Hirschsprung's disease: Difference between revisions

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*80% present within first month of life
*80% present within first month of life
**May have delayed presentation in milder disease
**May have delayed presentation in milder disease
*Chronic constipation
*Chronic [[Constipation (peds)|constipation]]
**Encopresis
**Encopresis
**Rectal prolapse
**[[Rectal prolapse]]
*Obstruction symptoms
*[[Small bowel obstruction|Obstruction]] symptoms
**Neonatal [[toxic megacolon]] may occur
**Neonatal [[toxic megacolon]] may occur
***Distended abdomen, [[Nausea and vomiting (peds)|bilious vomiting]], enterocolitis
***Distended abdomen, [[Nausea and vomiting (peds)|bilious vomiting]], enterocolitis

Revision as of 17:54, 9 July 2016

Background

  • Congenital aganglionosis of colon
    • Usually 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
  • Diagnosis requires rectal biopsy (consult GI)

Management

  • Consult pediatric surgeon for operative management
  • Treat enterocolitis with broad spectrum antibiotics

Disposition

  • Admit if symptoms of obstruction, enterocolitis

See Also

External Links

References