Respiratory 2

Respiratory & ENT

1-

A baby boy was diagnosed with bilateral severe sensorineural deafness at his newborn screening test.

He is now 8-months-old and despite optimal hearing aids he is not beginning to verbalise.

What is the best treatment option?

(Please select 1 option)

Bilateral cochlear implants at 1-year-old Correct

Bilateral cochlear implants at 2-years-old

Reassess hearing at 18 months

Unilateral cochlear implant at 1-year-old

Unilateral cochlear implant immediately

Hearing impairment causes delayed speech and language. Cochlear implants can allow a child to develop normal speech.

Bilateral implants give additional benefit over a unilateral implant; 1 year is the optimum age for implantation given the considerations of the size of the child and the potential to develop speech.

At 2-years-old it is too late to optimise speech development and reassessing at 18 months is unlikely to give any new information, especially given a trial of hearing aid has not worked.

Unilateral implants are not recommended in the NICE guidelines on Hearing impairment - cochlear implants (TA166).

2-

A day old baby, born at term, is noted to have ambiguous genitals with a small phallus and scrotal like structure but no palpable gonads. The genital skin is deeply pigmented.

What is the most likely diagnosis?


Congenital adrenal hyperplasia Correct

Congenital androgen insensitivity

Mixed gonadal dysgenesis

True hermaphroditism

Turner's syndrome

Congenital adrenal hyperplasia (CAH) is the commonest cause of ambiguous genitalia in western populations.

It is caused by an enzyme defect preventing the production of cortisol, the precursors are then diverted into androgens, virilisation the female fetus.

Pigmentation occurs because an excess of adrenocorticotropic hormone (ACTH) cross-reacts with melanocyte-stimulating hormone (MSH) and stimulates melanocytes.

It is a diagnosis that must not be missed as, with the withdrawal of maternal steroids, the baby can enter an addisonian crisis.

Congenital androgen insensitivity gives a normal female phenotype with an XY genotype.

Turner's syndrome also usually gives normal female genitalia.

Mixed gonadal dysgenesis and true hermaphroditism are both rare.

3-

Failure to pass meconium

AColonic atresia

BHirschsprung's disease

CImperforate anus

DJejunal atresia

EMeconium ileus

FMeconium plug

GVolvulus

From the given list what is the most likely diagnosis?

A 3-day-old baby boy has not passed meconium and his abdomen becomes distended

and he starts vomiting, initially milk and then green fluid. On rectal examination he

passes a large quantity of foul-smelling liquid stool.

Incorrect - The correct answer is Hirschsprung's disease

Hirschsprung's disease is a failure of the migration of the nerve supply to the distal bowel, causing a tonic contraction and a functional obstruction. Rectal examination typically causes a gush of liquid stool.

A 3-day-old baby boy has not opened his bowels and he develops abdominal distension

and bilious vomiting. On rectal examination there is just a small amount of creamy white

meconium.

Colonic atresia

Colonic atresia

Correct

Colonic atresia is uncommon and presents similarly to Hirschsprung's except it is not possible to decompress via the anus. The dark green/black colour of meconium is due to the presence of bile, in colonic atresia the meconium distal to the atresia has not be exposed to bile and so is white.

A 3-day-old baby girl has not passed meconium; her abdomen becomes distended, tense

and tender. Her cousin has cystic fibrosis.

Incorrect - The correct answer is Meconium ileus

This is a typical picture of meconium ileus where thick tenacious meconium obstructs the intestine, usually at the terminal ileum.

4-

Colonic atresia

A baby girl is born with a lumbar myelomeningocele which had been diagnosed antenatally.

What intracranial malformation will she certainly have in addition to her spinal lesion?


Absent corpus callosum Incorrect answer selected

Arnold-Chiari malformation This is the correct answer

Dandy-Walker malformation

Prosencephaly

Telencephalon

The Arnold-Chiari malformation is found in all cases of spina bifida cystica. This malformation is a displacement of the cerebellar tonsils through the foramen magnum; this obstructs the flow of CSF and causes hydrocephalus in the majority of children born with spina bifida.

Agenesis of the corpus callosum is a rare condition associated with various syndromes, including fetal alcohol syndrome, and craniofacial malformations.

Dandy-Walker malformation is another dysgenesis of the cerebellum and causes hydrocephalus but is not associated with spina bifida.

Prosencephaly is fusion of the cerebral hemispheres with a single lateral ventricle.

Telencephalon refers to the cerebral hemisphere and is not a pathological term.

5-

A 16-day-old baby born with a lumbar myelomeningocele had his back closed on day two of life.

He was initially well but now has a bulging fontanelle and reduced level of consciousness.

What is the most likely cause?


Hydrocephalus Correct

Intracranial tumour

Meningitis

Overly tight closure of the back defect

Urinary tract infection (UTI)

Hydrocephalus is common in spina bifida following closure of the defect. Aggressive treatment (shunting) will help to protect intellectual function.

Meningitis can give this clinical picture and is possible following surgery on the defect but is less likely than hydrocephalus, especially two weeks following surgery.

Intracranial tumours can also cause this presentation but this is extremely unlikely given the clinical scenario.

Babies with spina bifida are prone to UTIs but they do not usually present in this way.

6-

Antenatally diagnosed hydronephrosis

AImmediate ultrasound

BUrgent U&E

CUltrasound in 48 hours

DUrgent ultrasound and referral to urologist

EMicturating cystourethrogram

FDMSA

GMAG-3 scan

HUrethral catheterisation

For each scenario below, choose the appropriate course of action from the given list::

A 24-hour-old baby boy is born at 37 weeks gestation. At his 20 week scan he was noted

to have bilateral hydronephrosis with the transverse AP diameter measuring 35 mm on

the left and 45 mm on the right associated with oligohydramnios. He has been well since

birth but has not yet passed urine.

Incorrect - The correct answer is Urgent ultrasound and referral to urologist

This scenario describes a baby who is very likely to have posterior urethral valves. This is a lower outlet obstruction that can cause significant renal damage and requires surgical management to address the obstruction.

The local paediatric urologist should be informed urgently once a post-natal scan has confirmed ongoing hydronephrosis. Urethral catheterisation is also reasonable but catheterising these boys is often very difficult and introduces the risk of infection; it is better to seek a specialist opinion first.

A 6-hour-old baby girl who was noted at her 20 week scan to have right sided

hydronephrosis measuring 12 mm.

Incorrect - The correct answer is Ultrasound in 48 hours

Immediate ultrasound


This is a mild degree of unilateral hydronephrosis so there is no urgency. Immediate ultrasound is incorrect as an immediate ultrasound may well be misleading as newborns are relatively oliguric and so the hydronephrosis may not be seen in the first 48 hours of life, giving false reassurance. Similarly, urgent U&E is incorrect as at that age this will reflect the mother's renal function and not the baby's.

A 4-month-old baby girl who was reported to have right sided hydronephrosis measuring

16 mm at her 20 week scan, post-natal scans showed right sided hydronephrosis

measuring 18 mm at 1 week of age and 26 mm at 4 months.

Incorrect - The correct answer is MAG-3 scan

This scenario suggests a diagnosis of pelvi-ureteric junction (PUJ) obstruction and a MAG-3 scan is a dynamic scan that gives information on both function and drainage of the kidneys which can confirm the diagnosis and provide a split function for the kidneys which guides the need for surgical intervention.

DMSA is incorrect as a DMSA scan is a static test that shows functioning renal tissue but gives no information on drainage, making it unsuitable to confirm a diagnosis of PUJ obstruction.


7-

A 6-week-old baby boy with a history of projectile vomiting for ten days is diagnosed with pyloric stenosis. His latest capillary blood gas results are:

pH7.56(7.36 - 7.44)

Bicarbonate36 mmol/L(20 - 28)

Base excess+13.1 mmol/L(± 2)

Sodium132 mmol/L(137 - 144)

Chloride77 mmol/L(95 - 107)

Potassium3.2 mmol/L(3.5 - 4.9)

Which of the following is the most appropriate fluid regime for this child?


100ml/kg/24hours 0.45% saline and 5% dextrose Incorrect answer selected

120ml/kg/24hours 10% dextrose and 0.18% saline

150ml/kg/24hours 0.45% saline and 5% dextrose This is the correct answer

150ml/kg/24hours Hartmann's solution

200ml/kg/24hours Hartmann's solution

Pyloric stenosis leads to prolonged vomiting and subsequent dehydration and a hypochloraemic metabolic alkalosis. The baby needs water and salt replenishment. Once the hydration and chloride depletion are addressed the baby's kidneys will self-correct the acid-base disturbance.

Babies with pyloric stenosis may present severely dehydrated and require fluid bolus resuscitation with normal saline. Once acute dehydration has been addressed, further correction of the electrolyte imbalance should not occur too quickly to prevent rapid fluid shifts, 150ml/kg/day achieves the best

compromise between rehydration and over-rapid correction. Once urine output is established, potassium should be added to the infusion in addition to sodium chloride.

150ml/kg/24hours 0.45% saline and 5% dextrose will give the most appropriate rate of correction of the electrolyte and fluid imbalances.

100ml/kg/24hours 0.45% saline and 5% dextrose) would give too little fluid adequately to address the dehydration.

120ml/kg/24hours 10% dextrose and 0.18% saline is closer to an optimal rate of fluid replacement but does not give enough sodium chloride and would exacerbate the electrolyte depletion.

The remaining options are incorrect as Hartmann's solution contains bicarbonate and would exacerbate the alkalosis. The rate of 200ml/kg/24hours is also too great and would risk fluid overloading the baby.

8-

A term baby presented at 2 days of age with recurrent apnoeas.

He was noticed to have a flat nose and very small chin. The consultant felt he probably had Pierre Robin sequence and asked for the baby to be nursed prone with a nasopharyngeal airway in situ and requested a thorough family history and ophthalmic review.

What condition is he considering?


Apert syndrome Incorrect answer selected

Carpenter syndrome

Crouzon syndrome

Goldenhar syndrome

Stickler syndrome This is the correct answer

Pierre Robin sequence is a sequence of events in fetal development, leading to a small mandible (micrognathia), posterior displacement or retraction of the tongue and upper airway obstruction. Cleft palate is present in the majority of patients, and is commonly U-shaped.

Pierre Robin sequence is often part of an underlying syndrome, the most common being Stickler syndrome. Stickler syndrome is an autosomal dominant connective tissue disorder, specifically of collagen and is characterised by flat facial bones, Pierre Robin sequence ocular and joint problems and very near sightedness.

Other conditions associated with Pierre Robin sequence are fetal alcohol syndrome, velo-facial syndrome and Treacher Collins.

None of the other syndromes listed here classically cause Pierre Robin sequence and severe myopia.

Children with Apert syndrome have webbed fingers and toes, craniosynostosis and often brachycephaly, broadly spaced eyes and flat face.

Children with Carpenter syndrome have syndactyly or polydactyly, craniosynostosis of skull, causing cone shaped heads.

Regarding Crouzon syndrome, sutures in the head are prematurely fused, bulging eyes due to abnormal growth of mid face, usually receding upper jaw and protruding lower jaw.

With Goldenhar syndrome, the lower half of one side of the face does not grow normally. They are characterised by a partially formed or total absence of one ear.

9-

Cochlear implants can help people with a bilateral severe-to-profound sensorineural hearing loss.Which of the following is not a cause of sensorineural hearing loss?


Bacterial meningitis Incorrect answer selected

Early otosclerosis This is the correct answer

Gentamicin toxicity

Noise exposure

Skull fracture

Hearing loss is classified broadly by aetiology into conductive and sensorineural types.

Conductive losses affect outer and middle ear function, and sensorineural losses affect the cochlea in the inner ear.

Cochlear implants provide a direct electrical stimulation to the auditory nerve fibres in the cochlea to reproduce the action of the hair cells that are damaged in cases of sensorineural loss.

Causes of hair cell damage include:

gentamicin toxicity

bacterial meningitis

skull fracture

noise exposure

presbycusis

genetic syndromes

hereditary deafness, and

many of the 'unknown' causes of hearing loss.

Otosclerosis results from an overgrowth of bone in the middle ear fixing the foot plate of the stapes at the oval window, hence resulting in a conductive hearing loss.

If present untreated for many years, the cochlea can become affected and a 'mixed' (conductive and sensorineural) loss results.

10-

What is the maximum age by which intervention should ideally be in place if a pre-lingually deaf child is to acquire language in a manner as close as possible to a hearing child, in terms of both speed of development and completeness of development?


6 months Correct

12 months

18 months

24 months

36 months

Studies have shown that if congenital hearing loss is identified, diagnosed and appropriate intervention begun by the age of 6 months, a child's spoken language development will progress in the same way as that of a normal hearing child.

The intervention will consist of hearing aid fitting initially to allow all available sound to be delivered to the child's developing auditory system.

For children with a severe-profound hearing loss, for whom hearing aids are insufficient, cochlear implantation should be considered and, where

appropriate, carried out as early as is practicably possible.

11-

Chest pain in children

AAcute severe asthma

BCardiac failure

CCostochondritis

DDiabetic ketoacidosis

EHysterical over-breathing

FInhalation of foreign body

GPleura effusion

HPulmonary embolism

ITension pneumothorax

JUnilateral pneumonia

Select the most appropriate diagnosis from the options above that best explains the following cases.

A 14-year-old presents to the Emergency Department with a sudden onset of chest pain

and shortness of breath. Examination reveals reduced expansion and hyperresonance

over one side with reduced breath sounds.

Incorrect - The correct answer is Tension pneumothorax

A tension pneumothorax may result following trauma. It is a potentially life threatening event with air accumulating under the pleura in the pleural space, which pushes the mediastinum across the chest. Children may be shocked and will be hypoxic, with reduced air entry and hyperresonance. Distended neck veins may also be visible.

A 17-year-old presents with a sudden onset of chest pain. Examination reveals a sinus

tachycardia and a pleural rub.

Acute severe asthma

Incorrect - The correct answer is Pulmonary embolism

Pulmonary embolism is uncommon in infants and children. It may arise in children as a result of surgery, for example scoliosis surgery. It may also occur following prolonged inactivity.

In adolescents drug abuse, recent abortion or the oral contraceptive may be predisposing problems. Clinical manifestations may suggest pneumonia. Dyspnoea is common associated with pain. There may be no physical signs, although if the embolism is sufficiently large there may be a pleural friction rub.

A 13-year-old boy presents with tachypnoea and tachycardia. Examination of the chest

reveals hyperinflation, hyperresonance bilaterally and poor air entry, but no crackles or

wheeze. He has a pulsus paradoxus.

Correct

The clue in this case is the relatively silent chest associated with pulsus paradoxus. This would suggest severe asthma. Other causes of pulsus paradoxus include:

pericardial tamponade

effusion

constriction, and

myocarditis.

12-

12-

Dyspnoea in children

AAcute severe asthma

Acute severe asthma

Acute severe asthma

BCardiac failure

CCostochondritis

DDiabetic ketoacidosis

EHysterical over-breathing

FInhalation of foreign body

GPericardial effusion

HPleural effusion

IPulmonary embolism

JTension pneumothorax

From the given options select the most appropriate diagnosis to explain best each of the following cases:

A 3-month-old baby presents with tachycardia and tachypnoea with widespread

crepitations.

Incorrect - The correct answer is Cardiac failure

A 3-year-old presents with cough and haemoptysis. Examination reveals reduced breath

sounds over one lung and a localised wheeze.

Incorrect - The correct answer is Inhalation of foreign body

A boy presents with a dry cough, lethargy and shortness of breath. Examination reveals

reduced breath sounds over one side which is dull to percussion.

Incorrect - The correct answer is Pleural effusion

A number of congenital heart lesions will result in cardiac failure, commonly in the first year of life.

The baby:

Becomes breathless particularly after crying or feeding

May be having difficulty completing feeds and as a result there may be a problem

with poor weight gain

May also be seen to have sweating as a prominent symptom.

On examination the baby may be tachycardic and tachypnoenic. A murmur may be present or a gallop rhythm.

Acute severe asthma

Acute severe asthma

Acute severe asthma

Oedema may be seen around the dependent parts of the body.

Pulmonary crepitations may also be heard. It may be possible to pick up heart enlargement clinically and chest x ray may reveal cardiomegaly and plethoric lung fields.

In the second case the unilateral nature of the reduced breath sounds and wheeze strongly suggest inspissation of a foreign body.

The final case also has unilateral signs but on this occasion the dullness to percussion suggests a pleural effusion.

13-

Childhood Breathlessness

AAsthma

BBronchiolitis

CBronchiectasis

DBroncho pulmonary dysplasia

ECardiac failure

FLaryngomalacia

GTracheal haemangioma

HTracheal-oesophageal fistula

IVitamin D deficiency

JVocal cord palsy

Select the most appropriate choice from the above list that is the most appropriate diagnosis in the following cases.

A 4-month-old baby presents with cough and breathlessness with difficulty feeding.

Incorrect - The correct answer is Bronchiolitis

Bronchiolitis is an acute infection usually occurring in the winter months. It affects infants and toddlers and the respiratory syncytial virus is mainly responsible. Typically the child presents with coryzal symptoms which then develop in to an irritable cough, tachypnoea and feeding difficulties. Babies are tachycardic and have hyper inflation of the lungs and widespread crepitations are heard over the lung fields with high pitched rhonchi particularly on expiration.

A 5-year-old boy with bouts of respiratory distress and cough and a variable cough over

the year.

Correct

Asthma is a disorder characterised by hyper sensitivity of the airways. Exacerbations results in coughing, respiratory distress and wheeze. A nocturnal cough is often the presenting complaint.

A term infant is found to have an intermittent stridor which becomes louder, and worsens

when the baby cries.

Asthma

Asthma

Asthma

Incorrect - The correct answer is Laryngomalacia

This case describes a baby with laryngomalacia characterised by an inspiratory stridor at birth, which persists for months. It is due to a congenital floppy larynx, no treatment is required.

14-

Childhood breathlessness

AAsthma

BBronchiolitis

CBronchiectasis

DBroncho-pulmonary dysplasia

ECardiac failure

FLaryngomalacia

GTracheal haemangioma

HTracheal-oesophageal fistula

IVitamin D deficiency

JVocal cord palsy

From the given list select the most appropriate diagnosis in the following cases:

A premature baby presents with a cough and cyanotic episodes during feeds.

Incorrect - The correct answer is Tracheal-oesophageal fistula

The premature baby has tracheo-oesophageal fistula, which typically presents with a cough and choking cyanotic episodes during feeds.

A girl with hypogammaglobulinaemia develops haemoptysis and a purulent sputum.

Incorrect - The correct answer is Bronchiectasis

Asthma

Asthma

Bronchiectasis typically produces clubbing and haemoptysis as well as persistent crepitations in the chest. This process may follow a severe pneumonia, for example pertussis, measles, TB. It can also complicate immune-deficiency states and cystic fibrosis. Kartagener's syndrome is a triad of bronchiectasis, sinusitis and dextrocardia. The features result from abnormal ciliary function.

A baby is born by a ventouse delivery following failed forceps. She is born in poor

condition and stridor is noted shortly after birth.

Incorrect - The correct answer is Vocal cord palsy

Stridor from birth may occur as a result of birth trauma or an abnormality of the central nervous system, such as hydrocephalus.

15-

Emergency treatment

A100% oxygen plus nebulised salbutamol

BIV hydrocortisone

CAdrenaline

DDexamethasone

Asthma

EHyperbaric oxygen

FIntubation

GIV hydrocortisone plus oral antihistamines

HPulsed methylprednisolone

ISkin desensitisation

JSupportive treatment

From the given list select the most appropriate emergency treatment for the following cases:

A 16-year-old asthmatic is brought to the Emergency department severely breathless.

She is unable to complete a three word sentence.

Correct

Commonly used in the management of status asthmaticus following adequate

bronchodilatory treatment and oxygenation.

Incorrect - The correct answer is IV hydrocortisone

An asthmatic child is admitted to hospital. She receives bronchodilators and IV steroids.

She remains confused and agitated. Auscultation of her chest half an hour later reveals a

'silent chest'.

Incorrect - The correct answer is Intubation

These questions relate to the treatment of asthma which is worsening in severity.

Inpatient treatment of asthma includes oxygen, inhaled bronchodilators and steroids.

In the case of status asthmaticus, respiratory failure may develop and ventilation may be necessary.

The remaining case requires escalation of treatment and so would receive intravenous steroids and higher doses of oxygen as the next management step.

100% oxygen plus nebulised salbutamol



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Respiratory 2