31 Days of Awareness: Hirschsprung's Disease

Hirschsprung's Disease

Source

Awareness Color: Maroon/Burgundy
Awareness Day: September 25 (Illinois only)

Hirschsprung disease (HD) is a disease of the large intestine that causes severe constipation or intestinal obstruction. HD occurs when some of the nerve cells that are normally present in the wall of the intestine do not form properly during fetal development. HD affects 1 in 5000 newborns/toddlers, most often boys.

During digestion, intestinal muscles move food forward in a movement called peristalsis. In order for this movement to occur, special nerve cells called ganglion cells are required. Because these nerve cells are missing in children with Hirschsprung disease, normal peristaltic movement cannot occur.

Consequently, stool backs up, causing either partial or complete bowel obstruction.

Eventually, a bacterial infection can develop in the digestive tract, causing serious problems. Severe worsening of the obstruction can lead to a hole in the bowel (perforation) and severe infection.

All children with Hirschsprung disease require surgical treatment.

Symptoms

The main symptoms of Hirschsprung disease are constipation or intestinal obstruction, usually appearing shortly after birth. Constipation in infants and children is common and usually comes and goes, but if your child has had ongoing constipation since birth, Hirschsprung disease may be the problem.

Symptoms in Newborns

Newborns with Hirschsprung disease almost always fail to have their first bowel movement within 48 hours after birth. Other symptoms include

• green or brown vomit
• explosive stools after a doctor inserts a finger into the rectum
• swelling of the belly, also known as the abdomen
• lots of gas
• bloody diarrhea

Symptoms in Symptoms in Toddlers and Older Children

Symptoms of Hirschsprung disease in toddlers and older children include

• not being able to pass stools without laxatives or enemas. A laxative is medicine that loosens stool and increases bowel movements. An enema is performed by flushing water, or sometimes a mild soap solution, into the anus using a special wash bottle
• swelling of the abdomen
• lots of gas
• bloody diarrhea
• slow growth or development
• lack of energy because of a shortage of red blood cells, called anemia

Treatment

Surgery only. There are two types. Pull through surgery and ostomy surgery. In pull through surgery, the damaged parts of the colon are removed and the good parts are surgically reattached. In an ostomy surgery, the bowel is circumvented and wastes are expelled through the wall of the stomach into an ostomy bag.

.To learn more, please visit:

Hirschsprung Disease

Cinncinatti Children's