MRCP)

- MRCP is not the same as MRI liver/pancreas –

only selected cuts taken in order to reconstruct the biliary tree without contrast only T2 images, resolution is not as good as MRI

- Comparable to ERCP, and also minimally invasive Æ preferred to ERCP if patient does not require any therapeutic intervention that ERCP provides

4. Endoscopic retrograde cholangiopancreatography (ERCP)

- Will definitely involve cholangiogram - The largest value of ERCP lies in its therapeutic potential

1. Stone removal (using balloon catheter, or Dormia basket) 2. Sphincterotomy (in order to relieve obstruction or facilitate removal of stone) 3. Stenting

- High level of complications Overall risk is 10-15% a. Pancreatitis in 1-3%:

i. Due to inflammation from a) injection of contrast (increased p) and b) edema when removing stone ii. Increased risk in: younger, F, prev post-ERCP pancreatitis, cannulation/ injection of pancreatic duct,

sphincter of Oddi dysfunction b. Haemorrhage 2-3%: esp sphincterotomy c. Cholangitis 1-2% d. Perforation into bile duct, duodenum 0.1% (esp if sphincterotomy): (OGD 0.01% ie 1 in 10000– ERCP slightly

higher since it uses side viewing scope) e. Failed ERCP f. Over sedation (hypotension, resp depression, N/V)

Also: Cholecystitis, Contrast related reaction (allergy to iodine) rare - Before doing ERCP, need to assess the benefits and risks, and select patients carefully

5. Percutaneous transhepatic cholangiography (PTC) /biliary drainage (PTBD)

- PTC involves a tube being inserted under radiologic guidance into one of the biliary ducts (must be dilated duct) - Rarely done now; main indications:

1) Diagnostic: high obstruction not well visualised in ERCP; prev surgery with altered anatomy (eg gastrectomy) 2) Therapeutic: obstructed system that cannot be drained from below;

- Mostly for therapeutic rather than diagnostic purposes - Complications: bleeding (esp in biliary obs pts due to coagulopathy sec to decreased Vit K abs); leakage of bile when

tube is removed 6. HIDA scan

- No longer used commonly, except in biliary atresia

5 criteria for a normal cholangiopancreatogram (a) Normal intrahepatic ducts (b) No filling defects (c) Smooth common bile duct (d) No stricture/narrowing of the common bile duct (e) Good and free flow of contrast into duodenum

171

TREATMENT Asymptomatic - No surgery required unless patient has indications for surgery (see above) - Expectant management and close follow-up - Counsel patient about symptoms – biliary colic, acute cholecystitis, obstructive jaundice, etc Symptomatic Surgical = Cholecystectomy - Cholecystectomy is the only way to treat gallbladder stones that are symptomatic (esp if cxs arise Æ high risk of 2nd attack) - Open or laparoscopic ( preferred) - Adv of Lap: shorter hospital stay, less pain, less complications post-operatively - Risks of Lap: Conversion to open - up to 5%

Due to abnormal anatomy; difficult or complicated dissection; iatrogenic injury. Conversion rate is higher if there is ongoing infection e.g. cholecystitis – up to 1 in 3 to 1 in 4

Injury to surroundings: bowel & biliary structures e.g. CBD Haemorrhage Infection Spilled bile Æ peritonitis, sepsis

Non-Surgical - Non- surgical means of stone treatment Shockwave lithotripsy – more morbidiy cf renal lithotripsy as less fluid around to dampen waves; good results only for

cholesterol stones. Expensive. Bile salt therapy necessary following lithotripsy todissolve gallstone fragments. CI: more than 3 stones, large/calcified stones, non-functioning GB, Cx of gallstones

Medical Tx in radiolucent gallstones, <15mm, mod obesity, no/mild symptoms. (success in 75% who fulfil this criteria) Chemodissolution: LT oral bile acid ursodeoxycholic/ chenodeoxycholic acid (thought to reduce hepatic synthesis of

cholesterol and reduce chol secretion) 9 mths intensive followed by lifelong maintenance. Toxicity and S/E, high cost.

Liver diet: mod carbo, low fat and cholesterol, high fibre Æ All therapeutic regimens retaining the GB have 50% recurrence of stones aft 5yrs (no LT benefit)

172

4. ACUTE CHOLECYSTITIS

PATHOPHYSIOLOGY - Gallstone gets stuck in the cystic duct causing obstruction of biliary flow - Gallbladder becomes distended and inflamed - 50% of cultures are sterile (infection occurs eventually. In elderly, DM Æ severe with gas-forming organisms causing emphysematous

cholecystitis) PRESENTATION - Constant, severe RHC pain (less commonly epigastric) - Radiates to the inferior angle of the scapula, interscapular - Associated with nausea, vomiting - Tachycardia, Low grade fever (chills uncommon), Dehydration - RHC tenderness with guarding found on clinical examination;; Murphy’s sign positive (increased rigidity on inspiration) - Gallbladder may be palpable (30%) – omentum wrapping around GB; worst case scenario is empyema - Jaundice < 10%: due to Mirrizi’s or passage of stones into CBD (15% have CBD stones) INVESTIGATIONS

1. ULTRASOUND FEATURES OF ACUTE CHOLECYSTITIS a) Presence of gallstones in biliary system b) Contracted gallbladder (from chronic gallstone disease) c) Pericholecystic fluid (oedema of gallbladder wall) d) Thickened GB wall e) Sonographic Murphy’s positive

2. CT AP

x Fat stranding around gallbladder not seen on ultrasound but on CT x Exclude complications (empyema, perforation)

3. FBC: leukocytosis (except elderly) – if severe, indicates complications 4. Amylase (can be raised mildly, if >1000, pancreatitis!), 5. LFT (mild transaminitis)/ UECr (dehydration) 6. CXR, KUB:

x Radioopaque gallstones, aerobilia (due to fistula). x Exclude lower lobe pneumonia, perf viscus, abnormal right hemidiaph/thorax.

7. PFO: PT/PTT, ECG MANAGEMENT A) Conservative - Resuscitate the patient, senior to r/v if pt systemically unwell - Septic workup - NBM and intravenous fluids - Analgesia - Empirical intravenous antibiotics – IV ceftriaxone (1g bd) and metronidazole (500mg tds) - Bed rest - Careful monitoring for signs of failure (peritonism, non-resolving fever/pain) Cholecysitits usu resolves with medical management (60%), but inflammation may progress Æ complications, including peritonitis. - Definitive treatment – laparoscopic (better success if within 72hrs of symptom onset) cholecystectomy B) Timing of cholecystectomy - Dependent on several factors: Severity of illness Response to resuscitation and antibiotic therapy Logistical considerations (availability of OT, surgeon etc)

- Possibilities available:

i. Emergency (immediate; in very sick patients who are not doing well/ not responding to treatment) ii. Early (within few days of onset – ideally within 5 days) iii. Delayed/interval (after 6-8 weeks)

173

Early Delayed Advantages - Everything done in one admission - Easier to operate as the gallbladder is oedematous Disadvantages - Ongoing inflammation Æ higher risk of bleeding - Higher risk of injuring some other structure due to difficulty in

visualisation - Higher conversion rate to open chole - Increased risks of post-op infection

Advantages - Lower risks - Better laparoscopic success Disadvantages - Fibrosis Æ difficulty mobilising gallbladder - Need for another admission - Chance of recurrence / acute pancreatitis during the time

(20%)

Î Early surgery has been found to be more beneficial (È mortality, total duration of dz, length of hosp stay, cost)

Gallstone disease in elderly more severe - higher rate of complications - extremely high mortality for emergency cholecystectomy - Acute cholecystitis: timely diagnosis, early stabilization, semiurgent cholecystectomy C) Cholecystostomy (fastest way) - In moribund patients who are not fit for surgery / early surgery is difficult due to extensive inflammation - Can be done under LA, or more commonly under radiologic guidance (percutaneous) - Drains the gallbladder and alleviates the inflammation (resolves acute episode) Æ better outcomes - Can be followed by cholecystectomy 4-6 wks later COMPLICATIONS OF ACUTE CHOLECYSTITIS 1. Hydrops/ Mucocele

- Cystic duct obstruction leads to a tense gallbladder filled with mucus (slow distension from continuous mucus secretion) - May lead to gallbladder wall necrosis if pressure exceeds capillary bld pressure

2. Empyema

- Gallbladder is filled with pus due to bacterial infection of the stagnant bile (cystic duct being obstructed by a stone) - Patient is usually toxic, requiring urgent surgery

3. Gangrene and perforation – rare due to rich blood supply from hepatic and cystic arteries

- Localised perforation Æ abscess that is confined by the omentum (new mass) - Free perforation Æ generalised peritonitis and sepsis Æ sudden generalised abdo pain Æ emergency laparotomy

4. Cholecystenteric fistula

- Most commonly occurs in duodenum, then colon, and stomach; after repeated attacks of cholecystitis - Usually asymptomatic - On AXR, aerobilia is seen in 40% of cases - Symptomatic fistulas (malabsorption and steatorrhoea) should be treated with cholecystectomy and fistula closure

5. Gallstone ileus

- Stones causing cholecystenteric fistula pass into the enteric lumen causing intermittent bouts of small bowel obstruction - Accounts for 1-2% of IO overall - Ppt: unexplained gradual onset of SB obstruction - Small stones (<2-3cm) usually pass spontaneously without problems - If >2.5cm and migrated into gut Æ impact at terminal ileum (commonest), duod, sigmoid colon - Mortality is 10-15%, mostly in elderly patients in whom gallstone ileus is more common - Ix: AXR, Barium follow through - Tx: Small bowel enterotomy proximal to the point of obstruction is usually required to remove the stone (Immediate cholecystectomy

not warranted as <4% of patients will have further symptoms) PROGNOSIS - 5% mortality - Nearly all >60years, with DM - Causes: 2° cardiovascular pulmonary complications (older), uncontrolled sepsis with peritonitis and intraabdominal abscess ACALCULOUS CHOLECYSTITIS - Occurs in very ill patients with prolonged stay in ICU – prolonged fasting, poor nutrition, labile blood pressure, sepsis - Poor nutrition leads to biliary stasis, while dehydration and hypotension leads to formation of viscous bile and gallbladder ischaemia Æ bile

may get infected Æ cholecystitis - Treatment involves emergent cholecystectomy / percutaneous cholecystostomy to control acute disease followed by cholecystectomy

174

5. GALLBLADDER CANCER

- uncommon, increasing in China and North India - RF - F:M=2:1, obesity, chronic cholecystitis/ cholelithiasis - Mostly adenocarcinoma, small percentage SCC - Signs and symptoms

Early: mimic gallstone inflammation (RHC pain) Late: biliary and stomach obstruction (jaundice, vomiting, LOA, LOW) Mostly incidental histo finding after cholecystectomy

- Related to gallstones building up (95%) Æ porcelain gallbladder - Commonly spreads to liver, bile ducts, duodenum, stomach - Treatment: cholecystectomy + liver resection and LN dissection +/- chemoRT - Palliative: Endoscopic stent to biliary tree and stomach - Prognosis: poor (most die by 1 year after surgery)

6. CHOLEDOCHOLITHIASIS

PRESENTATION - Obstructive jaundice – tea-coloured urine, pale stools - Biliary colic - If infection sets in Æ cholangitis (see below) BLOODS - FBC (check TW for any rise suggestive of infection) - Amylase (CBD stone may cause pancreatitis) - LFTs (raised bilirubin – direct; ALP raised more than transaminases – also high if LT due to liver damage) ULTRASOUND - Gallstones in gallbladder - Gallstone in CBD (50% missed, esp in distal CBD) - Dilated CBD (normally <8-9mm) >10mm is abnormal In older patients, post-cholecystectomy, or patients on long-term opiates, the CBD may be larger, up to 11-12mm in size

MANAGEMENT - If unsure of presence of stone Æ less invasive investigation such as MRCP, EUS - If likelihood of CBD stone is high Æ ERCP with stone removal

ERCP successful Plan for lap cholecystectomy ERCP failed If patient is well and can tolerate another ERCP, try again (+ stent

in between to drain bile) Operative removal – Open CBD exploration – Lap CBD exploration

- If no facilities to do ERCP: open or laparoscopic cholecystectomy with CBD exploration When to do operative removal of stones (i.e. not suitable for ERCP) - Stone >25mm, Intrahepatic stone, Large number of stones, Impacted stone - Dual pathology, Tortuous duct, Previous Bilroth II (unsuitable anatomy for ERCP)

175

7. CHOLANGITIS

PRESENTATION - Classically Charcot’s triad: RHC pain, fever, jaundice (only 50-70% of patients have the classic triad) - Reynold’s pentad: Charcot’s triad plus mental obtundation and shock - A surgical emergency! (mention in exams!) PATHOLOGY - Usually results from obstruction to the biliary system with infection of stagnant bile - Most common cause is choledocholithiasis (60%);

Consider benign strictures (instrumentation), malignancy (pancreatic, biliary), foreign body (prev instrumentation), PSC, choledochocysts, Mirrizi’s, hemobilia, biliary enteric anastomosis

- Common causative organisms are gram negative bacteria and anaerobes – Klebsiella, E. coli, Enterobacter, Enterococcus - Small proportion (elderly, prev biliary surgery) – anaerobes (bacteriodes, clostridium), developing world – parasites (Clonorchis

sinensis, Ascaris lumbricoides) COMPLICATIONS - sepsis - electrolyte abnormality (dehydration) - infection - coagulopathy (Vit K) MANAGEMENT 1. Resuscitation – “In view of cholangitis being a surgical emergency, I will like to resuscitate the patient who may be in septic

shock”.Anticipate rapid deterioration. - Inform seniors - Obtain good intravenous access and fluid resuscitate as appropriate - Take bloods for investigations – cultures especially - Close monitoring of vitals in HD/ICU

Hrly para + SpO2 Æ Keep MAP > 65mmHg (or to inform doctors if SBP<100mmHg) Catheterise and watch urine output (hrly urine output) – hepatorenal! (keep >0.5mg/kg/hr) CVP line insertion if patient has shock unresponsive to fluid resuscitation (keep 10-12mmHg)

2. Antibiotics (after blood c/s)

- IV ceftriaxone (2g STAT + OM) & metronidazole (500mg STAT + Q8h); imipenem if pt in shock - 7 – 10 days

3. Investigate for cause - ultrasound (preferred if suspecting stones) vs CT (for non-stone cause) etc as above

4. Biliary DECOMPRESSION +/- definitive treatment

- Biliary decompression and definitive treatment can be combined or separate - Timing

usually deferred until 24-48h after admission when pt is stable/improved w Abx Emergency if deteriorating / Abx not improving infection (15%)

- ERCP a) Decompression commonly performed using ERCP Decompression is the primary objective

– endoscopic sphincterotomy + stenting/external drainage (nasobiliary drain: bile duct to nose for continuous drainage)

Success rate 90% b) If cause of obstruction can be treated in the same setting (e.g. stones to be removed) then treat the cause also

- Other methods to decompress: percutaneous transhepatic drainage (esp if neoplastic obstruction) operative decompression

5. Definitive treatment = Cholecystectomy +/- CBDE

- Definitive treatment dependent on: Medical condition of patient Success of biliary decompression Logistical considerations

176

- Choices for definitive treatment: (a) Open cholecystectomy with CBD exploration (b) Laparoscopic cholecystectomy (if CBD exploration is to be performed, need to convert to open – lap CBDE not done

in Singapore Æ must mention during consent taking) CBD EXPLORATION - Cholangiogram or choledochoscopy is performed

1. Cholangiogram involves injection of dye – can image higher ducts 2. Choledochoscopy involves using a scope to visualise the large biliary ducts – cannot image higher ducts, thus not as

sensitive, but can be used to remove stones visualised in the duct Choice of imaging depends on site of obstruction and the cause

- Removal of stones 1. Manual removal with stone-grasping forceps 2. Flushing out stones 3. Dredging stones out using balloon catheter or Dormia basket 4. Lithotripsy

- Consider use of biliary stent or T-tube after removal of stone(s) If there is a lot of instrumentation of the biliary system during the operation, one should anticipate swelling and oedema of the biliary system resulting in post-operative obstruction and buildup of bile Æ higher risk of biliary leakage

(a) Stent – removed later by endoscopy (b) T-tube (usually inserted after CBD-E)

A T-shaped tube with its horizontal limb placed in the CBD and the vertical limb leading out to drain bile Adv

i. Functions as a “pressure release valve” as most of the bile will flow through the horizontal limb of the tube into the distal part of the CBD; only when there is obstruction to flow will bile be diverted out through the vertical limb

ii. Allows for post-op cholangiogram (at POD 9-10) before removal 1. free flow of contrast into duodenum, 2. no residual stones, 3. no free leak of contrast into peritoneum

If all normal Æ release anchoring stitch & exert gentle traction on tube; tube should slip out easily, if not, call for help If stones are present Æ leave tube in for 4-6 weeks to form a fibrous tract Æ allows for instrumentation of tract with a

scope to remove the stones

BILIARY BYPASS (UNCOMMON)

- Consider biliary bypass if there are 1. multiple stones, 2. the CBD is more than 2cm in diameter, or 3. there are strictures (since the CBD has been chronically dilated, quite unlikely that it will function normally even after removal of the obstruction) Mostly replaced by endoscopic stenting. Cholecystojejunostomy preferred over Roux-en-Y choledochojejunostomy.

177

8. MIRIZZI’S SYNDROME

PATHOLOGY - Gallstone in the Hartmann’s pouch compressing the common hepatic resulting in obstructive jaundice - Compression effect is not just physical (the stone) but also contributed by the surrounding inflammation - One of the caveats to Courvoisier’s law GRADING

Type I - No fistula present Type IA - Presence of the cystic duct Type IB - Obliteration of the cystic duct

Types II-IV - Fistula present Type II - Defect smaller than 33% of the CBD diameter Type III - Defect 33-66% of the CBD diameter Type IV - Defect larger than 66% of the CBD diameter

MANAGEMENT - Grade 1: attempt laproscopic cholecystectomy - Grades 2-4: open cholecystectomy with CBD exploration

9. RECURRENT PYOGENIC CHOLANGITIS

BACKGROUND Cholangiohepatitis, or recurrent pyogenic cholangitis (RPC), is characterized by: 1. Recurrent bacterial cholangitis 2. Intrahepatic pigment stones 3. Intrahepatic biliary obstruction. PATHOPHYSIOLOGY x Helminthic infxn (eg Ascaris lumbricoides, Clonorchis sinensis) o epithelial damage o predispose to seeding of coliforms

into biliary system via bacterial translocation o repeated portal bacteraemia Æ cascade of events (biliary stasis, obstruction, stone formation) o recurrent cholangitis

x Malnutrition Æ deficiency of enzymes Æ calcium bilirubinate pigment stones formation Æ recurrent biliary obstruction o recurrent cholangitis

HISTORY: - A history of recurrent attacks of cholangitis – typical hx: 1-2 episodes of fevers, jaundice, and RUQ abdominal pain per year Hx of prev biliary surgery, endoscopic procedures, or percutaneous biliary drainage procedures.

- Complications of pyogenic cholangitis cirrhosis with portal hypertension cholangiocarcinoma

178

PHYSICAL EXAMINATION No specific physical findings are evident in RPC. Dx based on history. DIFFERENTIALS Primary Sclerosing Cholangitis INVESTIGATIONS For diagnosis For Complications Bloods - FBC - LFT with ALP>ALT, AST - Prothrombin time: N/n (if prolonged cholestasis causes fat malabsorption and vitamin K deficiency) Impt to exclude – correct with parenteral Vit K before invasive procedures

- Blood C/S: bacteremia – results help guide antibiotic choice. - Ova and parasites: RPC freq a/w Clonorchis infxn – look for it and treat when present. Radiology - U/S HBS segmental biliary dilatation hepatolithiasis liver abscesses helps determine choice of supplemental axial imaging techniques.

- ERCP or PTC – imaging modality of choice for delineating the biliary tree. - CT scan centrally dilated bile ducts with peripheral tapering bile duct stones pyogenic liver abscesses.

TREATMENT PRINCIPLES: - Treat current infection - Biliary drainage - Management of other complications e.g. dehydration etc Surgical - Usual surgical approach includes: Initial biliary decompression – ERCP sphincterotomy / stent placement Definitive biliary drainage procedure – e.g. Roux-en-Y choledochojejunostomy

PROGNOSIS - Death occurs in approximately 15-20% of patients over 5-6 years.

179

10. CHOLANGIOCARCINOMA

SITE - Intrahepatic/peripheral 10% - Distal 25% - Perihilar 65% (Altemeier-Klatskin tumour)

Bismuth classification i. Type I: below confluence of hepatic ducts ii. Type II: tumour reaching confluence iii. Type IIIA/B: involving common hepatic duct and either right or left hepatic duct iv. Type IV: multicentric or involving confluence and both hepatic ducts

ASSOCIATIONS - Related to chronic cholestasis: Primary sclerosing cholangitis / Ulcerative colitis Parasitic infection – Clonorchis sinensis, Opisthorchis viverrini Hepatolithiasis Recurrent pyogenic cholangitis

- Bile duct adenoma - Choledochal cyst (20-25% in Type 1)

Caroli’s disease = congenital cystic dilatation (multifocal segmental dilatation of large intrahepatic bile ducts) - Thorotrast exposure (radioactive Xray contrast) - Instrumentation PRESENTATION

- Painless jaundice (painful if there is cholangitis) - Acholic stools - Pruritus - Advanced signs and symptoms: Abdominal pain Fatigue, malaise Weight loss Hepatomegaly

DIAGNOSIS U/S detects biliary dilatation but not useful for ductal lesions. - CA 19-9 >100μl/ml (good sensitivity of 89%, specificity 86%) - Contrast CT - PTC: when biliary obstruction from distal end. 2 functions: 1) roadmapping for surgery; 2) drainage of obstructed system if

ERCP cannot drain - ERCP: can obtain brushings for cytology, less bile leak than PTC - Selective celiac angiography: aid in determination of surgical resectability by identifying tumor involvement with adjacent blood

vessels CURATIVE TREATMENT

- Surgery is the only chance of long-term cure - Only 25% of tumours are resectable - Contraindications to surgery Bilateral or multifocal intrahepatic disease (2nd order biliary radicles in both hepatic lobes) Invasion of portal vein trunk or hepatic artery Bilateral involvement of hepatic arterial or portal venous branches Unilateral hepatic vascular invasion with contralateral ductal spread Distant metastases

180

PROGNOSIS FOR RESECTABLE DISEASE (5-year survival)

- Intrahepatic: 35-45% - Distal 35-45% - Perihilar 10-30% (worse prognosis due to early lymphatic spread) PALLIATION

- Endoscopic/percutaneous transhepatic biliary stenting - Bilateral drainage for hilar cholangiocarcinoma - If after opening up and finding that tumour is not resectable, can perform surgical bypass

181

12. BREAST DISEASES 1. ANATOMY OF THE BREAST

- The breast is a modified sweat gland that lies in the subcutaneous tissue of the anterior chest wall between the superficial and deep layers of the superficial fascia

- The base of each breast extends from the lateral border of the sternum to the mid-axillary line, from the second to the sixth rib - The axillary tail pierces the deep fascia and enters the axilla - Each mammary gland consists of 15-20 lobules that are drained by lactiferous ducts that open separately on the nipple - Fibrous septa (Cooper’s ligaments) interdigitate the mammary parenchyma and extend from the posterior capsule of the breast

to the superficial layer of fascia within the dermis, and provide structural support to the breast (involvement of these ligaments by malignancy causes dimpling of the overlying skin)

- Lymphatic drainage:

1. Axillary nodes – 75% of ipsilateral breast drains to the axillary nodes

1. 40-50 nodes in 5 groups: Anterior, posterior, medial, lateral, apical 2. Drains into supraclavicular and jugular nodes

Anatomic/ Surgical division into levels I, II and III by the pectoralis minor muscle: Level I: lateral to pectoralis minor – usu e first LN where BrCA spreads to Level II: posterior to pectoralis minor Level III: medial to pectoralis minor, extending up to apex of axilla

2. Internal mammary nodes--20% of drainage from the ipsilateral breast

upper and lower inner quadrants About 4 nodes per side, with one node in each of the first three interspaces and one in the fifth or sixth

interspace 3. Interpectoral (Rotter’s nodes) – between pec major and pec minor muscles

2. PRESENTATION OF BREAST DISEASES

1. Lump (painful vs painless) 2. Pain (cyclical vs non-cyclical) [Î more likely benign] 3. Nipple changes or discharge

182

3. APPROACH TO A BREAST LUMP

DIFFERENTIALS (AGE DEPENDENT) Painless lump Painful lump

Elderly: (post-menopausal) 1. Carcinoma Younger: 2. Cyst 3. Fibroadenoma - can get cyclical pain (swell with the rest

of the breast cos it’s breast tissue) 4. Area of fibroadenosis (nodularity)

1. Area of fibroadenosis 2. Cyst 3. Abscess (usually in lactating women) 4. Fat necrosis (minor trauma) 5. Periductal mastitis 6. Galactocoele (lactating women) 7. Carcinoma (rare; ~10% & advanced)

May not be painful if pt has received treatment / some infx.

Cyst, fibroadenosis and carcinoma can be painful/ painless. HISTORY 1. History of lump

- Site of the lump? - Single or multiple? – multiple: fibroadenoma / cysts / fibroadenosis. - When & Why was it first noticed? (Pain, self-examination, etc)? - Painful or painless? - Overlying skin changes noted:

Erythema, warmth Dimpling: shortening of Cooper’s ligaments due to tumor pressure? Any general asymmetry of the breasts noticed? Discharge – ind CA invasion

- Duration since first noticed – long duration unlikely CA - Any increase in size from first noticed to now? - Any changes in the nipple e.g. retraction - Nipple discharge? If present, what is the colour and consistency? - Any other lumps elsewhere – other breast? Axilla? Neck? - Has the lump been treated before?

RF – to sieve out ppl carrying BrCA gene, ID high risk patient (to continue f/u even if lump benign)

2. Oestrogen exposure history Increased risk: - Age of menarche (early <12YO Æ increased risk) - Age of menopause if applicable? (>55YOÆ increased risk) - Use of HRT (>1yr) – cumulative risk with duration of therapy and/or Oestrogen based OCP (not proven) ‘Confers protective effect’: - How many full term pregnancies? (nulliparity Æ increased risk) - Age at which first child was born (>30 years old) - Whether patient breastfed her children, and if so, for how long

3. Other risk factors for cancer A. STRONGEST RF:

Family history of cancers* in paternal (BRCA2) and maternal side (BRCA 1&2), especially if cancer occurs in: a. first degree relative below the age of 40, in bilateral breasts b. >/= 2 relatives with Breast CA around 50yo

*Includes breast, ovarian, prostate and CRC. Lifetime risk in gene carrier – 80%.

B. Previous breast disease: a. Treated cancer b. Previous biopsy showing atypical ductal hyperplasia or LCIS

C. Exposure to ionising radiation (esp. RT for previous breast disease) D. Daily Alcohol intake, especially before age of 30 (link has been shown)

4. Systemic review

- LOA, LOW (constitutional) - Fever (infective cause) - Bone pain, SOB, headache (metastasis)

183

PHYSICAL EXAMINATION Preliminaries (HELP) - Hi: Introduce yourself & ask for permission to examine the breast

Always have a chaperone to accompany you if you are male - Expose patient adequately from the waist up with exposure of axillae - Lighting: good - Position the patient at 45o or sitting position if a bed is not available Inspection - General appearance - Patient’s hands relaxed at her sides – look for:

any asymmetry in the breast contours, any obvious skin changes

peau d’orange –infiltration of malignant cells into lymphatics causing edema (not compression), erythema, puckering

any scars of previous operation or procedure e.g. punch biopsy - Look for nipple changes (7 D’s):

Discolouration Discharge

Depression (retraction) Deviation Displacement

Destruction (Duplication – unlikely)

- Manouvres: 1. Ask patient to raise her arms (to see the axilla and underside of breasts, and accentuate any tethering to skin Î dimpling) 2. Ask the patient to contract the pectoralis major (push her hands against her hips) Î may reveal a previously unnoticeable lump if tethered to both skin and muscle 3. Press arms down & lean forward Æ the rest of the breast will flop fwd but not the CA that is attached to underlying muscle

Palpation - Patient should be lying down at 45 degrees to the horizontal with her hand tucked behind her head – this splays the breast out

so it can be palpated properly - Start with the normal side first! - Ask for any pain before starting to palpate - Use one hand to retract and stabilise the breast and palpate with the other - Palpate in a systematic manner e.g. quadrant by quadrant from centre outwards - Examine the entire breast including the axillary tail - When the lump is located, check with the patient whether this is the same lump - Characterise the lump: Site (which quadrant) Size Shape (hemispherical/ oval) Surface (smooth or nodular/irregular) Consistency (soft, firm, or hard) Tender or non-tender Warmth of overlying skin Fluctuance Margins (regular and smooth, or irregular and ill-defined) 1. Fixation to the skin – try to pick up the skin above the lump 2. Fixation to underlying muscle – Move lump in 2 perpendicular directions. Then ask patient to press her hands against

her hips to contract the pectoralis major muscle, then try to move the lump in 2 perpendicular directions. (no need to do if already attached to chest wall)

- Continue to examine carefully for other lumps - Ask patient if she can show you the discharge by expressing it herself (NEVER squeeze the nipple yourself!)

If patient cannot do it, then ask the chaperone to help / squeeze yourself by milking the breast from outside in (ideally with patient sitting forward)

Check list: - Breast - Nipple - SC LN - Spinal Tap - Lung effusion - Hepatomegaly

“I feel a lump in the upper outer quadrant of the Right breast. This lump is NOT WARM, and NON-TENDER, is hemispherical with poorly defined edges, measuring ___X___CM. It is firm in consistency with a irregularsurface and is NOT FLUCTUANT. It is NOT FIXED to the SKIN ………….(ask her to contract the pect maj)…. ………….And NOT FIXED to the underlying muscle”

184

Axillary lymph nodes - Palpate the normal side first - Rest the pt’s right forearm on your right forearm and use your left hand to palpate the right axilla (vice versa for the left side) - Palpate gently, slowly, and systematically, covering the major groups of nodes: anterior, posterior, medial, lateral, and apical - If any lymph nodes are found to be enlarged, note the number of lymph nodes, their site, size, tenderness, consistency (firm,

hard, matted), mobility To complete the examination - Examine the the supraclavicular LNs & cervical LNs - Examine the lungs for any pleural effusion - Percuss the spine for bony tenderness - Examine the abdomen looking for hepatomegaly FINDINGS FOR THE COMMON BREAST LUMPS

Type of lump Age Pain Surface Consistency Mobility Cyst 30-

55 Occ Smooth Soft to hard Not fixed

Nodularity 20-55

Occ Indistinct Mixed, fluctuant

Not fixed

Fibroadenoma 15-25

No Smooth, bosselated

Rubbery Very mobile

Cancer 35+ No Irregular Stony hard May be tethered or fixed

185

INVESTIGATIONS “The evaluation of a breast lump is via the TRIPLE ASSESSMENT – (i) Clinical examination;; (ii) Imaging;; and (iii) Histology.” Imaging 1. Mammography: always cf Prev MMG (alert if any CHANGE)

- Most sensitive of the proven breast imaging modalities (Gold std) - Purposes: Dx Screen the rest of the Breast Æ helps to plan treatment (possibility of breast conserving Sx) Screen contralateral breast (in case B/L BrCA – rare) Baseline for future f/u for contralateral breast (Br CA is RF for CA development)

- Usually performed in asymptomatic older women (>35YO) [breast tissue in younger women is denser; more difficult to pick up abnormalities], but <35YO in symptomatic women

- Normally, 2 views are done: craniocaudal (CC)

right /Left 70% tumours in lateral quadrant (upper)

mediolateral oblique (MLO) captures the tail right/ left 80% tumours in oblique milky way

- Additional specialised views: magnification and coned compression; done on request to

help magnify areas of abnormality or help visualise breast better - Abnormal features:

(a) Neo-density or asymmetric density (look for bilateral synchronous ca; satellite lesion) (b) Microcalcifications (<0.5mm in size)

- If calcifications >0.5mm Æ macrocalcifications; >5/mm2 Æ cluster - Sole feature of 33% of cancers detected on mammography - Causes:

A. DCIS, B. invasive cancer, C. fibrocystic disease, D. papilloma

- Features of malignancy: 1. pleomorphic microcals, heterogeneous appearance; segmental 2. closely grouped or arranged in a linear pattern (ductal distribution), 3. underlying density

- Features of Benign microcals: punctate, “tea-cup” appearance (c) Spiculated mass or stellate lesionwith poor outline or comet sign

- 95% of spiculated masses on mammography are due to malignancy - Stellate lesion is a localised distortion of the breast parenchyma without perceptible mass lesion – high chance of

it being malignant - Causes:

A. Invasive cancer, B. radial scar (benign), C. fat necrosis, D. abscess, etc

(d) Architectural distortion (of the contour), tent sign , nipple changes - Look at the axilla on the MLO view for any enlarged lymph nodes

All 3 must be concordant for benign to have >99% specificity to r/o malignancy

BI-RADS (Breast Imaging Reporting and Data System) classification Category 0: Need additional imaging evaluation Category 1: Negative (nothing to comment on, 0.05% risk still present) Category 2: Benign Category 3: Probably benign, short-term follow-up suggested (<0.2% risk) Category 4: Suspicious, biopsy should be considered (25-74% risk) Category 5: Highly suggestive of malignancy (75-99% risk) Category 6: Known malignancy

70%

80%

Comet sign: enhancing lesion with a tail (directed towards nipple). Indicates early DCIS. DDx – prev Bx / Sx to that area.

186

2. Ultrasound - Usually used as the 1st investigation in young patients (<35 years old) or pregnant, lactating patients – as mammogram has radiation

(small amount); not the gold standard for screening - Used when there is a breast lump:

A. Guide procedures e.g. Biopsy, drainage of abscess, aspiration of cyst B. Evaluates consistency (solid vs cystic) &margins C. Problems with Mammogram

Localisation of lesion seen in only one mammographic projection Evaluation of a palpable mass with a negative mammogram Evaluation in mammographically-difficult areas e.g. chest wall, axilla

- Pitalls: Operator dependent, non-standardised techniques, poor resolution, Unable to detect most microcalcifications

- Features of malignancy: 1. Markedly hypoechoeic with + thick echogenic halo 2. Irregular edges; Destruction of surrounding structures 3. Hypoechoeic shadowing; Posterior acoustic shadowing 4. Taller than it is wide (fir-tree appearance; invasion of fascia) 5. High central vascularity – Doppler to evaluate BV 6. Microcalcification (if large number)

3. MRI of the breast

- Expensive, but Good soft tissue definition without radiation (>90% sensitivity) - Useful for pre-menopausal women with dense breast - Look for contour, enhancement, washout kinetics (how fast/ whether dye stays) - Indications:

1. Pre screening: Screening in high risk patient (BrAC gene +: yearly MMG and MRI surveillance) 2. Post screening

a. Occult lesions: Axillary LAD but Mammogram & US -ve b. Suspicion of multifocal or bilateral malignancy (esp ILC)

3. Pre treatment: When planning for breast conservation surgery 4. Post treatment: Assessment of response to neoadjuvant chemotherapy

Histology - Options available:

(a) Fine needle aspiration cytology (b) Core biopsy (Trucut/ mammotome) (c) Incisional biopsy – remove part of lump (usually not done now except Frozen section intra-op) (d) Excisional biopsy – remove entire lump

(c) and (d) Æ GA, larger wound. - Mostly a choice between FNAC and core biopsy

FNAC is less invasive, less painful, smaller wound (23G needle), does not require any local anaesthetic, cheap, repeatable.

But only cells are obtained with no histology Æ cannot differentiate between in-situ cancer and invasive cancer, requires skilled cytopathologist If FNAC shows malignant cells, do frozen section intraop before proceeding with surgery (recommended).

Core biopsy is more invasive, requires local anaesthetic, will result in a larger wound (14G needle), more painful, risk of

complications higher (because biopsy needle is a spring-loaded firing mechanism, improper angling may result in puncture of the lung or heart), more ex as requires tissue processing. But can obtain tissue specimen, can stain for ER/PR status Æ better diagnostic value

Should have ‘tissue diagnosis’ if you are subjecting patient to cancer treatment! - Guided by clinical palpation or radiologic guidance[Æ more accurate, not 100%] US guidance Stereotactic guidance (stereotactic mammotome) – stereotactic = Xray from 2 angles, mammotome = vacuum-assisted biopsy device

(able to sample larger tissue volumes)

MANAGEMENT - If triple assessment suggests a benign lump (i.e. all 3 are concordant), follow up with physical examination for 1 year (q3-6mths) to ensure

the lump is stable or regresses - If all 3 concordant for malignancy Æ further staging and treatment - If 1 or 2 of 3 aspects suggest malignancy Æ further workup, Æ excisional Bx?

187

4. APPROACH TO NIPPLE DISCHARGE

CAUSES

Colour of discharge Cause Red or pink (blood + serum) Ductal papilloma (benign)

Ductal carcinoma Clear yellow (serous) Ductal papilloma

Duct ectasia (= periductal mastitis) Cyst Ductal carcinoma

Green, brown, black (cell debris) Duct ectasia Purulent, foul-smelling Mastitis/abscess Thin, white fluid (milk) Galactorrhoea/lactation

HISTORY 1. Is the discharge true?

- Exclude other conditions may mimick e.g. eczema, Paget’s, etc 2. Is the discharge significant?

- Spontaneous or only on pressing (spontaneous is sig) - Intermittent or persistent (persistent is sig) - Relation to breastfeeding (significant if >1yr after stopping breastfeeding)

3. Is the discharge worrisome?

- Unilateral or bilateral (unilateral more worrisome) - Discharge from multiple ducts or single duct (single duct more worrisome) - Nature of discharge (bloody more worrisome) - Age of the patient (more worrisome in older patient >60)

4. Is it troubling the patient? PHYSICAL EXAMINATION (as described above) INVESTIGATION 1. Discharge for cytology to detect malignant cells 2. Mammography/ US of both breasts to detect any underlying malignancy 3. Histology of biopsied lesion if found on imaging 4. Ductography, ductoscope & biopsy MANAGEMENT - If malignancy found, manage malignancy - Excision for intraductal papilloma (microdocholectomy, total ductal excision, hookwire locailised excision) – scarring may affect

other ducts too - Antibiotics for mastitis/abscess + incision and drainage for abscess - Conservative management for most other pathologies unless discharge persists and is troubling patient Æ microdochectomy

of offending duct

188

5. BREAST CANCER

EPIDEMIOLOGY - Most common cancer in females in Singapore: - Age-standardised incidence 55 per 100,000 in 2002; incidence is half that of the West - Bimodal age distribution: 45-55YO & older (>75YO) - Gender ratio is about 100-150 female :1 male RISK FACTORS 1. Age (increases with increasing age with two peaks as mentioned) 2. Genetic:

- Family history: maternal & paternal (breast or ovarian cancer, especially if in first degree relative, young onset <40 YO, bilateral cancer in relative affected)

- Known BRCA1 (on 17q; maternal side) or BRCA2 (both sides), - Li-Fraumeni syndrome involving p53 mutation)

3. High oestrogen exposure: - early menarche <12YO, nulliparity, late childbearing at >30YO, late menopause >55YO - Oral contraceptive usage (pure oestrogen type) - HRT (>5yrs, small increase in risk; reduced when stopped)

4. Previous breast disease: - Previous breast cancer (10X) - Previous biopsy with atypical ductal hyperplasia or LCIS (7-10X)

5. Ionising radiation to breast (previous RT) 6. Alcohol consumption (daily) PATHOLOGY (WHO classification: epithelial and non-epithelial tumours.) - Non-epithelial tumours arise from supporting stroma (e.g. angiosarcoma, malignant phyllodes tumour, primary sarcomas); very uncommon - Epithelial tumours arise from cells lining the ducts or lobules, and can be further divided into invasive and non-invasive based on invasion of

the basement membrane

Non-invasive Invasive

Ductal DCIS = malignant - From terminal duct lobular unit, - Cause distortion of lobules, - Do not invade BM - Non-palpable, detected microcals - 35% multicentric, occult invasive ca in 10-20% - Progress to CA within 10 yrs ~30% risk; considered

malignant - Good prognosis if treated - Tx similar to IDC

IDC - 70-80% of invasive breast cancer - Includes all cancers that cannot be subclassified into a

specialised type Æ “no special type” - Poorer prognosis than a carcinoma of specialised type - 2/3 express ER/PR, - 1/3 overexpress C-erbB2

Lobular LCIS = RF - From terminal duct lobular unit (like DCIS) - Do not distort lobular architecture - Usually presents with a lump, seldom detected by

mammo as rarely microcal. - 60-80% multicentric and bilateral - Not premalignant, but a marker for increased risk of

invasive disease in both breasts (7-10x increased risk) - If ca develops, will be IDC usually, occurs >15 years

after diagnosis - Usually proceed with excision biopsy.

ILC - 5-10% of invasive cancers - 10-20% multicentric and/or bilat - Cells morphologically similar to cells of LCIS:

monomorphic, bland round nuclei - Cells invade individually into stroma (due to loss of E-

cadherin, a cell-adhesion molecule) - Similar prognosis to IDC

Others Specialised types - Medullary, colloid (mucinous), tubular, papillary - Better prognosis than IDC Inflammatory carcinoma - Presents as erythematous. enlarged, swollen breast w/o palpable mass - Histologically not specialised - Diffuse invasion of breast parenchyma by ca cells blocking numerous dermal lymphatic spaces Æ swelling - No histo features of inflammation - Very poor prognosis, rapidly fatal

189

SPREAD

- Local: skin & subcut tissues, underlying ribs and muscle (chest wall) - Lymphatics: axillary, internal mammary LNs, supraclavicular LNs - Haematogenous: lungs, liver, brain, bone, adrenals, ovaries

PRESENTATION - Asymptomatic: detected on mammographic screening - Local:

- Self-detected lump in the breast (>1/3 of patients) - Nipple change: distortion, destruction, retraction, deviation, discharge, eczema - Overlying skin changes e.g. peau d’orange, tethering (means mass is still mobile but overlying skin will be indented when moving the

lump), fixation (means the mass is not mobile), even fungating ulcer - Other lumps in axilla - Pain is uncommon.

- Constitutional: LOW, LOA - Metastatic: bone pain/ #, SOB (metastases to lung, liver, LNs, bone, brain, adrenals) DIAGNOSIS – BY TRIPLE ASSESSMENT (see above) STAGING - Triple assessment can help divide it into:

- DCIS or early Breast Cancer (BC) (at most with small mobile axillary LNs) - locally advanced BC (matted LNs, skin and rib involvement)

o Î continue to stage to look for metastasis in advanced BC - Staging Investigations:

(i) CXR (for lung metastases; look for isolated hyperdensity) (ii) U/S HBS (iii) Bone scan (iv) LFT (raised ALP) + Hepatitis screen (may flare up with chemoTx) (v) CT or MRI brain – if patient symptomatic (vi) CT thorax , abdomen (for liver and adrenal metastasis) – in locally advanced BrCA

T stage N stage M stage

Tis: Carcinoma in-situ, Paget’s with no tumour

T1: <2cm T1a – 0.1 to 0.5 cm T1b – 0.5 to 1.0 cm T1c – 1.0 to 2.0 cm T2: 2 to 5 cm T3: >5cm T4: T4a – Chest wall involvmt T4b – Skin involvmt T4c – Both 4a and 4b T4d – Inflammatory ca

N1: Mobile ipsilat axillary nodes N2: Fixed/matted ipsilat axillary nodes N3: N3a – Ipsilat infraclav nodes N3b – Ipsilat int mammary nodes N3c – Ipsilat supraclav nodes

M1: distant mets

Stage 0 Stage I Stage II Stage III Stage IV Tis DCIS

T1N0

Early

T2N0, T3N0 T0N1, T1N1, T2N1

Breast cancer

*skin, rib inv., matted LNs T3 N1 T0N2, T1N2, T2N2, T3N2 Any T, N3 T4, any N

Locally advanced BC

M1

Advanced BC Aaaaa – Stage Xb (e.g. IIb, IIIb)

5 yr Survival

Stage I: 90% (70% in 10 yrs) Î 95% with adjuvant Rx Stage II: 60% (40-50%) Î 76% with adjuvant Rx Stage III: 30% (20-30%) Î 50% with adjuvant Rx Stage IV: 10% (<2%)

190

PROGNOSIS Prognostic factors: - to predict RECURRENCE rate to decide on the SYSTEMIC TREATMENT 1. Patient factors - ability to tolerate chemo - Age (younger patient Æ higher chance of recurrence, progress of disease) - Comorbidities - +/- Social support

2. Tumor factors

- Stage of disease – tumour size, lymph node involvement [Major prognostic factor] - Grade of tumour - Lymphovascular invasion

3. Predictive factors (predict response to a Tx):

- C-erbB2/Her-2 positivity indicates more aggressive tumour Æ worse - ER or PR positivity is good – predicts 90% response to treatment with tamoxifen; also means tumour is less

undifferentiated

p53 mutation NOT for prog purpose. Recurrence score: to identify people who will recur

Î using gene typing

THERAPEUTIC OPTIONS Options can be divided into aims of control (with either curative of palliative intent): (a) Locoregional control:

Surgery (WEAC vs SMAC) Radiotherapy

(b) Systemic control: (size >10mm; <70YO) Chemotherapy Hormonal therapy Targeted therapy

Surgery 1. Preparing for operation:

- Anaesthesia workup and necessary imaging. Mark out the site - Psychological counselling, consent taking, discuss breast reconstruction

2. Wide excision (breast-conserving surgery; standard of care) - Removal of tumour with clear margins, while achieving good cosmetic result - Criteria: [Nodal status does not influence decision for WE or SM]

1. Only 1 tumour, not multicentric/ multiple DCIS/ LCIS (multifocal) unless same quadrant 2. No metastatic disease 3. Appropriate tumour size-to-breast ratio (to achieve good cosmetic result) - not about T staging anymore 4. Previously conserved breast 5. Patient must agree to post-operative radiotherapy (daily RT in hosp) 6. No CI to RT

a. not PREGNANT (the only absolute CI) b. No CTD –underlying inflammation may get worse c. No previous RT to the chest

- Results: overall survival at 25 years for WEAC comparable to SMAC, with Slightly higher local recurrence rates (for WEAC: 1% per year, 4% in 5 years)

- Higher risk in younger patients as cancer tends to be more aggressive

Aim of adjuvant therapy: 1. Prevent local recurrence

a. RT b. ± CT, HT, TT

2. Eradicate micrometastasis a. CT, HT, TT b. ± RT

Î Reduces recurrence by 1/3; but if recurs have poor prognosis

191

2. Simple mastectomy (if any CI to Wide excision) - Removal of breast tissue, nipple-areolar complex, and overlying skin - Ind

1. CI to wide excision 2. Patient prefers to remove entire breast 3. Need to remove nipple (WE cannot give good cosmesis)

- Lower rates of local recurrence; similar long term prognosis as WE (Italian trial) - If done for DCIS, dont need to give adjuvant therapy

3. Axillary clearance

- Purpose of removal: NODAL STAGING (for prognostication) - does not confer survival benefit - Routinely be done in (1) Clinically palpable LN / detected on US (2) BrCA >/= T2 (5cm) - Not required for DCIS (theoretically cancer cells are confined to the breast) if diagnosed on excision biopsy / wide

excision. Recommended if diagnosed on Core biopsy (sampling error). - Complications: see below - Sentinel lymph node (SLN) biopsy (SLN) is a new standard of care Only in Early CA (</= 5cm) Principle: the sentinel lymph node, being the first lymph node draining the breast, is representative of the rest of the

axilla; if the SLN is negative for tumour cells, then the rest of the axillary nodes should be negative as well o Solitary internal mammary LAD is rare

Use of blue dye (isosulphan blue, methylene blue) or radioactive isotope (Tc-99 sulphur colloid or colloidal albumin) injected in the area of the breast just before surgery Æ concentrates in the first lymph node (sentinel node) that drains the breast after 5mins

During the op, look for the SLN by colour, or using a Geiger-Muller counter to detect the node with highest radioactivity. Send node for frozen section (FS)

If -ve do not clear axilla; if +ve, perform axillary clearance False –ve rate of SLN Bx <5%; false –ve rate of FS ~ 33% Î if so, re-explore if histology +ve and do AC No difference in axillary recurrence between AC vs SLN biopsy

4. Palliative surgery

- Palliative mastectomy for symptoms (bleeding, fungating, infected tumour) - 'toilet mastectomy' - Surgery at other sites:

Fixation of pathological fractures, decompression of spinal cord compression, surgical excision of brain metastases

5. Breast reconstruction

- Safe, can be done during breast surgery or at a later time - No delay in subsequent treatment and no increase in rates of relapse - Cx: abnormal sensation of breast, unable to breastfeed - Options:

(i) Prosthesis – 6 wks post-op (ii) Muscle flap from rectus abdominis or latissimus dorsi

Complications of surgery

Early Haemorrhage (POD1) Wound Infection (POD3) Seroma formation (accumulation of serum) in 50% Flap ischemia

Late Cosmetic deformity Complications of Axillary Clearance: - Lymphoedema – RT to axilla is contraindicated with AC as it worsens oedema - Cellulitis – even in minor trauma, due to lymphoedema. Need to clean even minor

wounds with antiseptic solution + prophylactic ABx vs staphstrep - Shoulder stiffness – require physiotherapy - Intercostobrachial nerve transection – numbness over inner aspect of arm.

Other forms of mastectomy (modified raical, radical or extended radical mastectomy) are not performed anymore.

192

Radiotherapy 1. Adjuvant: to decrease local recurrence rate

- (a) Usually done 6/52 after WE - (b) High risk of local recurrence: >/= 5cm OR >/= 4 LN +ve - Targeted at Breast with or without internal mammary, infra/supraclavicular LN (Axillary LN are tackled during Surgery) - CI: pregnancy, previous RT, patient choice - Regimen consists of 25 to 30 cycles in total, 1 cycle per day from Mon - Fri over 5-6/52 until max dose (no repeat RT for recurrences) - Cx: radiation injury (e.g. pneumonitis, skin changes), risk of cancers 1 in 2000 in 20yrs

2. Palliative

- Brain mets - Bone mets to painful areas / impending fractures

Chemotherapy (polyCT with 3 drugs is better; 4-6cycles, each over 1/12) - Main purpose: eliminate micrometastasis! 1. Neoadjuvant

(a) Given in Locally advanced breast cancer (stage III) to shrink the tumour before surgical resection (b) To shrink tumors before breast conserving Sx - 20% achieve complete clinical response (cCR) i.e. tumour is no longer palpable

further 20% will achieve complete pathological response (cPR) i.e. no more tumour cells = good prognosis - Place clip into tumour before neoadjuvant therapy to guide surgery in case tumour “disappears”;; operate according to preop staging - Cx: as for CT drug, e.g. mouth ulcers, N/V, hair loss, immunosuppression (main disadvantage pre-op)

2. Adjuvant

- Start 3/52 after surgery; given in stage III / LABC (LN+ve) & in some early breast cancers depending on stage (see below) - Premenopausal patients tend to have better response to chemotherapy than hormonal therapy (vice versa for postmenopausal patients) - Main active agents: anthracyclines (e.g. doxorubicin, epirubicin) and the taxanes (e.g. paclitaxel, docetaxel) - Common regimens: AC (anthracycline, cyclophosphamide), FAC (5-FU, anthracycline, cyclophosphamide), CMF (cyclophos,

methotrexate, 5-FU)

3. Palliative - Anthracyclines and taxanes are the mainstay - Helps to reduce load of disease to alleviate symptoms, increase survival

Hormonal therapy - Used in adjuvant setting to eradicate micrometasis (likewise with CT & TT) - For ER/PR +ve Î will have 90% response - Preferred for postmenopausal women as response to hormonal therapy is better - May render patient postmenopausal for better response to HT via medical ablation with LHRH-a or surgical oopherectomy - Mostly used as adjuvant therapy but can also be used as palliative treatment & preventive treatment in high risk patients

- Also reduces risk in contralateral breast (a) Selective oestrogen receptor modulators (SERMs): Tamoxifen

- 50% reduction in recurrence, 25% reduction in mortality - taken daily for 5 years - CI: PHx of CVA/DVT, immobile patients - Side effects: 1. menopausal symptoms (hot flushes, etc),

2. endometrial cancer (0.1% per year), 3. deep vein thrombosis

(b) Aromatase inhibitors: Lanastrazole, letrozole, exemestase

- Inhibit peripheral conversion of testosterone and androstenedione to oestradiol (still present in post-menopausal women) - Only suitable for post-menopausal patients as use of these agents will cause overactivity of the HPA axis in premenopausal women - Side effects: 1. musculoskeletal pain,

2. osteoporosis! Targeted therapy - Herceptin (trastuzumab)

- targets Her-2-neu a.k.a. C-erbB2 receptor (an epidermal growth factor receptor [EGFR] that is overexpressed in 18-20% of cancers) - Used in C-erbB2 positive tumours, early or late stage - Her-2-neu indicates worse prognosis Æ Herceptin improves prog to normal - Side effects of Herceptin: 1. cardiomyopathy & CCF

2. pulmonary toxicity, 3. infusion reactions, 4. febrile neutropaenia

- Avastin (or bevacizumab, targets vascular endothelial growth factor [VEGF] receptor, used in advanced cancer); - Lapatinib (targets Her-1 and Her-2, used in advanced cancers)

193

TREATMENT BY TUMOUR STAGE Tumour can be divided into

1. in-situ cancer, early BC, locally advanced BC Î curative intention 2. Advanced BC Î palliative intention

1. DCIS

- Same Tx as invasive carcinoma: WE vs SM - Sentinel LN biopsy if DCIS not very certain (ie diagnosed on Core biopsy) - ± hormonal therapy (only if WE) to reduce recurrence at surgical site; tamoxifen reduces overall breast cancer risk by

50% in both breast Æ not adjuvant therapy 2. Early breast cancer (stage 1 &2)

- T2 or less (<5cm), N1 or less (no nodes or non-fixed nodes) - Locoregional therapy: SMAC, or WEAC with postop RT - Adjuvant therapy Purpose of adjuvant therapy is to destroy systemic micrometastases Likelihood of patient having micromets is deduced from T and N stage (major prognostic factors):

Chemo Intermediate No chemo

T >20mm, N stage >1

11mm < T < 20mm, N=0

Look at histological grade

(minor prognostic factor); if high grade Æ chemo

T <10mm, N=0

Adjuvant therapy: CT if tolerable and/or HT if ER/PR +ve In general: In premenopausal pt Æ chemotherapy + hormonal

In postmenopausal pt Æ hormonal + chemotherapy 3. Locally advanced breast cancer (stage 3)

- T3 or T4 (tumour >5cm or skin/chest wall involvement), N2 or N3 (fixed lymph node involvement or supraclavicular node involvement)

- Surgical resection dependent on size of tumour and resectability (if tumour is too large, the skin defect will be very large Æ inoperable)

- Systemic therapy: Neoadjuvant chemo to (1) downstage inoperable tumour (in addition, it helps by predicting the tumour response to

chemotherapy before resection) and (2) eliminate micrometastasis Adjuvant therapy after resection – CT & HT (depends on the histology etc after surgery, ie does patient still require

adj tx) Usually not done if patient has completed the course of chemo during neoadjuvant. 4. Advanced breast cancer (stage 4) - aim for dz control

- Distant metastases: treat symptoms and prevent potential problems - Minimal locoregional therapy except for palliative purposes (new study shows that removing primary in metastatic BrCA

confers survival benefit) - Systemic therapy is the mainstay of treatment – CT, HT or TT (to prevent worsening of mets) - Mets specific:

1. Bone: RT to painful areas, Fixation/RT to impending fractures, Decompression if cord compression Treat hypercalcemia from mets

2. Brain: RT 3. Pleural: drain effusion 4. Liver (usually liver failure > obstructive jaundice)

FOLLOW-UP - symptoms + P/E

- 3-monthly for first 2 years - 6-monthly for the next 3 years (i.e. third to fifth years) - Yearly for another 5 years (to tenth year)

- MMG: same breast 1yr postop; then 2-yrly bilateral mammo for life (previous BC is a strong Risk factor for future Breast cancer) - CEA and CA15-3 are used in some centres for trending

194

BREAST SCREENING

Normal risk, asymptomatic

40-49 YO Annual mammogram 50-64 YO Biannual mammogram (by invitation) >65 YO Optional 2 yrly mammogram

Increased risk Start screening 5 yrs before

onset of breast dz in youngest family member

Monthly BSE 6 mthly CBE & U/S breast Annual mammography

HRT therapy 40-49 YO Annual mammogram 50-65 YO Biannual mammogram up to 5 yrs after cessation of HRT

6. PAGET’S DISEASE OF THE NIPPLE

- Presents as erythema and eczematous change of the nipple (not the areola) with crusting exudates, may develop into erosions and ulcerations

- Often associated with intraductal carcinoma (DCIS) or invasive carcinoma just beneath the nipple - Malignant cells invade across the epithelial-epidermal junction and enter the epidermis of the nipple, breaking the normal

epidermal barrier thus allowing fluid to be extruded onto the nipple - Examination and investigations should be targeted towards detecting an underlying tumour – may find a palpable mass and/or

mammographic abnormalities - Punch biopsy of the nipple may be required - Prognosis of the underlying cancer is not altered by the presence of Paget’s disease of the nipple - Treatment should be planned according to the underlying cancer if found - If no palpable mass or mammographic abnormality is detected, wide excision is an adequate treatment

7. GYNAECOMASTIA

- Causes: - Physiological: puberty (maybe painful) - Drugs:

Recreational drugs Cimetidine (H2 blockers) Digosin Spironolactone Antimicrobials (Isoniazid, ketoconazole)

- Endocrine disorders: Thyroid disorders Acromegaly Hypogonadism (testicular atrophy, Klinefelters’ syndrome)

- Malignancy: testicular tumours, lymphomas - Chronic liver disease: cirrhosis

- Investigation:

- TFT, testosterone/ LH - LFT, α-FP, ß-HCG

- Treatment

- Conservative management - Surgical excision

195

13. NECK MASSES 1. ANATOMY OF THE NECK

- The neck is composed of two triangles on each side – anterior and posterior triangles - The anterior triangle is bounded by the lower border of the mandible superiorly, the midline anteriorly, and the anterior border of

the sternocleidomastoid posteriorly - The posterior triangle is bounded by the posterior border of the sternocleidomastoid anteriorly, the anterior border of the

trapezius posteriorly, and the clavicle inferiorly

- Masses in the neck can be subdivided according to the triangle they occur in as there are pathologies peculiar to each triangle - Locations: (i) Midline (ii) Anterior triangle (iii) Posterior triangle - In general, enlarged lymph nodes are the most common cause of a lump in the neck, regardless of location MASSES BY LOCATION Midline (5) 1. Submental lymph node 2. Thyroglossal cyst 3. Thyroid nodule in the isthmus 4. Sublingual dermoid cyst 5. Plunging ranula (retention cyst of the sublingual) 6. Rarely, hyoid pathology e.g. bursa Anterior triangle 1. Lymph node – along anterior border of sternocleidomastoid (levels II, III, IV) 2. Thyroid nodule 3. Submandibular gland mass (see later section on Salivary gland swellings) 4. Branchial cyst + fistula 5. Chemodectoma (carotid body tumour) 6. Carotid aneurysm 7. Pharyngeal pouch 8. Laryngocoele (rare; an air-filled, compressible structure seen in glass-blowers) Posterior triangle 1. Lymph node (mainly) – level V and supraclavicular lymph node groups 2. Cystic hygroma 3. Cervical rib 4. Brachial plexus neuroma/schwannoma

Approach: - Does it move with swallowing – divides the thyroglossal cyst and

thyroid nodule from the other causes - If it moves with swallowing, does it move with tongue protrusion –

thyroglossal cyst moves with protrusion but a thyroid nodule does not

196

2. THYROGLOSSAL CYST Epidemiology: Equal in males and females. Occurs mostly in children and adolescents but up to one-third occur in patients older than 20 years. Pathology: Cystic expansion of the remnant thyroglossal tract (embryological origin of the thyroid which descends from the foramen caecum on the tongue). Features: Smooth, rounded, cystic lump. 75% are in the midline while 25% are slightly to the left or right. Usually asymptomatic but may become infected. Complications: 1. Infected with sinus formation and seropurulent discharge (occurs with incision or rupture of cyst) 2. Malignant change (carcinoma of the thyroglossal duct) Histology: Cyst with columnar or squamous epithelial lining which may be ciliated. The cyst may also contain thyroid and lymphoid tissue. If malignancy occurs, it is usually a papillary carcinoma (~90%). Treatment: Sistrunk procedure – resection of the (a) cyst and (b) mid-portion of the hyoid bone in continuity and resection of a (c) core of tissue from the hyoid upwards towards the foramen caecum (remove the entire tract to prevent recurrence!)

3. DERMOID CYST

Pathology: Can be congenital or acquired. (i) Congenital – developmental inclusion of epidermis along lines of fusion of skin dermatomes (seen in younger patients, present since birth).

Locations include: o medial and lateral ends of the eyebrows (internal and external angular dermoid cysts) o midline of the nose (nasal dermoid cysts) o midline of the neck and trunk

(ii) Acquired – due to forced inclusion of skin into subcutaneous tissue following an injury, usually on fingers. Seen in older patients, no previous history of mass, history of trauma to area (may have associated scar).

Histology: Cyst lined by epidermis, with evidence of adnexal structures such as hair follicles, sebaceous glands and sweat glands. Features: Small non-tender mobile subcutaneous lump, may be fluctuant, skin-coloured or bluish. Management - Imaging investigations (e.g. XR, U/S, CT) are important especially for cysts on the skull as they can communicate with cerebrospinal fluid. - Complete surgical excision of the cyst.

4. PLUNGING RANULA

Pathology: A pseudocyst associated with the sublingual glands and submandibular ducts. Ranulas (frog mouth) can be congenital or acquired after oral trauma. Typically appears as a blue-grey dome-like swelling beneath the tongue. A large ranula can present as a neck mass if it extends through the mylohyoid musculature of the floor of the mouth – termed a “plunging” ranula. Treatment: - Complete resection if possible, often in

continuity with assoc sublingual gland (but often difficult due to close association with the lingual nerve and submandibular duct).

- If complete resection not possible, marsupialisation (de-roofing the cyst so that it opens into the floor of the mouth) and suturing of the pseudocyst wall to the oral mucosa may be effective.

Incomplete removal leads to recurrence.

197

5. BRANCHIAL CYST/FISTULA

Epidemiology: Affects both sexes equally, usually in young adults in their 20s. Pathology: A branchial cyst is thought to develop because of failure of fusion of the embryonic second and third branchial arches. It is lined by squamous epithelium. Features: - Occurs anterior to the upper or middle third of the sternocleidomastoid muscle. - Smooth firm swelling that is ovoid in shape, with its long axis running downwards and forwards. - May be fluctuant, usually not transilluminable (due to desquamated epithelial cell contents). - Look for fistula in this area – a branchial fistula will run between tonsillar fossa and the anterior neck, passing between the

external and internal carotid arteries. - Fine needle aspiration of the cyst will yield opalescent fluid with cholesterol crystals under microscopy. - May be complicated by recurrent infections – purulent discharge, fixation to surrounding structures. Management: - If fistula present, perform fistulogram to delineate course. - Surgical excision of the cyst where possible. (perc drainage rarely permanently successful)

If fistula/sinus present, inject Bonney’s blue dye into tract prior to surgery to allow accurate surgical excision. - Treatment of infection with antibiotics. - Complications: cyst recurrence; chronic discharging sinus.

198

6. CHEMODECTOMA

Pathology: A chemodectoma is a tumour of the paraganglion cells (paraganglionoma) of the carotid body located at the bifurcation of the common carotid artery (into the internal and external carotids). They are usually benign, but locally invasive; the risk of malignancy is 10%, with metastasis to local lymph nodes (no histopathological features for malignancy, thus malignant nature can only be diagnosed by presence of metastasis). Features: - Solid, firm mass at the level of the hyoid bone (where the bifurcation is) – be gentle during palpation as pressure on the carotid

body can cause vasovagal syncope. - Mass is pulsatile but not expansile, due to transmitted pulsation from carotids. - Due to close association with carotid arteries, lump can be moved side to side but not up and down. - May be bilateral.

Differentials: - Main differential is carotid artery aneurysm - Aneurysm can occur at any level but carotid body tumour occurs at the level of the hyoid bone. - If suspecting aneurysm, (a) listen for bruit, look for (b) signs of Horner’s syndrome, (c) examine the rest of the peripheral

vascular system. Investigation: - DO NOT PERFORM FNA - Angiography (gold standard) – shows a hypervascular mass displacing the bifurcation. May also show vessel compromise by

tumour invasion, and undetected synchronous tumours. - CT and/or MRI can be used to delineate tumour anatomy in relation to surrounding structures; CT reveals homogenous mass

with intense enhancement following IV contrast administration. Treatment: - Surgical excision with pre-operative embolisation (reduces bleeding and complications, and facilitates resection); any enlarged

ipsilateral lymph nodes are also removed due to the small possibility of malignancy - Radiotherapy is an effective alternative for patients who are unfit for surgery or whose tumours are too large.

7. PHARYNGEAL POUCH

Pathology: A herniation of the pharyngeal mucosa (pulsion diverticulum) through its muscular coat at the weakest point – Killian’s dehiscence – between the cricopharyngeus muscle and the lower inferior constrictor muscles. Features - Occurs in older patients - A cystic swelling low down in the anterior triangle, usually on the left - Squelching sound on deep palpation - Patient complains of

o halitosis, o regurgitation of undigested food with coughing o dysphagia in the neck, o hoarseness, o weight loss

- Complications: aspiration pneumonia; diverticular neoplasm (<1%) Diagnosis by barium swallow Treatment - Leave it alone if small and asymptomatic - Minimally invasive treatment: endoscopic cricothyroid myotomy - Surgical approaches (several available)

o Diverticulectomy + cricothyroidotomy (diverticulectomy associated with risk of mediastinitis, dangerous) o Diverticulopexy (done in high risk patients, involves suspending the lumen of the pouch in the caudal direction so that food

and secretions cannot enter the pouch; as the diverticulum is still present, the risk for malignancy still remains)

199

8. CYSTIC HYGROMA

Pathology: A cystic hygroma is a congenital cystic lymphatic malformation found in the posterior triangle of the neck, probably formed during coalescence of primitive lymph elements. It consists of thin-walled, single or multiple interconnecting or separate cysts which insinuate themselves widely into the tissues at the root of the neck. Features: - 50-65% present at birth, but occasionally may present later in childhood or adulthood - cystic swelling that is soft, fluctuant, and compressible (usually into another part of the cyst), located in the posterior triangle

at the root of the neck - Classically “brilliantly transilluminable” - A large cyst may extend deeply into the retropharyngeal space Complications: - Cystic hygroma seen on prenatal ultrasound in the first trimester suggests chromosomal abnormality (50% of foetuses, usually

trisomy 21) or other structural abnormalities (33% of foetuses with no chromosomal abnormality, usually congenital heart anomalies)

- May obstruct delivery - Compressive problems after delivery – respiratory, swallowing Management: - Radiological investigations e.g. CXR, CT to delineate extent of cyst - Non-surgical treatment – aspiration and injection of sclerosant (usually unsuccessful) - Surgical excision – partial (to alleviate symptoms) or complete

9. CERVICAL RIB

Features: - Usually more symptoms than signs as it causes thoracic outlet syndrome (diagram below) - A hard mass in the posterior triangle at the root of the neck - Symptoms/signs:

o Arterial: pallor, gangrene or necrosis of the tips of the fingers o Venous: oedema, cyanosis o Neurological: complaints of radicular symptoms (pain, paraesthesia), wasting of the small muscles of the hand

- Adson’s test can be done – ask patient to extend neck and rotate it towards side of symptoms Æ radial pulse will be diminished, occasionally with reproduction of radicular symptoms in the limb

- Diagnosis by CXR 10. NEUROMA/SCHWANNOMA

Features: - Slow growing tumour arising from peripheral neural structures of the neck e.g. brachial plexus, cervical plexus, vagus nerve,

phrenic nerve, etc. - Fusiform, is mobile in plane perpendicular to axis of nerve but not parallel - Usually benign - May be Tinnel’s positive – tap on the mass for any paraesthesia occurring in distribution of the nerve - DO NOT PERFORM FNA – excruciatingly painful

200

11. CERVICAL LYMPHADENOPATHY

There are six levels of lymph nodes in the neck, and different structures drain to different groups of nodes: Level Ia – submental Ib – submandibular II – long internal jugular vein from skull base to bifurcation of carotids (includes jugulodigastric nodes) III – along internal jugular vein from carotid bifurcation to omohyoid IV – along internal jugular vein from omohyoid to clavicle Va – Posterior triangle Vb – Supraclavicular VI – Tracheo-oesophageal groove (not palpable) VII – Superior mediastinum Drainage: - Oral cavity and oropharynx Æ levels I – III - Thyroid and larynx Æ levels II – VI - Nasopharynx Æ II – V (usually upper neck – level II and high level V) CAUSES:

1. Cancer (3): SCC, NPC, Adenocarcinoma 2. Lymphoma 3. TB

Infective Viral (esp 1-2cm firm LN. No need Abx unless obvious infx)

Epstein-Barr virus, cytomegalovirus (infectious mononucleosis); HIV Bacteria

Streptococcus, Staphylococcus, Klebsiella (from intraoral pathology e.g. dental abscess, tonsillitis) Tuberculosis

Parasitic Toxoplasma

Fungi Actinomycosis

Neoplastic Metastatic Head and neck primary Nasopharyngeal carcinoma Oral cavity, oropharynx, larynx, hypopharynx, thyroid, etc.

Other primary sites (4B’s) Bowel (stomach, colon), breast, bronchus (lung), balls (testicular). Also UL.

Primary - lymphoma Inflammatory SLE

Kikuchi’s (necrotising lymphadenitis occurring in young females, presenting as painful cervical lymphadenopathy) Sarcoidosis

201

HISTORY - Age - RF for CA: smoking, alcohol. Betel nut - The lump itself

o onset, duration, associated symptoms, lumps elsewhere o Pain: inflammatory > CA o Growth pattern / rate of growth:

last few days = infx / inflammatory / haemorrhage into cyst last few months = CA

- Constitutional symptoms o Fever, malaise, arthralgia, myalgia (viral prodrome); o Night sweats, low-grade fever (TB, B symptoms of lymphoma); o Loss of appetite, loss of weight (chronic infection, malignancy)

- Local symptoms – intra-oral diseases e.g. tooth decay, oral/tongue ulcer, tonsillitis. Use of dentures. - Past medical history – cancer, TB (contact? Diagnosed? treated or untreated?) - Social history: travel and contact history, sexual history for HIV, ORAL SEX PHYSICAL EXAMINATION Inspection - SITE - Size, shape, surface: erythema, discharging sinus (multiple lymph node enlargement with discharging sinuses can be TB or actinomycosis;

sulphur granules seen in actinomycosis) “Is there any pain? I am going to feel the lump, if any pain, let me know” Palpate from behind, one side at a time – start at submental, then submandibular, preauricular, postauricular, along anterior border of

sternocleidomastoid, supraclavicular, posterior triangle, lastly occipital. Use pulps of the fingers in a gentle rolling movement. - Tenderness to palpation - Consistency – hard, matted nodes are more suspicious for malignancy - Fixation to overlying skin or underlying structures Potential drainage sites: - Upper LN (5) : MOUTH!!, face, scalp, thyroid, salivary glands - Lateral LN: thyroid - Lower LN: UL, Breast, Lungs, Abdomen (if Virchows), Oesophagus - Susp lymphoma (3): axilla,parotid, inguinal + liver/spleen To complete the examination: - Formal ear, nose, throat examination especially looking at the post-nasal space for nasopharyngeal carcinoma (NPC being the most

common cancer causing enlarged cervical lymph nodes) - Full respiratory and abdominal examination especially if supraclavicular lymph node found - Examination of the thyroid gland - Examination of lymphoreticular system – other lymph node groups, liver, spleen - Breast examination in female patient INVESTIGATIONS - Fine needle aspiration

o Able to definitively diagnose CA and TB. (still the possibility of false positive and false negative results) o Only lymphoma requires excision Bx to diagnose (to be done after FNAC) o Do not do excision Bx first as it can compromise resection later if LN mets is from H&N CA, because LN mets counted as

locally advanced dz (still resectable). - Radio: CT, ultrasound (esp thyroid), MRI (mainly CA as pre-op w/u) Æ to assess nature of lump, extent, other enlarged nodes that are not clinically palpable, and can be used to visualise primary tumour if present - Panendoscopy: to look for synchronous tumors as smoking/alc affect squamous cells

o Nose – airway: nasolaryngoscopy, bronscopy o Mouth – CE junction: OGD

MANAGEMENT - According to FNAC results - Malignant – work up for primary if present (e.g. squamous cell carcinoma – look for oral cavity, skin, ENT, lung malignancy;

adenocarcinoma – look for breast, GI tract malignancy) and treat as appropriate - Infective/Inflammatory – treat underlying condition

202

14. SALIVARY GLAND SWELLINGS 1. ANATOMY OF THE PAROTID GLAND

- Surrounded by tough fibrous capsule – the parotid sheath (thus mumps is painful as the gland swells within a tight envelope) - Sandwiched between the posterior border of the ramus of the mandible and the mastoid process - Important structures that pass through the gland in order from lateral to medial:

(i) Facial nerve and its branches (ii) Retromandibular vein (formed as the maxillary veins drain into the superficial temporal vein) (iii) External carotid artery (branching into its two terminal branches, the superficial temporal and maxillary arteries)

- Nerve supply: (i) Parasympathetic secretomotor supply from auriculotemporal nerve carrying postganglionic fibres from the otic ganglion (preganglionic

fibres from inferior salivary nucleus); (ii) Somatic sensory supply of the gland from auriculotemporal nerve; sensory supply of the capsule from the great auricular nerve.

- Parotid duct (of Stensen) runs 5cm across the masseter (surface marking: along the line joining the intertragic notch to the midpoint of the philtrum), drains into the mouth opposite to the upper second molar tooth

- Histology: predominantly serous acini, many ducts (other glands have few ducts) 2. ANATOMY OF THE SUBMANDIBULAR GLAND

- Consists of a large superficial part & a small deep part that are continuous with one another around the free posterior border of the mylohyoid - The deep part of the gland is closely associated with the lingual nerve (with the attached submandibular ganglion) above it, and the

hypoglossal nerve and submandibular duct below it – surgery may injure these nerves - Nerve supply: parasympathetic secretomotor supply from lingual nerve carrying postganglionic fibres from the submandibular ganglion

(preganglionic fibres in superior salivary nucleus) - Submandibular duct (of Wharton) arises from the superficial part of the gland, runs forwards deep to mylohyoid and drains into the oral

cavity at the sublingual papilla just adjacent to the frenulum - Histology: mixed serous and mucous acini, few ducts

3. ANATOMY OF THE SUBLINGUAL GLAND

- A small almond-shaped gland sitting just under the mucosa of the floor of the oral cavity - Each gland has 15 or so ducts, half of which drain into the submandibular duct, the rest draining directly into the oral cavity - Nerve supply is similar to the submandibular gland - Histology: almost solely mucous acini, few ducts

203

4. APPROACH TO SALIVARY GLAND SWELLINGS

HISTORY - About the lump: onset, duration, progress, associated symptoms e.g. pain

o If pain is present, is it precipitated by food ingestion? (suggestive of sialolithiasis) o Intermittent swelling a/w food (inflammatory)

- Symptoms of infection e.g. fever, malaise; if considering mumps, ask about testicular pain and swelling (orchitis), abdominal pain (pancreatitis)

- Any noticed asymmetry of the face – incomplete closure of the eye on one side, drooping corner of the mouth, drooling - Does the patient have symptoms of Sjogren: xerostomia (e.g. cannot eat a piece of biscuit or bread without water),

xerophthalmia - History of connective tissue disease e.g. rheumatoid arthritis, SLE PHYSICAL EXAMINATION Inspect - Put yourself at the level of the patient’s face and look from front for any asymmetry with an obvious mass on one side

o parotid mass is located between the angle of the jaw and the ear, and lifts the earlobe if large; o submandibular mass is located just under the mandible

- Look for scars – parotidectomy scar runs anteriorly to the ear, below the earlobe and around posteriorly before looping forward again under the jaw

- Look for fistula/sinus - Look at the patient’s face for asymmetry (facial nerve palsy) “Is there any pain? I am going to feel the lump, if any pain, let me know” Palpate from behind - Palpate the obviously enlarged gland:

- Check for warmth of overlying skin, tenderness, consistency, surface, margins - Fixation to underlying structures – for parotid, ask pt to clench the teeth to contract the masseter, then try to move the gland - Fixation to overlying skin

- Palpate the contralateral gland for any swelling - Palpate for cervical lymphadenopathy Other tests

1. Examination of the duct openings: Æ Using a torch and a tongue depressor, examine opposite the second upper molar tooth (opening of the

parotid duct), and under the tongue (opening of the submandibular duct) Æ Look for (1) red inflamed opening, (2) discharge-purulent, (3) visible stone. Æ For the parotid duct, can palpate the duct along the masseter for stone, and look for discharge inside the

mouth while palpating 2. Tonsillar fossa: enlargement of deep lobe of the parotid (in retropharyngeal space) pushes tonsils and archs aside Æ see

asymmetry of the arches 3. Palpate the gland openings for stones. 4. Bimanual palpation of the submandibular gland 5. Facial nerve examination

Suspicious features of malignancy: 1. Facial nerve involvement 2. LN involvement 3. Skin involvement: eg Hyperaemic hot skin over lump 1+2+3 = CA until proven otherwise - Pain - Fixation to underlying structures or skin - Hard consistency - Irregular surface or ill-defined border

204

CAUSES OF SWELLING OF THE PAROTID Parenchymal swelling

Neoplasia Benign - pleomorphic adenoma - Warthin’s (10% bilateral) Malignant tumours Lymphoma and leukaemia: bilateral

Stones Sialolithasis Infection/ Inflammation

Mumps: bilateral Acute sialadenitis Chronic recurrent sialadenitis HIV

Autoimmune Sjogren’s syndrome: bilateral Infiltration Sarcoidosis: bilateral Systemic disease: bilateral (MADMAP)

Alcoholic liver disease Diabetes mellitus Pancreatitis Acromegaly Malnutrition

Non-parenchymal swelling

Lymph node Facial neuroma Temporal artery aneurysm Skin and soft tissue swellings e.g. sebaceous cyst, lipoma

INVESTIGATIONS - FNAC: malign vs benign - CT: confirm salivary gland (vs LN)

o esp parotid – multiple swelling likely LN (DDx Warthin) 5. SIALOLITHIASIS

Epidemiology - Stones of the salivary gland that may be impacted within the gland itself or in the duct. - Usually occurs in males more than females, and between the ages of 30 and 60. - 80% occur in the submandibular gland (due to its higher mucus and calcium content with a long duct, and slow flow of the saliva against

gravity); 10% occur in the parotid, 7% sublingual. - Most submandibular gland stones occur in the duct, while 50% of parotid stones occur in the gland itself. - 80-95% of submandibular stones are radio-opaque and can be seen on an X-ray of the floor of the mouth, and 60% of parotid stones are

radio-opaque. Presentation - Complete obstruction

Acute pain & swelling of the gland involved at meal times, rapid onset within minutes of starting to eat, resolves about an hour after the meal. - Partial obstruction

Occasional symptomatic episodes interspersed by asymptomatic periods of days-weeks, chronically enlarged mass in submandibular region - Cx: sialadenitis, and even abscess formation Æ worsening of symptoms of pain and redness; systemic symptoms such as fever, chills;

purulent discharge from duct opening - Stone may be palpable along the duct or at the opening of the duct Investigations - CT scan (Noncontrast) – can pick up almost all stones when fine cuts are requested - Plain X-rays can pick up radio-opaque stones - Sialogram (rarely done as it is invasive and technically demanding; CT is better. Contraindicated in acute sialadenitis and contrast allergy.) Management - General measures:

o Good hydration, good oral hygiene, soft diet, avoid sour food (increase salivation) o Massage of the gland, milking the duct, application of moist hot towel o Lifestyle changes: activities that exacerbate the pain eg. Blowing instruments o Analgesia – NSAIDs such as ibuprofen

- If sialadenitis present: o Antibiotics– usually to cover Staph and Strept e.g. Augmentin o Refer specialist treatment if symptoms persist for several days, or sialadenitis persists despite antibiotic therapy

- Surgical removal o Transoral removal of stones for submandibular duct stones (50% can be removed thus), less for parotid duct stones o If stones cannot be removed via transoral surgery or is intraglandular, partial gland resection can be performed

- Other options: Lithotripsy, wire basket removal, sialoendoscopy

205

6. SALIVARY GLAND TUMOURS

Epidemiology: (80% rule for parotid) - 80% occur in the parotid, of which 80% are benign (80% of the benign tumours are pleomorphic adenomas) - 10% occur in the submandibular, of which 60% are benign (95% pleomorphic adenoma) - 15% occur in minor salivary glands, of which 50% are benign (all benign tumours are pleomorphic adenomas) - 0.3% occur in sublingual glands, of which all are malignant Pathology

Epithelial Non-epithelial Adenomas (benign) Carcinomas (malignant) Pleomorphic adenoma Warthin’s tumour

Adenoid cystic ca Pleomorphic adenoca Mucoepidermoid ca Acinic cell ca Adenoca Squamous cell ca Undifferentiated

Haemangioma Lymphangioma Neurofibroma Neurilemmoma Lipoma Sarcoma Malignant lymphoma

PLEOMORPHIC ADENOMA Epidemiology: - Most common benign tumour - 85% occur in the parotid gland - Equal sex ratio, occurs in younger patients (<50 years old) Histology: Very heterogeneous appearance, containing epithelial cells surrounded by loose stroma with islands of chondromyxoid (mesenchymal components), and interspersed islands of myoepithelial cells. The tumour appears to be encapsulated, but histology shows multiple sites of capsular penetration by tumour cells. Features: - Slow-growing, painless swelling occurring in the lower pole of the parotid - Irregular and lobulated surface, texture of cartilage (slightly harder than Warthin’s) - Does not invade or metastasise - Cx

o Malignant transformation (carcinoma ex pleomorphic) if left for 10-15 years (1-6% risk) o If not completely excised, can recur due to frequent capsular penetration (recurrence rate of 2%)

Diagnosis by clinical, FNAC, + MRI Treatment – surgical excision (sufficient margin) - Parotid: Superficial parotidectomy for superficial tumour; if tumour is deep or large, total parotidectomy with preservation of the facial nerve

(or any way possible as long as margins are sufficient) - Submandibular: Total gland excision together with adjacent connective tissue, sparing lingual and hypoglossal nerves WARTHIN’S TUMOUR AKA PAPILLARY CYSTADENOMA LYMPHOMATOSUM / ADENOLYMPHOMA Epidemiology: - Only occurs in the parotid gland (10% of parotid tumours) - More common in males than females (4:1) - Occurs in older patients (>50 years) - Related to cigarette smoking Histology: Consists of cleftlike or cystic spaces lined by two-tiered epithelium, containing mucin, surrounded by a stroma of well-developed lymphoid tissue with germinal centres. Features: - Slowly enlarging, soft to firm cystic fluctuant swelling in parotid tail (inferior) - Invariably benign with no risk of malignant change - 10% bilateral (check contralateral side) Diagnosis by clinical, FNA + MRI Treatment - Can be left alone if absolutely certain that the entire mass is composed of only Warthin’s tumour cells, since there is no malignant potential - Superficial parotidectomy if causing trouble to patient

206

MALIGNANT TUMOURS

x Tumor grade x Nerve involvement

Most common malignancies

1. Mucoepidermoid (34%) - most common malignant tumour in the parotid. Younger patients. 2. Adenoid cystic carcinomas (22%) – most common in the submandibular, sublingual and minor salivary glands. Equal sex

ratio, can occur in any salivary gland, in older patients (usually >60 yrs). Tends to spread along nerves. 3. Malignant pleomorphic adenoma

o Usually occurs in pre-existing pleomorphic adenoma, rarely de novo o Worst prognosis of any salivary gland tumour o 30-70% recurrence and metastasis rate

TREATMENT OF SALIVARY GLAND CANCERS Parotid: - Total parotidectomy with sacrifice of facial nerve if tumour has infiltrated it (may be grafted with great auricular nerve) - Radical neck dissection if neck nodes positive - Postoperative radiotherapy If localised in superficial lobe - Superficial parotidectomy only (if sufficient margin) - + RT (in higher grade tumor / adenoid cystic – cos may have spread to nerves) Submandibular: - Radical excision of gland with lymphatic clearance of submandibular triangle - Radical neck dissection if neck nodes positive - Postoperative radiotherapy COMPLICATIONS OF PAROTIDECTOMY Immediate (within 24 hrs) 1. Intraoperative facial nerve transection – lower motor neurone palsy (in surgery to the submandibular gland, damage to the

hypoglossal and/or lingual nerves can occur intraoperatively) 2. Reactionary haemorrhage: early post-op bleeding due to displacement of a clot in a BV / slippage of a ligature

Early (1 to 30 days) 1. Wound infection 2. Skin flap necrosis 3. Temporary facial weakness (neuropraxia of facial nerve) 4. Salivary fistula 5. Division of great auricular nerve Æ loss of sensation over pinna 6. Trismus (inability to open mouth due to spasm of masseter)

Late (more than 30 days) 1. Wound dimple, cosmetic problems 2. Hyperaesthesia of local skin 3. Frey’s syndrome (gustatory sweating syndrome / auriculotemporal syndrome)

a. Increased sweating and redness of facial skin when eating b. Auriculotemp nerve: symp branches (sweat glands of the scalp), parasympathetic branches (parotid gland secretion) c. due to reinnervation of divided sympathetic nerves to the facial skin sweat glands by fibres of the secretomotor branch

of the auriculotemporal nerve

207

15. THYROID DISEASES 1. ANATOMY OF THE THYROID GLAND

Structure: 2 lateral lobes joined by an isthmus that lies in front of the 2nd, 3rd and 4th tracheal rings. Strap muscles of the neck lie superficial to the thyroid gland. Nerves and vessels: x Superior thyroid artery (from external carotid) x Inferior thyroid artery (from thyrocervical trunk, a branch of the first part of the subclavian artery). x External laryngeal nerve – supplies the cricothyroid muscle which controls pitch of voice; runs close to superior thyroid artery. x Recurrently laryngeal nerve – supplies all the other intrinsic muscles of the larynx (except for cricothyroid) and runs close to

the branches of the inferior thyroid. The nerve runs behind the pretracheal fascia and so will not be damaged if the fascia is not breached during operation. Important to visualise nerve and avoid damaging it!

Embryonic origin: Thyroglossal tract from foramen caecum of the tongue (in the midline, at the junction between anterior two-thirds and posterior one-third of the tongue) descends close to the hyoid bone Æ expansion of the caudal end of the tract forms the thyroid gland. Parathyroid glands: 2 superior and 2 inferior glands that lie behind the lateral lobes. Level VI lymph nodes – first nodes that a thyroid malignancy spreads to; they lie in the tracheo-oesophageal groove and are not palpable.

208

2. APPROACH TO THYROID PROBLEMS

1. Problem with configuration/anatomy (i) Solitary thyroid nodule (most common in exam) (ii) Multinodular goitre (iii) Diffuse enlargement

2. Problem with function (usually hyperfunctioning)

(i) Graves’ disease (ii) Toxic adenoma (iii) Toxic multinodular goitre (iv) Hashimoto’s disease

AIMS OF ASSESSMENT IN THYROID NODULE/ENLARGEMENT: - Exclude cancer! - Address issues of thyroid function - Look for any complications e.g. compression (of airway, oesophagus, rarely nerve) - Cosmesis – is patient bothered by lump? HISTORY-TAKING About the LUMP - Onset (gradual or sudden), duration - Size (Diffuse or one side predominant? Any sudden increase in size? – malignant growth; ddx includes haemorrhage into

necrotic nodule or cyst, subacute thyroiditis) - Any pain – bleeding into cyst can result in sudden increase in size and pain; rarely pain can occur in anaplastic carcinoma and

thyroiditis - Compressive symptoms: difficulty swallowing, difficulty breathing, hoarseness of voice (benign pathologies almost never

compress the recurrent laryngeal nerve) - Cosmetic effects About THYROID FUNCTION

Hyperthyroid Hypothyroid Weight loss despite increased appetite Decreased appetite, weight gain, lethargy Heat intolerance Cold intolerance Increased sweating Dry skin, loss of outer third of eyebrows Proximal myopathy (Graves’) Muscle fatigue Diarrhoea, frequent bowel movement Constipation Tachycardia, atrial fibrillation Bradycardia Oligomenorrhoea, amenorrhoea Menorrhagia Nervousness; easily irritable; emotional lability; insomnia

Slow thought, speech and action; depression; dementia

Fine tremor Carpal tunnel syndrome symptoms About RISK FACTORS - History of autoimmune disease e.g. type I DM, SLE, RA, pernicious anaemia (associations with Graves and Hashimoto’s) - History of cancer elsewhere – metastatic disease to thyroid; lymphoma; papillary cancer is associated with familial polyposis

syndromes Æ ask about GI polyps/ca - History of thyroid disease – long-standing MNG can progress to lymphoma - Occupational history – any exposure to radiation (papillary cancer risk) - Family history of thyroid cancer – ~20% of medullary cancers are familial (MEN2, AD inheritance), ~5% of papillary cancers About previous TREATMENT for any thyroid disease - Medications given e.g. propylthiouracil, carbimazole, propranolol – for how long, efficacy, side effects - Radioactive iodine treatment – what was the result? Is the patient receiving replacement? - Surgery – what kind of surgery, any complications? - Follow-up – what investigations done?

209

PHYSICAL EXAMINATION A. THYROID GLAND GREET PATIENT, ASK FOR PERMISSION to examine (and listen to the voice – is it hoarse?) POSITION PATIENT – on a chair with space behind the chair for you to stand. INSPECT FROM THE FRONT 1. Any swelling? Where is it? (if unable to see, ask patient to swallow) 2. Any scars (thyroidectomy scar may be difficult to spot as it is often hidden in a skin crease)? Sinuses? 3. Any skin changes over the mass? 4. Check if mass moves on swallowing by asking patient to take a sip of water – “Please take a sip of water and hold it in your

mouth, do not swallow until I tell you to.” 5. Check if mass moves on protruding the tongue – “Please open your jaw slightly. Now, without moving your jaw, please stick

your tongue out and back in again.” NB. A thyroid lump moves only on swallowing; a thyroglossal cyst will move on both swallowing & protrusion of the tongue.

6. Check for plethora of face, distended neck veins – may be due to compressive nature of mass (but rarely seen). PALPATE FROM BEHIND – one side at a time, the opposite hand stabilises the gland. Ask for pain before palpating! 1. Characteristics of lump: site (anterior triangle), size (discrete nodule or multinodular enlargement or diffuse enlargement?),

consistency (soft, cystic, hard, multinodular?), mobility (fixed to skin? Fixed to underlying structures?), tenderness. 2. Check swallowing while palpating to confirm mass moves on swallowing. 3. Check tongue protrusion. 4. Palpate lymph nodes PALPATE TRACHEA from in front for tracheal deviation. PERCUSS – any retrosternal extension? AUSCULTATE – bruit in Graves’ OFFER to do Pemberton’s sign to check for thoracic inlet obstruction; check thyroid status; ask patient about compressive symptoms. B. THYROID STATUS HANDS (get patient to stretch arms out in front of him, palms down) 1. Feel palms – warm sweaty palms 2. Nails – thyroid acropachy, onycholysis (both seen in Graves’) 3. Feel pulse – tachycardia, atrial fibrillation (AF more in toxic MNG than Graves’) 4. Fine postural tremor – accentuate by placing a sheet of paper on the hands 5. Palms up – palmar erythema FACE 1. Expression – staring, unblinking (hyperthyroid); lethargic, apathetic (hypothyroid) 2. Complexion – dry, ‘peaches-and-cream’ complexion, loss of outer third of eyebrows (hypothyroid) 3. Eyes

- Lid retraction (can see sclera between upper limbus of iris and upper eyelid) - Exophthalmos (sclera between lower limbus and lower eyelid) - Chemosis (oedema and erythema of conjunctiva) - Ophthalmoplegia (restriction of eye movements; ask about diplopia!) - Lid lag (eyelid lags behind eye when patient follows your finger downwards) - Proptosis (look from above patient’s head – eye visible over supraorbital ridge)

NEUROMUSCULAR 1. Proximal myopathy (Graves’) 2. Reflexes – slow to relax in hypothyroidism 3. Legs for pretibial non-pitting oedema (Graves’ or hypothyroid)

210

3. APPROACH TO THE SOLITARY THYROID NODULE

Prevalence: About 4-8% of population in US have palpable thyroid nodules; prevalence in Singapore not known.

Differential diagnoses: 1. Cancer (only 10-20% of nodules is malignant,

but need to exclude!) 2. Follicular adenoma 3. Cyst (simple, colloid, or haemorrhagic) 4. Dominant nodule of a multinodular goitre

Clinical features suspicious of malignancy: 1. Male gender (thyroid nodules less common in male but more likely to be malignant) 2. Age <15yrs or >60yrs (majority of nodules occurs in 3rd to 6th decades – likely benign) 3. History of head and neck radiation or thyroiditis 4. Family history of thyroid cancer (or MEN2, Gardner’s syndrome, FAP) 5. Rapidly enlarging nodule 6. Hard, single nodule and/or nodules fixed to surrounding structures 7. Hoarseness (i.e. recurrent laryngeal nerve invasion) 8. Cervical lymphadenopathy 9. Other symptoms of invasion e.g. haemoptysis, stridor, dysphagia

INVESTIGATIONS 1. FINE NEEDLE ASPIRATION CYTOLOGY - The most important investigation modality!

- 90-95% sensitivity and specificity - 4 possible results:

(i) Benign (thyroiditis, dominant nodule of MNG) (ii) Malignant (papillary, medullary, anaplastic, mets) (iii) Suspicious (follicular, Hurthle cell change in follicular lesion) (iv) Inadequate Æ repeat FNAC

- Can be both therapeutic and diagnostic for cyst – chocolate-brown fluid aspirated; feel lump after aspiration to check for resolution - Cannot differentiate follicular adenoma from follicular carcinoma as the mark of malignant disease is capsular invasion – can only tell from a

histological specimen of the nodule - Procedure: inject local anaesthetic in area, insert 20-22G needle and apply suction while fanning needle in region of nodule, release suction

before pulling out needle, expel contents onto slide, then fix - Best to have experienced cytologist on hand to view slides and re-do FNAC if the sample is inadequate

2. ULTRASOUND OF THYROID

- Advantages: (i) Objective measurement of nodule (ii) Detection of subclinical nodule/screening – of value in papillary carcinoma since multicentric disease occurs in 15% (iii) Detection of lymph node enlargement (especially level VI nodes) (iv) Can define consistency of nodule – solid, cystic, or complex

- Suspicious sonographic features: (i) Microcalcifications (in psammoma bodies Æ papillary cancer) (ii) Indistinct margins (iii) Sonolucent halo around lesion (iv) Hypoechoeic or anechoeic lesion – carcinoma is almost never hyperechoeic (v) Increased intranodular vascularity

- Ultrasound still does not provide as good diagnostic value as FNAC

3. THYROID FUNCTION TEST - Easy to perform, establish baseline, detect any abnormal function - No real diagnostic value

4. RADIO-ISOTOPE SCAN

- Hot nodule: only 1% malignant; but cold nodule: 10-20% malignant - But not very useful diagnostically

5. BASELINE TUMOUR MARKERS (IF SUSPECTED OR CONFIRMED MALIGNANCY)

- For differentiated thyroid cancer: thyroglobulin - For medullary thyroid cancer: calcitonin, carcinoembryonic antigen (CEA)

6. CT SCAN OR MRI

- Not routine in thyroid nodular study - Uses: (i) Evaluating invasion of surrounding structures

(ii) Retrosternal extension (iii) Lymph node involvement

- Care to be taken with CT as contrast contains iodine and will affect post-op radioactive iodine body scan once given - MRI has same functions as CT but higher cost

7. ENT EXAMINATION OF VOCAL CORDS

- In the rare occasion that there is pre-existing vocal cord palsy on one side Æ take extra care not to injure opposite recurrent laryngeal nerve as that can cause bilateral vocal cord palsy

MANAGEMENT OF BENIGN NODULE - Soft, small, round nodule with benign FNAC results, non-functional, not causing any symptoms Æ can follow-up and monitor for Ç in size - A lump >4cm has a greater risk for malignancy

211

4. THYROID CANCERS

Differentiated thyroid carcinoma Medullary carcinoma Anaplastic

carcinoma Lymphoma Papillary carcinoma Follicular carcinoma

Proportion 75% 10% 7% 3% 5% Age 25-40 years 40-50 years >50 yrs for sporadic type;

20-30 years for familial 60-70 years >50 years

F:M ratio 3:1 3:1 1:1 3:2 2:1 Risk factors - Radiation exposure

- Polyposis syndromes (FAP, Gardner’s, etc)

- Positive family history in 5%

- Follicular adenoma is NOT a risk factor

- Iodine deficiency may be associated

- Significant family history in the familial type – MEN2 (AD, complete penetrance, associated with parathyroid adenoma and phaeochromocytoma – see notes below)

- Longstanding goitre

- History of previous differentiated thyroid ca (30% of anaplastic ca)

- History of lymphoma or MALT elsewhere

- Hashimoto’s thyroiditis (60X increased risk)

Pathological features

- Characteristic Orphan Annie nuclei, nuclear pseudoinclusions

- Papillary architecture with psammoma bodies

- Tall cell variant (nuclear features of papillary ca within follicular lesion) behaves like papillary ca, has worse prognosis

- Follicular structures similar to normal thyroid

- Diagnosis of cancer made on evidence of capsular or vascular invasion by tumour cells (vs follicular adenoma)

- Hurthle cell variant – worse prognosis

- Arise from parafollicular C cells (which produce calcitonin)

- Distinctive deposits of acellular amyloid material – altered calcitonin collections

- Multicentric C-cell hyperplasia may be seen in familial cases

- Small blue round cells that are highly anaplastic – may resemble lymphoma

- FNAC may suggest lymphoma but definitive diagnosis requires trucut or excision biopsy

- Almost always non-Hodgkin’s of B-cell type

Clinical features

- Slow-growing tumour

- Spread by lymphatics

- 30-50% multicentric - LN involvement in

80% of disease at diagnosis (level VI first)

- Solitary - Haematologic

spread to bone, lung, liver, brain

- LN involvement in 10% (rare)

- Sporadic cases usually solitary, worse prognosis

- Familial cases all multicentric, better prognosis

- Aggressive growth; spread via local, lymphatic, haematological routes

- 95% produce calcitonin, 80% produce CEA

- Unilat LN involved in 60-80%, contralat LN in 40%

- Always exclude MEN2 – serum calcium, 24hr urinary catecholamines

- Large bulky mass involving neck structures – locally advanced

- Aggressive growth

- Multiple metastases probably present at presentation

- Usually presents as rapidly enlarging goitre with compressive symptoms

- 60-80% aggressive and 30% more indolent

- Very good prognosis - Poor prognostic factors (AMES): Age>40,

presence of metastases, extra-thyroid invasion, size>4cm (more details on risk stratification below)

Treatment Surgical resection - Hemithyroidectomy for selected low-risk patients

(see below) - Total thyroidectomy for the majority - LN clearance: tracheo-oesophageal nodes

cleared, and neck dissection if neck nodes are positive

- For suspicious lesion – hemithyroidectomy with histology, KIV TT

Adjuvant therapy - Radioactive iodine at ablative levels to ablate

remnant thyroid and any cancer tissue (only for total thyroidectomy)

- External radiotherapy (only shown to have good results in pts with locally advanced follicular ca)

TSH suppression – give L-thyroxine to suppress TSH levels to <0.005U/L Follow-up - Check TSH levels - Thyroglobulin as a tumour marker of recurrence - Radioactive iodine scan to detect recurrence,

followed by ablation

Surgical resection - Aggressive resection – total

thyroidectomy with level VI node clearance

- Sampling of cervical and mediastinal nodes and modified dissection where positive

No good adjuvant therapy Follow-up - Thyroxine replacement (not for

TSH suppression but to maintain euthyroid state)

- Serum calcitonin and CEA six mths after surgery (if normal, considered cured – 5% 5yr recurrence)

- High calcitonin – screen for residual or metastatic disease, treat surgically, with RT or chemo as appropriate

Palliative therapy for compressive effects

- Chemotherapy to shrink tumour

- Surgical debulking

- Tracheostomy

Chemotherapy and/or radiotherapy depending on type of lymphoma

5yr survival 95% in low-risk pts, 88% in intermediate-risk pts, 50% in high-risk pts Slightly worse for follicular ca

60-70% Median survival <6mths

Dependent on histo, stage, treatment, etc.

212

RISK STRATIFICATION: - Risk factors can be divided into patient factors and disease factors - Patient factors: Age – >45 years old is high risk; Gender – male is high risk - Tumour factors:

Size – nodule >4cm has higher risk Histology – tall cell variant of papillary ca and Hurthle cell variant of follicular ca are considered unfavourable Extrathyroidal extension into surrounding structures – worse Lymph node or distant metastases – worse

- Various score systems have been formulated to stratify risk:

AMES – Age, Metastases, Extent, Size AGES – Age, Grade (Histological), Extent, Size) – rarely used as histological grading is not commonly performed MACIS – Metastasis, Age, Completeness of resection, Invasion, Size

- Patients can be divided into three groups:

(i) Low risk – low risk patient and low risk disease (i.e. no high risk features) (ii) Intermediate risk – low risk patient with high risk disease, or high risk patient with low risk disease (iii) High risk – high risk patient and high risk disease

- Risk helps to guide treatment – low risk patients can undergo hemithyroidectomy without ablative radioiodine therapy post-op, while

high risk patients undergo total thyroidectomy with post-op ablative RAI treatment; treatment in intermediate risk patients is tailored to the disease, but usually is similar to that in high risk patients

- 5 year survival is also prognosticated by the risk: low risk patients have a survival of 95-98%, intermediate risk patients 88%, and high risk patients 50%

TOTAL THYROIDECTOMY VERSUS HEMITHYROIDECTOMY Advantages of TT: - Evidence for microfoci of disease and multicentricity of cancer – removal of the entire thyroid decreases risk of recurrence - Ability to use adjuvant radioiodine to ablate any residual cancer tissue after surgery - Ability to use radioiodine to detect recurrent disease (normal thyroid picks up iodine better than cancer cells, thus the presence of the thyroid

gland will decrease the ability of RAI to pick up recurrent cancer) and as treatment for recurrence - Ability to use serum thyroglobulin as a cancer marker for recurrence Disadvantages of TT: - Risk of bilateral recurrent laryngeal nerve injury and permanent hypoparathyroidism - Very low incidence of cancer recurrence in residual thyroid – microfoci probably not clinically significant - Limited thyroidectomy may spare patient from having to be on lifelong thyroid hormone replacement Risk stratification helps to guide the extent of surgical resection in differentiated thyroid cancer according to the patient’s disease. Lymph node clearance - Tracheo-oesophageal groove (level VI) node clearance usually done - Radical neck dissection or modified radical neck if:

(i) Tracheo-oesophageal groove nodes histologically positive for cancer (ii) Clinically positive nodes in the neck – palpable or enlarged on ultrasound

Radical neck dissection - The removal, en-bloc, of the entire ipsilateral lymphatic structures of the neck, from the mandible superiorly to the clavicle inferiorly, from the

infrahyoid muscles medially to the anterior border of the trapezius laterally - Classic radical neck dissection (Crile’s) – internal jugular vein, sternocleido-mastoid muscle, and accessory nerve are resected.

Structures not resected: carotid arteries, vagus nerve, hypoglossal nerve, brachial plexus, phrenic nerve - Modified radical neck

(i) Type I: one of the three structures not removed, usually accessory nerve (ii) Type II: two of the structures not removed – accessory and IJV (iii) Type III: all of the three structures not removed (iv) Extended radical neck dissection: resection of lymph nodes and/or structures not included in the classic neck dissection

- Complications of radical neck dissection: (i) Injury to nerves – vagus (vocal cord paralysis), cervical sympathetic chain (Horner’s), mandibular branch of facial (lower lip weakness) (ii) Haematoma Æ bring back to OT to find source of bleeding and stop it (iii) Salivary fistula (usually when pt has received RT to the neck, and if the upper GI tract was opened during the surgery) – infection can

result (iv) Wound infection – risk factors: previous irradiation, if upper aerodigestive tract is opened during surgery with salivary contamination,

salivary fistula (v) Carotid blowout – risk factors: infection, irradiation Æ resus, apply constant pressure all the way to the OT! (vi) Poor healing – usually in irradiated skin; weakest point is the junction of the trifurcate incision

213

Multiple endocrine neoplasia A group of inherited diseases resulting in proliferative lesions (hyperplasia, adenomas, carcinomas) of multiple endocrine organs. FEATURES: - Tumours occur at younger age than sporadic cancers - Multiple endocrine organs involved, either synchronously or metachronously - Multifocal tumours in each organ involved - Tumour usually preceded by asymptomatic stage of endocrine hyperplasia - More aggressive and higher chance of recurrence compared to sporadic type of tumours in the same organs MEN 1 - Autosomal dominant inheritance - Gene involved is the tumour suppressor gene MEN1 located on chromosome 11q13 where mutations cause loss of function of

the gene - Three P’s:

Parathyroid (95%) – hyperparathyroidism from hyperplasia of parathyroid glands Pancreas (>40%) – aggressive metastatic tumours (e.g. gastrinoma, insulinoma), leading cause of death in MEN 1 patients Pituitary (>30%) – most commonly prolactin-secreting macroadenoma; some have growth hormone-secreting tumours

MEN 2 - Autosomal dominant inheritance - Gene involved is RET protooncogene at 10q11.2 where activating mutations occur - Two distinct groups of disorders:

1. MEN 2a (Sipple syndrome) Medullary carcinoma of the thyroid (almost all) Phaeochromocytoma (50%, of which less than 10% are malignant) Parathyroid hyperplasia and hyperparathyroidism (30%)

2. MEN 2b (William syndrome)

Thyroid and adrenal involvement like MEN 2a, but no hyperparathyroidism Neurocutaneous manifestations: ganglioneuromas on oral mucosa, lips eyelids Other features: Marfanoid habitus, SCFE, delayed puberty

214

5. SURGERY IN BENIGN THYROID DISEASE

Indications for surgery: 1. Cannot be treated medically - failed medical therapy or unsuitable for medical tx 2. Cancer 3. Compression on neighbouring structures 4. Cosmesis 5. Compliance/cost problems – with long-term medical therapy (but patient may still require long-term therapy after op if he/she becomes hypothyroid or

is still hyperthyroid) 6. Child-bearing (not a very strong indication since medical therapy can still be given, but not RAI) Types of surgery available: 1. Hemithyroidectomy – removal of one lobe of the gland, including the isthmus and the pyramidal lobe; usually for suspicious thyroid nodules 2. Total thyroidectomy – entire gland removed completely; usually done in MNG 3. Subtotal thyroidectomy

- Conventional subtotal thyroidectomy – leave a thumb-sized amount (about 4-6g) of remaining thyroid tissue on both sides - Harley-Dunhill subtotal thyroidectomy – leave a thumb-sized amount on one side with removal of the rest of the gland

Total versus subtotal thyroidectomy (for hyperfunctioning thyroid disease) - Result of total thyroidectomy is always hypothyroidism, thus the patient will require life-long thyroid replacement and follow-up Æ problems with compliance, cost, inconvenience

- Results of subtotal thyroidectomy (at 5 years): o 60-70% euthyroid (do not require medication but still have to be followed up closely) o 16-20% hypothyroid (usually becomes evident within 1 year of surgery) o 8-10% hyperthyroid (percentage increases proportionately with time Æ failure of surgical therapy) Æ Difficulty in managing post-operatively and in the long term as patients need close monitoring (better off to just replace everyone after TT?), but weigh this against the benefits of not requiring any medication (for which there is a good chance)

Complications of thyroid surgery: (Mostly H’s, one I and one T) IMMEDIATE (<24HRS) 1. Haemorrhage with haematoma formation

- Haematoma forms in the paratracheal region, mostly below the strap muscles Æ compression of airway if not released (patient can die!) - Cut the subcuticular stitches and also the stitches holding the strap muscles opposed to let the blood drain out

2. Hoarseness or airway compromise from recurrent laryngeal nerve injury

- Risk of nerve injury is <1% - Unilateral nerve injury for hemithyroidectomy, bilateral nerve injury for total or subtotal thyroidectomy - If bilateral nerve palsy resulting in compromised airway, will require tracheostomy

3. Hyperthyroidism

- Resection of gland can release large amounts of stored thyroid hormone into bloodstream - May result in thyroid storm (see Management of thyroid storm)

4. Tracheomalacia

- Floppiness of trachea resulting from chronic compression e.g. by large goitre - Requires intubation to secure airway

INTERMEDIATE (1 DAY TO 1 MTH) 1. Infection

2. Hypoparathyroidism leading to hypocalcaemia

- Risk of permanent hypoparathyroidism is 1-4% (only in total or subtotal thyroidectomies); 10-20% of patients may have temporary Ç Ca2+ - Important to check the serum calcium levels post-operatively – POD 1,3,5 - Ask patient for any symptoms and look for signs of hypocalcaemia – paraesthesia around the mouth, difficulty breathing, carpopedal spasm,

Chvostek’s sign (spasm of the facial muscles on tapping the facial nerve), Trousseau’s sign (carpopedal spasm on inflating BP cuff over arm) - Dangerous as it can cause laryngeal spasm and airway compromise - Check serum calcium together with albumin to get corrected calcium!

Measured serum calcium + 0.02 (40 – Albumin)

- Replacement: 5mmol/6h if symptoms mild, 10ml of 10% calcium gluconate over 30 minutes if severe - Hypocalcaemia may also occur due to “hungry bone syndrome” after thyroidectomy in long-standing thyrotoxicosis

LATE (MORE THAN 30 DAYS) 1. Hypothyroidism 2. Hyperthyroidism (failed treatment) 3. Permanent hypoparathyroidism 4. Hypertrophic scarring or keloid formation – ask patient if he/she has keloids

215

16. PERIPHERAL ARTERIAL DISEASES 1. ANATOMY OF THE LOWER LIMB ARTERIES

- External iliac artery continues as the femoral artery after crossing the inguinal ligament

(surface landmark: the mid-inguinal point i.e. midway between the pubic symphysis and the anterior superior iliac spine)

- The femoral artery then divides into the superficial femoral and the profunda femoris (or deep femoral) arteries about 4cm below the inguinal ligament

- The profunda femoris supplies compartments of the thigh via 2 main branches, the medial & lateral circumflex femoral arteries - The superficial femoral runs distally and passes through the adductor hiatus to reach the popliteal fossa, where it changes its

name to become the popliteal artery - The popliteal artery divides into the anterior tibial artery and the posterior tibial (also called tibioperoneal trunk by some), and

the posterior tibial will give off the peroneal artery - - The anterior tibial crosses into the anterior compartment of the leg and supplies the muscles there, and then continues as the

dorsalis pedis in the foot (surface landmark: one third of the way down a line joining the midpoint of the two malleoli to the cleft between the first and second toes)

- The posterior tibial supplies the posterior compartment of the leg and passes posterior to the medial malleolus (surface landmark: one third of the way down a line joining the medial malleolus to the heel) before dividing into medial and lateral plantar arteries to supply the sole of the foot

- Refer to diagram – important to know the arrangement of the anterior tibial, posterior tibial and peroneal vessels at the trifurcation as you may be asked to read an angiogram of these vessels.

- From lateral to medial: Anterior tibial, Peroneal, Posterior tibial PAD is a very powerful predictor of CVD/CAD, and mortality.

216

2. ACUTE LIMB ISCHAEMIA

Acute limb ischemia is defined as a sudden decrease in limb perfusion that causes a potential threat to limb viability (manifested by ischemic rest pain, ischemic ulcers, and/or gangrene) in patients who present within 2/52 of the acute event (if >2/52, it is considered chronic ischaemia). The decrease in perfusion is usually due to sudden occlusion of a feeding arterial vessel, and this may be in a setting of already narrowed vessel lumen (acute on chronic ischaemia) or in a normal lumen. CAUSES 1. Arterial embolism

- Most common cause of acute limb ischaemia (60-80% of the time) - The most likely source of embolus is the heart (80%), of which 70% is due to atrial fibrillation, 20% to AMI with left

ventricular mural thrombus, and a small proportion to prosthetic heart valves - Non-cardiac emboli arise from other arteries where there are atherosclerotic plaques or an aneurysm (the embolic

material may be thrombus or part of a plaque, but atheroemboli are less likely to cause complete arterial occlusion) - Most common sites where emboli lodge: Bifurcation of the femoral artery (most common site) Trifurcation of the popliteal artery (next most common site in the lower limb) Aortic bifurcation External and internal iliacs Arm (about 20% of emboli)

- Emboli usually cause lower limb ischaemia mostly - After emboli obstructs the vessel, thrombus can propagate distally (due to stasis of blood) and proximally (due to

turbulence of incoming blood hitting embolus) by derangements in the Virchow’s triad 2. Acute thrombosis

- Thrombosis of a previously stenotic but patent artery (atherosclerotic vessel) - Less common cause of acute limb ischaemia - When thrombotic occlusion of a vessel does occur, the resulting ischaemia is usually less severe than in an embolic

occlusion, because collaterals have had time to form around the chronically stenosed vessel - Other less common causes of acute thrombosis include the arteritides (usually affecting medium-sized arteries), ergotism,

and hypercoagulable states (notably antiphospholipid syndrome). 3. Arterial trauma

- Increasing incidence of acute arterial occlusion due to endovascular diagnostic or interventional procedures - Trauma can cause development of an arteriovenous fistula that shunts blood away from the limb - Fracture or dislocations can stretch an artery and cause an intimal tear while the media and adventitia layers are intact

(because they contain elastin and can stretch) Æ a thrombus forms at the site of the tear where underlying thrombogenic collagen is exposed

- Compartment syndrome can result from trauma as well 4. Dissecting aortic aneurysm

- As blood dissects between the intima and media of the aorta, it can cause occlusion of the aortic branches at their origins

PATHOPHYSIOLOGY In order of sensitivity to ischaemia, the tissues affected are nerves (most sensitive), muscle, skin, and bone (least sensitive); thus early signs of ischaemia involve pain and numbness, and muscle paralysis as well as skin changes occur later. The lower limb can survive about 6 to 8 hours in an ischaemic state before injury becomes irreversible.

217

PRESENTATION The classic 6 P’s of acute limb ischaemia: Pain, Paraesthesia, Pallor, Pulselessness, Paralysis, Perishingly cold Pain - Develops acutely - Starts off in a distal part of the extremity and then progresses proximally, increasing in severity with time - Further progress leads to decrease in pain as the nerves die off from ischaemia - Important to ask for any previous claudication pain (10% of claudicants can develop acute ischaemia due to thrombosis of the

stenosed vessel) Paraesthesia - Starts off with paraesthesia (develops relatively early in the course of ischaemia) and develops to complete loss of sensation - Progression: Light touch ÆVibration Æ Proprioception Æ (late) Deep pain Æ Pressure sense Pallor - Assess skin colour, temperature (Perishingly cold), and capillary refill - The limb may still be slightly pink though pale, but in severe ischaemia it can be marble-white (especially in embolus where

there are no collaterals) - Other colours: Pale Æ Cyanosis Æ Mottling Æ Fixed cyanosis and mottling Mottling/Marbling (patches of blue on white): deoxygenation of stagnated blood; surrounding areas of pallor are due to

vasoconstriction Duskiness: due to deoxygenation of stagnated blood; if there is fixed staining (i.e. does not blanch on pressure) then the

limb is non-viable Black: gangrene

- The disclouration usually affects a large part of the distal limb e.g. the toes, foot; rarely does it only affect one toe (more in chronic ischaemia)

- The site of arterial occlusion is usually one joint above the line of demarcation between normal and ischaemic tissue Pulselessness - If able to feel one good pulse (PT or DP), quite unlikely that the limb is ischaemic, but still possible - If unable to feel, assess with a handheld Doppler the arterial and venous flow in the limb – there can still be flow without a

palpable pulse - Also feel the pulses on the other limbs – gives a clue as to whether the cause is embolic or thrombotic (see below) Paralysis - Initial: heavy limb, Late irreversible ischemia: muscle turgidity - Total paralysis occurs late and usually indicates that the limb is non-viable - Intrinsic foot muscles > Leg muscles (toe mvmts produced by leg muscles Æ detect late) - Can assess viability of muscle by making a cut – viable muscle will be shiny and twitches in response to flicking, while dead

muscle will be dull and will not twitch - Dangerous to save dead muscle as reperfusion can cause circulation of toxic metabolites in the muscle P/E Search for sources of embolisation: - Cardiac: AF, murmur (MDM), prosthetic click - Non-cardiac: AAA? Bruit?

218

DIFFERENTIATING BETWEEN EMBOLIC AND THROMBOTIC CAUSE (IMPT AS THE MANAGEMENT IS DIFFERENT) -

Embolic Thrombotic Identifiable source Present – AF, recent AMI Less common Claudication hx Negative Positive Physical findings Contralat pulses present

White limb (no blood) Contralat pulses diminished Dusky limb (collaterals still supplying limb)

Angiography Minimal atherosclerosis, sharp cut-off, few collaterals

Diffuse atherosclerosis, irregular cut-off, well-developed collaterals

CLASSIFICATION OF SEVERITY (SVS/ISCVS) Three categories: viable, threatened and non-viable (i) Viable: No immediate threat of tissue loss (ii) Threatened: Salvageable if revascularised promptly (iii) Non-viable: Limb cannot be salvaged and has to be amputated, no emergency to operate. Patient may require

revascularisation to allow lower amputation or help the amputation to heal Severity depends on: - capability of existing collaterals to carry blood around occlusion - location of obs relative to no. of axial arteries (pop=1, fem=2, tib=3) - extent of obs: larger Æ lose more collaterals - duration

Viable Threatened Non-viable Pain Mild Severe (rest pain) Variable (anaesthesia) Capillary refill Intact Delayed Absent (fixed stain) Motor deficit None Partial Complete Sensory deficit None Partial Complete Arterial Doppler Audible Inaudible Inaudible Venous Doppler Audible Audible Inaudible Treatment Urgent work-up Emergency

surgery Amputation

219

ACUTE MANAGEMENT 1. DOPPLER U/S: viable vs threatened vs non-viable + level of obstruction 2. EARLY ANTICOAGULATION - Important to start anticoagulation with heparin if the suspicion of acute limb ischaemia is high, to avoid clot propogation - Give IV heparin bolus 3000-5000 units - Follow with IV heparin infusion at 1000 units/hour - Ideal PTT is 2 to 2.5 times normal 3. ANALGESIA 4. MEASURES TO IMPROVE EXISTING PERFUSION

- Keep foot dependent - Avoid pressure to heel, extremes of temp - Max tissue oxygenation (O2 supp) - Correct hypotension

5. TREAT OTHER ASSOCIATED CONDITIONS (CHF, AF) INVESTIGATIONS - Pre-operative investigations - FBC, U/E/Cr, PT/PTT, GXM - CXR and ECG if patient is older than 40 yrs old - If suspecting an AMI with mural thrombus, do cardiac enzymes - Biochemical abnormalities (muscle necrosis): K, CK, lactic acidosis - ANGIOGRAM

Can be done in pts with viable limb; in pts with threatened limb there is no time for angiogram Æ may do on-table angiography High clinical probability of embolism does not need angiography Useful in 1. confirming an occlusion, 2. cause – thrombotic or embolic 3. pinpointing the level of occlusion and the anatomy

DEFINITIVE TREATMENT OPTIONS

Surgical Endovascular - Embolectomy - Endarterectomy - Bypass grafting - Fasciotomy - Primary amputation

- Thrombolysis - Angioplasty - Stenting

In general, embolectomy is done for embolic occlusion, while thrombolysis is done for thrombotic occlusion. Embolectomy - Can be done under LA but still require anaesthetist to monitor patient as he may be quite sick (e.g. AMI), and hyperkalaemia

with cardiac arrhythmia can occur after reperfusion - Involves clamping of the involved artery and making an arterotomy - A Fogarty balloon catheter is inserted into artery until distal to the clot, then balloon is inflated to trawl clot out of the artery - Check for forward-bleeding and back-bleeding of the vessel (i.e. free spontaneous flow from proximal and distal ends of the

artery when unclamped) - Flush with heparinised saline - Check foot – warm foot with good pulse indicates reperfusion - Important to monitor ECG for any arrhythmias! - Closure of arterotomy with meticulous haemostasis as patient is on heparin - Post-op: patient monitored in high-dependency; look out for reperfusion injury The reperfused muscles become oedematous (due to ROS tt injure cells), Çing pressure in the compartments of the leg,

like compartment syndrome Patient complains of calf pain Unable to dorsiflex ankle as the anterior compartment is affected first Requires three compartment fasciotomy to release pressure

- Need to convert to full warfarin anticoagulation, uptitrating dose until INR 2-2.5 before stopping heparin (pt at risk of further embolic events) Æ discharge patient to anticoagulation clinic for follow-up with warfarin advice

220

Catheter directed thrombolysis - Angiogram done before thrombolysis to locate occlusion - Thrombolysis catheter inserted into the clot, and the thrombolytic agent is infused (Streptokinase, Urokinase, tPA) - Patient will be in high-dependency with thrombolytic infusion for 6 hours (~1000-4000 units per minute) - After 6 hrs, redo angiogram to check for residual clot; if some clot remains, adjust catheter into clot and infuse for 6 more hrs - After complete lysis of the clot, can do angioplasty - Takes much longer than embolectomy - Thrombolysis may be preferred for embolism in a diseased artery, since it may be difficult to trawl out the clot in a diffusely

stenosed vessel – the clot may get caught on a proximal stenosed segment - CI

Absolute 1. CVA within past 2 months 2. Active bleeding / recent BGIT past 10 days 3. Intracranial trauma/ neuroSx past 3 months

Relative 1. CPR past 10 days 2. Major Sx / trauma past 10 days 3. Uncontrolled HTN

Results: - Embolectomy has a 20% mortality, almost full success rate - Thrombolysis has a 10% mortality, only 35% successful DDx: - Acute DVT: Phlegmasia cerulean dolens = painful blue edema - Blue toe syndrome: atheroembolism from AAA or more proximal - Purple toe syndrome: Cx of warfarin therapy - Venous insufficiency - Venous occlusion - Acrocyanosis

221

3. CHRONIC LIMB ISCHAEMIA

Chronic limb ischaemia can be divided into critical and non-critical limb ischaemia, and non-critical ischaemia further subdivided into that which causes symptoms (usually claudication) and that which is asymptomatic. Most common cause is atherosclerosis with gradually developing diffuse stenosis of the peripheral arteries resulting in diminished blood supply to the lower limb (imbalance between supply and demand). Multiple collaterals form to bypass the obstructed vessels as a compensatory mechanism. Progression:

CRITICAL LIMB ISCHAEMIA Critical limb ischaemia is defined as decrease in limb perfusion that causes a potential threat to limb viability (manifested by ischemic rest pain, ischemic ulcers, and/or gangrene) in patients who present more than two weeks after the acute event (the converse of the definition of acute limb ischaemia). FEATURES: 1. Rest pain requiring regular opioid analgesia (e.g. codeine) lasting >2 weeks

+/- Tissue loss (Gangrene or ulcers over the toes or feet) 2. Objective indication of poor vascular supply to the lower limbs

(a) Ankle brachial pressure index <0.5 (b) Toe pressure index < 0.3 (c) Toe pressure <30 mmHg, Ankle pressure <55mmHg

I. Rest pain

- Severe pain in the distal portion of the lower limb (usually toes, foot but may involve more proximal areas if disease is severe) occurring at rest (calf pain without toe pain is not)

- Pain is aggravated or precipitated by lifting the limb, relieved by dependency of the limb – many patients sleep with the leg hanging over the side of the bed to relieve the pain

- So severe as to disturb sleep at night – È blood supply as pt is not in dependent position and decrease BP during sleep - Not easily controllable with analgesia – requires opioids to control pain

II. Ischaemic ulcers (most are neuroarteropathic ulcers)

- Usually arise from minor traumatic wounds with poor healing - Often painful - Most often occur on the tips of the toes, bunion area, over the metatarsal heads (ball of the foot), lateral malleolus (as opposed to

venous ulcers that occur over the medial malleolus) - Usually deep, dry, punctate (unlike venous ulcers that tend to be superficial, moist, diffuse) - May become infected Æ cellulitis / abscess, and spread to involve the underlying bone and joints Æ osteomyelitis, septic arthritis

III. Gangrene

- Cyanotic, anaesthetic tissue associated with or progressing to necrosis - Occurs when arterial blood supply falls below that which is necessary to meet minimal metabolic requirements - Either dry or wet:

DRY – hard, dry texture. Often has a clear demarcation between viable and necrotic tissue. Occurs in patients with atherosclerotic disease. Safe and can be allowed to autoamputate after demarcation with precautions against infection. WET – = infected dry gangrene. Moist, swollen, frequently blistered. Often occurs in diabetics with decreased sensation and unrecognised trauma, requiring an emergency surgical debridement or amputation.

222

NON-CRITICAL LIMB ISCHAEMIA WITH CLAUDICATION Intermittent claudication is defined as a reproducible discomfort of a defined group of muscles that is induced by exercise and relieved with rest. Usually described as the patient as a cramping, aching pain in the muscle group on exertion such as walking, and alleviated on stopping (patient does not have to sit down for pain to go away) – “shop window to shop window”. - Calf claudication Å Usually affects the superficial femoral near to the adductor hiatus, or the popliteal artery - Foot claudication Å tibial & peroneal arterial disease, but rarely do patients with claudication due to atherosclerosis get foot pain alone (more

common in Buerger’s) - Thigh claudication Å common femoral artery or aortoiliac disease - LeRiche’s syndrome arises from occlusion of the aortoiliacs, and is composed of a classical tetrad of buttock claudication, impotence in men,

absent femoral pulses (and distal pulses), and occasionally presence of aortoiliac bruits. - Determine “claudication distance” – within a short period of time it is usu fairly constant, but can shorten as the disease progresses - Need to differentiate the various causes: vascular vs neurogenic vs musculoskeletal CAUSES OF VASCULAR CLAUDICATION - Mainly an atherosclerotic disease; pain is due to acidosis & buildup of metabolites - Other less common causes: ergot toxicity, Takayasu’s arteritis, Buerger’s disease (thromboangiitis obliterans), vasospasm DDX NEUROGENIC CLAUDICATION!!! Vascular intermittent claudication needs to be differentiated from neurogenic claudication (can also present as pain in the lower limb on exertion) The characteristic of neurogenic claudication is “park bench to park bench”

1. patient has to sit down and flex the spine to relieve the pain (pain results from compression of the cord and spinal nerves in spinal stenosis; extension of the spine further narrows the spinal canal while flexion widens it)

2. “Claudication distance” of neurogenic claudication is more variable 3. Pulses will be absent/diminished in vascular but not in neurogenic claudication 4. Parasthesia is common in neurogenic claudication

TAKING A HISTORY OF CHRONIC LIMB ISCHAEMIA 1. Claudication = muscular pain, reproducible, incr on walking/dec w rest

- Which part of the lower limb does the pain occur in – depends on level of obstruction - Nature of the pain - Radiation - Severity - Aggravating factors – exertion - Relieving factors – rest (just standing is sufficient) - Associated symptoms e.g. impotence in LeRiche’s - Duration: When did pain first start (>2/52?) - Progress since first noticed until currently (worsening pain, Çing areas of lower limb affected, pain on less exertion, rest pain) - Current claudication distance - How has symptoms affected lifestyle e.g. impaired mobility

2. Any rest pain

- Site, nature, severity - Aggravating factors – raising the limb - Relieving factors – putting limb in a dependent position - Able to relieve with normal analgesics? Or require opioid analgesia? - How long has rest pain lasted for requiring opioid analgesia (if > 2 wks, considered a feature of critical limb ischaemia)

3. Any ulcer or gangrene in the lower limb?

- Ask about onset of ulcer/gangrene - Progress (stable, or increasing in size, getting worse) - If ulcer, any preceding trauma? Ill-fitting shoes? Altered sensation in the foot? Does patient take care to protect foot? Pain?

Redness/swelling/warmth in surrounding skin? Purulent/foul-smelling discharge from the ulcer? - If gangrene, is it wet or dry? Redness/swelling/warmth in surrounding skin? Any feeling in the toe involved? Any sensory changes in the other

normal toes, foot, limb? - Any systemic signs of infection – fever, chills, rigors, malaise

4. Risk factors (“Arteropath”)

- Diabetes mellitus – take a full diabetic history including other complications - Hyperlipidaemia / Heart disease (IHD) / Stroke (CVA) - Smoking (very strong RF; 3-6X risk of claudication, higher than the risk for IHD) - Family history

5. Drug history

- Aspirin and statin intake – commonly prescribed for vasculopath - Any allergies to contrast (for angiography) - Ergots

6. Social history

- Premorbid function and current function - Social support and home condition (need to climb stairs?)

223

PHYSICAL EXAMINATION Examine the patient’s lower limbs in a warm room, exposed optimally (from the thighs to the feet, wearing underwear). Patient is supine with the bed flat. Look 1. Colour of the lower limb

- White (pallor); pink (normal); blue/dusky (cyanosed); mottled 2. Trophic changes

- Loss of hair - Dry, shiny skin - Nail changes - Wasting

3. Presence of any diabetic dermopathy 4. Presence of ulcer

- Look carefully at the entire lower limb, including the heels (ask pt to flex at the knee) & between the toes - Site of the ulcer Venous ulcers form at the medial malleolus Arterial ulcers are more distal, usually between the toes, and at pressure points such as the lateral malleolus; Neuropathic ulcers form at areas such as the ball of the foot and the heel

- Size, shape - Edges (punched out – arterial; sloping – venous) - Base Depth of the ulcer (can see underlying tendon? Down to bone?) Appearance of the base – Necrotic? Granulating (beefy-red)? Sloughy? Any discharge – pus, blood?

- Surrounding skin Erythema (cellulitis) – there may be an underlying abscess (confirm on palpation) Blistering, purplish colour (possibility of necrotising fasciitis)

5. Presence of gangrene - Wet (infected) or dry (not infected) - Extent of gangrene (level of demarcation)

6. (If the patient has diabetes, may see deformities – Charcot’s joint) Feel 1. Warmth of the skin

- Use the dorsum of the fingers of both hands to simultaneously run up the patient’s feet to the shins and thighs bilaterally - Compare the temperature on both sides (note if one side is cooler) - If one limb feels cool, feel for the level where the skin becomes warm

2. Capillary refill - Press hard on a toe for a few seconds, then release - Normal capillary refill should be 2 seconds or less - If a toe is blue, check for blanchability (fixed staining = dead toe)

3. Palpating the ulcer if present - Any surrounding tenderness (infection) - Bogginess of surrounding tissue (may have abscess formation) - See if any discharge from the ulcer when palpating

4. Pulses – compare both legs. Check regularity of pulses. - Feel the distal pulses and work your way proximally - Posterior tibial pulse: one-third of the way down a line joining the medial malleolus to the heel - Dorsalis pedis pulse: just lateral to the ext hallucis longus tendon/ one third of the way down a line joining the midpoint

of the two malleoli to the cleft between the first and second toes - Popliteal pulse: Ask patient to bend the knee ~60-90 degrees, then palpate deeply in the popliteal fossa with the fingers

of one hand pressing the fingers of the other. Thumbs on the tibial tuberosity. [If the pulse is very well felt, suspect a popliteal aneurysm]

- Femoral pulse: Midpoint of the line joining the pubic symphysis to the anterior superior iliac spine (mid-inguinal point), just below the inguinal ligament

- Abdominal aorta

Grading of pulses: 2+ normal; 1+ diminished (but may be normal for popliteal); negative if not felt

224

Move 1. Buerger’s test

- Lift both legs together to compare - Holding the heel of the foot, with the pt’s LL straightened, slowly lift the entire LL, looking at the colour of the toes - Stop when the toes become pale (white) - Estimate the angle the lower limb makes with the horizontal – this is the Buerger’s angle Normal lower limb can be raised to 90 degrees without turning white;; if the Buerger’s angle is less than 30-40 degrees,

this indicates critical ischaemia - There may be venous guttering of the lower limb at this angle as well - Hold for 1 min (to induce ischemic environment) - If the patient is lying near the side of the bed, tell the patient that you’re going to put his leg over the edge of the bed

before gently abducting the hip and then letting the leg drop over the edge of the bed - Look at the leg for reactive hyperaemia (the leg turns purple-red) – due to ischemic env causing increased acidity and

triggering autonomic response Complete the exam - Examine the rest of the pulses - Offer to auscultate over the femoral and popliteal arteries for bruits - Examine the abdomen for any abdominal aortic aneurysm - Measure the ankle-brachial pressure index (ABPI) INVESTIGATIONS 1. Ankle-brachial pressure index

- How the ankle-brachial pressure index is done Lie patient supine (so that ankle pulse will not increase sec to dependency) Brachial pressure is measured with a blood pressure cuff around the arm and a Doppler probe at the brachial artery –

cuff is inflated until the arterial signal is obliterated, then slowly deflated until the signal just starts being detected, at which the pressure is recorded

Ankle pressures are measured in a similar manner, with the cuff around the calf and the Doppler at the dorsalis pedis and posterior tibial arteries – one reading for each artery

The ankle pressure to be used for each leg is the higher of the two taken This ankle pressure is then divided by the brachial pressure (the higher of the two brachial pressures for both upper

limbs) to get the ankle-brachial pressure index

- Interpreting the values Normal ABPI is > 0.9 (can be more than 1.0 as ankle pressures tend to be higher than brachial; ABPI between 0.5 - 0.9 – occlusion, often associated with claudication ABPI <0.5: Critical ischaemia Rest pain if >1.25, suggests non-compressible calcified vessel esp seen in DM patients

Æ do Toe pressure index instead (every value minus 0.2)

- Accuracy of the index ABPI below 0.9 has 95% sensitivity and 100% specificity for detecting angiogram-positive peripheral arterial disease

and is associated with >50% stenosis in one or more major vessels

- Exercise treadmill testing Measure ABPI before and after patient exercises on a treadmill If the ABPI falls by >0.2 Æ claudication

2. Arterial Duplex ultrasound

- Non-invasive test, good alternative to angiogram - Duplex (means 2 modalities) = 2D ultrasound plus Doppler ultrasound (measures flow and waveforms) - Normal arterial flow waveform should be triphasic (rapid antegrade flow reaching a peak during systole, transient reversal

of flow during early diastole, and slow antegrade flow during late diastole.); biphasic & monophasic waves are abnormal - Distal to stenosis: rate of rise is delayed, the amplitude decreased, and the transient flow reversal in early diastole is lost - A twofold increase in peak systolic velocity compared with the velocity in an adjacent segment of the artery usually

signifies a stenosis of 50% or more - Can define anatomy of occlusions and also look for relatively good arteries distally for “landing zone” of bypass graft

225

3. Angiogram (arteriogram) - Invasive and associated with risks of

1. bleeding from arterial puncture, 2. dissection 3. damage to artery with worsening ischaemia

- Usually only done if planning intervention e.g. angioplasty, stenting - Preparing for angiogram:

Take informed consent from patient Ask about contrast allergy, asthma, renal disease, metformin Investigations: FBC (platelets impt), PT/PTT, UECr

- Angiogram with digital subtraction – the images of the underlying bone are removed so as to better visualise the arteries (if the bones are visible, then it is a normal angiogram, without digital subtraction)

4. Basic laboratory investigation

- FBC, UECr, PT/PTT, septic workup: bld c/s, wound c/s

ASSESSMENT OF SEVERITY The three L’s of peripheral arterial disease: (i) Life – does disease threaten life (e.g. sepsis; other cxs of atherosclerosis e.g. stroke, AMI;) or will intervention cause risks (ii) Limb – will patient lose the limb (iii) Lifestyle – is the lifestyle of the patient severely handicapped, does it require intervention Fontaine system Stage I: Asymptomatic Stage IIa: Mild claudication Stage IIb: Moderate to severe claudication Stage III: Ischaemic rest pain Stage IV: Ulceration or gangrene (A) TREATMENT OF CLAUDICATION – BASED ON QOL Conservative Mainstay for all cases of claudicants, esp. foot and calf claudicants - RF control

Assessment and treatment to optimise control of CVS risk factors– cardiologist Antiplatelets [e.g. aspirin] and statins (target lipid levels are much lower) Smoking cessation – not shown to be useful

- Exercise training to stimulate collateral formation Æ symptoms get better Exercise at least half to one hour every day Walk until pain comes, rest 2-3 minutes, walk again Keep a walk diary recording daily claudication distance in paces Supervised exercise 3X/wk helps. Advice to exercise alone does not.

- Patient educaton: Teach patient to go to ED if symptoms of critical ischaemia arises Podiatrist to teach foot care

- Medications Craxilen and Cilasterzol shown to increase claud dist - Ex. Use of Vasteral (methoxyphylline) is controversial

- Monitor regularly with measurement of ABPI Intervention (endovascular or surgical)

- At least 6 months of conservative treatment first; mainly for aortoiliac disease - Monitor claudication distance & ABPI – usually intervene if claudication distance <50m (poor QOL, almost critical limb ischemia) - Confirm disease outline with CT angiogram or angiogram - If parameters improve but then plateau, discuss with patient about whether he can accept the level of symptoms, and the risks

of intervention Æ weigh risks against benefits - Usually do angioplasty rather than bypass as it is less invasive, though may not be as effective in treating the symptoms

226

1. Angioplasty 1. Stenting usually not done for lower limbs except in aortoiliacs (since stent needs to be placed in a vessel which is relatively fixed and won’t be kinked/bent by movement) – not true: can even stent foot arteries!

2. Angioplasty only effective for focal stenotic lesions and better for large vessels. Problem with angioplasty is that it is not long-lasting – restenosis can occur

3. New method: subintimal angioplasty – if lumen is so occluded that guide wire cannot pass through, the guidewire is threaded into the subintimal space to create a dissection around the occluded segment, and this space is then angioplastied to create a channel parallel to the actual lumen for blood to flow through

2. Bypass grafting

Consider bypass when lesions cannot be treated by angioplasty i.e. lesion extends for long distance through the vessel and/or no lumen for guide wire to pass through (complete occlusion)

Needs a good “landing zone” for graft distally – if vessel is diffusely diseased, difficult to perform bypass (B) TREATMENT OF CRITICAL LIMB ISCHAEMIA

- Need to revascularise – either angioplasty or bypass grafting (C) AMPUTATION Indications (3 D’s) 1. Dead: Necrotic tissue 2. Dangerous: Gangrene, ascending sepsis 3. Damn nuisance: Non-functional limb; bad smell; pain; constant need to dress wound - Level of amputation depends on

vascularity of the limb (palpable pop pulse: BKA) indication (e.g. if infected, need to amputate above level of infection)

- As far as possible try to preserve function of the lower limb - May require revascularisation interventions before amputation to ensure good healing, or to enable lower amputation - Do not simply amputate without ensuring good va - scular supply to the surgical site, otherwise the wound will not heal

227

17. ABDOMINAL AORTIC ANEURYSM

EPIDEMIOLOGY More common in men than in women (4:1 ratio) Predominantly in older patients (>60 years old) Other risk factors: smoking, hypertension, strong family history (Marfan, Ehler-Danlos) PATHOLOGY - An aneurysm is a localised abnormal dilatation of a blood vessel wall or the heart - True aneurysms are bound by all layers of the blood vessel wall, while a false aneurysm is a breach in the blood vessel wall

leading to an extravascular haematoma that freely communicates with the intravascular space - Atherosclerosis is the most common aetiological factor – plaque formation results in destruction of the tunica media (and the

elastin fibres in it) Æ arterial wall thinning and loss of elastic recoil Æ dilatation - Other causes: cystic medial degeneration (in Marfan), trauma, infection (mycotic) - Location: 95% of cases are infrarenal, may extend to involve common iliac arteries, rarely beyond. 5% are juxtarenal, thoracic

or both - Size: 3 to 15 cm (normal aorta is 2cm in diameter) - Shape: Usually fusiform – long dilated segment (versus saccular which is spherical) - Often contains mural thrombus due to turbulence and stasis RISK OF RUPTURE - Small aneurysms <5cm have a 2-3% chance of rupture per year, while aneurysm larger than 5.5 cm will have a 10% risk of

rupture per year 75% of aneurysms 7cm or larger will rupture in 5 years

PRESENTATION - Asymptomatic (Most commonly; found incidentally during imaging) - Rupture: intense abdominal pain radiating to the back, becomes rapidly hypotensive and goes into shock (Most feared

presentation) - Trash feet: distal Thromboembolism Æ gangrene of feet - Local compression on neighbouring structures e.g. ureter - Obstruction of branches from aorta e.g. iliac, renal, mesenteric, vertebral - Also ask for vascular risk factors (DM, smoking, HPT, hyperlipidemia, previous CVA, IHD), AAA risk factors (smoking, HPT,

Marfan’s/ Ehler-Danlos) & GA risk factors (any heart, lung or kidney diseases) PHYSICAL EXAMINATION - Ensure vitals stable - Visible pulsation over abdomen - Pulsations and mass in epigastric region felt on deep palpation - Mass is expansile – when fingers of both hands are placed at the edges on either side of the mass, the fingers are pushed

upwards and outwards - Auscultate for bruit over the mass - Check the other arteries – femoral, popliteal – for any aneurysm, and listen for bruits - Look at the lower limbs for any gangrene, infection, etc DIFFERENTIAL DIAGNOSIS OF UPPER ABDOMINAL PAIN WITH SHOCK

- Pale: ruptured AAA, ruptured hepatoma, BGIT, ruptured spleen in trauma, mesenteric bleed - Not pale: sepsis, pancreatitis, perforated viscus, AMI , pyonephrosis

INVESTIGATIONS Imaging: CT AbdoPelvis Other investigations: FBC, UECr, PT/PTT, GXM for 4 units, ECG & CXR MANAGEMENT Dependent upon clinical context: asymptomatic, symptomatic, or ruptured?

228

Ruptured AAA - Very high mortality – nearly 100% if frank rupture (will not get to ED in time) - Most of the patients who reach the ED (about 50% reach ED alive) have a leaking AAA with a tamponade effect by the

retroperitoneal structures - High suspicion in unstable hypotensive patient complaining of severe sharp pain radiating to the back; may feel a pulsatile

mass in the abdomen

1. Stabilise patient – resuscitation with fluid and blood products x Do not intubate as neuromuscular blocking agents will reduce tamponade effect, worsening haemorrhage

2. Call for vascular surgeon 3. Bring to OT for open repair – quickly isolate the aorta and clamp it proximally

x can be clamped for about 30 minutes without significant visceral ischaemia x AAA is incised, the surrounding haematoma and mural thrombus within the AAA are cleared out x Synthetic graft (Dacron – polytetrafluoroethylene) is placed within the aorta and the vessel wall closed up over the

graft i.e. the graft forms the lumen of the aorta o Mortality rate of repair operation in this setting is about 50%

4. Most common complication postoperatively is renal insufficiency – can be reduced by giving frusemide or mannitol pre-operatively before anaesthesia induction

Non-ruptured AAA - Time available for investigation of size of AAA and related anatomy - Indications for surgery:

(a) Aneurysm > 5.5 cm in largest diameter [risk of surgery outweighs that of AAA] (b) Increase in diameter of more than 1cm per year (c) Symptomatic aneurysm – back pain, tenderness on palpation, distal embolism, ruptured/leaking aneurysm

- Patient’s fitness for surgery needs to be properly assessed because it is a major operation – need to optimise CVS function - Operation is the same except that it is done under elective setting - Mortality is <5% in the elective setting, serious morbidity ~10% Endovascular stenting - An alternative to open repair which is less invasive, can be done under GA - Mortality ~1%, but serious morbidity rate is similar to open repair: 10% - Involves deployment of a non-porous stent within the aneurysm to form the lumen of the aorta – requires adequate “neck”

proximally and good landing site distally - Not as good results as open surgery; need to do an angiogram every 6 months to check position of stent (ensure not migrated) COMPLICATIONS OF SURGERY Intraoperative/early 1. Acute myocardial infarction – most patients already have atherosclerotic disease of coronary vessels and are at risk of AMI

(responsible for 50-60% of mortality) 2. Stroke (due to hypotension or embolism) 3. Renal insufficiency 4. Colon ischaemia – occurs in 2-6% 5. Trash foot – embolism of thrombus from the aneurysm 6. Infection of graft 7. Spinal cord ischaemia (quite uncommon) 8. Haemorrhage Late 1. Aortoenteric fisula – frank PR bleeding, torrential 2. Late infection of prosthetic graft material 3. Sexual dysfunction Post operative investigations:

x FBC: Hb and plt (blood loss and consumptive coagulopathy) x UECr: renal insufficiency or contrast nephropathy x KUB: check position of stent

229

18. PERIPHERAL VENOUS DISEASES 1. ANATOMY OF THE VENOUS SYSTEM OF THE LOWER LIMB

- The venous drainage of the lower limb is divided into a deep venous system and a superficial venous system separated by the

deep fascia of the lower limb - The deep venous system is composed of veins corresponding to the arterial supply e.g. anterior and posterior tibial veins,

popliteal vein, femoral vein - The superficial venous system is composed of two major veins, the great saphenous vein and the small saphenous vein - Course of the great saphenous vein: Arises from the medial end of the dorsal venous arch of the foot Passes anterior to the medial malleolus Runs up the leg posteriorly to pass behind the medial surface of the knee Then runs anteriorly and laterally up the thigh Pierces the cribriform fascia at the saphenofemoral junction to drain into the femoral vein

230

- Course of the small saphenous vein: Arises from the lateral end of the dorsal venous arch of the foot Passes posterior to the lateral malleolus Runs up the midline of the calf Pierces the deep fascia over the popliteal fossa to drain into the popliteal vein

- The superficial system and the deep system communicate through communicating veins that contain valves which allow only

one-way flow of blood from the superficial vein into the deep vein (Foot perforators allow bi-directional flow directed by muscle movements)

- Locations of the communicating veins:

a. Saphenofemoral junction (great saphenous drains into femoral vein): located 2.5 cm below and lateral to the pubic tubercle [HDB]

b. Hunterian perforator: mid-thigh c. Dodd’s perforator: distal thigh d. Boyd’s perforator: knee e. Calf perforators: at 5, 10, and 15 cm above the medial malleolus

- Physiology of venous drainage: Main mechanism is the calf muscle pump:

Contraction of the calf muscles compresses large venous sinuses in the muscles, squeezing the blood into the popliteal vein

and back to the heart

(The deep veins have many valves to prevent backflow, so blood only flows towards the heart) During calf muscle relaxation, the intramuscular veins open and suck blood in from the superficial system through the

communicating veins, thus draining the superficial veins

231

2. CHRONIC VENOUS INSUFFICIENCY

Chronic venous insufficiency develops when there is venous hypertension, which can result from:

1. Obstruction to venous flow e.g. tumour compression in the pelvis, pregnancy, deep vein thrombosis 2. Dysfunction of venous valves e.g. varicose veins 3. Failure of the “venous pump” – dependent on adequate muscle contraction (stroke, muscular weakness can cause failure) as

well as competent venous valves MANIFESTATIONS OF CHRONIC VENOUS INSUFFICIENCY: [4] 1. Venous dilatations[4]

(a) Telangiectasias (spider veins or venous stars – intradermal veins)

15 % of men and 25 % of women in the general population.In the classification of veins, telangiectasias are classified as type I veins. Spider veins are amenable to laser therapy and sclerotherapy.

(b) Reticular veins (slightly larger intermediate veins) also known as blue veins and intradermal varices. In the classification of veins, reticular veins are considered type III veins.

(c) Varicosities (visible, dilated tortuous superficial veins; formed by main tributaries of the saphenous veins because these do not have a strong coat of smooth muscle in their walls, unlike the saphenous veins; they are more superficial and not bound down to the deep fascia) Trunk varicosities – along main trunk of LSV/SSV.

(d) Corona phlebectactica (a network of small dilated venules beneath the lateral and/or medial malleolus with severe venous hypertension)

2. Oedema – pitting: The hallmark of CVI; present in all but the earliest stages

Unilateral oedema worsened by dependency (worse at the end of the day) and better with recumbency 3. Skin changes[5]

(a) Hyperpigmentation over medial lower third of the leg (gaiter area) – extravasation with haemosiderin deposits (b) Atrophie blanche – hypopigmented scars of healed venous ulcers (avascular and fibrotic skin) (c) Venous stasis eczema – pruritic, weeping, scaling, with erosions and crusting (d) Lipodermatosclerosis (fats and skin hardening) – a fibrosing panniculitis of the subcutaneous tissue that results in a

firm area of tender, hyperpigmented skin that is fixed to subcutaneous tissue. Results from severe venous hypertension Æ hemosiderin deposits at fats Æ inflammation Æ chronic inflammation Starts in the gaiter area and extends circumferentially to surround the leg If severe can result in an “inverted champagne bottle” appearance of the leg with brawny oedema above and below

the area of lipodermatosclerosis (e) Cellulitis

4. Venous ulcer formation

- Typical location is over the medial malleolus - Shallow, flat ulcer with sloping edges; base may be sloughy or granulating, usually quite moist-looking - Surrounding skin will show signs of CVI - In long-standing ulcer SCC can develop (Marjolin’s ulcer) – If ulcer

1. enlarges, 2. becomes painful 3. malodorous, 4. edge becomes thickened or raised, 5. inguinal lymph nodes are enlarged

SYMPTOMS

1. asymptomatic 2. leg fullness / swelling 3. heaviness, 4. non-specific pain: aching discomfort, dull, shooting 5. nocturnal leg cramps, 6. itch (40%) 7. bursting pain upon standing.

232

CEAP CLASSIFICATION OF CHRONIC VENOUS INSUFFICIENCY

3. VARICOSE VEINS

Varicose veins are dilated, tortuous veins of the superficial venous system (secondary to incompetent leg valves resulting in reflux).

x Primary varicose veins: cause is unknown (may be related to posture and components and structure of the vein wall) GENETIC! (20% risk in gen pop, 50% 1 parent, 80% both parents)

x Secondary varicose veins: 1. destruction of the valves by thrombosis (DVT most common secondary cause), 2. proximal venous obstruction, 3. increase in flow and pressure caused by an arteriovenous fistula, 4. pelvic masses – compress on deep veins

PATHOPHYSIOLOGY - Inherent weakness in the vein wall, leading to dilation and separation of valve cusps so they become incompetent - This may be aggravated by obstruction to venous return (as above) RISK FACTORS (NOT CAUSES!) - in already predisposed people - Age - Parity - Occupation – requiring long periods of standing - Weight - Posture – crossing legs all the time - Increased abdominal pressure – constipation, chronic cough, etc - Family history: 1 parent (50% risk, both parents (up to 80% risk) HISTORY x Asymptomatic x Symptomatic: local (non specific pain, swelling, itching, heavy legs) x Complications :

1. ulceration +/- cancer 2. thrombophlebitis (= thrombosis Æ inflammation, chemical rather than infective. commonly mistaken as cellulitis) 3. bleeding, 4. hyperpigmentation, 5. eczema, 6. infection (due to dependent oedema)

x Risk factors: Ask for Past history of DVT & thrombophlebitis, obstetrics history, family history

233

EXAMINATION Examine patient STANDING with adequate exposure of the lower limbs Inspection (look all around the limb!) 1. Presence of signs of chronic venous insufficiency (as above)

- Oedema - Skin changes, stasis eczema, lipodermatosclerosis (feels like leather, v tight) - Venous ulcers from pressure necrosis from insude

2. Look at course of great saphenous vein and short saphenous vein for varicosities 3. Look at the inguinal region for any saphena varix Palpate 1. Feel any dilated varicosities: Direct tenderness that can account for patient’s pain symptoms

(if patient’s varicosities are not tender, warn patient that ankle/calf pain may not go away after surgery as the pain may not be directly from the veins)

2. Palpate along the course of the saphenous veins and their tributaries to feel any varicosities and for tenderness (may be more palpable especially in fat legs)

3. Palpate the inguinal region for a saphena varix (compressible lump that refills when released) 4. Do the cough test to feel for reflux at the saphenofemoral junction (2.5 cm below and lateral to the pubic tubercle) 5. Percussion (tap test) – test for valvular incompetence (not a very valuable test) – Ensure good distance between both hands

first, to cover valves in between. Positive if the distal hand can feel the wave of blood flowing retrogradely after tapping the proximal varicosities (normal: tap bottom, feel thrill on top)

6. Feel the peripheral pulses!!! of Lower limb to exclude any ischaemia as the management will involve compression of limbs (ABI >0.8)

Special tests TOURNIQUET TEST

- Lie the patient down and empty the varicosities & tie a tourniquet just below the SFJ - Ask the patient to stand up - Look for filling up of the varicosities above and below the tourniquet - If the veins dilate above but not below the tourniquet, this indicates that the perforators below the level of the tourniquet are not

incompetent and that the SFJ is incompetent Æ confirm this by releasing the tourniquet and watching the veins dilate - If veins below the tourniquet are dilated when pt stands up, then the incompetent perforator is below level of the tourniquet - Repeat the test, placing the tourniquet at different sites:

(i) Mid thigh (just below the Hunterian perforator) (ii) Below the knee (iii) Mid-calf

- The incompetent perforator is located between just above the level where the tourniquet prevents dilation of the veins in the limb on standing

[The alternative is the triple tourniquet test, where three tourniquets are tied with the patient lying down and then released from the bottom up to locate the site of insufficiency] TRENDELENBURG TEST

- The SFJ is occluded (landmark is 2.5 cm below and lateral to the pubic tubercle) with the patient lying down - Get the patient to stand while holding the SFJ occluded - If varicosities do not fill up, the SFJ is the site of incompetence; if they fill up, there are other sites of incompetence (the SFJ

may or may not be incompetent) PERTHES’ TEST (Test of deep venous obstruction)

- Tie a tourniquet around the calf or thigh and ask patient repeatedly stand on tiptoe and then relax - In a person with normal deep venous drainage and competent venous valves in the communicating veins the superficial veins

should drain into the deep veins - Positive test: patient will complain of pain and stop after ard 20 times because of increased swelling as blood cannot drain out

through the deep veins

234

Completing the examination - Auscultate over the varicosities for any bruit (indicate arteriovenous malformation) - Examine the abdomen for any mass that may be causing the varicosities Use of a handheld Doppler probe to detect incompetence 1. Doppler probe is placed along groin crease over SFJ (caution in obese ppl whose groin crease are lower) with patient standing 2. Look for arterial signal then move medially Æ SFJ 3. Squeeze the calf to empty the veins – should hear a whoosh as blood flows through the small saphenous vein 4. When the calf is relaxed there should not be a prolonged sound – a second whoosh >0.5s indicates reflux of blood i.e. there is

valvular incompetence of SFJ (90% accurate prediction) Can also perform test over popliteal fossa for SPJ but less accurate as the junction is variable. Therefore, you may be getting signals from the deep perforators instead. INVESTIGATIONS Venous duplex ultrasound - Indications:

1. Recurrent varicose veins 2. Complications of CVI: History of superficial thromobophlebitis 3. Skin manifestations of CVI: Venous eczema, Haemosiderin staining, Venous ulceration , Lipodermatosclerosis, 4. Everyone with varicosities to exclude subclinical DVT

- Ask for

(a) SFJ and SPJ reflux, (b) perforator, deep venous incompetency (c) DVT screen

- Can delineate deep and superficial venous systems and locate sites of incompetence - Exclude presence of deep vein thrombosis – stripping is contraindicated MANAGEMENT Conservative

1. Lifestyle changes

- Decrease amount of time spent standing - If due to job, change job or ask for change to position to stand & walking less

2. Graduated compression stockings, usually grade II Î ensure good pulses 3. Medications e.g. Daflon (only symptomatic, like painkillers) Surgical – the only definitive treatment

Indications: 1. Cosmesis – large unsightly varicosities 2. Symptoms – pain, discomfort 3. Complications – signs of chronic venous insufficiency, venous ulceration Contraindications:

1. Infected ulcers. (slow healing ulcers that are not infected can still operate as it may improve the ulcer) 2. DVT: can rescan after medical treatment. If DVT resolves, can opt for selective stripping. 3. Thrombotic tendencies: patient may dev DVT in the future.

Available modalities: 1. High tie with great saphenous vein stripping, and stab avulsion of varicosities 2. Ultrasound-guided foam sclerotherapy 3. Endovenous laser therapy (burns vein from within) 20% recurrence: due to large number of perforators left over even after stripping.

235

4. VENOUS ULCERS

CAUSE – ANY CAUSE OF CHRONIC VENOUS INSUFFICIENCY 1. Obstruction to venous flow – thrombosis 2. Incompetent valves – varicose veins, deep vein reflux (post-DVT) 3. Muscle pump failure – stroke, neuromuscular disease INVESTIGATIONS 1. Exclude infection of the ulcer and other complications

- FBC for raised total white count - Swabs of the ulcer for Gram stain and cultures - X-ray of the area to exclude underlying gas, bone involvement

2. Venous duplex to map out venous system 3. Check for peripheral arterial disease by doing ABPI 4. Biopsy if cannot exclude malignant transformation (Marjolin’s ulcer) MANAGEMENT Conservative 1. 4 layer compression stockings (change 2X/week if ulcer, 1X/wk if no ulcer)

(a) Non-adherent wound dressing over ulcer (e.g. Menolin) followed by wool bandage – most impt layer to protect ulcer (b) Crepe bandage (c) Blue-line bandage (Elset) (d) Adhesive bandage (Coban)

Aim: ankle pressure around 30mmHg (can vary according to needs) Nowadays, 2 layer stockings are used too – can achieve the same ankle pressure but not as effective as it loses pressure more quickly so less consistent pressure. 2. Stockings: Once healed (cannot use with ulcer/wound), compression stockings should be fitted and continued for life 3. Symptomatic: Analgesia 4. Antibiotics if infected 5. Lifestyle

(a) Warn patient to avoid trauma to affected area (b) Encourage rest and elevate leg

Surgical If ulcer fails to heal: - First, exclude malignancy or other causes of ulcer (biopsy) - Split skin graft can be considered with excision of dead skin and graft attached to healthy granulation tissue - Venous surgery for the underlying pathology

236

237

19. UROLOGICAL DISEASES 1. APPROACH TO HAEMATURIA

DEFINITION: - >3 RBC / hpf. - DDx: haemoglobinuria, myoglobinuria, pseudohaematuria (menstruation), meds causing discoloration (eg rifampicin, phenytoin) CAUSES

Pre-Renal

Drugs Analgesics (NSAIDs) Anticoagulants Cytotoxic/immunosuppressive agents (eg cyclophosphamide) OCP Penicillin Quinine Warfarin

Systemic Bleeding diathesis Sickle cell disease

Metabolic Hypercalciuria Hyperuricosuriia

Vascular AV malformations Renal artery disease – thromboembolism, dissecting aneurysms, malignant

hypertension Renal vein thrombosis

Renal Vasculitis HSP PAN Wegener granulomatosis

Glomerular Post-strep GN Post-infectious GN IgA nephropathy Lupus nephritis Other GNs

Tubulo-interstitial dz

Polycystic kidney disease Nephrolithiasis Malignancy – RCC, metastatic Pyelonephritis Renal cysts

Post-renal

Infxns of ureter, bladder, prostate, urethra – eg schistosomiasis, TB etc Cancers of ureter, bladder (TCC), prostate, urethra Nephrolithiasis

HISTORY Post-renal Causes 1. Which part of urine stream is blood stained?

- Beginning – urethra distal to UG diaphragm - End – bladder neck or prostate - Throughout – upper urinary tract or upper bladder

2. Painful vs painless haematuria

Painful Painless - UTI - Pyelonephritis - Tumour - Hydronephrosis - Renal cysts - Ureteric stone / clot - Bladder outflow obstruction (e.g. BPH,

strictures)

- Malignancy – TCC, RCC, Prostate - Drugs - GN - Bleeding diathesis - ITP / HSP - Infections – malaria, schistosomiasis - Exercise (jogger’s hematuria)

3. Severity

- Clots - S/S of anemia

238

4. Frequency + dysuria + haematuria - DDx: nephrolithiasis (colicky), malignancy, UTI (women & children), bladder outflow obstruction (men e.g. BPH – vascular

prostate may bleed) 5. Other urological symptoms

- Storage problem – frequency, urgency, nocturia, incontinence - Voiding problem – strangury, hesitancy, dribbling, incomplete emptying etc - Others – polyuria, oliguria, urethral discharge

Pre-renal & Renal Causes 6. Associated fever – pyelonephritis, malaria 7. Screen for pre-renal causes

LOW / bone pain / sickness Malignancies, TB, systemic illnesses Rash, arthritis, arthralgia, myalgia, fever, oedema Autoimmune causes, vasculitis Sore throat, skin infxns, URTI Post-strep / post-infective GN Ongoing URTI or GE IgA nephropathy Iatrogenic Drug causes, radiotherapy Travel history Schistosomiasis, malaria PMHx Renal disease, HPT, diabetic nephropathy, bleeding diathesis, sickle cell dz Family history PKD, sickle cell disease, renal dzes (eg Alport syndrome – ask for

deafness), HTN, urolithiasis Other necessary history 1. Infection - Fever, travel and contact history 2. Sorethroat - Post-strep/infective GN, IgA nephropathy 3. Autoimmune - Fever, rash, joint pain, oedema 4. Malignancy - LOW, bone pain, neuro deficits, SOB, liver function 5. PMHx - Renal dz, systemic dz (DM HPT Bleeding sickle cell) 6. Drug history / Hx of radiation 7. Family history – PKD, renal dz, Sickle cell, HPT PHYSICAL EXAMINATION 1. Check patient’s vitals- stable? 2. Conjunctival pallor 3. Abdomen – renal/bladder mass, palpable bladder (clot causing retention) 4. Scrotum – varicocoele on the left (may have RCC of the left kidney with extension of tumour into renal vein, blocking the

testicular vein where it drains into the left renal vein) 5. External genitalia – blood from urethra 6. Digital rectal examination – prostate enlargement (BPH versus cancer) INVESTIGATIONS URINE 1. Urine dipstick

- Causes of false-positive for blood: haemoglobinuria, beetroot, drugs (rifampicin), metabolic (alkaptonuria, porphyria)

2. UFEME (normal (all below 5): RBC 3-5, WBC 2-5, EC <5) - Confirm presence of red blood cells - Elevated WBC (pyuria is >5 WBC per hpf), organisms Æ infection - Casts Æ nephritis

3. Urine cytology for malignant cells - useful for: - High grade urothelial carcinoma (LG hardly shed cells) - Carcinoma in situ (imaging cannot see)

4. Urine phase contrast - RBCs – isomorphic or dysmorphic? Dysmorphic RBCs suggest a tubular source, while isomorphic RBCs suggest post-

renal source (ureter, bladder, etc) 5. Urine culture and sensitivity

239

BLOOD 6. Full blood count

- How low is the Hb? - Elevated TW – infection

7. Urea, electrolytes and creatinine - Any renal impairment and electrolyte abn (renal or pre-renal dz more likely)

8. PT/PTT - Anycoagulopathy

9. GXM!!! IMAGING (HEMATURIA – IMAGE ENTIRE URINARY SYSTEM) Upper tract: imaging, lower tract: cystoscopy 10. Plain KUB

- Margins: Superiorly needs to be above upper pole of the right kidney (T12), inferiorly needs to show pubic symphysis - Stones, size of kidney

11. Ultrasound of the kidneys - Renal size - Presence of any hydronephrosis, hydroureters - Renal stones

12. Intravenous urogram (IVU) - Look for

1. Distortion of renal OUTLINE and pelvic calyces by RCC, may have specks of calcification 2. Contrast UPTAKE: present or not (no contrast in obstruction / non-functioning), equal and symmetrical uptake 3. Configuration of the kidneys eg. Horseshoe kidneys 4. Stones (filling defect, proximal dilatation, decreased distal passage of contrast) + hydroureter and/or hydronephrosis 5. Filling defect in bladder due to TCC 6. Increased residual volume in bladder after micturition due to BPH

- Intravenous contrast used to delineate anatomy of the kidneys and urinary system - Various phases:

(i) Control film – plain KUB (ii) Tomogram – zoom into kidneys before contrast (iii) Nephrogram phase (1 min after contrast) – contrast fills kidney parenchyma so kidneys become more visible Æ

measure size, outline (iv) Pyelogram phase (3-5 min) – contrast fills calyces & pelvis, can detect dilated calyces/pelvis (hydronephrosis), any

filling defects (v) Release film (abdominal binder which was placed to slow the flow of contrast into the bladder is released) – shows

ureters, any hydroureter, filling defects (vi) Cystogram - any filling defects, abnormal appearance of the bladder (fir-tree appearance in neurogenic bladder) (vii) Post-micturition – any residual urine in bladder after voiding

- Contraindicated in:

(a) Contrast allergy (b) Renal impairment (Cr >200) (c) Patients on metformin (can cause lactic acidosis; patients need to stop metformin 2 days before and after study) (d) Patients with asthma (given steroids for 3 days before study) (e) Pregnancy: ask LMP

13. CT urogram / IVP - Non-contrast phase (stones) Æ Nephrogram (tumors) Æ Delayed phase - Adv: Ability to see renal parenchyma tumors (IVU only sees outline) - Disadv: Radiation ++ (3 CT scans) 14. Cystoscopy

- Detection of bladder tumour (IVU may not pick up small tumours <1cm) - Biopsy can be taken at the same time

240

2. RENAL CELL CARCINOMA

EPIDEMIOLOGY - 3% of adult malignancy - Most frequent occurring solid lesion within kidney - 2:1 male predominance - Peak incidence 60-70 years PATHOLOGY - Most common primary renal tumour (80-85% of all tumours of the kidney) - Arise from the renal tubular epithelium - Three cell types:

1. clear cell carcinoma (70-80%), 2. papillary renal cell carcinoma (10-15%), 3. chromophobe renal cell carcinoma (5%)

Other renal tumours: TCC of renal pelvis, Wilms’ tumour, lymphoma RISK FACTORS - Smoking - Exposure to cadmium (industrial) - Family history von-Hippel Lindau syndrome Æ clear cell carcinomas

Due to mutation of the VHL gene on chromosome 3p25 resulting in abnormal growth of BV ie. angiomas (associated with CNS haemangioblastomas (usually cerebellar), bilateral multicentric retinal angiomas, phaeochromocytomas, etc)

Hereditary papillary RCC (HPRCC) due to mutation of the MET proto-oncogene on chromosome 7q31 Æ multifocal bilateral papillary carcinomas

- Acquired polycystic kidney disease (secondary to chronic dialysis) PRESENTATION - Initially asymptomatic (may be detected incidentally) 1. Local S/S - Painless gross haematuria is the most common – >50% of cases - When tumour has grown large enough, dull flank pain and palpable mass may result Æ Classical triad of RCC: flank pain, painless haematuria, palpable renal mass (indicates late stage disease)

2. Complications 1. May have fever a/w night sweats, LOA, LOW, malaise 2. Polycythaemia occurs in 1-5% (due to increased erythropoietin) 3. For left renal tumour, extension of tumour into left renal vein can cause a left varicocoele as the left testicular vein

becomes occluded 4. Extension into IVC can cause (a) lower limb oedema, (b) ascites, (c) liver dysfunction, (d) pulmonary embolism 5. Paraneoplastic syndromes are uncommon – Cushing’s, hypercalcaemia, hypertension

3. Metastasis - Symptoms of metastases – lungs, liver, bones, brain, lymph nodes

241

INVESTIGATIONS DIAGNOSTIC 1. Imaging – CT and/or ultrasound

- Presumptive diagnosis is made on imaging – a renal parenchymal mass with (a) thickened irregular walls and (b) enhancement after contrast injection suggests malignancy

2. Pathological diagnosis

- Needle biopsy usually not done for resectable lesions due to fears of tumour seeding - In these resectable lesions, a partial or total nephrectomy is often performed, and provides the tissue diagnosis post-

operatively - In tumours with metastatic disease on presentation, biopsy of the metastatic site may be easier

STAGING 1. CT scan of the abdomen

- Perinephric invasion, adjacent organ invasion - Extension into renal vein, IVC - Lymph node enlargement - Liver metastases

2. CT scan of the chest

- For lung metastases Also, Bone scan

- Only done if patient complains of bone pain and/or alkaline phosphatase is raised MRI of abdomen and heart

- Superior to CT for evaluation of IVC and right atrium involvement

T1 Tumour <7cm, limited to the kidney T1a: tumour <4cm T1b: tumour >4cm but <7cm

T2 Tumour >7cm, limited to the kidney

T3 Tumour extends into major veins or invades adrenal gland or perinephric tissues, but not beyond Gerota’s fascia

T4 Tumour invades beyond Gerota’s fascia

Fuhrman grading Æ prognosis (not tx) - size and shape of nucleus - no and size of nucleoli - chromatin clumping I (best) Æ IV (worst) Prognosis Stage I (T1N0): >90% 5 year survival Stage II (T2N0): >75% Stage III (T3N0/N1): >60% Metastatic disease: <10%

242

TREATMENT A. RESECTABLE TUMOURS Surgery + Adj chemo + Surveillance after resection to detect relapse early - Laparoscopic versus open methods - Retroperitoneal versus transperitoneal approach 1. Partial nephrectomy (T1a)

- spares part of the kidney that is not involved Æ nephron-saving 2. Total nephrectomy (T1b)

- entire kidney removed 3. Radical nephrectomy (T2 and beyond)

- entire kidney together with Gerota’s fascia - In T3 disease, aim for radical nephrectomy and removal of structures affected e.g. adrenal gland

Patients who cannot undergo resection - Most small tumours grow slowly and do not become symptomatic or metastasise – reasonable to manage conservatively

with periodic re-evaluation - Alternatives: radiofrequency ablation, cryotherapy of lesions

B. ADVANCED TUMOURS Immunotherapy - High dose interleukin-2 – associated with good results in patients whose tumours respond to treatment, as treatment can induce

long-term remissions without relapse. However, associated with high toxicity and often not tolerable - Cytoreductive nephrectomy performed prior to starting immunotherapy can improve survival Molecular targeted therapy: Sorafenib, Bevacizumab 1. Sorafenib – an inhibitor of tyrosine kinase Æ blocks intracellular domain of the vascular endothelial growth factor (VEGF) receptor 2. Bevacizumab – monoclonal antibody against VEGF

243

3. BLADDER TRANSITIONAL CELL CARCINOMA EPIDEMIOLOGY - Ninth most common cancer in Singaporean males - Increasing incidence with age (80% diagnosed in patient >60 years old) - 4:1 male predominance PATHOLOGY - TCC is the most common tumour of the bladder (>90%)

Thought to arise due to exposure to carcinogenic substances in the urine Æ urothelial cancers think FIELD CHANGE effect, thus urothelial tumours often occur multifocally Æ Screen ENTIRE urothelium every f/u

Papillary in nature (more differentiated) - Adenocarcinoma (1%, arises from remnant of the urachus in the dome of the bladder), - SCC (<5%, due to chronic irritation e.g. long term indwelling catheter or untreated bladder stone) RISK FACTORS - Cigarette smoking (Urothelial CA!) – ask secondary smoking - Industrial chemicals – naphthylamine, aniline-containing dyes, etc - Occupational (hairdressers – exposure to hair dyes) - Chronic cystitis (SCC)

Schistosomiasis Radiation (pelvic) Chemotherapy (cyclophosphamide)

- Analgesic abuse (phenacetin) PRESENTATION - Haematuria most common (90%) – typically gross, painless, intermittent, occurring throughout the stream - LUTS

– irritative symptoms suggestive of carcinoma in-situ, - obstructive symptoms: indicate a tumour at the bladder neck or prostatic urethra

- Pain – in locally advanced or metastatic tumour (a) flank pain due to urinary obstruction, (b) suprapubic pain due to local invasion, (c) bone pain due to metastasis

- Constitutional symptoms – LOW, LOA, fatigue DIAGNOSIS 1. Urine cytology for malignant cells (not very sensitive but very specific) 2. Cystoscopy with cell brushings and biopsy 3. IVU or CT urogram to detect SYNCHRONOUS LESIONS (3% chance of proximal tumour) STAGING

1. CT abdo/pelvis for T, N and M staging 2. Transurethral resection of bladder tumour (TURBT) with histopathology – can reach the muscular layer Æ thus used for staging

Ta Superficial, does not involve lamina propria Tis Carcinoma in-situ: “flat tumour” T1 Superficial, involves lamina propria (up to muscularis propria) T2a Superficial involvement of muscularis propria – up to inner half of muscle T2b Deep involvement of muscularis propria – up to outer half of muscle T3a Microscopic extension outside bladder (from TURBT specimen) T3b Macroscopic extension outside bladder T4a Invasion of prostate, vagina, uterus T4b Invasion of lateral pelvic walls, abdominal wall

Generally can be divided into 2 main groups: (a) Superficial tumour (70-80% of patients) – Ta, Tis, T1 (b) Muscle-invasive tumour (20-30%) – >T2

Grading is also important T1 Æ 15% becomes invasive T1G3 Æ 75-80% invasive

244

MANAGEMENT DEPENDENT ON STAGE SUPERFICIAL TUMOUR - Primary treatment is TURBT of the tumour - Intravesical therapy

BCG – 1 instillation per week for 6 weeks Mitomycin C – single instillation within 24hrs of TURBT, or weekly/monthly treatments for up to 2 years Indicated in patients with high risk of tumour recurrence or tumour progression:

1. high grade, 2. multiple primary sites, 3. multiple recurrences, 4. tumour size >3cm, 5. primary or coexisting carcinoma in-situ, 6. prostatic urethral involvement

- Follow-up:

Urine cytology with cystoscopy 3-monthly for 1 year 6-monthly for next 4 years Yearly thereafter

IVU every 2 years MUSCLE-INVASIVE0 - Radical cystectomy with urinary diversion Radical cystoprostatectomy with pelvic lymphadenectomy in male

Anterior exenteration with pelvic lymphadenectomy in female (Cx: perineal hernia) Ways of diverting urine output

o Ileal conduit (a segment of ileum with ureters attached, as a stoma; not continent) o Neobladder construction using ileum (only if urethra not removed; continent, better quality of life) o Cutaneous ureterostomy (use ureters to create stoma, but easily stenosed due to small calibre; not continent) o Stoma with pouch construction under abdominal wall (not continent)

- Radiotherapy (not as good as surgery) METASTATIC Chemotherapy

245

4. UROLITHIASIS STONE COMPOSITION - Calcium oxalate or calcium phosphate stones – 75% - Magnesium ammonium phosphate (struvite = MAP) stones – 15% - Uric acid and cystine stones – 10% - Can occur at any level in the urinary tract, but most commonly in the kidney PATHOLOGY - Most important cause of stone formation is increased urine concentration of the stone’s constituents, such that they exceed their

solubility Æ precipitate as stones E.g. hypercalciuria with or without hypercalcaemia, hyperuricuria

- Urinary tract infections– struvite stones form in Proteus vulgaris infections ( splits urea into ammonium), generating alkaline urine Bacteria can also form nidi for the formation of any kind of stone

PRESENTATION DEPENDS ON SITE Renal stones - Most often asymptomatic unless the stone gets lodged in the pelviureteric junction causing hydronephrosis and subsequent

infection Æ pyonephrosis - Vague flank pain may occur. Bleeding? Ureteric stones - Even small stones can cause severe symptoms as the ureter is narrow (a) Classically ureteric colic pain – severe, intermittent loin-to-groin pain (b) Haematuria – gross or microscopic (c) Irritative symptoms – frequency, urgency (d) Can cause upper urinary tract infection Æ Pyonephrosis!

(infected purulent urine in an obstructed collecting system) fever with C&R, pain, can be very ill (sepsis) Management:

- Broad spec Abx eg. Ceftriaxone - DECOMPRESSION using PCN or URS with stenting (no need to remove stone in same setting)

Loin pain DDx: MSK (diff to differentiate) Bladder stones - May be asymptomatic (a) Can cause irritative urinary symptoms – frequency, urgency (b) Haematuria (c) If infection is present – dysuria, fever, etc PHYSICAL EXAMINATION - In ureteric colic, symptoms are often out of proportion to signs – no guarding, rebound, etc - If the patient has pyelonephritis, renal punch may be positive (DDx spinal pain by pressing on the spine) - Otherwise unremarkable examination

246

INVESTIGATIONS Diagnosis 1. KUB

- May be able to see radio-opaque stone (90% of renal stones are radio-opaque) - False neg: too small. False positive: Phlebolith (perfectly round, sometimes with lucent centre. Found in pelvis near ureters),

stools - K: Look at kidney size, any renal stones - U: Trace path of ureter along tips of transverse processes, across sacroiliac joint, in front of bifurcation of common iliac

arteries and medially into bladder at ischial tuberosity, looking for ureteric stones - B: Look for bladder stones

2. Intravenous urogram - Can also help to visualise a stone - Can show dilated urinary system secondary to stone obstruction – hydroureter and/or hydronephrosis

3. Ultrasound of kidney or bladder - Features of stone: echogeneic rim, posterior acoustic shadowing

Complications 4. Urine tests – dipstick, UFEME, urine culture/sensitivity

- Haematuria - Pyuria, micro-organisms (UTI)

5. Intravenous urogram - as above

6. MAG-3 renogram - If pyelonephritis present due to stone obstruction, it is valuable to measure the renal function using the MAG-3 renogram - The renogram gives the differential function of each kidney – in normal individuals the function should be approximately 50%

on each side (out of 100% for both kidneys combined) - If one kidney has less than 15% of total renal function, it is not worth salvaging the kidney

TREATMENT CONSERVATIVE Stones smaller than 5mm can be treated conservatively as 60% will be passed out; only treat if they (a) do not pass out after 4 to 6 weeks, and/or (b) cause symptoms (a) Treatment of any urinary tract infection (b) If underlying disease present that causes increased urinary concentration of stone components e.g. hypercalcaemia Æ treat

disease if possible (c) Diet:

High fluid intake Low salt intake Restriction of red meat, dairy produce, refined sugars Increase citrus fruit intake

SURGICAL INTERVENTION Indications: - S/S: Constant pain - Cx:

Obstructs urine flow Causes urinary tract infection Damages renal tissue or causes significant bleeding Increase in size

- Unlikely to resolve with conservative Tx: Does not pass after one month Too large to pass spontaneously

247

Types of treatment available: [from top to bottom] 1. Percutaneous nephrolithotomy (PCNL)

- Done for renal stones that are too large for ESWL to disintegrate - CI : uncorrected bleeding diathesis, patients unfit for GA

2. Extracorporeal shock wave lithotripsy (ESWL)

- Calcium oxalate, uric acid and struvite stones fragment easily, but calcium phosphate and cystine do not - Used for stones below 2cm in size - Used for renal stones and upper ureter stones – not so good for lower system due to difficulty in access - CI:

(a) pregnancy, (b) untreated UTI, (c) untreated bleeding diathesis, (d) Total obstruction ( need stone-urine interface for it to work) (e) distal obstruction that cannot be bypassed with a stent (f) ESRF (no urine to flush stones down)

- Cx: (a) Bleeding (capsular hematoma) (b) Sepsis (if stone is infected) (c) Injuries to surr tissues

3. Ureterorenoscopy with lithotripsy (URS + LL) (usually laser lithotripsy, can also be done by pneumatic drill, electrohydraulic

means) - For stones along the ureter - Retrograde pyelogram to see the stone?

4. Cystolitholapaxy (stone crushing) for bladder stone Open surgery (pyelolithotomy or ureterolithotomy) – rarely done; only if failed other management strategies, altered anatomy, performing open surgery for another reason anyway, non-functioning kidney Adjuncts: Double-J stent (or DJ stent) – inserted to stent the urinary system when - worried that stone fragments after ESWL may cause obstruction e.g. when ESWL used for treatment of a large stone; or if system

is obstructed to begin with, may want to stent to ensure good drainage after surgery - to prevent stricturing Summary of treatment modalities (ESWL: any level of ureter)

Location Size Treatment Renal: 2cm < 5mm Conservative management unless symptomatic/persistent

5-10mm ESWL 10-20mm Either ESWL or PCNL > 20mm PCNL

Upper ureter: 1cm

< 5mm Conservative management unless symptomatic/persistent 5-10mm ESWL > 10mm URS with lithotripsy

Mid/Distal ureter: 5mm

< 5mm Conservative management unless symptomatic/persistent > 5mm URS with lithotripsy

ESWL Bladder: 3cm/ multiple

< 30mm Cystolitholapaxy > 30mm Open cystolithotomy (also if there are multiple stones)

248

5. APPROACH TO ACUTE RETENTION OF URINE CAUSES: BPH, FAECES, ANTICHOL, UTI, STRICTURE, STONES Mechanical Extraluminal (5) Prostate enlargement (benign/malignant/prostatitis) – BPH is the commonest cause!

Faecal impaction Pelvic tumour, ovarian cyst, fibroid Pregnancy, retroverted gravid uterus UV prolapsed

Intramural 54) Tumour of the bladder neck (TCC) Urethritis (UTI) Urethral stricture from STD, prev instrumentation Phimosis Urethral trauma/rupture

Intraluminal (3) Stones Blood clot (clot retention in haematuria) Foreign body

Non-mechanical

Cord disease/ injury Æ neurogenic bladder

Cord compression, cauda equina Multiple sclerosis Tabes dorsalis (Tertiary syphilis)

Neuropathy Diabetic autonomic neuropathy Drugs (4A) Anticholinergics (cough medicine, Overactive bladder Tx), antihistamines, anti-depressants, alcohol Others (3P) Prolonged immobility

Post-anaesthesia Pain

HISTORY 1. Confirm Dx (Symptoms of ARU) [3] - Sudden inability to pass urine - Suprapubic pain (unlike chronic retention of urine which is painless) – severe! - Suprapubic distension 2. Precipitating factors: (if present, higher the chances of retaining bladder abilities after Tx with TURP) [4] (a) UTI: dysuria, frequency, urgency, nocturia, haematuria (b) Constipation (c) Drugs e.g. cough mixture, antihistamines (d) Immobility 3. History suggestive of aetiology: - Previous history of obstructive symptoms Æ BPH - Previous history of ureteric colic pain or stones - Previous urethral instrumentation or STD Æ stricture - Gross painless haematuria recently Æ TCC, bladder stone, blood clot - Recent trauma to urethra - Inguinotesticular pain, urethral burning, dribbling Æ Prostatitis - Lower limb weakness/paralysis, bowel incontinence, back trauma, history of spinal disease e.g. PID, spinal stenosis Æ

neurogenic bladder - Constitutional symptoms: LOW, LOA, malaise (any tumour in general) 4. Complications: [3] - Infection – symptoms of UTI - Stone disease (if in the bladder, usually asymptomatic) - Renal failure (more likely in chronic retention) – vomiting, lethargy, drowsiness

249

PHYSICAL EXAMINATION - General condition Vitals (fever) sallow appearance, scratch marks, pedal oedema, etc (uraemia)

- Abdomen Palpable bladder – tense, dull, rounded, tender, pressure Ç desire to micturate (large bladder up to umb suggests b/g of

chronic retention) Other pelvic masses – fibroid, gravid uterus, ovarian cyst Faecal loading Bilateral enlarged kidneys (hydronephrosis)

- External genitalia: narrow maetus, tight foreskin - Digital rectal examination Any saddle anaesthesia (buttock and perineum – ind cauda equina) Anal tone Prostate enlargement (>2FB) – firm and smooth? Or hard, craggy, irregular, rectal mucosa not mobile? Tender? (prostatitis) Stool impaction

- Neurological examination LMN paralysis of the lower limbs? Any sensory level present?

IMMEDIATE MANAGEMENT – PROMPT BLADDER DECOMPRESSION - 1st choice: URETHRAL CATHETERISATION (14F)

(CI!!: S/S of urethral injury – (a) blood at urethral meatus, (b) high-riding prostate – more relevant in the trauma setting) If cannot pass into bladder:

a) enlarged prostate Æ use thicker catheter (stiffer, easier to pass through) b) urethral stricture (clue: catheter is stuck quite proximally along the penile urethra / PHx of instrumentation/STD) Æ

smaller gauge catheter Do not push too hard – may cause false passage creation if the obstruction is due to a stricture

- If urethral catheterisation fails Æ suprapubic catheterisation Requires distended bladder which pushes the surrounding bowel loops away so that risk of bowel injury is lower: Method:

Local anaesthetic injected 2 fingerbreadths above pubic symphysis Small incision made in the skin and fascia, and trocar inserted When a gush of urine is seen, the suprapubic catheter is inserted and secured

INVESTIGATIONS 1. Bloods/Urine

a. Urine dipstick, UFEME and culture/sensitivity (UTI, hematuria) b. UECr: raised creatinine (renal impairment secondary to obstructive nephropathy) c. FBC: raised TW (infection) – not usually done

2. Imaging 1) KUB for stones, faecal loading 2) U/S bladder: (1) stones, (2) tumour, (3) prostate volume, (4) intravesical protrusion of prostate 3) U/S kidney, ureters: hydronephrosis, hydroureters (obstructive complication)

PSA - to be done 4-6/52 later (as ARU can cause raised PSA) - PSA velocity (rate of rise), free/total PSA, PSA density (= PSA/prostate volume) - >/= 2 readings 2/52 apart - Causes of raised PSA

i. cancer ii. BPH [usually <40] iii. prostatitis iv. instrumentation /catheterization >11days v. ARU

250

TREATMENT 1. Treat reversible causes

- Stop drugs that may have precipitated ARU - Relieve constipation with fleet enema, lactulose, senna etc - Treat any urinary tract infection if present

2. Monitor for complications

(a) Post-obstructive diuresis = diuresis that persists after decompression of bladder

Urine output >200ml/hr for 2 hours or more Due to tubular damage from chronic obstruction of drainage of the pelvicalyceal system, resulting in transient

impairment of concentrating function. Usually seen in CRU, representing appropriate attempt to get rid of excess fluid in the body during period of obstruction.

Can result in hypotension and electrolyte abnormalities (hyponatraemia, hypokalaemia, hypovolaemia) Î close monitoring of urine output and fluid/electrolyte status with appropriate replacement and resuscitation

(b) Haemorrhage ex-vacuo (transient hematuria) Bladder mucosal disruption with sudden emptying of greatly distended bladder Usually self-limiting, rarely clinically significant Î drain urine in 500-750ml aliquots, with 15-20min intervals between each

(c) Hypotension secondary to vasovagal response or relief of pelvic venous congestion

3. Trial-off catheter

- Take off catheter and watch patient’s output, as well as perform bladder scan to measure bladder volume - If patient cannot pass urine and bladder volume >400ml Æ re-catheterise - When patient passes urine, can perform

1) uroflow to investigate severity of outlet obstruction, and also do 2) bladder scan post-micturition to check residual volume

Failed Trial of void: after how long can try TOC?

1. Long term catheterization 2. Clean intermittent catheterization:

a. Adv: improved rate of spontaneous voiding (pt can PU in btw), decrease UTI 3. TURP (if sec to BPH): refer to indications

1 and 2 are done while waiting for medical therapy to work. Urinary obstruction + Fever Æ Admit! (Uro emergency)

251

6. BENIGN PROSTATIC HYPERPLASIA EPIDEMIOLOGY - Very common problem in men - Frequency rises with age after the age of 30, reaching 90% in men older than 80

PATHOLOGY - Results from proliferation of both the epithelial and stromal components of the prostate with resultant enlargement of the gland - Commonly occurs in the central zone of the prostate 1) Major stimulus: dihydrotestosterone (produced from testosterone by 5-alpha reductase) 2) Age-related increases in oestrogen levels may also contribute to BPH by increasing the expression of dihydrotestosterone

receptors on prostatic parenchymal cells - Chronic obstruction leads to hypertrophy of the detrusor muscle and trabeculation of the bladder mucosa as the bladder tries to

empty against increased resistance PRESENTATION (APPROACH TO OBSTRUCTIVE UROPATHY) - Lower urinary tract symptoms (LUTS):

Obstructive Irritative [FUNI] Hesitancy Straining to pass urine (strangury) Weak stream Prolonged micturition Terminal dribbling Feeling of incomplete voiding Double voiding (pis-en-deux) Obstructive S/S predominate.

Frequency Urgency Nocturia Urge incontinence Dysuria Irritative S/S significant for Cx of urine retention: UTI, stones

- Haematuria (UTI or rupture of enlarged veins) - Fever (UTI - pyelonephritis from ascending infection) - Rule out other DDx (refer ARU):

Stricture/ bladder neck contractures: previous instrumentation or STDs causing urethritis/ post- TURP Drug causes: codeine (cough mixture), BB, anti-cholingerics, TCAs Chronic constipation Ca bladder neck/ Ca Prostate: LOW, LOA, bone pain, haematuria Neurogenic bladder

- 6 COMPLICATIONS of an obstructive uropathy: a) Acute urinary retention (previous admissions and IDC) b) UTI: irritative symptoms, haematuria c) Stones: irritative symptoms, haematuria d) Hydronephrosis, pyelonephrosis: loin pain, fever Æ Renal impairment: polyuria/ anuria. e) Overflow incontinence 2o to Chronic urinary retention with high post-void residual volume in the bladder f) Hernia sec to chronic straining

- Other aspects of history: Social history (effect on lifestyle)

252

253

PHYSICAL EXAMINATION Inspection - Vitals: BP for HPT? (CRF) Fever? (UTI) Urine output? (CRF) - Already on IDC? (ARU) Diapers? (incontinence) Hematuria? (UTI, stones) - Sallow? Anaemia? (of CRF/ underlying malignancy) Cachexia (CA) - Hernia repair scar? (hernia) Palpation - Check for hernias - Abdominoperineal masses (fecal loading, masses to compress) - Any ballotable kidneys? (Hydronephrosis, Pyelonephritis) - Renal punch? (Pyelonephritis) - Palpable tender bladder in (ARU) non-tender (CRU) - Pedal/ sacral oedema (CRF) - Bony tenderness (tumor) Confirm diagnosis and R/O DDx: - DRE impacted stools prostate (benign):

1. smooth enlarged 2. median sulcus, 3. rubbery, 4. non-tender, 5. mobile mucosa

INVESTIGATIONS Blood - FBC: anaemia, raised WBC - UECr: dehydration, raised creatinine Î renal impairment due to chronic obstruction - UFEME, cytology, urine c/s: for UTI and screen for cancer - PSA (done 4-6/52 later to avoid false +ves): normal <4 Imaging - KUB for bladder stone - US kidney – hydronephrosis - US bladder – post-void residual volume >100ml, bladder stone, measure intravesical prostatic protrusion (IPP) - Cystoscopy to rule out stones, strictures/ bladder neck obstruction or cancer, - Uroflowmetry to confirm obstruction to urinary outflow (IMPT!!)

1. normal bell shaped curve, saw tooth appearance 2. Volume voided (>100ml to be valid): too low (falsely low peak flow rate), too high (falsely long duration, increase RU) 3. Normal peak flow rate (Qmax) > 15ml/sec 4. Residual urine 0 in young males, can accept up to 50ml in elderly

- ± urodynamic studies, TRUS with biopsy TRO prostate cancer if PSA >10

A) BOO with normal detrusor muscle: max flow rate 10ml/sec, prolonged flow. B) BOO with abdo straining: intermittent flow, peak flow rate may be normal or high, especially if outlet resistance is reduced

254

COMPLICATIONS - Acute/chronic urinary retention Æ Bladder stones & Recurrent UTI - Gross haematuria (after excluding other causes) - Renal impairment secondary to outflow obstruction - Co-existence of prostate cancer MANAGEMENT - Divided into watchful waiting, medical management, and surgical management - Objectives of treatment: Rapid and sustained relief of symptoms, prevent long-term complications, improve patient’s quality of life

I. Watchful waiting - Suitable for patients with minimal symptoms, no complications and normal invx - Monitor patient’s symptoms and clinical course annually II. Medical treatment 1. Alpha blockers (Prazosin, Terazosin, Doxazosin, Alfuzosin) - dynamic

- Treatment of symptoms (blocking the α-1 adrenergic receptors in the bladder neck, prostate and urethra – relaxing the area) - Result in decreased outflow resistance and decreased bladder instability - SEs: include postural hypotension, dizziness, lethargy, lightheadedness

2. 5-alpha reductase inhibitors (Finasteride, Dutasteride) – static - Treats the disease (not just symptoms) by inhibiting conversion of testosterone to dihydrotestosterone by 5-alpha reductase Æ È prostate size - Proven to decrease need for surgery and acute retention rates - Only effective after 6 /12 (counsel the patient!), and in prostates >40g - Most common side-effect is Sexual dysfunction. Warn patient about Gynaecomastia (1%) and stop med when they notice it

developing. Hair growth. - Ex: $3/pill. Combi therapy better than monotherapy.

III. Surgery Types - Transurethral resection of prostate (TURP)is the gold standard - Transurethral incision of the prostate (TUIP): decision made during TURP when the prostate does not appear to be enlarged Æ make small

cuts around the bladder neck area to open it up. Adv: 50% chance of Retrograde ejaculation

- Laser prostectomy is promising (pending long term results) - Other techniques do not show good results: stenting, cryoablation, etc. Indications: 1. Failed medical treatment 2. Significant Complications (4): Refractory urinary retention, Recurrent UTI, Bladder calculi, Obstructive uropathy 3. Recurrent gross haematuria Aim: to widen bladder neck Complications of TURP: bold = must mention during consent taking 1. Bleeding, infection, risk of GA/ spinal analgesia 2. Local injury causing incontinence (1%), stricture / bladder neck stenosis 3. Perforation of the urethra or bladder dome Æ can form fistula 4. Retrograde ejaculation (100%) Æ impotence: (incompetent bladder neck). Always check if pt is planning to have children! 5. TUR syndrome (<1%) = hyponatremia

- Symptoms: Nausea, vomiting, confusion, hypertension, visual disturbance, giddiness, seizure - Hyponatraemia due to constant irrigation during TURP (glycine used for irrigation – cannot use N/S, as ionic solutions make

diathermy non-functional) - Irrigation fluid is hypotonic, thus water enters open vasculature during surgery - Risk Ç with prolonged operation & Ç pressure of irrigation, thus op is kept to < 1 hour, and irrigation pressures <60mmHg - Patient usually given spinal anaesthesia during TURP so the surgeon can assess the patient’s mental status during the operation - Now uncommon as new technology allows isotonic irrigation

6. Failure of procedure: S/S recur Caution: Must rule out Neurogenic bladder / Detrusor hypotonia before TURP!! Æ Do Urodynamic studies 1. Insert narrow catheter with pressure gauge at the end into

a. Bladder: intravesical pressure b. Rectum: intraabdominal pressure Detrusor pressure = a – b

2. Fill up bladder: look for detrusor contractions 3. Cough when erect: stress incontinence 4. Void: detrusor pressure should be sufficient, good flow (Qmax)

255

7. PROSTATIC CANCER EPIDEMIOLOGY - Prostate Cancer is the 6th commonest cancer among men in Singapore. - 5th common cancer in Singapore - Peak incidence between 65 and 75 years of age PATHOLOGY - Adenocarcinoma - Arise in the outer parts of the prostate 70-80% of the time and are thus palpable on digital rectal examination RISK FACTORS - Hormonal – growth of tumour can be inhibited by orchidectomy or administration of oestrogens - Genetic – racial variations in onset and prevalence, family history - Environmental – industrial chemical exposure, diet containing high animal fat PRESENTATION - Asymptomatic (mostly): incidentally picked up on DRE or due to elevated prostate-specific antigen (PSA) level (>100 is highly

suggestive) - Local symptoms: obstructive LUTS, bladder outlet obstruction; (uncommon as most cancers arise in peripheral zones) haematuria,

haematospermia, new onset erectile dysfunction - Metastatic symptoms – lower back pain (from Batson’s venous plexus) PHYSICAL EXAMINATION - DRE: Asymmetric area of induration, or frank hard irregular nodule - Percuss spine for any bone pain Staging (TNM staging)

x T1: non-palpable lesions o 1a: diagnosed on TURP (Gleason score <7, <5% involvement) o 1b: diagnosed on TURP (Gleason score >7, >5% involvement) o 1c: diagnosed with PSA screening and TRUS biopsy

x T2: confined to prostate o 2a: confined to 1 lobe o 2b: both lobes of prostate involved

x T3: extra-prostatic spread (beyond prostatic capsule) x T4: prostate stuck down to pelvic structures

Gleason score for prostate Ca is based on its microscopic appearance. Higher scores means more aggressive lesion and worse prognosis. DIAGNOSIS 1. Transrectal ultrasound (TRUS) with biopsy- gold standard

- Histology of prostate carcinoma is graded by the Gleason score looking at glandular architecture at low magnification 2. PSA level

- >10ng/ml: biopsy recommended as >50% of patients will have prostate cancer - 4-10ng/ml: biopsy advised, though only 20% will have prostate cancer - <4ng/ml: majority will have negative biopsies, and yet there is a significant proportion of men with prostate cancer with PSA

<4ng/ml Æ biopsy if the rate of rise of PSA is >0.75ng/ml per year

STAGING 1. Clinical examination (palpable tumour Æ T2) 2. TRUS biopsy for staging purpose 3. CT scan of the abdomen and pelvis to assess extent of tumour invasion and nodal status (regional, non-regional) 4. Bone scan for metastasis

256

TREATMENT LOCALISED DISEASE (T1/2, NO N/M) AND <75YEARS OLD 1. Radical prostatectomy

- Open, laparoscopic or robot-assisted - Open – retropubic or perineal approaches

2. Radiotherapy

- External beam radiotherapy (EBRT) or Brachytherapy LOCALLY ADVANCED DISEASE (T3/4) 1. Radiotherapy with androgen ablation METASTATIC DISEASE 1. Androgen ablation

A. Castration (decrease Testosterone by 90-95%) a. Surgical orchidectomy b. Medical – LHRH agonist (luteinizing hormone releasing hormone)

i. 7-10 days: hormone flare (raised testosterone) ii. After: pituitary stops signaling testicles Æ castration level testosterone

B. Anti-androgen a. Non-steroidal e.g. Flutamide b. Steroidal – cyproterone acetate

C. Combined androgen blockade (A + B) D. Oestrogen therapy (diethylstilbestrol)

a. binds Testosterone-R at hypothalamus Æ decrease LHRH b. S/E: CVS

HORMONAL REFRACTORY PROSTATIC CANCER - 2 consecutive PSA rises no less than 2 wks apart and/or documented dz progression based on clinical/radiological findings in pts

with castrate levels of testosterone - Management:

1. Secondary hormonal manipulation Glucocorticoids – prednisone, dexamethasone, hydrocortisone Progesterone – megestrol acetate Adrenal suppressives – ketoconazole, aminoglutethimide

2. Chemotherapy (relieves pain from bone mets + slow dz progression) Docetaxel + Prednisone (gold standard) Mitoxantrone + Prednisone

Watchful waiting is possible in elderly (>75yo).

257