Hirschsprung Dx

Question 1

Mention gold standard of inv

Answer 1

Suction rectal biopsy

Question 2

What is Hirschsprung Disease?

Answer 2

Congenital A ganglionic Megacolon

It is characterized by the absence of ganglionic cells in the bowel.

Question 3

What is the pathogenesis of Hirschsprung Disease?

Answer 3

Absence of ganglionic cells in the myenteric & submucosal plexuses of rectum, leading to failure of relaxation of the bowel wall

This results in obstruction.

Question 4

What are the three extents of lesions in Hirschsprung Disease?

Answer 4

1. Recto-sigmoid (80%)
2. Long segment (15%)
3. Total bowel affection (5%)

The majority of cases are limited to the recto-sigmoid region.

Question 5

What segments is Hirschsprung Disease divided into?

Answer 5

1. Rectum: Spastic a ganglionic segment
2. Sigmoid: Transitional zone
3. Colon: Dilated & Hypertrophied proximally

This classification helps in understanding the disease’s impact on bowel function.

Question 6

What is the incidence of Hirschsprung Disease?

Answer 6

Usually sporadic, 1:5,000 live births (Male: Female = 4:1)

This indicates a higher prevalence in males.

Question 7

What are the clinical signs of Hirschsprung Disease in the neonatal period?

Answer 7

Delayed passage of meconium, intestinal obstruction

These symptoms are often the first indications of the disease.

Question 8

What are the childhood symptoms of Hirschsprung Disease?

Answer 8

Progressive chronic constipation, progressive abdominal distension, visible peristalsis terminated at left iliac fossa, fecal mass in the left iliac fossa

Symptoms may vary in severity and presentation.

Question 9

What investigations are used for diagnosing Hirschsprung Disease?

Answer 9

1. PR examination
2. Barium enema
3. Anorectal manometry
4. Suction rectal biopsy (Gold standard)

The suction rectal biopsy is crucial for definitive diagnosis.

Question 10

What findings are expected during a PR examination in Hirschsprung Disease?

Answer 10

Narrow segment, empty rectum
Gush fluid and gases

This indicates the absence of ganglionic cells in the affected area.

Question 11

What is a characteristic finding during a barium enema for Hirschsprung Disease?

Answer 11

Gush of fluids and gases

This is due to the obstruction and abnormal motility.

Question 12

What is the primary treatment for Hirschsprung Disease?

Answer 12

Surgical intervention: a primary pull-through procedure

This procedure aims to remove the aganglionic segment and restore normal bowel function.

Question 13

When is a colostomy indicated in Hirschsprung Disease?

Answer 13

If associated enterocolitis

This is a complication that may require temporary diversion of stool.