Difference between revisions of "Hirschsprung's disease"

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
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*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
  • 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
  • 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

See Also

External Links

References