Hirschsprung's disease: Difference between revisions
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==Background== | ==Background== | ||
*Congenital aganglionosis of 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 | **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 | |||
*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
- Delayed meconium passage after birth
- 80% present within first month of life
- May have delayed presentation in milder disease
- Chronic constipation
- Encopresis
- Rectal prolapse
- Obstruction symptoms
- Neonatal toxic megacolon may occur
- Distended abdomen, bilious vomiting, enterocolitis
- Neonatal toxic megacolon may occur
- Enterocolitis
Differential Diagnosis
Infant Constipation
- Constipation
- Hirschsprung's disease
- Congenital anorectal malformations
- Imperforate anus
- Bowel obstruction
- Neurologic disorders
- Encephalopathy
- Spinal cord abnormalities: myelomeningocele, spina bifida, tethered cord
- Meconium ileus
- Metabolic causes
- Heavy-metal poisoning
- Medication side effects
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