Hirschsprung's disease


(A) Showing point of calibre change in the colon. (B) Resection of the aganglionic bowel and the severely dilated proximal colon.
  • Congenital aganglionosis of colon
  • Usually affects rectosigmoid area
    • Short segment affects rectum and small amount of distal colon
    • Long segment affects more of colon, proximal intestine
    • Rarely involves more proximal intestine
  • 30% associated with a syndrome
  • 1:5,000 live births

Clinical Features

Three patients with late-diagnosis disease demonstrating abdominal distention.

Differential Diagnosis

Infant Constipation


Abdominal X-ray showing: (A) Faecal stasis and huge dilated transverse colon. (B) Air fuid levels and cut-off sign.
Barium enema in neonate with Hirschsprung disease shows transitional zone and cobblestone appearance.
Barium enema in a 1-month-old male with Hirschsprung disease depicts bizarre irregular contraction.
  • KUB distended colon, empty rectum
    • Enterocolitis: distention, bowel wall thickening
    • Obstruction: air fluid levels
  • Barium/contrast enema will show narrowed colon and dilated intestine above
    • Sensitivity 70% and specificity of 83%
  • Diagnosis requires rectal biopsy (consult GI)


  • Consult pediatric surgeon for operative management
  • ED management may include gastric and rectal decompression
  • Treat enterocolitis with broad spectrum antibiotics
  • Supportive fluid and electrolyte replacement


  • Admit if symptoms of obstruction, enterocolitis

See Also

External Links