Hirschsprung's disease

Question 1

Define Hirschsprung’s disease

Answer 1

Aganglionosis of distal colon and rectum

Question 2

What is the embryology of Hirschsprung’s disease?

Answer 2

Failure of neural crest cells to migrate into GIT from cranial to caudal in myenteric and submucosal plexus (5th-10th week GA)

Question 3

What is another name for the myenteric plexus?

Answer 3

Auerbach

Question 4

What is another name for the submucosal plexus?

Answer 4

Meissner

Question 5

Name the types of Hirschsprung’s disease

Answer 5

Short segment (common)
Long segment

Question 6

Which anomalies are associated with Hirschsprung’s disease?

Answer 6

Cardiac
Urogenital
Anorectal
T21 syndrome

Question 7

Name the clinical features of Hirschsprung’s disease

Answer 7

No meconium within 24h of life 
Resonant abdominal distention
Explosive stool discharge on DRE
Poor feeding
Vomiting
Enterocolitis
Perforation

Question 8

How do you diagnose Hirschsprung’s disease?

Answer 8

Full thickness rectal biopsy

Question 9

Discuss the management of Hirschsprung’s disease

Answer 9

NGT
IV fluids
Colostomy

If enterocolitis

• saline enemas
• antibiotics

Question 10

Which other muscle does degenerative visceral myopathy affect?

Answer 10

Urinary tract muscles

Question 11

In which population does degenerative visceral myopathy occur?

Answer 11

Black Africans

Question 12

What is the treatment of degenerative visceral myopathy?

Answer 12

None

Patients die as teenagers due to kidney failure

Question 13

Name the 5 causes of not passing meconium

Answer 13

Anorectal malformation
Hirschsprung's disease
Small bowel atresia
Small bowel malrotation
Meconium ileus