Hirschsprung's disease is a condition of the large intestine that causes severe constipation and intestinal obstruction. The constipation comes from the lack of nerve cells in part or all of the large intestine. Hirschsprung's disease is mainly found in infants but sometimes a less severe case develops in older children. An adult case of the disease is extremely rare.
Symptoms
About 80 percent of children who have Hirschsprung's disease show symptoms within the first six months of life. Children who have only a short segment of intestine lacking nerve cells may not show symptoms for up to several months or years. The most common symptoms of the disease are: no bowel movement in the first 48 hours of life, gradual abdominal bloating, gradual onset of vomiting and a fever. Children who do not show early symptoms may have constipation that becomes worse over time, a loss of appetite, delayed growth and passing small, watery stools.
Diagnosis
In order to determine whether a child has Hirschsprung's disease, a doctor will perform tests that may include an abdominal X-ray, a barium enema, an anorectal manometry or a biopsy of the rectum or large intestine. The abdominal X-ray may show a lack of stool in the large intestine or near the rectum and dilated segments on both the large and small intestine. Barium, which is a metallic, chalky liquid, is given into the rectum as an enema to coat the organs so that they show up on an X-ray. This will show whether there are any narrowed areas, blockages or dilated intestines above the obstruction. An anorectal manometry is a test that measures nerve reflexes that are missing in Hirschsprung's disease, and the biopsy of the rectum or large intestine is a test that takes a small sample of the cells in the rectum or large intestine to be examined under a microscope.
Treatment
Two surgeries may be performed to correct Hirschsprung's disease --- ostomy surgery and pull-through surgery. Soon after a child is diagnosed as having the disease, she will undergo one of these procedures to remove the affected section. Although most children with Hirschsprung's may not need ostomy surgery, a child who has been very sick may need to undergo the surgery so his health can improve before he undergoes the pull-through procedure. Ostomy allows the stool to leave the body through an opening in the abdomen. The surgeon will take out the diseased segment of the large intestine, and the healthy end of it will be moved to the opening of the abdomen, where a stoma is created. A stoma is created by rolling the intestine end back on itself, similar to a shirt cuff, and stitching it to the abdominal wall. An ostomy pouch is attached to the stoma and is worn outside the body to collect the stool. The pull-through procedure is when the surgeon removes the affected segment of the large intestine and connects the healthy segment to the anus.
Diet and Nutrition
After the pull-through procedure is performed, children will need to drink more fluids to avoid the risk of dehydration. Because the colon absorbs water from food in the last stages of digestion, when a portion of the colon is removed, the child may have trouble absorbing enough water. Drinking adequate amounts will help the child stay hydrated and may help constipation. Some infants may need to be fed through feeding tubes for awhile. The tube allows an infant's formula or milk to be pumped directly into the stomach or small intestine. Breast milk is recommended due its known laxative effect. If a child is under 12 months of age and is still breastfed, remember that once food is introduced, the frequency and consistency of the stool will change because the breast milk will no longer have its laxative effect.
For babies between 4 and 8 months old, some foods they can eat to help avoid constipation are baby oatmeal, baby prunes, green beans, blueberries, apple juice and carrots. Babies between 8 and 10 months old can have canned peas, fresh strawberries, watermelon, honeydew melon, peeled grapes, kiwi, whole-wheat toast, waffles and breads, whole-wheat macaroni and cheese or pasta, steamed broccoli, green beans, cauliflower and green peas. Avoid the following for babies 4 months and older: rice cereal, applesauce, papayas, tangerines, yams, oranges and potatoes without skins.
For patients with an ostomy bag, include complex carbohydrates, which can be found in foods like whole-wheat cereals, breads and pasta. Make sure to give plenty of caffeine-free fluids to stay hydrated, and avoid all fruit juices and sugary drinks. Juices offer little to no nutritional benefits and can increase stool and ostomy output. Avoid sources of simple sugars, such as jams, jellies, snack cakes, fruit roll-ups and candies. Large amounts of high-fat dairy products may cause constipation in some children and have no effect in others. Be sure to provide enough dairy foods to meet the calcium requirements needed for the age. For children between 0 and 6 months, 210 mg of calcium a day is needed; for children 7 to 12 months, 270 mg; and for children 1 to 3 years, 500 mg.
References
- National Digestive Diseases Information Clearinghouse: What I need to know about Hirschsprung's Disease
- MedlinePlus: Hirschsprung's Disease
- Lucille Packard Children's Hospital at Stanford: Hirschsprung's Disease
- Cincinnati Children's Colorectal Center: Nutrition for Patients with Hirschsprung's Disease
