3: GI - Hirschsprungs

Question 1

What is Hirschsprung’s Disease

Answer 1

Inherited disorder where there is failure of migration of parasympathetic neuroblasts that form Auerbach and meissner plexus causing a failure of bowel to relax and functional obstruction.

Question 2

In which gender is Hirschsprung’s disease more common

Answer 2

Male (4:1)

Question 3

What are three RF for hirschsprungs disease

Answer 3

• FH
• Trisomy 21
• MEN2

Question 4

What are 4 early ways in which hirschsprungs may present

Answer 4

• Delayed meconium
• Billous vomitting
• Tight anal sphincter
• FTT

Question 5

What defines delayed meconium

Answer 5

> 48h

Question 6

What is a late sign of hirschsprungs

Answer 6

Chronic absolute constipation

Question 7

Which part of the colon is affected in 80% of hirschsprungs

Answer 7

Rectosigmoid

Question 8

What is first-line investigation of hirschsrpungs

Answer 8

Abdominal x-ray

Question 9

What may be seen on x-ray in hirschsprungs

Answer 9

1. Dilation of bowel proximal to aganglionic segment.

2. Absent air in rectum

Question 10

What is second-line investigation for hirschsrpungs

Answer 10

Barium enema

Question 11

Why is a barium enema performed in hirschsprungs

Answer 11

Determine length of aganglionic segment prior to surgery

Question 12

When may anorectal mamoetry be used in hrischsprungs

Answer 12

Older children

Question 13

What is the confirmatory test for hirschsprungs

Answer 13

Rectal biopsy

Question 14

What will a biopsy in Hirschsprung’s show

Answer 14

Absence of ganglia

Question 15

How may hirschsprungs be managed

Answer 15

Surgically by two stages:

1. Diverting colostomy to relieve the bowel
2. Removal of aganglionic segment

Question 16

What is a risk if hirschsprungs is not treated

Answer 16

Hirschsprungs enterocolitis which can lead to toxic megacolon