Hirschsprung’s Disease
What is it?
Hirschsprung’s disease, also known as congenital aganglionic megacolon, is a rare congenital disease where the nerve cells in the colon are missing. This causes difficulties in passing stool and can lead to accumulation of stool. Treatment is bowel resection of the affected area. Megacolon can occur, which is a complication of Hirschsprung’s disease where the colon becomes severely enlarged.
Symptoms
- Failing to pass meconium (stool) in the first 48 hours of life
- Chronic constipation
- Abdominal distension
- Vomiting
- Foul-smelling ribbon-like stools
- Intestinal obstruction or perforation (in severe cases).
Treatment
Temporary colostomy may be needed to relieve obstruction then a pull-through surgical repair with excision of affected bowel.
Nursing Priorities
- NPO
- IV hydration and electrolyte monitoring
- Monitor bowel function
- Measure abdominal girth
- I & O
- Daily weight
- No rectal temperatures
Hirschsprung’s Disease