Intestinal Blockage Caused by Missing Nerves

Hirschsprung disease is a condition that occurs when certain nerves are missing from a portion of the bowel and the contents of the intestines cannot push through that area. Although the intestines are not physically blocked or obstructed in Hirschsprung disease, they behave as if there is an intestinal blockage. 

During normal development before birth, the nerve cells that control a child’s intestinal function work their way from the upper part of the gastrointestinal (GI) tract to the anus. In some cases, this nerve cell migration is incomplete and normal nerve cells are missing, leading to Hirschsprung disease.

Symptoms, Diagnosis and Outlook

The main symptoms of Hirschsprung disease are intestinal obstruction or constipation that appears shortly after birth and does not respond to medication.

Other symptoms include:

  • Difficulty having bowel movements
  • Failure to pass meconium shortly after birth
  • Failure to pass a first stool within 24 to 48 hours after birth
  • Infrequent but explosive stools
  • Jaundice
  • Poor feeding
  • Poor weight gain
  • Vomiting
  • Watery diarrhea in newborns

Diagnosis of Hirschsprung disease includes a physical exam and review of your family’s health history.

Tests may include:

  • Rectal biopsy to diagnose or rule out Hirschsprung
  • Abdominal X-ray to check for obstructions
  • Anorectal manometry, which uses special sensors and balloons to measure well your child’s rectum is working
  • Lower GI series to observe the large intestine

Surgical treatment improves or eliminates symptoms of Hirschsprung disease for most children. A small number may have constipation issues or experience fecal incontinence. Children who are treated early or who have a shorter segment of bowel involved tend to have the best outcomes.

Our Experts in Care

The pediatric surgeons at Penn State Children’s Hospital have advanced expertise in the diagnosis, treatment and management of patients with all varieties of Hirschsprung disease.

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