Gastrointestinal Development

Question 1

What is the pathophysiology and anatomy of Hirschsprung’s disease?

Answer 1

1. Lack of parasympathetic innervation of rectosigmoid colon, constant contraction.
2. Obstruction and dilation of bowel proximal to affected segment.
3. Internal anal sphincter always involved.

Question 2

What is this a presentation of?
Newborn fails to pass meconium within 48 hours of birth, distended abdomen, vomiting, tight anal sphincter, explosive passage of stool on removal after PR.

Answer 2

Hirschsprung’s disease

Question 3

How is Hirschsprung’s disease diagnosed?

Answer 3

1. Rectal suction biopsy an aganglionic section gold standard.
2. Rectal contrast enema is a good screening test.

Question 4

What is the management of Hirschsprung’s disease?

Answer 4

1. Resection of aganglionic segment is gold standard.
2. Initial bridging management with rectal washouts.
3. Colon anastomosed to anal canal or colostomy.

Question 5

What are the complications of Hirschsprung’s disease?

Answer 5

Perforation, bleeding, ulcers, enterocolitis, incontinence.

Question 6

What is this describing?

Blind-ending oesophagus and an abnormal connection between the oesophagus and the trachea.

Answer 6

Oesophageal atresia and tracheo-oesophageal fistula (most often occur together)

Question 7

What is this a presentation of?

Saliva pooling in mouth, blowing bubbles, choking, cyanosed when feeding (milk spills into lung), cough.

Answer 7

Oesophageal atresia and tracheo-oesophageal fistula

Question 8

What are the clinical features of prenatal oesophageal atresia and tracheo-oesophageal fistula?

Answer 8

Polyhydramnios, small stomach

Question 9

How is oesophageal atresia and tracheo-oesophageal fistula diagnosed?

Answer 9

1. Inability to pass NG tube into stomach, coiling of tube in oesophagus.
2. In isolated OA - no gastric bubble on CXR
3. If gastric bubble +ve - suggests distal TOF

Question 10

What is the management for oesophageal atresia and tracheo-oesophageal fistula?

Answer 10

1. NBM
2. Suction of oropharynx to prevent aspiration of saliva.
3. Reanastomosis surgery within 24 hours

Question 11

What is the main complication a congenital diaphragmatic hernia?

Answer 11

Pulmonary hypoplasia

Question 12

What is this a presentation of?
Neonate, severe respiratory distress, scaphoid (concave) abdomen, no breath sounds on one side, heart sounds displaced to one side, bowel sounds in one hemithorax.

Answer 12

Congenital diaphragmatic hernia

Question 13

How is prenatal and postnatal congenital diaphragmatic hernia diagnosed?

Answer 13

Prenatal - USS

Postnatal - CXR, bowel loops in hemithorax, mediastinal shift

Question 14

What is the management for a congenital diaphragmatic hernia?

Answer 14

1. Immediate intubation and ventilation
2. Large NG tube to enable air to escape to aid lung expansion
3. Surgery - delay until respiratory status has been established

Question 15

What is the prognosis of a congenital diaphragmatic hernia?

Answer 15

50%

Question 16

What is this describing?

A paraumbilical defect in the abdominal wall with evisceration of the intestine.

Answer 16

Gastroschisis

Question 17

How is gastroschisis identified?

Answer 17

Antenatally on USS

Question 18

What is the management of gastroschisis?

Answer 18

1. Cover exposed bowel in cling film

2. Close defect surgically

Question 19

What is this describing?
Defect in the umbilical ring with herniation of abdominal viscera, covered in peritoneum. Often associated with other congenital malformations.

Answer 19

Exomphalos

Question 20

How is exomphalos identified?

Answer 20

Antenatally on USS

Question 21

What is the management for exomphalos?

Answer 21

Surgery, reduce gradually with delayed closure to prevent respiratory insufficiency.

Question 22

What is the pathophysiology of malrotation (congenital bowel issue)?

Answer 22

Bowel fixed in abnormal position in abdomen, absent attachment of small intestine mesentery causes mid gut volvulus/obstruction.

Question 23

What is this a presentation of?

Bilious neonatal vomiting and bowel obstruction.

Answer 23

Malrotation of the bowel

Question 24

How is malrotation of the bowel in neonates diagnosed?

Answer 24

Upper GI contrast studies

Question 25

What is the management for malrotation of the bowel in neonates?

Answer 25

1. IV fluids, NG tube | 2. Surgical fixation of the bowel

Question 26

What is the complication of malrotation of the bowel in neonates?

Answer 26

Volvulus - surgical emergency, bowel can become necrotic due to occlusion of blood supply form mesentery.

Question 27

What is this describing? | Blind-ending passage of the duodenum.

Answer 27

Duodenal atresia (most common bowel atresia)

Question 28

What is this a presentation of? | Vomiting (can be bilious), failure to pass flatus and faeces.

Answer 28

Intestinal atresia

Question 29

How is intestinal atresia diagnosed?

Answer 29

AXR - dilated bowel loops proximal to atresia, 'double bubble' sign in duodenal atresia.

Question 30

What causes the 'double bubble' sign on the AXR in duodenal atresia?

Answer 30

Air distends the stomach and first part of duodenum forming two separate bubbles.

Question 31

What is the management of intestinal atresia?

Answer 31

Surgical anastomoses of proximal and distal ends.

Question 32

What is the pathophysiology of biliary atresia?

Answer 32

1. Atresia of common bile duct/hepatic ducts. 2. Bile produced but unable to be secreted into small bowel. 3. Inflammation of the bile ducts and liver

Question 33

What is this a presentation of? | Presents in first few weeks of life with prolonged jaundice (>14 days), pale stools, dark urine, poor weight gain.

Answer 33

Biliary atresia

Question 34

How is biliary atresia diagnosed?

Answer 34

1. Conjugated hyperbilirubinaemia, deranged LFTs 2. USS to exclude other causes of cholestasis. 3. Gold standard - radioisotope scans showing no excretion of radioisotope-labelled bile from liver.

Question 35

What is the management for biliary atresia?

Answer 35

Hepatoportoenterostomy (Kasai's procedure)

Question 36

What is a hernia?

Answer 36

A protrusion of an internal organ/tissue through a weakness in its containing cavity into an abnormal position.

Question 37

What is the most common type of hernia in children and why?

Answer 37

Inguinal hernia due to patent processus vaginalis

Question 38

Which gender is more likely to get an inguinal hernia in children?

Answer 38

Male (M:F = 9:1)

Question 39

Which type of inguinal hernia are children most likely to get and at what age does it change?

Answer 39

1. Almost always indirect | 2. >25 years old direct is more likely

Question 40

``` What is this a presentation of? Painless swelling (may be intermittent in inguinal region/scrotum, exacerbated by crying), impalpable from above, can be reduced. ```

Answer 40

Inguinal hernia

Question 41

What is this a presentation of and how would you manage it? | Painful, firm mass in inguinal region/scrotum, intestinal obstruction, irreducible.

Answer 41

Incarcerated inguinal hernia - emergency surgery as there is a risk of strangulation

Question 42

How is an inguinal hernia diagnosed?

Answer 42

1. Clinical examination - can you palpate above it? (to differentiate from hydrocele) 2. USS if diagnostic uncertainty

Question 43

What is the management for an inguinal hernia in an infant?

Answer 43

Six/two rule: 1. Baby <6 weeks - operate within 2 days 2. <6 months - within 2 weeks 3. <6 years - within 2 months

Question 44

What is the management for an umbilical hernia?

Answer 44

1. 80% spontaneously close by age 5, observe. 2. If small - elective repair at 4-5 years of age 3. If large - elective repair at 2-3 years of age