Hirchsprung’s Disease at a glance

Hirschsprung’s Disease is a condition that effects the large intestine, causing problems during bowel movements.
Hirschsprung’s Disease is when babies or children are unable to empty out their bowels at all. This condition occurs when there is a birth defect where due to missing or incorrectly formed nerve cells in the colon or the intestine, during fetal development. In most cases, problems with the bowel movements start at birth, but if it is a milder case, problems may present themselves in a few months, or sometimes, years time.

During the process of digestion, the food reaches the intestines, at which point the are pushed outside by what is known as peristalsis. Specific cells called ganglions are required for this step, which when notably missing, causes a backup, causing Hirschsprung’s Disease.

Symptoms and Risk Factors

Symptoms of Hirschsprung’s Disease include:

• Prominently, swelling of the abdomen
• Lots of gas
• Blood in the stools
• Vomiting, often in green or brown colour
• Anemia due to shortage of RBCs
• Loss of appetite and delayed growth as a result

This disease is more frequently seen in boys more than girls, almost as often as four times. Some of the other risk factors include having a family member who has a history of the disease, or even a sibling. Children with Down’s Syndrome are also at a higher risk for Hirschsprung’s Disease.

Tests

Diagnosis of Hirschsprung Disease is done based on results of various tests, that include but are not limited to X Rays to determine where the obstruction is in the large intestine, and it is normally done with barium inserted for a better picture.

A manometry may also be done, where a reflexes of the anus is measured. This is mainly done on older children. A balloon is placed in the rectum and inflated to see how they react. Once deflated, if the muscles don’t relax, Hirschsprung’s Disease may be the problem. A biopsy of the affected cells are also a common test that is done, where a small piece of the intestine is removed and checked for missing nerve cells. The doctor may also make the diagnosis following a conversation about the frequency of the child’s bowel movements.

Treatment

In all cases of Hirschsprung’s Disease the definitive treatment is surgery, where the segment of the intestine without the nerve endings is removed a segment which is normal is pulled down to the anus. When it is diagnosed at an early stage where there is no infection, the prognosis is favourable and the procedure itself is minimally invasive and done laparoscopically.

Although it is usually a single procedure, in some cases, doctors may recommend also a colostomy. This is usually when there is a severe infection or there is a severe obstruction in the intestine. It could also be recommended if the person has an enlarged bowel.

Prognosis

The usual outlook for children who have had a surgery as treatment for Hirschsprung’s Disease is rather positive as they go on to live normal lives with no health problems. In some cases some symptoms may continue, which should be looked at by a doctor again.

Why Yashoda?

The Yashoda Hospitals’ Center for Paediatrics has evolved with comprehensive multidisciplinary programmes for the diagnosis and treatment and prevention of conditions and illnesses that affect children. The specialists here work inclusively with other departments to arrive at a treatment plan that is unique to each patient. It is this attention to detail that makes the Yashoda Hospitals’ Center for Paediatrics the best in the city.