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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
- Delayed meconium passage after birth
- 80% present within first month of life
- May have delayed presentation in milder disease
- Chronic constipation
- Obstruction symptoms
- Enterocolitis
Differential Diagnosis
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