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Hirschsprung's disease: a cause of chronic constipation in children

S Worman1, T G Ganiats

  • 1University of California, San Diego, School of Medicine, La Jolla.

American Family Physician
|February 1, 1995
PubMed

Insights

Hirschsprung's disease is a rare cause of chronic constipation in children, characterized by absent nerve cells in the intestine. Early diagnosis and surgical treatment are crucial for a favorable outcome in this serious condition.

Area of Science:

  • Pediatric Gastroenterology
  • Surgical Neonatology
  • Developmental Biology

Background:

  • Constipation is a frequent pediatric concern, with most cases being benign.
  • Hirschsprung's disease, a form of intestinal dysganglionosis, presents as an unusual but recognized cause of chronic constipation.
  • This condition involves the absence of intramural ganglionic cells in the intestinal wall.

Observation:

  • The disease course exhibits variability with five distinct patterns of severity.
  • Untreated Hirschsprung's disease carries a high mortality rate, approaching 50% by one year of age.
  • The underlying pathology is the lack of nerve cells crucial for intestinal motility.

Findings:

  • Hirschsprung's disease is defined by the absence of submucosal and myenteric ganglionic cells.
  • The condition's presentation varies significantly among affected neonates and children.
  • Surgical intervention is the established treatment modality for this condition.

Implications:

  • Early diagnosis and prompt surgical intervention are critical for improving patient outcomes.
  • Understanding the disease's patterns aids in tailoring treatment strategies.
  • Effective management can significantly reduce the mortality associated with Hirschsprung's disease.

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