Hirschsprung's disease: Difference between revisions

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==Evaluation==
==Evaluation==
[[File:PMC4389175 Iranjradiol-12-02-12451-g001.png|thumb|Barium enema in neonate with Hirschsprung disease shows transitional zone and cobblestone appearance.]]
*[[KUB]] distended colon, empty rectum
*[[KUB]] distended colon, empty rectum
**Enterocolitis: distention, bowel wall thickening
**Enterocolitis: distention, bowel wall thickening

Revision as of 06:17, 8 January 2022

Background

  • 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

Differential Diagnosis

Infant Constipation

Evaluation

Barium enema in neonate with Hirschsprung disease shows transitional zone and cobblestone appearance.
  • 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)

Management

  • 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

Disposition

  • Admit if symptoms of obstruction, enterocolitis

See Also

External Links

References