Hirschsprung’s Disease: A Team Approach to Surgery and Care

Hirschsprung’s disease is an intestinal disorder that often requires surgery and long-term bowel management. http://www.chop.edu/pace

In This Video:
0:31 What is Hirschsprung’s Disease?
0:55 Signs and Symptoms of Hirschsprung’s Disease
1:33 Diagnosing Hirschsprung’s Disease
2:40 Pull-Through Procedure for Hirschsprung’s Disease
4:17 Long-Term Care for Children with Hirschsprung’s Disease
4:50 Antegrade Continence Enema (ACE)
5:27 Living with Hirschsprung’s Disease: School, Sports

The Pediatric Anorectal Continence Evaluation (PACE) Program at The Children's Hospital of Philadelphia brings together top pediatric specialists in general surgery and gastroenterology to provide comprehensive care in one location for children with Hirschsprung’s disease and other long-term bowel management needs. Abnormal bowel function interferes with daily life and health, and long-term coordinated care can help improve health and well-being.

Hirschsprung’s disease is a rare intestinal disorder, also referred to as congenital megacolon, in which ganglion cells fail to develop in the portion of the large intestine that descends toward the rectum. Ganglion cells are what stimulate the bowel to relax. Without these cells, the bowel tightens up and a child is unable to defecate (poop). Newborns will often have an enlarged abdomen or swollen stomach (abdominal distension). Other Hirschsprung’s disease symptoms may include excessive vomiting, inability to tolerate feeding and weight loss. Older children may suffer from chronic constipation. If left untreated, Hirschsprung’s disease can be life threatening.

The Pediatric Anorectal Continence Evaluation (PACE) Program treats more than 300 patients annually in our multidisciplinary clinic. We are experienced at diagnosing Hirschsprung’s disease and tailor treatment to meet a child's specific needs.

Hirschsprung’s disease treatment includes surgical correction, typically using the pull-through procedure, a type of anorectal surgery where the diseased bowel is either removed and bypassed and replaced with normal colon. After a pull-through procedure for Hirschsprung’s disease, children are monitored closely for surgical complications such as enterocolitis, anastomotic leak, complications from bloating, bowel obstruction (blocked intestine), or a narrowing of the bowel (stenosis).

In addition to surgery, we incorporate medications, diet, activity and lifestyle changes depending on a child's underlying diagnosis, associated anomalies and overall lifestyle. Hirschsprung’s disease requires lifelong management to monitor for problems like chronic constipation and other digestive problems. Our PACE Program team works with families to establish a successful bowel management program in the years following surgery so that a child can lead a normal, healthy life. This may include anything from antegrade colonic enemas (ACE), used to irrigate the bowel from the top down, to making sure children have the accommodations they need at school and in the community so they can thrive.