Hirschsprung’s disease is a disease involving mainly the large intestine. It affects the nerve cells (Ganglion cells) in the large intestine and sometimes also the small intestine. These nerve cells control the muscles that move (Peristalsis) food and waste, or stool, through the large intestine and finally stools are excreted through the anus. During development, a baby’s nerve cells form along the intestines. They begin in the mouth and end in the anus. In babies with Hirschsprung’s disease, the nerve cells don’t grow past a certain part of the large intestine.And so the muscles of that part can’t move food and stool and become partly or fully blocked.The intestine begins to grow larger than normal. This then leads to constipation, abdominal distension, and infection.
Boys are more likely to have Hirschsprung’s disease than girls.
Most babies with Hirschsprung’s disease have symptoms in the first few weeks of life. In some cases only a short part of the intestine may be affected. Each child’s symptoms may vary depending on the length of the involved intestinal segment. Symptoms in new-borns may include:
Not having a bowel movement in the first 48 hours of life
Slow distension of the abdomen
Vomiting green or brown fluid
Older Children who don’t show early symptoms may also have:
Constipation that gets worse over time
Loss of appetite
Slow or delayed growth
Small, watery stools
Some tests are done to find out if child has Hirschsprung’s disease. These tests include:
Abdominal X-ray. This test may show a lack of stool in the large intestine or near the anus. It can also show if part of the large intestine is distended..
Barium enema (Dye Test). This X-ray examination checks the large intestine for any problems. The child is given a contrast called barium.. The barium is put into a tube and inserted into your child’s rectum as an enema. An X-ray of the abdomen will show any narrowed areas or any blockages.
Biopsy of the rectum or large intestine. A tiny piece of the Rectum/large intestine is removed and checked under a microscope to see if any nerve cells are missing.This is the most diagnostic test for Hirschsprung’s disease.
The treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.
Hirschsprung’s disease is treated with surgery called as pull-through procedure which can be performed by open as well as laparoscopic technique. A surgeon removes the part of the large intestine that lacks nerve cells. Healthy part that is left is connected to the anal opening.
It can be done as a single stage procedure although some cases surgery may require two stage procedure. A child who is very sick because of Hirschsprung’s disease may first need ostomy surgery (Stoma – opening of the intestine in the abdomen). This can help the child’s intestine to heal before the pull-through surgery.
The common long-term problems of pull through procedure include bowel control and leaking stool, constipation, and infections.