Everything you Need to Know about Hirschsprung’s Disease

Everything you Need to Know about Hirschsprung’s Disease

Aziz Q Shaikh

Hirschsprung’s disease is a congenital intestinal disorder. This mostly affects males 3 to 4 times more than females. It is present in approximately 1 out of 5,000 live births in which the nerve cells present in the colon do not develop well. In fact, 15-20% of all intestinal obstructions are due to Hirschsprung’s disease. Children born with it do not have the ganglion cell in the rectum and other parts. The disease and its symptoms can be treated with pediatric surgery.

What exactly is Hirschsprung’s Disease

Also known as congenital aganglionic megacolon; the disease prevents proper bowel movement in the infant. It occurs when the nerves in the intestines are not well-developed before birth. The condition is usually not identified before the first few months.

In normal children, the enteric nerves promote muscle contractions that help the gas and stool to move out of the intestine. The absence of such nerves can lead to blockage and cause severe constipation.

Symptoms of Hirschsprung’s Disease

Each child experience different symptoms. But the common ones in infants are the following:

·        Swelling or pain in the abdominal region

·        Frequent bouts of fever

·        Poor eating behaviour and appetite loss leading to malnutrition

·        No discharging of bowels within the first 2 days of birth

·        Gradual vomiting of green or brown substances

·        Constipation and inability to defecate

·        Stomach bloating

·        Improper growth accompanied with fatigue

This is not one of the common birth defects, and so parents and care-givers must be mindful of these symptoms. Children are also more prone to enterocolitis; a serious intestinal infection, with this condition.

Often pediatric surgery can save the child from long term health complications.

Causes of Hirschsprung’s Disease

Certain genetic conditions like Down syndrome or similar birth abnormalities are associated with Hirschsprung’s disease. It can also be inherited if the parents have a family history or one of the siblings have been affected. Additionally, for unknown reasons, a male child has more chances of being born with this condition.

Treatment of Hirschsprung’s disease

It is usually cured by two kinds of effective surgeries. Get in touch with the best children’s hospitals for the following:

Pull-Through Procedure

The pediatric surgeon removes the diseased lining of the large intestine. Then, the healthy portion is pulled out of the colon and attached to the anus. This is the most non-invasive technique and can be performed with laparoscopic method.

Ostomy Surgery

The abnormal colon portion is removed and the top part is connected to an opening in the abdomen. This helps the stool pass out of the body without the help of the anus. This opening is called the stoma. Once the colon heals, it is closed and the healthy part of the intestine is connected to the rectum.

Doctors perform abdominal X-rays, biopsy or anal manometry to diagnose the conditions. There are fewer chances of surgical problems. After the surgery, some children might experience problems like constipation, diarrhoea and lack of control over bowel movements. But, with proper care, these symptoms usually resolve. Hirschsprung’s disease is completely curable, if diagnosed on time.

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