Hirschsprung's disease: Difference between revisions
m (Rossdonaldson1 moved page Hirschsprung's Disease to Hirschsprung's disease) |
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*30% associated with a syndrome | *30% associated with a syndrome | ||
*1:5,000 live births | *1:5,000 live births | ||
==Clinical Features== | ==Clinical Features== | ||
*Delayed meconium passage after birth | *Delayed meconium passage after birth | ||
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*Enterocolitis | *Enterocolitis | ||
**Diarrhea, rectal bleeding, abdominal distention, fever | **Diarrhea, rectal bleeding, abdominal distention, fever | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*Meconium ileus | *Meconium ileus | ||
| Line 25: | Line 27: | ||
*Constipation | *Constipation | ||
*Bowel obstruction | *Bowel obstruction | ||
==Evaluation== | ==Evaluation== | ||
*KUB distended colon, empty rectum | *[[KUB]] distended colon, empty rectum | ||
**Enterocolitis: distention, bowel wall thickening | **Enterocolitis: distention, bowel wall thickening | ||
**Obstruction: air fluid levels | **Obstruction: air fluid levels | ||
*Diagnosis requires rectal biopsy (consult GI) | *Diagnosis requires rectal biopsy (consult GI) | ||
==Management== | ==Management== | ||
*Consult pediatric surgeon for operative management | *Consult pediatric surgeon for operative management | ||
*Treat enterocolitis with broad spectrum antibiotics | *Treat enterocolitis with broad spectrum [[antibiotics]] | ||
==Disposition== | ==Disposition== | ||
*Admit if symptoms of obstruction, enterocolitis | *Admit if symptoms of obstruction, enterocolitis | ||
==See Also== | ==See Also== | ||
Revision as of 17:48, 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
- Usually rectosigmoid area
- 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
- Diarrhea, rectal bleeding, abdominal distention, fever
Differential Diagnosis
- Meconium ileus
- Cystic fibrosis
- Imperforate anus
- Constipation
- Bowel obstruction
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