Difference between revisions of "Hirschsprung's disease"

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==Background==
 
==Background==
 
*Congenital aganglionosis of colon
 
*Congenital aganglionosis of colon
**Usually rectosigmoid area
+
*Usually affects rectosigmoid area
***Short segment affects rectum and small amount of distal colon
+
**Short segment affects rectum and small amount of distal colon
***Long segment affects more of colon, proximal intestine
+
**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
 
*1:5,000 live births
 
*1:5,000 live births
 +
 
==Clinical Features==
 
==Clinical Features==
 
*Delayed meconium passage after birth
 
*Delayed meconium passage after birth
 
*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, bilious vomiting, enterocolitis
+
***Distended abdomen, [[Nausea and vomiting (peds)|bilious vomiting]], enterocolitis
 
*Enterocolitis
 
*Enterocolitis
**Diarrhea, rectal bleeding, abdominal distention, fever
+
**[[Diarrhea (peds)|Diarrhea]], [[GI Bleeding (Peds)|rectal bleeding]], abdominal distention, [[fever]]
 +
 
 
==Differential Diagnosis==
 
==Differential Diagnosis==
*Meconium ileus
+
{{Infant Constipation}}
**Cystic fibrosis
+
 
*Imperforate anus
 
*Constipation
 
*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
 +
*Barium/contrast enema will show narrowed colon and dilated intestine above
 +
**Sensitivity 70% and specificity of 83%
 
*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
+
*ED management may include gastric and rectal decompression
 +
*Treat enterocolitis with broad spectrum [[antibiotics]]
 +
*Supportive fluid and electrolyte replacement
 +
 
 
==Disposition==
 
==Disposition==
 
*Admit if symptoms of obstruction, enterocolitis
 
*Admit if symptoms of obstruction, enterocolitis
 +
 
==See Also==
 
==See Also==
 +
*[[Constipation (peds)]]
  
 
==External Links==
 
==External Links==
 +
  
 
==References==
 
==References==
 
<references/>
 
<references/>
 +
 +
[[Category:Pediatrics]]
 +
[[Category:GI]]

Latest revision as of 21:18, 29 September 2019

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

Differential Diagnosis

Infant Constipation

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