Pedia 2

Question 1

What is congenital umbilical hernia? Why does it happen

Answer 1

It occurs due to failure of the mid gut to return to the abdominal cavity

Not covered with skin, contents are visualized

Question 2

What are the 2 types of congenital umbilical hernia?

Answer 2

Exomphalos major and exomphalos minor

Question 3

What is the difference between Exomphalos Major and exomphalos minor?
[size of defect? Covering? Contents? Treatment?]

Answer 3

Defect:
Exomphalos major: Large (>5cm)
Exomphalos minor: Small (<5cm)

Sac:
Exomphalos major: Ambiotic membrane
Exomphalos minor: Amniotic membrane & Wartons jelly

Contents:
Exomphalos major: Any abdominal viscera (up to liver)
Exomphalos minor: Usually small loop of intestine

Treatment:
Exomphalos major: Complex and needs staged repair
Exomphalos minor: Simple, reduction, herniotomy and herniorraphy

Question 4

Cause of infantile umbilical hernia?

Answer 4

Imperfect closure of the umbilical scar

Question 5

What is the covering of the infantile umbilical hernia?

Answer 5

Skin

Question 6

Treatment of infantile umbilical hernia?

Answer 6

-Spontaneous closure
-Surgical repair may be indicated if persists for more than 2 years or with defect more than 2 fingers

Question 7

What is gastrochisis?

Answer 7

Congenital defect of the anterior abdominal wall with intact peritoneum

May occur at umbilicus or away from it

(Picture of baby with intestines poking out)

Question 8

What is Prune belly syndrome

Answer 8

Congenital defect of the anterior abdominal wall with lack of abdominal musculature so skin is wrinkled

Question 9

Treatment of prune belly syndrome?

Answer 9

Surgical reconstruction and repair of the abdominal wall + management of the associated anomalies

Question 10

What medical conditions are associated with prune belly syndrome?

Answer 10

Undescended testis
Urinary tract malformations (especially mega-ureter) with vesico-ureteric reflux

Question 11

What happens in Ectopia vesica?

Answer 11

Absent lower abdominal wall
Absent anterior bladder wall
Wide separation of the symphysis pubis
Other urinary anomalies especially episadius

(Picture of absent wall over the bladder)

Question 12

Complications of ectopia vesica?

Answer 12

Severe UTI & metaplasia (precancerous)

Question 13

Treatment of ectopia vesica?

Answer 13

Temporary closure of defect
Then> definitive reconstruction of the bladder and pelvic wall

Question 14

From where can congenital diaphragmatic hernia herniate?

Answer 14

Posterior hernia: foramen of bochdalek (most common)

Anterior hernia: foramen of morgagni

Question 15

Clinical presentation of diaphragmatic hernia?

Answer 15

Maternal polyhydraminos

Neonatal cyanosis & respiratory distress

Signs: scaphoid abdomen
Diminished air entry with audible intestinal sounds on the chest
Heart sounds heard better on the right side

Question 16

What investigations would you do for Congenital diaphragmatic hernia

Answer 16

Plain chest x ray (diagnostic)
-presence of bowel in the chest
-shift of the mediastinum to the contra lateral side
-Apparent dextrocardia

Gastrograffin meal

Blood gases: hypoxia hypercapnia &acidosis

Question 17

Treatment of congenital diaphragmatic hernia (pre-operative and operative)

Answer 17

Pre-operative:
•NGT & IV fluids: to deflate the stomach→decrease the lung compression & the mediastinal shift.

•Endotracheal intubation

•Extracorporeal membrane oxygenation (ECMO).

•Pulmonary vasodilators:
o Nitric oxide (NO) inhalation.
o Non-selective α-adrenergic blockers.
o Phosphodiestrase inhibitors (Sildenafil).

Operative repair:
• Open (through subcostal incision).
• Thoracoscopic.

✓ Principles:
• Reduction of the abdominal contents.
• Repair of the diaphragmatic defect either by sutures or by application of Gortex patch.
• Closure should be performed without tension.

Question 18

Which type of biliary atresia is correctable?

Answer 18

Portion of the extrahepatic tree only is occluded

Question 19

Which type of biliary atresia is non-correctable?

Answer 19

When it’s both Intrahepatic and extrahepatic atresia

Question 20

Presentation of biliary atresia?

Answer 20

Neonatal obstructive jaundice (since birth)

Question 21

Treatment for correctable and non correctable biliary atresia?

Answer 21

• Correctable: Kasai operation (Roux en Y hepatico-jejunostomy)
• Non-correctable: liver transplantation

Question 22

What investigations would you do for biliary atresia

Answer 22

US (initial)
HIDA scan (confirmatory)
ERCP

Question 23

Definition & clinical presentation of choledocal cyst?

Answer 23

Congenital cystic dilatation of the intra &/or extra hepatic biliary system

Presentation:
Triad of:
- Childhood obstructive jaundice. (Not since birth)
- Rt. hypochondrial swelling.
- +/- Pain.

Question 24

What can predispose you to cholangiocarcinoma?

Answer 24

Choledocal cyst

Question 25

Treatment for choledocal cyst?

Answer 25

▪Treatment:
- Extra-hepatic only: Excision of the cyst with Roux-en-Y Choledocho jejunostomy.
- Intra-hepatic affection (Caroli’s disease): liver transplantation.

Question 26

Investigations for Choledocal cyst?

Answer 26

-US (initial)
-CT
-HIDA scan (confirmatory)
-ERCP

Question 27

What are the complications of congenital diaphragmatic hernia?

Answer 27

Ipsilateral pulmonary hypoplasia
Contralateral lung compression
Strangulation (of the bowel)

Question 28

Cause of congenital diaphragmatic hernia?

Answer 28

Normal embryology:
Fusion of:
• septum transversum
• pleuroperitoneal folds

Rt. fuses before Lt. making Lt. weaker