Surgery · Pediatric Surgery

A 1-day-old neonate presents with failure to pass meconium, abdominal distension, and bilious vomiting. Contrast enema shows a narrow rectosigmoid with proximal dilation. Suction rectal biopsy confirms the diagnosis. What will histology show?

  • A Presence of normal ganglion cells in the submucosal and myenteric plexuses
  • B Absence of ganglion cells (aganglionosis) with hypertrophied nerve trunks in the narrowed segment
  • C Mucosal inflammation with crypt abscesses
  • D Smooth muscle hypertrophy without neural abnormality
Correct answer: B. Absence of ganglion cells (aganglionosis) with hypertrophied nerve trunks in the narrowed segment

Explanation

Hirschsprung's disease results from failure of neural crest cell migration to the distal bowel during embryogenesis, causing aganglionosis of the affected segment (typically rectosigmoid). Suction rectal biopsy demonstrating absence of ganglion cells in the submucosal (Meissner's) and myenteric (Auerbach's) plexuses with hypertrophied nerve trunks is diagnostic. Acetylcholinesterase histochemistry shows excess staining in nerve trunks. The aganglionic segment causes functional obstruction, and the proximal normal bowel dilates.

Reference: Bailey & Love's Short Practice of Surgery, 27th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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