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Hirschsprung’s disease

OVERVIEW

Hirschsprung’s disease is a rare condition that affects the large intestine in children. The disease may be caused by a lack of nerve cells in the colon, which makes it difficult for stool to pass through. This condition is usually diagnosed in newborns or infants. Early diagnosis is important to prevent complications and improve outcomes. Although the disease is rare, it can have serious consequences if left untreated. Therefore, it is important for parents and healthcare providers to be aware of the signs and symptoms of the disease and to seek medical attention promptly if there is a concern.

Symptoms of Hirschsprung’s disease in children

Symptoms of Hirschsprung’s disease in children can include the following:

Failing to pass meconium (the baby's first stool) within 48 hours after birth
Chronic constipation
Abdominal distension (swelling)
Vomiting
Poor appetite
Slow growth
Diarrhoea (in rare cases)

These symptoms may appear shortly after birth or develop later in infancy or childhood. If your child shows any of these symptoms, seeking medical advice from a healthcare professional for accurate diagnosis and treatment is crucial.

Treatment

The first step is to establish a diagnosis, and this is by having a contrast enema study which is done in the X-ray department. The next step is to confirm the suspected diagnosis with a rectal biopsy which is a surgical procedure where the paediatric surgeon takes a biopsy of the wall of the rectum via the anus.

The treatment of Hirschsprung’s disease in children usually involves surgery to remove the affected portion of the colon and create a new connection to the rectum. This surgery is called a pull-through procedure and can be done in one or more stages depending on the severity and length of the affected colon.

In some cases, a temporary colostomy may be needed before the pull-through surgery. A surgical procedure called colostomy involves bringing a portion of the colon to the abdominal surface, forming an opening or stoma for the elimination of stool into a bag that is attached to the skin. This procedure is typically performed when it is not possible to remove the affected portion of the colon in a single surgery due to its size or other complications.

After the surgery, the child may stay in the hospital for a couple of days to a week. They will be monitored closely to ensure that the bowel is functioning properly and that there are no complications. Some children may require a special diet or medications to help manage symptoms and promote healing.

Early diagnosis and treatment are important for the best outcomes in children with Hirschsprung’s disease. With appropriate treatment, most children are able to lead healthy and normal lives.

REQUEST A CONSULTATION

Dr Mangray, the founder of KZN Specialised Paediatric Surgical Service, and his associates, Dr Ngobese, Dr Govender and Dr Dube-Zvirikuzhe, have a well-established reputation for caring for children right until late adolescence.

FREQUENTLY ASKED QUESTIONS

FAQ

1What are the long-term effects of Hirschsprung’s disease?

If left untreated, Hirschsprung’s disease can lead to serious complications such as bowel obstruction, a potential infection, and even death. Even with treatment, some children may experience ongoing bowel problems such as constipation or diarrhoea.

2Can Hirschsprung’s disease recur after treatment?

Recurrence of Hirschsprung’s disease after treatment is rare, but it can occur if a portion of the affected colon is missed during surgery or if there are complications such as scar tissue formation.

3Can Hirschsprung’s disease be inherited?

Hirschsprung’s disease can sometimes run in families, but it is usually not inherited in a simple pattern. Genetic testing and counselling may be recommended for families with a history of the condition.