Correct Answer

a) Male and female have equal incidence

Explanation

Hirschsprung’s disease is a congenital disorder characterized by absence of ganglion cells in the distal bowel, leading to functional intestinal obstruction.

• Sex distribution:
  Hirschsprung’s disease shows a clear male predominance. The male-to-female ratio is approximately 4:1 in short-segment disease. Therefore, equal incidence in males and females is false.
• Extent of disease:
  Total colonic aganglionosis occurs in about 5–10% of patients, making option (b) true.
• Postoperative outcome:
  Following pull-through surgery, bowel dysfunction is common. Constipation is the most frequent long-term problem, so option (c) is true.
• Genetic associations:
  Hirschsprung’s disease is associated with chromosomal anomalies. Down syndrome is seen in approximately 2–10% of cases, making option (d) true.

Teaching Points

• Hirschsprung’s disease has a strong male predominance
• Total colonic aganglionosis occurs in about 5–10% of cases
• Constipation is the most common long-term complication after surgery
• Down syndrome is the most common genetic association
• Enterocolitis is the most feared complication, especially in infants