UMEM Educational Pearls

Category: Pediatrics

Title: Hirschsprung's disease

Posted: 12/13/2014 by Rose Chasm, MD (Updated: 2/27/2020)
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  • Irregular bowel movements and constipation are a common complaint pediatric complaint.
  • The majority of cases are functional, but providers should take extra care to rule out organic causes like Hirschsprung's disease particularly during the neonatal period. 
  • 1 in 5000 incidence, with abnormal innervation of the distal colon resulting in tonic contraction, and obstruction of feces.
  • In most cases, the agangionic segment is limited to the rectosigmoid area.
  • Symptoms usually begin in the first month of life and consist of obstuctive complications such as abdominal distension, bilious vomiting, and poor feeding.
  • Rectal examination should be done in all patients with constipation, and often reveals a narrowed high-pressure region adjacent to the anal sphincter.
  • Barium enema, anal manometry, and rectal biopsy all aid in the diagnosis.

References

NMS Pediatrics. Lippincott Williams and Wilkins. 4th Edition. Paul Dworkin editor.