Hirschsprung's disease: Difference between revisions

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==Background==
==Background==
*Congenital aganglionosis of colon
*Congenital aganglionosis of colon
**Usually rectosigmoid area
*Usually affects rectosigmoid area
***Short segment affects rectum and small amount of distal colon
**Short segment affects rectum and small amount of distal colon
***Long segment affects more of colon, proximal intestine
**Long segment affects more of colon, proximal intestine
**Rarely involves more proximal intestine
**Rarely involves more proximal intestine
*30% associated with a syndrome
*30% associated with a syndrome
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==Management==
==Management==
*Consult pediatric surgeon for operative management
*Consult pediatric surgeon for operative management
**ED management may include gastric and rectal decompression
*ED management may include gastric and rectal decompression
**Treat enterocolitis with broad spectrum [[antibiotics]]
*Treat enterocolitis with broad spectrum [[antibiotics]]
*Supportive fluid and electrolyte replacement
*Supportive fluid and electrolyte replacement


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==External Links==
==External Links==


==References==
==References==

Revision as of 05:31, 4 November 2018

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

  • 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