APIL Conference 2012

High alimentary tract obstruction

Topics

Development of GIT oesophageal atresia oesophageal stricture gastro-oesophageal reflux gastrostomy diaphragmatic hernia pyloric stenosis abdominal wall defects intestinal atresia intestinal perforation (NAI) X

Overview

• What they are• Why they occur• How they present - signs and symptoms• Diagnosis• Treatment

Development of the Alimentary Tract

alimentary tract 10 -12m

oral cavity

Anus


alimentary tract 10 -12m

oral cavityoesophagus 0.5m

stomach 0.3m

small intestine 7mduodenumjejunumileum

large intestine 1.5mcaecumcolon

rectum 0.1


stomach

duodenum

small intestinejejunum / ileum

oesophagus

http://en.wikipedia.org/w/index.php?title=File:Commons-logo.svg&page=1


• Intestinal tract– tube– runs from mouth to anus

• Oesophagus & Trachea are formed from the proximal gut– runs through chest cavity

mouth

anus




• Stomach distends asymmetrically– rotates anticlockwise

anterior posterior

right left


Development of the Gastro-intestinal Tract (GIT)



• Stomach distends asymmetrically– rotates anticlockwise

• Small & Large intestine elongate– herniates through umbilicus

– rotate anticlockwise– returns to the abdomen

http://php.med.unsw.edu.au/embryology/index.php?title=File:Normal_intestinal_rotation_cartoon.jpg


Oesophageal Atresia

oesophageal atresia

• oesophageal • esophagus (american)• gullet

➜ pharynx to stomach

What is Oesophageal Atresia?

What is Oesophageal Atresia?• the proximal and distal parts of the oesophagus do not communicate

What is Tracheo-oesophageal Fistula?• a communication between the oesophagus and trachea

Aetiology

INCIDENCE1 in 4000 live births

• Genetic factors • Vitamen deficiency• Drug and alcohol exposure• Viral and chemical influences• External physical events

Anatomy

• anterior thoracic wall removed

• mediastinum– heart & great vessels– trachea bronchi– oesophagus

Anatomy

• mediastinum– heart & great vessels

– trachea / bronchi

– oesophagus

Pathogenesis



mouth

anus

Pathogenesis

• Trachea & Oesophagus are formed from the proximal gut in upper chest

• 4th week of intrauterine growth

• Lateral mesodermal ridges form in the proximal gut

• Fuse in the midline separating the Oesophagus from Trachea

mouth

anus

Pathogenesis

➜ Lateral mesodermal ridges form in the proximal gut

➜ Fuse in the midline separating the Oesophagus from Trachea

oesophagus trachea

This process fails to occur or occurs incompletely

This process fails to occur or

occurs incompletely

Pathogenesis

• Trachea & Oesophagus are formed from the foregut

• 4th week of intrauterine growth• Lateral mesodermal ridges form in the

proximal foregut• Fuse in the midline separating the

Oesophagus from Trachea• Occurs before D34 probably D26• This process fails to occur or occurs

incompletely

ALSO abnormal• Trachea

• deficient cartilage rings• floppy trachea• respiratory difficulty• Tracheomalacia

• Oesophagus• proximal oesophagus

• very dilated • thick wall

• dysmotility• reflux

Classification

5 different types and combinations

OA with Distal TOF Pure OA without a fistula ‘H’ Type without OA

OA; proximal & distal fistula OA with proximal fistula

Classification

5 different types and combinations

• OA with Distal TOF 84%

• Isolated (Pure) OA without a fistula 8%

• ‘H’ Type without OA 4%

• OA with both proximal & distal fistula 3%

• OA with proximal fistula 1%

Classification

In Oesophageal Atresia with distal TOF

• Proximal oesophagus blind ending dilated thick (hypertrophied) walled

• Distal segment

small diameter thin walled extends a variable distance above the diaphragm usually just above carina

OA with Distal TOF

85%

Classification

In Pure Oesophageal Atresia without a fistula

• Proximal oesophagus blind ending dilated thick (hypertrophied) walled

• Distal segment

no fistula no air in stomach usually just above diaphragm long gap Primary repair not possible difficult repair

Pure OA without a fistula

8%

Classification

In ‘H’ Type TOF

• NO Atresia

Can feed diagnosed later easier repair better prognosis

4%

‘H’ Type without OA

Clinical Features & Diagnosis

Defect occurs 4th week of intrauterine growth

• Antenatal Diagnosis ?

not usually diagnosed antenatally occasionally dilated upper pouch seen

most common type – pure oesophageal atresia absence of a gastric bubble

Polyhydramnios should alertPolyhydramnios

• 1% ‘normal’ pregnancies•35 - 85 % TOF pregnancies


• Postnatinatal Diagnosis

Usually term baby clinical suspicion – polyhydramnios drooling / excessive salivation ‘Mucosy’ – not coping with saliva On feeding – inability to swallow/not coping with feeds Pass a Naso-gastric tube. It must be a good size reasonably thick and rigid (10 FG) It will get stuck at about 10 cms (or curl up in upper pouch) Plain abdominal film. – tube seen in upper thorax AP and lateral




• Postnatal Diagnosis

include abdomen confirm air in stomach

• not isolated OA

and small bowel • not duodenal atresia

• Barium rarely required– Risk of aspiration

• Endoscopic examination of trachea +/- upper pouch– exclude upper pouch TOF– identify position of upper pouch fistula


• Postnatal Diagnosis

• suspected – other associated abnormalities

• occasionally late diagnosisfed

• orally• Naso-gastric tube

Treatment

Initial Treatment• Arrange transfer to Paediatric Surgical Unit• Do not feed• Intravenous fluids• consider associated abnormalities• Nurse head flat / head down • Right lateral position• Continuous suction / intermittent aspiration of upper pouch

– Replocle Tube• Ventilate only if required

– Care not to distend stomach• Consent• Reassure mother – not her fault

Treatment

Definitive Treatment• Surgical

– Difficult– Oesophagus inaccessible– Posterior mediastinum– behind the heart and lungs

• Oesophageal Atresia and Tracheo-oesophageal Fistula– Primary repair

• Isolated Oesophageal Atresia– Ends too far apart– Primary repair not possible– gastrostomy tube– Delayed primary repair– Different options– Oesophageal substitute

Detailed discussion of surgical issuesbeyond scope of this presentation

Outlook

Surgical complications

• Anastomotic leak– May close spontaneously– Major early leak – May need re-repair

• Anastomotic Stricture– dilatation

• Gastro-oesophageal Reflux– drugs

• Tracheomalacia– occassionally operation– surgery

Oesophageal Stricture


A narrowing of the wall of the oesophagus

Due to • Gastro-oesophageal Reflux• Oesophageal Atresia • Ingestion of Caustic• Congenital oesophageal stricture – very rare

Treatment• Treat the cause• Oesophageal dilatation• Resect Stricture• Replace oesophagus


Reflux stricture

Repeated dilatations

Rigid dilators


Reflux stricture


Rigid dilators


Reflux stricture


Rigid dilators


Reflux stricture


Rigid dilators

Balloon dilators

Gastro-oesophageal Reflux


Gastro-oesophageal reflux disease acid reflux

a chronic condition due to inflammation of the oesophagus caused by stomach acid coming up from the stomach into the oesophagus



Gastro-oesophageal junction Hiatus hernia




Symptoms• Pain• Vomiting

Complications• Stricture• Aspiration


Gastro-oesophageal reflux disease

InvestigationBarium X-rayspH studyendoscopy


TreatmentMedicationSurgery– Anti-reflux surgery


Anti-reflux procedure

Gastrostomy

Gastrostomy

➜ Opening into the stomach usually using a tube

➜ Initially performed more than 100 years ago, remains in use➜ Gauderer performed the first percutaneous endoscopic gastrostomy (PEG) in 1980

Gastrostomy

➜ An opening into the stomach➜ Using a tube➜ permanent or long term➜ open operation➜ inserted with gastroscope➜ inserted with laperoscope

Gastrostomy

➜ Reasons for gastrostomy

➜ Inability to swallow➜ Inability to consume adequate oral intake

Gastrostomy

➜ Conditions requiring gastrostomy

➜ 365 children

➜ CNS lesions: 214➜ Inflammatory bowel disease: 25➜ Renal disease: 19➜ Cystic fibrosis: 15➜ Metabolic conditions: 13➜ Oropharyngeal/tracheal: 12➜ Others: 67

Gastrostomy

➜ Usually placed endoscopically➜ General Anaesthetic

➜ Need a good Assistant

Digital Indentation

Complications

➜ Death➜ tension pneumoperitoneum ➜ bleeding➜ gastro-colic fistula➜ colo-cutaneous fistula➜ small bowel obstruction➜ perforation of the stomach➜ duodenal haematoma➜ peritonism➜ peritonitis

➜ pharyngeal injur y➜ oesophageal injury➜ tract infection➜ necrotising faciatis➜ surgical emphysema➜ catheter migration➜ catheter leakage

➜ up to 50% minor complications➜ up to 12% mod/severe complications

Congenital Diaphragmatic Hernia

What is Congenital Diaphragmatic Hernia


is a defect or hole in the diaphragm that allows the abdominal contents to move into the chest cavity.



is a defect or hole in the diaphragm that allows the abdominal contents to move into the chest cavity.



Bochdalek HerniaDefect in Postero-lateral Diaphragm

Inferior vena cava

Oesophagus

Aorta

Postero-lateral defectBochdalek Hernia

Epidemiology

Bochdalek HerniaDefect in Postero-lateral Diaphragm

1 in 3000 births 8% of all major congenital abnormalitiesrisk of recurrence 2% (nonsyndromic)Familial is rare (< 2% of all cases),

Mortalityhidden mortality – in utero 40% (1/3 terminated)

60% live born- high 50% live births

Present at birth• late presentors

Pathophysiology

Development of the Diaphragm• Complex• 4th to 6th week of foetal life• complete partition of chest and abdominal cavities• Fusion

– Septum transversum– pleuro-peritoneal membranes– dorsal mesentery of oesophagus– body wall

• Right side closes slightly earlier than Left• The diaphragm separates the abdominal viscera

from the thoracic organs

Pathophysiology

Development of the Diaphragm• Complex• 4th to 6th week of foetal life• complete partition of chest and abdominal cavities• Fusion

– Septum transversum– pleuro-peritoneal membranes– dorsal mesentery of oesophagus– body wall

• Right side closes slightly earlier than Left• The diaphragm separates the abdominal viscera

from the thoracic organs

Bochdalek Hernia – abdominal viscera in the chest cavity

Pulmonary hypoplasiaPulmonary hypertension

Clinical - Antenatal Period

50% antenatal diagnosis

should prompt detailed foetal scanassociated abnormalities

Stomach

Heart

headchestabdomen

Clinical - At Birth

Prognostic indicators

• Prenatal diagnosis

• Apgar scores

• Birth weight & maturity

• Side of lesion

• Presence of stomach in chest

• Other lesions

Clinical - Postnatal

• 50% undiagnosed at birth

• Present with Respiratory Distress

• Scaphoid Abdomen

• Mediastinal Shift– trachea– heart

Diagnosis

Chest X-ray

• absent diaphragmatic outline

• Loops of bowel in chest

• NG tube passed

Left Diaphragmatic

Hernia

Diagnosis

Chest X-ray



• NG tube passed

➜ mediastinal shift

LEFTMediastinal shift as

indicated by position of nasogastric tube

Diagnosis

Chest X-ray



• NG tube passed


➜ tip may be in the chest

LEFT

Diagnosis

Chest X-ray



• NG tube passed


➜ tip may be in the chest

Contrast occasionally necessary(cystadenomatoid lung malformation)

LEFT

Diagnosis

LEFT

Chest X-ray

➜ Right sided Hernia

➜ Eventration of diaphragm

Diagnosis

LEFT

Contrast Swallow X-ray

➜ Right sided Hernia

➜ Eventration of diaphragm

This was late presenting with chronic GI symptoms

Contrast occasionally necessary(cystadenomatoid lung malformation)

Treatment

Immediate Treatment

Naso-gastric tube

Endo-tracheal tube – ventilate

Goaladequate tissue oxygenationuse low ventilation pressuresavoid barotrauma

sedation / paralyse

prevent acidosis

avoid pulmonary hypertension

Treatment

Ventilation

• conventional ventilation

• High Frequency Oscillatory Ventilation

• Inhaled Nitric Oxide

• Extracorporeal Membrane Oxygenation

Treatment

Surgery

Not an emergency

Abdominal approach

Reduce the hernia

Suture with interrupted non-absorbable sutures

‘Gortex’ patch sometimes required

Treatment

LEFT

Treatment

LEFT

Treatment

RIGHT

Infantile Pyloric Stenosis

Infantile Pyloric Stenosis

• may be called• Infantile Hypertrophic Pyloric Stenosis

• sometimes called (incorrectly)• Congenital Pyloric Stenosis

Epidemiology

• Age between 2 to 8 weeks – usually 4-6 weeks

• incidence – 2-3 per 1000 live births

• sex – M:F 4:1

Aetiology

• Cause of Pyloric Stenosis• multifactorial

• family history

• more common in firstborn

• Affected mother– 20% risk for son, 7% risk for daughter• Affected father– 5% risk for son, 2.5% risk for daughter

Pathogenesis

pylorus

Pathogenesis

• Hypertrophy of the circular layer of smooth muscle surrounding the pyloric opening

• Narrowing of the pyloric lumen

• Obstructs the passage of food

• Severe projectile vomiting

Clinical Presentation - History

• Effortless vomiting initially, becoming projectile

• Non-bilious

• ‘Well child’ - Hungry

Later• lost weight• dehydrated• lethargic

Clinical Presentation - Examination

• Well Child / thin & dehydrated

• crying; hungry

• lethargic – semiconscious

• Abdomen

• Visible peristalsis



• crying; hungry


• Abdomen




• crying; hungry


• Abdomen


• Test feed– pyloric tumour (olive)

Investigations

• Ultrasound– >4mm thickness, – >18mm long, – non-passage of fluid despite gastric peristalsis

Management

Erect IV infusion

Check serum electrolytes– sodium– potassium– chloride

Rehydrate– when electrolytes normal

OPERATION– Ramstedt’s Pyloromyotomy

Management

Erect IV infusion


Rehydrate– when normal


Management

Erect IV infusion




Management

Erect IV infusion




Management

Erect IV infusion




Post-op

feed after a few hours

observe until feeding begins to be established

out-patient review is not required

Complicationsperforation of duodenal mucosa

duodenal fornix

Duodenal Atresia

Duodenal Atresia

• Failure of canalization

• Early – 8-10 weeks

• Jejunoileal Atresia

• Late event

• Mesenteric Vascular accidents• volvulus / malrotation • internal hernia,• intussusception, • bands, • gastroschisis

What is Duodenal Atresia

Duodenal atresia is the congenital absence or complete closure of a portion of the lumen of the duodenum

Anatomy

Duodenal atresia congenital absence or complete closure

of a portion of the lumen of the duodenum

• behind transverse colon

• Four parts

• retroperitoneal – except 1st part

Anatomy

Duodenum


• Four parts


• Atresia in SECOND part

Bile & pancreatic juice

Ampulla of Vater

Anatomy

Duodenum


• Four parts


• Atresia in SECOND part

duodenum

Epidemiology

Duodenal Atresia

• 1 in every 5,000 live births• M = F• Race – no significant differences• Approximately 30% have Downs Syndrome• Approximately 8% all infants with Down syndrome have duodenal atresia

• Overall survival – 70%

• Mortality– Associated abnormalities– Prematurity– Low Birth Weight

Associated Conditions

Duodenal Atresia

• Down’s- third• Cardiac- 20%• Malrotation – 20%• TOF – 10%• Genito-urinary – 10%• Ano-rectal – 5%

• Not associated with other small bowel atresias.

Clinical - Antenatal

Antenatal Considerations– Polyhydramnios– Ultrasound scan

• double bubble

– 50% diagnosed– Persistent polyhydramnios

• Repeat scan

– Downs Syndrome• karyotyping

– Counselling– Planning delivery


Antenatal Considerations– Polyhydramnios– Ultrasound scan

• double bubble

– 50% diagnosed

– Counselling– Planning delivery


Duodenal Atresia

• vomiting – early– bile stained

• scaphoid abdomen

• epigastrium– fullness– mass

Investigations

Duodenal Atresia

• Abdominal x-ray– Double bubble

• Contrast study – rarely required

DuodenumStomach


Operation

• Do not excise the obstruction• Bypass the obstruction

• Duodenoduodenostomy side-to-side anastomosis

• Complications uncommon

• Hospital 2-3 weeks

• Following discharge

• no long-term sequelae

Atresia of the Small Bowel

What is Small Bowel Atresia?

Jejunoileal atresia and stenosis

congenital obstruction

major causes of neonatal intestinal obstruction.

Compare with Duodenal Atresia

Duodenal Atresia

• Failure of canalization

• Early – 8-10 weeks

• Jejunoileal Atresia

• Late event

• Mesenteric Vascular accidents• volvulus / malrotation • internal hernia,• intussusception, • bands, • gastroschisis

Anatomy

Small intestine

• intra-abdominal

• from: duodeno-jejunal flexure• to: ileocaecal valve

• adults: 6 m• children 3 m

• jejunum 40%• ileum 60%

Epidemiology

• 1 in 300 live births

• More common than Duodenal atresia

• Much more common that large bowel atresia

• not associated with Down’s syndrome

Aetiology

• intrauterine vascular accident resulting in necrosis of the affected segment, with subsequent resorption.

• Meconium Ileus

• Mid-gut volvulus

Types

Types

• Type I – in continuity; mesentery intact• Type II – band; mesentery intact (usually)• Type IIIa – ends separate; V shaped defect in mesentery• Type IIIb – Ends separate “apple peal”; Christmas “Tree deformity”• Type IV – multiple atresias

• Defect in mesentery– missing bowel– short gut

Types

Types 4 Types

• Type I • Type II • Type IIIa • Type IIIb • Type IV

• Defect in mesentery– missing bowel– short gut


• Polyhydramnios

• Diagnosed on foetal ultrasound– dilated or obstructed foetal intestine


• features of intestinal obstruction Bilious vomiting Abdominal distension

• Diagnosed on day 1 – 2

• Distal obstruction more delayed


• features of intestinal obstruction Bilious vomiting Abdominal distension

• Diagnosed on day 1 – 2

• Distal obstruction more delayed

• Present with perforation

Diagnosis

• Radiology

Diagnosis

• Radiology

Treatment

Surgery Type I

Treatment

Surgery Type III

Treatment

Surgery

Treatment

Surgery

Treatment

Surgery

Treatment

Surgery

• Discrepancy in bowel diameter

• Short gut

Procedure

• anastomosis

• Resect very dilated segment

• Stoma

Prognosis

Post-op

• Anastomotic leak• progress• difficult and slow• parenteral nutrition• dysmotility• re-operation common

• Survival• 80 – 90%

Abdominal Wall Defects

Overview

➜ Types➜ Epidemiology➜ Pathophysiology➜ Clinical➜ Treatment➜ Prognosis

Types

➜ Exomphalos➜ Major ➜ Minor

➜ Gastroschisis

ExomphalosOmphalocele

Gastroschisis

sac

other congenital abnormalities

no other congenital abnormalities

no sac

Types



sac

other congenital abnormalities

Types

➜ Gastroschisis Gastroschisis

no other congenital abnormalities

no sac

Types



Exomphalos MinorHernia of the cord

Exomphalos Major

Epidemiology & Pathophysiology

➜ Incidence1 in 3000 births

Exomphalos ➜ Associated abnormalities 50%➜ Association with Trisomy 13 and 18

➜ Pathophysiology➜ not known➜ Wk 5 to 10 intestine lies in a peritoneal extension in the umbilical cord➜ ?? Gut does not return to abdominal cavity➜ Does Gastroschisis result from a ruptured Exomphalos?

➜ But Exomphalos is associated with other major early developmental abnormalities➜ Gastroshisis is not➜ In Exomphalos, in utero - liver appears before gut in sac


➜ Diagnosis➜ no problem at birth

➜ Antenatal diagnosis➜ major structural abnormalities➜ occur early in development

➜ Can tell➜ if membrane is present➜ if liver is outside abdomen

➜ If antenatal diagnosis made➜ Search for other abnormalities➜ Counsel parents➜ Plan delivery➜ C/S only for obstetric reasons➜ Repeated frequent scans especially in last trimester


➜ Delivery➜ At or near paediatric surgical centre➜ Diagnosis

➜ no problem at birth➜ cord clamped and cut !!


➜ Delivery➜ At or near paediatric surgical centre➜ Diagnosis

➜ no problem at birth➜ cord clamped and cut !!

➜ Naso-gastric tube ➜ IV fluids

➜ account for increased fluid loss➜ Protect exposed gut

➜ clean ➜ prevent evaporation ➜ prevent kinking of bowel

➜ Keep infant warn➜ significant increased risk of heat loss

➜ Look for other abnormalities

Treatment

Exomphalos Minor➜ Hernia of the cord➜ Small defect < 4cms diameter➜ Contains only midgut➜ Midgut rotational abnormalities are common➜ Associated abnormalities are rare➜ Persistent vitello-intestinal duct frequent➜ Associated with other midline defects

➜ Pentalogy of Cantrell

Cantrell’s PentalogyThoraco-abdominal ectopia cordia

➜Cleft lower sternum➜anterior diaphragm defect➜absence of parietal pericardium➜exomphalos➜congenital heart defect

Treatment


➜ Pantalogy of Cantrell➜ Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome

➜Macroglossia➜Exomphalos➜visceromegaly➜giantism➜hypoglycaemia➜hemihypertrophy➜Renal tumours➜Beckwith-Wiedemann syndrome

Treatment


➜ Pantalogy of Cantrell➜ Beckwith-Wiedemann syndrome➜ cloacal exstrophy

➜cloacal exstrophy

➜Exomphalos➜bladder exstrophy➜vesical cleft➜intestinal fistula

Treatment


➜ Pantalogy of Cantrell➜ Beckwith-Wiedemann syndrome➜ cloacal exstrophy

➜ Primary closure usually possible

Treatment

Exomphalos Minor

Treatment

➜ Exomphalos Major➜ Large defect > 4 cms➜ in addition to midgut contains

➜ Liver➜ ? stomach➜ ? spleen

➜ Closure – difficult➜ Close skin only – leave abdominal wall➜ Close skin only – patch abdominal wall➜ leave to granulate

9 weeks

Treatment

Gastroschisis➜ defect small usually < 4cms➜ contains

➜ midgut➜ ? stomach➜ ? bladder / uterus / ovaries / testes

➜ Bowel wall – thickened / matted➜ parts ischaemic / necrotic➜ Closure may increase intra-abdominal pressure➜ Complicate gastroschisis➜ Associated atresia

Treatment

Gastroschisis

bowel matted fibrinous coat

necrotic bowel

stalk

Treatment

Gastroschisis➜ primary closure➜ just close skin

Treatment

Gastroschisis➜ primary closure➜ just close skin➜ Delayed primary closure

silo

Treatment

Gastroschisis➜ primary closure➜ just close skin➜ Delayed primary closure➜ Abdominal wall patch abdominal wall patch

Outcome

Gastroschisis➜ 90 % survive➜ most cosmetically acceptible

Outcome

Gastroschisis➜ 90 % survive➜ most cosmetically acceptible

Documents

APIL Conference 2012