GI Malformations

Question 1

Presentation of Hirschprung’s disease?

Answer 1

Failure or delay to pass meconium
Constipation
Abdominal distention
Faeces may be felt per abdomen
PR may reveal tight anal sphincter and explosive discharge of stool

Question 2

What are complications of Hirschprungs?

Answer 2

GI perforation
Bleeding
Ulcers
Enterocolitis
Short gut syndrome

Question 3

How is Hirschprungs diagnosed? Mx?

Answer 3

Recta suction biopsy of aganglionic section staining for acetylcholisterase positive nerve excess

Excision of aganglionic segment

Question 4

What are prenatal signs of oesophageal atresia + tracheo-oesophageal fistula

Answer 4

Poly hydramnios

Small stomach

Question 5

What are postnatal signs of oesophageal atresia and tracheo-oesophageal fistula?

Answer 5

Cough
Airway obstruction
Increased secretions
Blowing bubbles
Distended abdomen
Cyanosis 
Aspiration

Question 6

How is oesophageal atresia/TOF diagnosed:?

Answer 6

Inability to pass catheter into stomach, x-ray shows it coiled in oesopagus

Question 7

Mx of oesophageal atresia?

Answer 7

Stop feeding
Suck out oesophageal pouch
Primary surgical repair

Question 8

What is congenital diaphragmatic hernia?

Answer 8

Developmental defect in the diaphragm allowing herniation of abdominal contents into the chest
Leads to impaired lung development: pulmonary hypoplasia and pulmonary hypertension

Question 9

How is congenital diaphragmatic hernia diagnosed?

Answer 9

Prenatal US

Postnatal CXR

Question 10

What are signs of congenital diaphragmatic hernia?

Answer 10

Difficult resuscitation at both
Respiratory distress
Bowel sounds in one hemithroax - usually the left
pH < 7.3
Cyanosis

Question 11

What is associated with congenital diaphragmatic hernia?

Answer 11

Trisomy 18
Pierre Robin
Other malformation - NTDs

Question 12

What is prenatal and post natal treatment for congenital diaphragmatic hernia?

Answer 12

Prenatal:
Consideration of fetal surgery - tracheal obstruction by balloon

Postnatal:
Inset large bore NG tube when diagnosis suspected
Aim is to keep all air out of the gut
Facemask ventilation is contraindicated so immediately intubate, ventilate and paralyse
Surgery

Question 13

What causes inguinal hernias? How do they present?

Answer 13

Patent processus vaginalis (passage which ushers the descending testicle into the scrotum)

Present as a lateral bulge to the pubic tubercle e..g during crying

Question 14

Management for inguinal hernia?

Answer 14

Surgical repair

baby < 6w - operate within 2 days
<6 months operate within 2 weeks
< 6 years operate within 2 months

Question 15

What is hydrocele?

Answer 15

Processus vaginalis patent at birth allowing only fluid from the peritoneal cavity to pass down it
Generally closes during the first year of life so no action is usually needed

If it persists until 2 years, surgical correction may be needed

Question 16

What should you do for billions vomiting in neonates?

Answer 16

Surgical referral
Pass NGT
May be a midgut malrotation

Question 17

What does acute gastric volvulus cause?

Answer 17

Non-bilious vomtiing
Epigastric distention
signs of pain

Question 18

What is gastroschisis?

Answer 18

Paraumbilical (lateral to umbilicus) defect with evisceration of the abdominal contents

Most diagnosed antenatally on US

Question 19

What is management for gastroschisis?

Answer 19

At delivery cover exposed bowel in clingfilm
Keep baby warm and hydrated

Surgical correction ASAP
May involve a staged procedure because the abdomen at birth is too small to accommodate gut

Question 20

What is exomphalos?

Answer 20

Omphalocele

Ventral defects of the umbilical ring with herniation of abdominal viscera - covered in peritoneum and amnion

Often associated with other malformations

May contain bowel, stomach, liver, bladder

Question 21

What is management of exomphalos?

Answer 21

Protect herniated viscera
Maintain fluids and electrolytes
Prevent hypothermia
Gastric decompression
Prevent sepsis

Primary or staged closure may be used to repair