A 2-day-old baby girl is noted to become cyanotic whilst feeding and crying.

A
diagnosis of congenital heart disease is suspected. What is the most likely cause?

A. Transposition of the great arteries

B. Coarctation of the aorta

C. Patent ductus arteriosus

D. Tetralogy of Fallot

E. Ventricular septal defect

Congenital heart disease

 cyanotic: TGA most common at

birth, Fallot's most common overall
 acyanotic: VSD most common
cause

Theme from April 2011 exam

For Surgeons it is important to be aware of common congenital cardiac abnormalities.

The main differentiating factor is whether the patient is cyanotic or acyanotic. The
key point to this question is that whilst tetralogy of Fallot is more common than
transposition of the great arteries (TGA), Fallot's doesn't usually present until 1-2
months following the identification of a murmur or cyanosis. In the neonate, TGA is
the most common presenting cause of cyanotic congenital heart disease

The other 3 options are causes of acyanotic congenital heart disease

Congenital heart disease

Acyanotic - most common causes

 Ventricular septal defects (VSD) - most common, accounts for 30%

 Atrial septal defect (ASD)
 Patent ductus arteriosus (PDA)
 Coarctation of the aorta
 Aortic valve stenosis

VSDs are more common than ASDs. However, in adult patients ASDs are the more
common new diagnosis as they generally presents later

Cyanotic - most common causes

 Tetralogy of Fallot
  Transposition of the great arteries (TGA)
 Tricuspid atresia
 Pulmonary valve stenosis

Fallot's is more common than TGA. However, at birth TGA is the more common
lesion as patients with Fallot's generally presenting at around 1-2 months
S

A 6 month old boy is brought to the clinic by his mother. She is concerned that his
testes are not located into the scrotum. She has noticed them only when he is in the
bath, but not at any other time. What is the most likely underlying diagnosis?

A. Rectractile testis

B. Ectopic testis

C. Undescended testis

D. Testicular agenesis

E. Intersex child

Theme from April 2012 Exam

A testis that appears in warm conditions or which can be brought down on clinical
examination and does not immediately retract is usually a retractile testis.

Cryptorchidism

A congenital undescended testis is one that has failed to reach the bottom of the
scrotum by 3 months of age. At birth up to 5% of boys will have an undescended
testis, post natal descent occurs in most and by 3 months the incidence of
cryptorchidism falls to 1-2%. In the vast majority of cases the cause of the maldescent
is unknown. A proportion may be associated with other congenital defects including:

Patent processus vaginalis

Abnormal epididymis
Cerebral palsy
Mental retardation
Wilms tumour
Abdominal wall defects (e.g. gastroschisis, prune belly syndrome)

Reasons for correction of cryptorchidism

 Reduce risk of infertility

 Allows the testes to be examined for testicular cancer
 Avoid testicular torsion
 Cosmetic appearance
Males with undescended testis are 40 times as likely to develop testicular cancer
(seminoma) as males without undescended testis
The location of the undescended testis affects the relative risk of testicular cancer
(50% intra-abdominal testes)

Treatment

 Orchidopexy at 6- 18 months of age. The operation usually consists of

inguinal exploration, mobilisation of the testis and implantation into a dartos
pouch.
 Intra-abdominal testis should be evaluated laparoscopically and mobilised.
Whether this is a single stage or two stage procedure depends upon the exact
location.
 After the age of 2 years in untreated individuals the Sertoli cells will degrade
and those presenting late in teenage years may be better served by
orchidectomy than to try and salvage a non functioning testis with an
increased risk of malignancy.

Which of the following statements relating to omphalocele is false?

A. The herniated organs lie outside the peritoneal sac

B. Cardiac abnormalities co-exist in 25%

C. Intestines are almost always malrotated

D. The defects occurs through the umbilicus

E. Mortality may be as high as 15%

Gastroschisis: Isolated abnormality, bowel lies outside abdominal wall through defect
located to right of umbilicus.
Exomphalos: Liver and gut remain covered with membranous sac connected to
umbilical cord. It is associated with other developmental defects.

They are contained within the peritoneal sac and therefore do not have the fluid losses
seen in gastroschisis. Because the intestines are not located in a intra abdominal
location, a degree of intestinal malrotation is almost inevitable. However, this aspect
does not always result in a requirement for surgery.

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

  When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

Theme: Bilious vomiting in neonates

A. Biliary atresia
B. Intestinal malrotation
C. Ileal atresia
D. Necrotising enterocolitis
E. Duodenal atresia
F. Meconium ileus
G. Viral gastroenteritis
H. Pyloric stenosis

Please select the most likely underlying cause of bilious vomiting for the situation
described. Each option may be used once, more than once or not at all.

4. A male infant is born prematurely at 26 weeks gestation by emergency cesarean

section. Following the birth he develops respiratory distress syndrome and is
ventilated. He begins to improve twelve days after birth. Then he becomes
unwell and develops abdominal distension and passes bloody stools and vomits
a small quantity of bile stained vomit.

You answered Intestinal malrotation

The correct answer is Necrotising enterocolitis

Necrotising enterocolitis often has a delayed presentation and affected infants

will typically pass bloody stools. Plain films may show air in the intestinal wall
(Pneumatosis).

5. A male infant is born by spontaneous vaginal delivery at 39 weeks gestation. He

is well after the birth, established on bottle feeding and discharged home. His
parents are concerned because he subsequently becomes unwell and vomits a
large quantity of bile stained vomit approximately 2 days after discharge home.
On examination he looks ill and his abdomen is soft and non distended.

You answered Viral gastroenteritis

The correct answer is Intestinal malrotation

Intestinal malrotation with volvulus will typically compromise the

vascularisation and lumenal patency of the gut. This will cause bilious vomiting
and the vascular insufficiency will produce a clinical picture of illness at odds
with the lack of overt abdominal signs. Delay in diagnosis and surgery will
result in established necrosis, perforation and peritonitis.

6. A female infant is born by cesarean section at 38 weeks gestation for foetal

distress. The attending paediatricians notice that she has a single palmar crease
and an anti mongoloid slant to her eyes. Soon after the birth the mother tries to
 feed the child who has a projectile vomit about 10 minutes after feeding. On
examination she has a soft, non distended abdomen.

You answered Meconium ileus

The correct answer is Duodenal atresia

Proximally sited atresia will produce high volume vomits which may or may not
be bile stained. Abdominal distension is characteristically absent. Whilst under
resuscitated children may be a little dehydrated they are seldom severely ill. The
presence of Trisomy 21 (palmar and eye signs) increases the likelihood of
duodenal atresia.

Bilious vomiting in neonates

Causes of intestinal obstruction with bilious vomiting in neonates

Disorder Incidence and Age at Diagnosis Treatment
causation presentation
Duodenal 1 in 5000 Few hours after AXR shows Duodenoduodenostomy
atresia (higher in birth "double
Downs bubble sign,
syndrome) contrast study
may confirm
Malrotation Usually cause Usually 3-7 Upper GI Ladd's procedure
with by incomplete days after birth, contrast study
volvulus rotation during volvulus with may show DJ
embryogenesis compromised flexure is
circulation may more
result in medially
peritoneal signs placed, USS
and may show
haemodynamic abnormal
instability orientation of
SMA and
SMV
Jejunal/ ileal Usually caused Usually within AXR will Laparotomy with
atresia by vascular 24 hours of birth show air-fluid primary resection and
insufficiency in levels anastomosis
utero, usually 1
in 3000
Meconium Occurs in Typically in first Air - fluid Surgical decompression,
ileus between 15 24-48 hours of levels on serosal damage may
and20% of life with AXR, sweat require segmental
those babies abdominal test to resection
with cystic distension and confirm
 fibrosis, bilious vomiting cystic fibrosis
otherwise 1 in
5000
Necrotising Up to 2.4 per Usually second Dilated bowel Conservative and
enterocolitis 1000 births, week of life loops on supportive for non
risks increased AXR, perforated cases,
in prematurity pneumatosis laparotomy and resection
and inter- and portal in cases of perforation of
current illness venous air ongoing clinical
deterioration
Theme: Paediatric gastrointestinal disorders

A. Meconium ileus
B. Biliary atresia
C. Oesophageal atresia
D. Pyloric stenosis
E. Intussusception
F. Malrotation
G. Hirschsprung disease
H. Mesenteric adenitis.

What is the most likely diagnosis for each scenario given? Each option may be used
once, more than once or not at all.

7. A 3 day old baby presents with recurrent episodes of choking and cyanotic
episodes. There is a history of polyhydramnios.

Oesophageal atresia

Diagnosis is confirmed when an nasogastric tube fails to reach the stomach.

8. A 3 day old neonate is developing increasing problems with feeding. On

examination she has a pan systolic murmur and her forearms have not
developed properly.

Oesophageal atresia

This child has VACTERL, which is a combination of Vertebral, Ano-rectal,

Cardiac, Tracheo-oesophageal, Renal and Radial limb anomalies. Half of babies
with oesophageal atresia will have VACTERL.

9. A 2 year old child has central abdominal pain. He has had a recent upper
respiratory tract infection.
 Mesenteric adenitis.

Mesenteric adenitis may complicate upper respiratory tract infection and clinical
exclusion of appendicitis can be difficult.

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

  X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

Theme: Neonatal gastrointestinal disease

A. Ano-rectal atresia
B. Pyloric stenosis
C. Hirschbrungs disease
D. Duodenal atresia
E. Meconium ileus
F. Intussusception
G. Necrotising enterocolitis
H. Intestinal volvulus
I. Tracheo-oesophageal fistula

Please select the most likely diagnosis to account for the case described. Each option
may be used once, more than once or not at all.

10. A newborn baby boy presents with mild abdominal distension and failure to
pass meconium after 24 hours. X- Ray reveals dilated loops of bowel with
fluid levels. The anus appears normally located.

Hirschbrungs disease

Hirschsprung's disease is an absence of ganglion cells in the neural plexus of

the intestinal wall. It is more common in boys than girls. The delayed passage
of meconium together with distension of abdomen is the usual clinical
presentation. A plain abdominal x ray will demonstrate dilated loops of bowel
with fluid levels and a barium enema can be helpful when it demonstrates a
cone with dilated ganglionic proximal colon and the distal aganglionic bowel
failing to distend.

11. A premature infant (30-week gestation) presents with distended and tense
 abdomen. She is passing blood and mucus per rectum, and she is also
manifesting signs of sepsis.

Necrotising enterocolitis

Necrotising enterocolitis is more common in premature infants. Mesenteric

ischemia causes bacterial invasion of the mucosa leading to sepsis. Terminal
ileum, caecum and the distal colon are commonly affected. The abdomen is
distended and tense, and the infant passes blood and mucus per rectum. X -Ray
of the abdomen shows distended loops of intestine and gas bubbles may be
seen in the bowel wall.

12. A newborn baby boy presents with gross abdominal distension. He is

diagnosed with cystic fibrosis and his abdominal x ray shows distended coils
of small bowel, but no fluid levels.

Meconium ileus

One in 15,000 newborns will have a distal small bowel obstruction secondary
to abnormal bulky and viscid meconium. Ninety percent of these infants will
have cystic fibrosis and the abnormal meconium is the result of deficient
intestinal secretions. This condition presents during the first days of life with
gross abdominal distension and bilious vomiting. x Ray of the abdomen shows
distended coils of bowel and typical mottled ground glass appearance. Fluid
levels are scarce as the meconium is viscid.

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
  Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

Which of the following statements relating to biliary atresia is untrue?

A. It most commonly presents as prolonged conjugated jaundice in the

neonatal period.

B. Evidence of portal hypertension at diagnosis is seldom present in the

UK.

C. It may be confused with Alagille syndrome.

D. The Kasai procedure is best performed in the first 8 weeks of life.

 E. Survival following a successful Kasai procedure is approximately
45% at 5 years.
Alagille syndrome autosomal dominant disorder characterised by presence of paucity
of bile ducts and cardiac defects. Only the embryonic form of biliary atresia is
associated with cardiac and other embryological defects.

Biliary atresia usually presents with obstructed jaundice. A Kasai procedure is best
performed in the first 8 weeks of life. If a Kasai procedure is successful most patients
will not require liver transplantation. 45% of patients post Kasai procedure will
require transplantation. However, overall survival following a successful Kasai
procedure is 80%.

Biliary atresia

 1 in 17000 affected
 Biliary tree lumen is obliterated by an inflammatory cholangiopathy causing
progressive liver damage

Clinical features

 Infant well in 1st few weeks of life

 No family history of liver disease
 Jaundice in infants > 14 days in term infants (>21 days in pre term infants)
 Pale stool, yellow urine (colourless in babies)
 Associated with cardiac malformations, polysplenia, situs inversus

Investigation

 Conjugated bilirubin (prolonged physiological jaundice or breast milk

jaundice will cause a rise in unconjugated bilirubin, whereas those with
obstructive liver disease will have a rise in conjugated bilirubin)
 TEBIDA radionuclide scan

Management

 Early recognition is important to prevent liver transplantation.

 Nutritional support.
 Roux-en-Y portojejunostomy (Kasai procedure)
 If Kasai procedure fails or late recognition, a liver transplant becomes the only
option.

Theme: Administration of intravenous fluids

A. 0.9% Saline
B. 5% Dextrose
C. 20% Glucose
D. 0.18% saline/ 4% glucose
E. 0.45% saline/ 15% glucose
F. 0.45% saline/ 2.5% glucose
G. 4.5% albumin
H. 10% Pentastarch
I. 10% Dextrose

For the scenario given please select the most appropriate type of intravenous fluid for
the scenario given. Each option may be used once, more than once or not at all.

14. A 4 year old boy is undergoing an elective orchidopexy.

You answered 5% Dextrose

The correct answer is 0.9% Saline

Isotonic fluids should be used in this setting and 0.9% saline is the safest
option.

15. A 2 day old boy is recovering from an inguinal herniotomy he has yet to feed
and the nursing staff would like a prescription for an initial fluid to be given on
return to the ward. His potassium is within normal limits.

You answered 5% Dextrose

The correct answer is 10% Dextrose

Neonates require 10% dextrose solutions as they are at risk of developing

hypoglycaemia.

16. A 4 year boy with learning difficulties has developed swallowing problems
and is awaiting a PEG tube. He required maintenance IV fluids and the nursing
staff require choice of fluid for the next bag. He has just been given 250ml of
0.9% saline.

5% Dextrose

5% Dextrose would the routine choice for water replacement.

Paediatric fluid management

Since 2000 there have been at least 4 reported deaths from fluid induced
hyponatraemia in children. This led to the National Patient Safety Agency introducing
revised guidelines in 2007.
Indications for IV fluids include:

 Resuscitation and circulatory support

 Replacing on-going fluid losses
 Maintenance fluids for children for whom oral fluids are not appropriate
 Correction of electrolyte disturbances

Fluids to be avoided
Outside the neonatal period saline / glucose solutions should not be given. The
greatest risk is with saline 0.18 / glucose 4% solutions. The report states that 0.45%
saline / 5% glucose may be used. But preference should be given to isotonic solutions
and few indications exist for this solution either.

Fluids to be used

 0.9% saline
 5% glucose (though only with saline for maintenance and not to replace
losses)
 Hartmans solution

Potassium should be added to maintenance fluids according patients plasma

potassium levels (which should be monitored).

Intraoperative fluid management

Neonates should receive glucose during surgery.
Other children should receive isotonic crystalloid.

Maintenance fluids
Weight Water Na K mmol/kg/day
requirement/kg/day mmol/kg/day
First 10Kg body weight 100ml 2-4 1.5-2.5
Second 10Kg body 50ml 1-2 0.5-1.5
weight
Subsequent Kg 20ml 0.5-1.0 0.2-0.7

Glucose will need to be given to neonates- usually 10% at a rate of 60ml/Kg/day.

Reference
NPSA -reducing risk of hyponatraemia when administering intravenous fluids to
children. Issue date March 2007. Further references included in this document.
Theme: Paediatric gastrointestinal disorders
A. Duodenal atresia
B. Pyloric stenosis
C. Budd Chiari Syndrome
D. Annular pancreas
E. Oesophageal atresia
F. Congenital diaphragmatic hernia
G. Cystic fibrosis

Please select the most likely diagnosis for the scenario given. Each option may be
used once, more than once or not at all.

17. A 31 year old women gives birth to a male infant weighing 2.5kg by induction
of labour at 38 weeks. During the third trimester of her pregnancy she has been
troubled by polyhydramnios but otherwise her pregnancy proceeded
uneventfully. Instrumental delivery was instituted for foetal distress, but
initially the obstetricians were reassured by the absence of meconium in the
liqor. In the hours following birth the baby is struggling to feed and no
meconium has been passed.

You answered Cystic fibrosis

The correct answer is Oesophageal atresia

Polyhydramnois is a feature of oesophageal atresia. This condition occurs

during the 4th foetal week when separation of the trachea and oesophagus
occur. It is associated with other birth defects including vertebral anomalies,
imperforate anus (hence the lack of meconium) , cardiac anomalies, tracheal
anomalies, renal and limb problems (VACTERL syndrome)

18. A 6 week old baby is developing well and develops profuse and projectile
vomiting after feeds. After assessment by the paediatricians he is taken for an
ultrasound scan which demonstrates an abnormality for which he is due to
undergo a Ramstedts procedure. His blood tests reveal a hypochloraemic
metabolic alkalosis.

Pyloric stenosis

This is a typical scenario for pyloric stenosis. A Ramstedts pyloromyotomy is

the eponymous name given to the procedure. Diagnosis is often confirmed by
USS.

19. A 25 year old women delivers a Downs syndrome baby of 38 weeks gestation.
She has polyhydramnios in the latter stages of pregnancy and after the birth the
infant begins to feed. However, he begins to vomit and the vomit itself is
 copious and bile stained. His blood tests reveal a hypochloraemic metabolic
acidosis and aciduria.

Duodenal atresia

This is the typical scenario for duodenal atresia. About 1/3 cases occur in
patients with Downs Syndrome. Pre natal USS with show a double bubble
deformity. The majority of abnormalities occur distal to the Ampulla and so
vomiting is bile stained although this is not the case with more proximal
obstructions.

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
  PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

A 3 day old baby develops dyspneoa. A chest x-ray is performed and shows a radio-
opaque shadow with an air-fluid level in the chest. It is located immediately anterior
to the 6th hemivertebra. Which of the following is the most likely underlying
diagnosis?

A. Bronchogenic cyst

B. Congenital diaphragmatic hernia

C. Infection with Staphylococcus aureus

D. Oesphageal duplication cyst

E. Hiatus hernia

A midline cystic mass of an infant in this age group is most likely to be a

bronchogenic cyst. Hiatus hernia is unusual in the neonatal period. Oesophageal
duplication cysts are very rare and respiratory symptoms are less common than with
bronchogenic cysts.

Bronchogenic cysts

Overview
Bronchogenic cysts most commonly arise as a result of anomalous development of the
ventral foregut. They are most commonly single, although multiple cysts are
described.
They often lie near the midline and most frequently occur in the region of the carina.
They may be attached to the tracheobronchial tree, although they are seldom in direct
connection with it.

Cases may be asymptomatic or present with respiratory symptoms early in the

neonatal period.

They are the second most common type of foregut cysts (after enterogenous cysts) in
the middle mediastinum. Up to 50% of cases are diagnosed prior to 15 years of age.

Investigation
Many cases are diagnosed on antenatal ultrasound. Others may be detected on
conventional chest radiography as a midline spherical mass or cystic structure. Once
the diagnosis is suspected a CT scan should be performed.

Treatment
Thorascopic resection is the ideal treatment. Very young babies can be operated on
once they reach six weeks of age.
Theme: Paediatric umbilical disorders

A. Omphalitis
B. Umbilical hernia
C. Umbilical granuloma
D. Paraumbilical hernia
E. Persistent vitello-intestinal duct
F. Persistent uranchus

Please select the most likely underlying disorder for the umbilical condition
described. Each option may be used once, more than once or not at all.

21. A 2 week old baby is referred to the surgical team by the paediatricians. They
are concerned because the child has a painful area of macerated tissue at the
site of the umbilicus. On examination a clear- yellowish fluid is seen to be
draining from the umbilicus when the baby cries.

Persistent uranchus

A patent uranchus will present with umbilical urinary discharge. The skin may
become macerated if not properly cared for. The discharge is most likely to be
present when intra-abdominal pressure is raised. It is associated with posterior
urethral valves.

22. A premature neonate is born by emergency cesarean section at 29 weeks

gestation. He initially seems to be progressing well. However, the team are
concerned because he becomes systemically septic and on examination has a
swollen and erythematous umbilicus.
 Omphalitis

Infection from omphalitis may spread rapidly and cause severe sepsis
especially in immunologically compromised, premature neonates.

23. A baby boy is born by elective cesarean section at 39 weeks gestation. He

initially seems to progress well and is discharged from hospital the following
day. The parents bring the child to the clinic at 10 days of age and are
concerned at the presence of a profuse and foul smelling discharge at the site
of the umbilicus. On examination the umbilicus has some prominent
granulation tissue. When the baby cries a small trickle of brownish fluid is
seen to pass from the umbilicus.

You answered Umbilical granuloma

The correct answer is Persistent vitello-intestinal duct

A persistent vitello-intestinal duct may allow the persistent and ongoing

discharge of small bowel content from the umbilicus. This fluid may be very
irritant to the surrounding skin.

Paediatric umbilical disorders

Embryology
During development the umbilicus has two umbilical arteries and one umbilical vein.
The arteries are continuous with the internal iliac arteries and the vein is continuous
with the falciform ligament (ductus venosus). After birth the cord dessicates and
separates and the umbilical ring closes.

Umbilical hernia
Up to 20% of neonates may have an umbilical hernia, it is more common in premature
infants. The majority of these hernias will close spontaneously (may take between 12
months and three years). Strangulation is rare.

Paraumbilical hernia
These are due to defects in the linea alba that are in close proximity to the umbilicus.
The edges of a paraumbilical hernia are more clearly defined than those of an
umbilical hernia. They are less likely to resolve spontaneously than a paraumbilical
hernia.

Omphalitis
This condition consists of infection of the umbilicus. Infection with Staphylococcus
aureus is the commonest cause. The condition is potentially serious as infection may
spread rapidly through the umbilical vessels in neonates with a risk of portal pyaemia,
and portal vein thrombosis. Treatment is usually with a combination of topical and
systemic antibiotics.

Umbilical granuloma
These consist of cherry red lesions surrounding the umbilicus, they may bleed on
contact and be a site of seropurulent discharge. Infection is unusual and they will
often respond favorably to chemical cautery with topically applied silver nitrate.

Persistent uranchus
This is characterised by urinary discharge from the umbilicus. It is caused by
persistence of the uranchus which attaches to the bladder. They are associated with
other urogenital abnormalities.

Persistent vitello-intestinal duct

This will typically present as an umbilical discharge that discharges small bowel
content. Complete persistence of the duct is a rare condition. Much more common is
the persistence of part of the duct (Meckels diverticulum). Persistent vitello-intestinal
ducts are best imaged using a contrast study to delineate the anatomy and are
managed by laparotomy and surgical closure.
Which one of the following is least associated with Tetralogy of Fallot?

A. Right ventricular outflow tract obstruction

B. Overriding aorta

C. Ejection systolic murmur

D. Left-to-right shunt

E. Right ventricular hypertrophy

Right-to-left shunting is characteristic of Fallot's. It is however known that a small

number of asymptomatic infants may initially have a degree of left-to-right shunting
through the ventricular septal defect.

Tetralogy of Fallot

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart
disease*. It typically presents at around 1-2 months, although may not be picked up
until the baby is 6 months old

The four characteristic features are:

 ventricular septal defect (VSD)

 right ventricular hypertrophy
 right ventricular outflow tract obstruction, pulmonary stenosis
 overriding aorta
The severity of the right ventricular outflow tract obstruction determines the degree of
cyanosis and clinical severity

Other features

 cyanosis
 causes a right-to-left shunt
 ejection systolic murmur due to pulmonary stenosis (the VSD doesn't usually
cause a murmur)
 a right-sided aortic arch is seen in 25% of patients
 chest x-ray shows a 'boot-shaped' heart, ECG shows right ventricular
hypertrophy

Management

 surgical repair is often undertaken in two parts

 cyanotic episodes may be helped by beta-blockers to reduce infundibular
spasm

*however, at birth transposition of the great arteries is the more common lesion as
patients with TOF generally present at around 1-2 months
A 3 year old boy is brought to the clinic with symptoms of urinary hesitancy and poor
stream. Which of the following is the most likely underlying diagnosis?

A. Benign prostatic hypertrophy

B. Posterior urethral valves

C. Neurogenic bladder

D. Urethral calculus

E. Hypospadias

Posterior urethral valves are one of the commonest causes of poor urinary stream and
hesitancy in children. Prostatic disorders are rare.
Hypospadias is associated with urine that is difficult to control, but should not
produce hesitancy.

Urethral valves

Posterior urethral valves are the commonest cause of infravesical outflow obstruction
in males. They may be diagnosed on ante natal ultrasonography. Because the bladder
has to develop high emptying pressures in utero the child may develop renal
parenchymal damage. This translates to renal impairment noted in 70% of boys at
presentation. Treatment is with bladder catheterisation. Endoscopic valvotomy is the
definitive treatment of choice with cystoscopic and renal follow up.
Which of the following is not a feature of oesphageal atresia in neonates?

A. High incidence of polyhydramnios

B. Risk of recurrence in subsequent pregnancies of 80%

C. Distal tracheoesphageal fistula is the commonest variant

D. High incidence of associated imperforate anus

E. Absence of gastric bubble on antenatal ultrasound

Most are sporadic and risk in subsequent pregnancies is not increased.

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension
Oesophageal atresia  Associated with tracheo-oesophageal fistula and
polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

A 12 day old infant is brought to the emergency department by his anxious mother
who notices that he has developed a right sided groin swelling. On examination the
testes are correctly located but it is evident that the child has a right sided inguinal
hernia. It is soft and easily reduced. What is the most appropriate management?

A. Surgery over the next few days

B. Reassure and discharge

C. Surgery at 1 year of age

D. Surgery once the child is 6 months old

E. Application of a hernia truss

Inguinal hernia in infants =
Urgent surgery

The high incidence of strangulation necessitates an urgent herniotomy be performed.

In infants with a reducible hernia this can be performed on a daycase list during the
same week. Deferring surgery on the basis of age is not justified.

Paediatric inguinal hernia

Inguinal hernias are a common disorder in children. They are commoner in males as
the testis migrates from its location on the posterior abdominal wall, down through the
inguinal canal. A patent processus vaginalis may persist and be the site of subsequent
hernia development.
Children presenting in the first few months of life are at the highest risk of
strangulation and the hernia should be repaired urgently. Children over 1 year of age
are at lower risk and surgery may be performed electively. For paediatric hernias a
herniotomy without implantation of mesh is sufficient. Most cases are performed as
day cases.
Theme: Paediatric gastrointestinal disorders

A. Meckel diverticulum
B. Pyloric stenosis
C. Acute appendicitis
D. Mesenteric adenitis
E. Intussusception
F. Malrotation
G. Hirschsprung disease

What is the most likely diagnosis for each scenario given? Each option may be used
once, more than once or not at all.

4. A 48 hour old neonate develops increasing abdominal distension. He had a

normal delivery but has yet to pass any meconium. Following digital rectal
examination liquid stool is released.

Hirschsprung disease

Hirschsprungs may present either with features of bowel obstruction in the

neonatal period or more insidiously during childhood. After the PR there may
be an improvement in symptoms. Diagnosis is by full thickness rectal biopsy.

5. A 7 month old girl presents with vomiting and diarrhoea. She is crying and
drawing her legs up. There is a a sausage shaped mass in the abdomen.

Intussusception

Sausage shaped mass (colon shaped) is common in intussusception. The other

common sign is red jelly stool.

6. A 1 month old baby girl presents with bile stained vomiting. She has an
exomphalos and a congenital diaphragmatic hernia.

Malrotation

Exomphalos and diaphragmatic herniae are commonly associated with

malrotation.
Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure
Necrotising  Prematurity is the main risk factor
enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

Theme: Paediatric gastrointestinal disorders

A. Liver transplant
B. Air insufflation
C. Roux-en-Y portojejunostomy
D. Ramstedt pyloromyotomy
E. Appendicectomy
F. Administration of antibiotics
G. Distal gastrectomy

What is the best management option for these children? Each option may be used
once, more than once or not at all.

7. A 2 year old child has central abdominal pain. He has had a recent upper
respiratory tract infection. On examination he is febrile and has a soft abdomen
with some paraumbilical tenderness.

Administration of antibiotics

This child has mesenteric adenitis. Symptoms should resolve. Should they fail
to do so then appendicectomy will be required as it can present insidiously in
this age group.

8. A 2 month old baby presents with jaundice. He has an elevated conjugated

bilirubin level. Diagnosis is confirmed by cholangiography during surgery.

Roux-en-Y portojejunostomy

This child has biliary atresia. The aim is to avoid liver transplantation (however,
most will come to transplant in time).

9. A 6 week old baby boy presents with non bile stained projectile vomiting. He is
otherwise developmentally normal. His abdomen is soft and non tender.

Ramstedt pyloromyotomy
 This baby has pyloric stenosis and the treatment of choice is a Ramstedt
pyloromyotomy.

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid
Biliary atresia  Jaundice > 14 days
 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

A 6 year old child develops ballooning of the foreskin on micturition and is brought to
the clinic by his anxious mother. One examination the foreskin is non retractile but
otherwise normal. By which age are 95% of all foreskins retractile

A. 2 years

B. 16 years

C. 8 years

D. 5 years

E. 10 years

By 16 years of age almost all foreskins should be retractile and if they are not
circumcision should be considered at around this time.

Paediatric urology- foreskin disorders

Disorders of the foreskin

At birth and in the neonatal period the normal foreskin is non retractile due to the
presence of adhesions between the foreskin and glans. In most cases these will
separate spontaneously. By the end of puberty 95% of foreskins can be retracted. In
some children the non-retractile foreskin may balloon during micturition. This is a
normal variant and requires no specific treatment.

Balanitis This is inflammation of the glans penis. It may occur in both

circumcised and non-circumcised individuals.
Posthitis This is inflammation of the foreskin. It may occur as a result of
infections such as gonorrhoea and other STD's. It may also
complicate diabetes. Posthitis may progress to phimosis and as this
may make cleaning of the glans difficult allow progression to
balanoposthitis.
Paraphimosis Prolonged retraction of the foreskin proximal to the glans may allow
oedema to occur. This may then make foreskin manipulation
difficult. It can usually be managed by compression to reduce the
 oedema and replacement of the foreskin. Where this fails a dorsal
slit may be required and this followed by delayed circumcision.
Phimosis This is inability to retract the foreskin and may be partial or
complete. It may occur secondary to balanoposthitis or balanitis
xerotica obliterans. Depending upon the severity and symptoms
treatment with circumcision may be required.
Balanitis This is a dermatological condition in which scarring of the foreskin
xerotica occurs leading to phimosis. It is rare below the age of 5 years.
obliterans Treatment is usually with circumcision.

What is the investigation of choice to look for renal scarring in a child with
vesicoureteric reflux?

A. Abdominal x-ray

B. Ultrasound

C. DMSA

D. CT KUB

E. Micturating cystourethrogram

Vesicoureteric reflux

Vesicoureteric reflux (VUR) is the abnormal backflow of urine from the bladder into
the ureter and kidney. It is relatively common abnormality of the urinary tract in
children and predisposes to urinary tract infection (UTI), being found in around 30%
of children who present with a UTI. As around 35% of children develop renal scarring
it is important to investigate for VUR in children following a UTI

Pathophysiology of VUR

 ureters are displaced laterally, entering the bladder in a more perpendicular

fashion than at an angle
 therefore shortened intramural course of ureter
 vesicoureteric junction cannot therefore function adequately

The table below summarises the grading of VUR

{Grade}
I Reflux into the ureter only, no dilatation
II Reflux into the renal pelvis on micturition, no dilatation
III Mild/moderate dilatation of the ureter, renal pelvis and calyces
IV Dilation of the renal pelvis and calyces with moderate ureteral tortuosity
V Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity

Investigation

 VUR is normally diagnosed following a micturating cystourethrogram

 a DMSA scan may also be performed to look for renal scarring

Which of the following statements relating to necrotising enterocolitis is false?

A. It has a mortality of 30%

B. Most frequently presents in premature neonates less than 32 weeks

gestation.

C. Should be managed by early laparotomy and segmental resections in

most cases.

D. Pneumostosis intestinalis may be visible on plain abdominal x-ray.

E. May be minimised by use of breast milk over formula feeds.

Most cases will settle with conservative management with NG decompression and
appropriate support. Laparotomy should be undertaken in patients who progress
despite conservative management or in whom compelling indications for surgery exist
(eg free air).

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation
Malrotation  High caecum at the midline
 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

A 6 week old baby boy is brought to the clinic by his mother. She is concerned
because although the left testis is present in the scrotum the right testis is absent. She
reports that it is sometimes palpable when she bathes the child. on examination the
right testis is palpable at the level of the superficial inguinal ring. What is the most
appropriate management?

A. Discharge

B. Re-assess in 5 years

C. Laparoscopy

D. Re-assess in 6 months

E. Orchidopexy
Undescended testes are not uncommon in young children. They may be present in 4%
of term infants, but only in 1.3% children at 3 months of age. In this scenario the testis
is retractile and can be managed expectantly.

Cryptorchidism

A congenital undescended testis is one that has failed to reach the bottom of the
scrotum by 3 months of age. At birth up to 5% of boys will have an undescended
testis, post natal descent occurs in most and by 3 months the incidence of
cryptorchidism falls to 1-2%. In the vast majority of cases the cause of the maldescent
is unknown. A proportion may be associated with other congenital defects including:

Patent processus vaginalis

Abnormal epididymis
Cerebral palsy
Mental retardation
Wilms tumour
Abdominal wall defects (e.g. gastroschisis, prune belly syndrome)

Reasons for correction of cryptorchidism

 Reduce risk of infertility

 Allows the testes to be examined for testicular cancer
 Avoid testicular torsion
 Cosmetic appearance

Males with undescended testis are 40 times as likely to develop testicular cancer
(seminoma) as males without undescended testis
The location of the undescended testis affects the relative risk of testicular cancer
(50% intra-abdominal testes)

Treatment

 Orchidopexy at 6- 18 months of age. The operation usually consists of

inguinal exploration, mobilisation of the testis and implantation into a dartos
pouch.
 Intra-abdominal testis should be evaluated laparoscopically and mobilised.
Whether this is a single stage or two stage procedure depends upon the exact
location.
 After the age of 2 years in untreated individuals the Sertoli cells will degrade
and those presenting late in teenage years may be better served by
orchidectomy than to try and salvage a non functioning testis with an
increased risk of malignancy.

An 8 week old infant is brought to clinic with a history of 18 days of jaundice. The
mother is breast feeding. He was a full term baby. There is no family history of liver
disease. What is the most appropriate next step?
 A. Liver USS

B. Unconjugated bilirubin measurement

C. Conjugated bilirubin measurement

D. Reassure and discharge

E. ERCP

This baby is a full term and has > 14 days of jaundice, therefore needs an urgent
conjugated bilirubin check to rule out biliary atresia. If physiological jaundice the
unconjugated bilirubin levels will be increased. Isotope scanning may be used in
diagnosis, but a definitive diagnosis is normally made during a laparotomy.

Biliary atresia

 1 in 17000 affected
 Biliary tree lumen is obliterated by an inflammatory cholangiopathy causing
progressive liver damage

Clinical features

 Infant well in 1st few weeks of life

 No family history of liver disease
 Jaundice in infants > 14 days in term infants (>21 days in pre term infants)
 Pale stool, yellow urine (colourless in babies)
 Associated with cardiac malformations, polysplenia, situs inversus

Investigation

 Conjugated bilirubin (prolonged physiological jaundice or breast milk

jaundice will cause a rise in unconjugated bilirubin, whereas those with
obstructive liver disease will have a rise in conjugated bilirubin)
 TEBIDA radionuclide scan

Management

 Early recognition is important to prevent liver transplantation.

 Nutritional support.
 Roux-en-Y portojejunostomy (Kasai procedure)
 If Kasai procedure fails or late recognition, a liver transplant becomes the only
option.
Which of the following statements relating to Hirschsprungs disease is false?

A. It is more common in males.

B. Is typically associated with a dilated aganglionic segment of bowel.

C. May present with delayed passage of meconium.

D. Mucosal biopsies are inadequate for diagnosis.

E. Disease extending beyond 30cm of colon and rectum is unusual.

There is a transition zone from the contracted aganglionic segment (the abnormal
area) to dilated normal bowel on barium enema. Males are more frequently affected
than females. Surgery may involve a pull through procedure. A number of patients
will have ongoing evacuatory disturbance.

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F

 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
  Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
 Increased risk when empirical antibiotics are given to
infants beyond 5 days

A 4 year old is admitted with right iliac fossa pain and is due to undergo an
appendicectomy. The nursing staff would like to give the child an infusion of
intravenous fluid whilst waiting for theatre. Assuming electrolytes are normal, which
of the following is an appropriate fluid for infusion in this situation?

A. 10% Dextrose solution

B. 0.9% Saline solution

C. 0.45% saline/ 5% glucose solution

D. Gelofusin

E. None of the above

0.45% saline/ 5% glucose solutions carry a risk of hyponatraemia and is contra

indicated- see below.

Paediatric fluid management

Since 2000 there have been at least 4 reported deaths from fluid induced
hyponatraemia in children. This led to the National Patient Safety Agency introducing
revised guidelines in 2007.
Indications for IV fluids include:

 Resuscitation and circulatory support

 Replacing on-going fluid losses
 Maintenance fluids for children for whom oral fluids are not appropriate
 Correction of electrolyte disturbances

Fluids to be avoided
Outside the neonatal period saline / glucose solutions should not be given. The
greatest risk is with saline 0.18 / glucose 4% solutions. The report states that 0.45%
saline / 5% glucose may be used. But preference should be given to isotonic solutions
and few indications exist for this solution either.

Fluids to be used

 0.9% saline
 5% glucose (though only with saline for maintenance and not to replace
losses)
 Hartmans solution

Potassium should be added to maintenance fluids according patients plasma

potassium levels (which should be monitored).

Intraoperative fluid management

Neonates should receive glucose during surgery.
Other children should receive isotonic crystalloid.

Maintenance fluids
Weight Water Na K mmol/kg/day
requirement/kg/day mmol/kg/day
First 10Kg body weight 100ml 2-4 1.5-2.5
Second 10Kg body 50ml 1-2 0.5-1.5
weight
Subsequent Kg 20ml 0.5-1.0 0.2-0.7

Glucose will need to be given to neonates- usually 10% at a rate of 60ml/Kg/day.

Reference
NPSA -reducing risk of hyponatraemia when administering intravenous fluids to
children. Issue date March 2007. Further references included in this document.
Theme: Paediatric gastrointestinal disorders

A. Appendicectomy
B. Active observation
C. Discharge
D. Ultrasound of the abdomen
E. Colonoscopy
F. Hydrostatic reduction under fluoroscopic guidance
G. Laparotomy

Please select the most appropriate form of management from the list above. Each
option may be used once, more than once or not at all.

17. A 5 year old girl has been unwell for 3 days with occasional vomiting and
lethargy, she had one episode of diarrhoea. On examination she has a soft
abdomen with tenderness in the region of the right iliac fossa. Her temperature
is 38.1. Urine dipstick shows leucocytes (+) and protein (+).

Appendicectomy

The most likely diagnosis is appendicitis. This can often present with less
robust signs in paediatric than adult practice.

18. 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

Laparotomy

He has necrotising enterocolitis and whilst this is often initially managed

medically a laparotomy is required if the situation deteriorates.

19. 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'.

Hydrostatic 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 barium or water is the first line
option.

Paediatric Gastrointestinal disorders

Pyloric stenosis  M>F
 5-10% Family history in parents
 Projectile non bile stained vomiting at 4-6 weeks of life
 USS diagnosis
 Treatment: Ramstedt pyloromyotomy

Acute appendicitis  Uncommon under 3 years

 When occurs may present atypically

Mesenteric adenitis  Central abdominal pain and URTI

 Conservative management

Intussusception  Telescoping bowel

 Proximal to ileocaecal valve
 6-9 months age
 Colicky pain, diarrhoea and vomiting, sausage shaped
mass, red jelly stool.
 Treatment: reduction with air insufflation

Malrotation  High caecum at the midline

 Feature in exomphalos, congenital diaphragmatic hernia,
intrinsic duodenal atresia

Hirschsprung's  Absence of ganglion cells from myenteric and

disease submucosal plexuses
 Occurs in 1/5000 births
 Full thickness rectal biopsy for diagnosis
 Delayed passage of meconium and abdominal distension

Oesophageal atresia  Associated with tracheo-oesophageal fistula and

polyhydramnios
 PC choking and cyanotic episodes
 VACTERL

Meconium ileus  Majority have cystic fibrosis

 X-Rays will not show a fluid level as the meconium is
viscid

Biliary atresia  Jaundice > 14 days

 Increased conjugated bilirubin
 Urgent Kasai procedure

Necrotising  Prematurity is the main risk factor

enterocolitis  Early features include abdominal distension and passage
of bloody stools
 X-Rays may show pneumatosis intestinalis and evidence
of free air
  Increased risk when empirical antibiotics are given to
infants beyond 5 days

A 4 year old boy presents with symptoms of dysuria and urinary frequency. A urine
dipstick is positive for blood and nitrites. A UTI is suspected. Which of the following
follow up strategies is most appropriate?

A. Watchful waiting

B. Cystoscopy

C. DMSA scan

D. CT scan of pelvis

E. Renal MRI

A first presentation of an uncomplicated UTI (even in male children) may be

managed expectantly. More than 1 UTI in a six month period should prompt further
investigation.

Urinary tract infection- paediatric

 UTI's may occur in 5% of young girls and 1-2% males. The incidence is
higher in premature infants.
 E-Coli accounts for 80% cases.
 In children with UTI it is important to establish whether there is underlying
urinary stasis or vesico-ureteric reflux (or both).
 Pyelonephritis in children carries the risk of renal scarring 10% and this
translates into a 10% risk of developing end stage renal disease.

Diagnosis

 Pyrexia lasting for more than 3 days mandates urine testing.

 Samples may be taken from mid-stream urine samples or supra pubic
aspiration. Urine collected from nappies usually have faecal contaminants. In
samples showing mixed growth contamination of the sample has usually
occurred.
 As in adults >105 colony forming units of a single organism are usually
indicative of a UTI.

Management

 A single isolated UTI (in girls) may be managed expectantly.

 > 2 UTI's (or 1 in males) in a 6 month period should prompt further testing.
  Voiding cystourethrograms show the greatest anatomical detail and is the ideal
first line test in males; isotope cystography has a lower radiation dose and is
the first line test in girls.
 USS should also be performed. Renal cortical scintigraphy should be
performed when renal scarring is suspected.

An 18 month old boy is brought to the emergency room by his parents. He was found
in bed with a nappy filled with dark red blood. He is haemodynamically unstable and
requires a blood transfusion. Prior to this episode he was well with no prior medical
history. What is the most likely cause?

A. Necrotising enterocolitis

B. Anal fissure

C. Oesophageal varices

D. Meckels diverticulum

E. Crohns disease
Meckels diverticulum is the number one cause of painless massive GI bleeding
requiring a transfusion in children between the ages of 1 and 2 years.

Other causes of GI bleeding include:

Site Newborn 1 month to 1 year 1 to 2 years Older than 2
years
Upper Haemorrhagic Oesophagitis/ Peptic ulcer Varices
GI tract disease, swallowed gastritis disease
maternal blood
Lower Anal fissure/ NEC Anal fissure/ Polyps / IBD/ Polyps/
GI tract Intussusception Meckels Intussusception
diverticulum

Meckel's diverticulum

 Congenital abnormality resulting in incomplete obliteration of the vitello-

intestinal duct
 Normally, in the foetus, there is an attachment between the vitello-intestinal
duct and the yolk sac.This disappears at 6 weeks gestation.
 The tip is free in majority of cases.
 Associated with enterocystomas, umbilical sinuses, and omphaloileal fistulas.
 Arterial supply: omphalomesenteric artery.
 2% of population, 2 inches long, 2 feet from the ileocaecal valve.
 Typically lined by ileal mucosa but ectopic gastric mucosa can occur, with the
risk of peptic ulceration. Pancreatic and jejunal mucosa can also occur.
Clinical

 Normally asymptomatic and an incidental finding.

 Complications are the result of obstruction, ectopic tissue, or inflammation.
 Removal if narrow neck or symptomatic. Options are between wedge excision
or formal small bowel resection and anastomosis.