PAEDIATRIC QUESTIONS 5 AND
ANSWERS.
What is congenital diaphragmatic hernia - Answers-It is characterised by the herniation
of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm.
This can result in pulmonary hypoplasia and hypertension which causes respiratory
distress shortly after birth.
What is the most common type of congenital diaphragmatic hernia - Answers-The most
common type of CDH is a left-sided posterolateral Bochdalek hernia which accounts for
around 85% of cases.
Only around 50% of newborns with CDH survive despite modern medical intervention.
Charlie is a 7 month old baby boy who presents to you with poor weight gain (50th to
10th centile), on examination he has an erythematous, blanching rash over his
abdomen, colicky abdominal pain and vomiting after feeds. He has been breast feeding
with top ups of 'Aptamil' formula. What is the most likely diagnosis - Answers-cow's milk
protein intolerance
The following clues in the history would suggest the diagnosis of cows' milk protein
intolerance:
-Multi-system involvement
-7 months would suggest the new introduction of top up feeds which correlates with the
symptoms
-Faltering growth along with the multi-system involvement would suggest cows' milk
protein intolerance
Charlie is older than the classical age of presentation for pyloric stenosis (2 to 8 weeks
very rare above 6 months)
The presentation is unusual for eczema, infantile colic and reflux due to the multi-
system involvement in the history making cows' milk protein intolerance more likely.
A 6 day old baby was born prematurely at 33 weeks. He has been suffering from
respiratory distress syndrome and has been receiving ventilatory support on NICU. He
has developed abdominal distension and is increasingly septic. Ultrasound of the
abdomen shows free fluid and evidence of small bowel dilatation. His blood pressure
has remained labile despite inotropic support - Answers-Laparotomy
He has necrotising enterocolitis and whilst this is often initially managed medically a
laparotomy is required if the situation deteriorates.
, A 5-year-old child has been unwell with a sore throat and fever for several days. He
progresses to develop periumbilical abdominal discomfort and passes diarrhoea. This
becomes blood stained. The paediatricians call you because the ultrasound has shown
a 'target sign'. - Answers-Pneumatic reduction under fluoroscopic guidance
This child has an intussusception. The lymphadenopathy will have initiated it. A target
sign is seen on ultrasound and is the side on view of multiple layers of bowel wall.
Reduction using fluoroscopy with air is the usual first line management. Ileo-colic
intussceceptions are generally most reliably reduced using this method, long ileo-ileal
intussceceptions usually result in surgery.
What is the commonest cause of pulmonary hypoplasia - Answers-congenital
diaphragmatic hernia
You are asked to speak to a family who have just received a diagnosis of pulmonary
hypoplasia on fetal MRI. Which of the following conditions is the most common cause of
pulmonary hypoplasia? - Answers-congenital diaphragmatic hernia
Pulmonary hypoplasia in CDH occurs alongside the hernial development rather than as
a direct result of it, as part of a sequence
nfants with congenital diaphragmatic hernia (CDH) have associated pulmonary
hypoplasia.
This is not necessarily due to direct compression of the lung by herniated viscera but
rather occurs as part of a sequence alongside the hernial development.
Oligohydramnios, rather than polyhydramnios, leads to pulmonary hypoplasia. It is
believed that oligohydramnios decreases the size of the intrathoracic cavity, thus
preventing foetal lung growth.
Diaphragm agenesis, tetralogy of Fallot and osteogenesis imperfecta are all less
common causes of pulmonary hypoplasia.
What are the causes of pulmonary hypoplasia - Answers--oligohydramnios
-congenital diaphragmatic hernia
A 16-year-old boy presents to the emergency room with a history of groin pain for the
past three hours. He has associated nausea and has vomited three times. He reports
that he recently had unprotected vaginal sex. On examination there is tenderness and
swelling of the scrotum and left testicle, with absence of the cremaster reflex on the left
side. Elevation of the affected testicle causes increased pain.
What is the most likely diagnosis? - Answers-testicular torsion
ANSWERS.
What is congenital diaphragmatic hernia - Answers-It is characterised by the herniation
of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm.
This can result in pulmonary hypoplasia and hypertension which causes respiratory
distress shortly after birth.
What is the most common type of congenital diaphragmatic hernia - Answers-The most
common type of CDH is a left-sided posterolateral Bochdalek hernia which accounts for
around 85% of cases.
Only around 50% of newborns with CDH survive despite modern medical intervention.
Charlie is a 7 month old baby boy who presents to you with poor weight gain (50th to
10th centile), on examination he has an erythematous, blanching rash over his
abdomen, colicky abdominal pain and vomiting after feeds. He has been breast feeding
with top ups of 'Aptamil' formula. What is the most likely diagnosis - Answers-cow's milk
protein intolerance
The following clues in the history would suggest the diagnosis of cows' milk protein
intolerance:
-Multi-system involvement
-7 months would suggest the new introduction of top up feeds which correlates with the
symptoms
-Faltering growth along with the multi-system involvement would suggest cows' milk
protein intolerance
Charlie is older than the classical age of presentation for pyloric stenosis (2 to 8 weeks
very rare above 6 months)
The presentation is unusual for eczema, infantile colic and reflux due to the multi-
system involvement in the history making cows' milk protein intolerance more likely.
A 6 day old baby was born prematurely at 33 weeks. He has been suffering from
respiratory distress syndrome and has been receiving ventilatory support on NICU. He
has developed abdominal distension and is increasingly septic. Ultrasound of the
abdomen shows free fluid and evidence of small bowel dilatation. His blood pressure
has remained labile despite inotropic support - Answers-Laparotomy
He has necrotising enterocolitis and whilst this is often initially managed medically a
laparotomy is required if the situation deteriorates.
, A 5-year-old child has been unwell with a sore throat and fever for several days. He
progresses to develop periumbilical abdominal discomfort and passes diarrhoea. This
becomes blood stained. The paediatricians call you because the ultrasound has shown
a 'target sign'. - Answers-Pneumatic reduction under fluoroscopic guidance
This child has an intussusception. The lymphadenopathy will have initiated it. A target
sign is seen on ultrasound and is the side on view of multiple layers of bowel wall.
Reduction using fluoroscopy with air is the usual first line management. Ileo-colic
intussceceptions are generally most reliably reduced using this method, long ileo-ileal
intussceceptions usually result in surgery.
What is the commonest cause of pulmonary hypoplasia - Answers-congenital
diaphragmatic hernia
You are asked to speak to a family who have just received a diagnosis of pulmonary
hypoplasia on fetal MRI. Which of the following conditions is the most common cause of
pulmonary hypoplasia? - Answers-congenital diaphragmatic hernia
Pulmonary hypoplasia in CDH occurs alongside the hernial development rather than as
a direct result of it, as part of a sequence
nfants with congenital diaphragmatic hernia (CDH) have associated pulmonary
hypoplasia.
This is not necessarily due to direct compression of the lung by herniated viscera but
rather occurs as part of a sequence alongside the hernial development.
Oligohydramnios, rather than polyhydramnios, leads to pulmonary hypoplasia. It is
believed that oligohydramnios decreases the size of the intrathoracic cavity, thus
preventing foetal lung growth.
Diaphragm agenesis, tetralogy of Fallot and osteogenesis imperfecta are all less
common causes of pulmonary hypoplasia.
What are the causes of pulmonary hypoplasia - Answers--oligohydramnios
-congenital diaphragmatic hernia
A 16-year-old boy presents to the emergency room with a history of groin pain for the
past three hours. He has associated nausea and has vomited three times. He reports
that he recently had unprotected vaginal sex. On examination there is tenderness and
swelling of the scrotum and left testicle, with absence of the cremaster reflex on the left
side. Elevation of the affected testicle causes increased pain.
What is the most likely diagnosis? - Answers-testicular torsion
