ABOUT THE DISEASE

Hirschsprung`s disease is a congenital / birth defect of the large intestine (bowel). In a normal intestine, nerve cells called ganglion cells enable the intestinal muscles to push stools to the anus from where it leaves the body. In a child affected with this disease these cells are missing. As a result, stool is not able to move properly through the intestine and severe constipation and even bacterial infection can develop. Sometimes the ganglion cells are missing from just a small part of the large intestine near the anus. Other times, the cells may be missing from a large part of the large intestine.

If HD is not treated, stool can fill up the large intestine, causing serious problems such as infection, bursting of the colon, and even death.

SYMPTOMS

The first and the most obvious symptom is the absence of the first bowel movement, called the meconium, in the first 24 to 48 hours after birth. Other associated symptoms could be:

• • Constipation or gas
• • Poor appetite
• • Vomiting bile (a green liquid) after eating
• • Diarrhea
• • Fever / Infection
• • Swollen belly
• • Lack of weight gain

CAUSES

It is unclear why the nerve cells stop growing, and there is no evidence that it is caused by any of the mother’s actions or activities while she is pregnant. HD occurs more often in boys than in girls. HD occurs in 1 out of every 5,000 births. It is sometimes associated with genetic disorders such as Down`s syndrome.

DIAGNOSIS

- Physical examination
- Abdominal X-ray
- Rectal exam
- Barium enema X-ray
- Manometry
- Rectal Biopsy

TREATMENT METHODS

A surgery is the only option for treatment (Hirschsprung disease surgery), where the abnormal section of rectum and colon are removed and the healthy part is pulled down and attached to anus.

Before surgery, a procedure called serial rectal irrigation helps relieve pressure in (decompress) the bowel.

In some cases, it may be done in stages. First a colostomy is done. In this procedure, the diseased portion of intestine is removed and a small hole (stoma) is created in the abdomen. The normal intestine is then connected to it allowing stool to leave the body while the bottom portion heals. This is followed by the pull through surgery at a later date.

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