Hirschsprung’s Disease

What is Hirschsprung’s Disease?

Hirschsprung’s disease is a congenital condition that affects the large intestine (colon), causing severe constipation, intestinal blockage, and other serious digestive problems. The condition occurs when nerve cells in the intestinal wall are missing, preventing proper muscle movement in the bowel.

Key Characteristics

  • Absence of nerve cells (ganglion cells) in parts of the intestine
  • Affects bowel movement and intestinal muscle function
  • Usually detected shortly after birth
  • More common in males than females

Common Symptoms

  • Failure to pass meconium within 48 hours of birth
  • Chronic constipation
  • Swollen abdomen
  • Vomiting
  • Poor feeding
  • Slow growth

Diagnostic Methods

Healthcare providers use several tests to diagnose Hirschsprung’s disease:

  • Rectal biopsy to examine tissue samples
  • Contrast enema x-ray
  • Anorectal manometry to measure muscle pressure
  • Genetic testing

Treatment Options

Treatment typically involves surgical intervention:

  • Pull-through procedure to remove affected portion of the bowel
  • Temporary ostomy in some cases
  • Post-surgical care and monitoring
  • Dietary management

Long-term Management

  • Regular follow-up with healthcare providers
  • Monitoring for complications
  • Dietary adjustments as needed
  • Support for families and caregivers

Complications

Without proper treatment, Hirschsprung’s disease can lead to serious complications:

  • Enterocolitis (intestinal inflammation)
  • Intestinal perforation
  • Malnutrition
  • Growth delays