Paediatric Surgery

Hirschprung’s Disease

Introduction to Hirschprung’s Disease

Hirschprung’s disease is an intestinal disorder where the baby is unable to pass stools due to an obstruction of their bowels.

Written by Doctify Team 27/04/2020

What is Hirschprung’s Disease?

Hirschprung’s disease is an intestinal disorder where the baby is unable to pass stools due to an obstruction of their bowels. This is because they are unable to systematically contract and relax their intestines as in healthy people, and a part of their bowel is permanently held in the contracted state. This leads to obstructions, most commonly in the last part of the large intestine just before the rectum and the subsequent difficulty in excreting stools.

In most cases this is rapidly detected at birth and treated with surgery immediately upon diagnosis.

What causes Hirschprung’s Disease?

During development the nervous system spreads throughout the growing embryo to supply the relevant organs and tissues in the body. However, due to a genetic defect nerve cells fail to migrate to the intestines. The normal function of these nerve cells is to allow the muscles of the intestine to relax and contract yet these are absent in children suffering from Hirschprung’s Disease. This absence leads to them being unable to relax their intestinal muscles, causing the constant contraction and subsequent serious constipation and intestinal blockage.

Because of this genetic component it is often seen alongside other congenital conditions such as congenital heart defects or chromosomal issues. It tends to be more frequent in boys and you are more likely to be born with it if other members of your family have suffered from it in the past. Many genetic defects have this pattern of pathology.

What are the symptoms of Hirschprung’s Disease?

Babies with Hirschprung’s disease will be unable to pass meconium within the first two days of being born, as healthy babies do. Meconium is the first stool a baby passes and is distinctively greenish and tarry due to what the baby will have ingested in the womb. The baby will also have a swollen belly because of the buildup of faecal matter and may also vomit due to the internal pressure and reflux.

Sometimes the condition isn’t immediately discovered at birth and in older cases the child may find that he/she is quickly full after eating and frequently complains of stomach aches, the course of treatment is similar in both cases.

How is Hirschprung’s Disease treated?

Hirschprung’s Disease is treatable via surgery. After the bowel is washed out with warm saline the malfunctioning section of bowel is surgically removed and the healthy parts then connected to reform the continuous digestive tract. Since the affected portion is most commonly just before the rectum (the portion of the digestive tract that stores faecal matter before using the bathroom) the healthy bowel is usually attached directly to it.

Sometimes the surgery may take place over two stages if the relevant medical professionals deem it more appropriate. In the interval stage the baby may have a colostomy bag to collect stools through an opening in their belly before the surgery is completed safely.

In most cases surgical intervention provides effective treatment, however sometimes the child may experience milder constipation in which case a high fibre diet and laxatives will be recommended. During the immediate recovery period the baby will require painkillers and will need to be fed fluids few a few days.

Find out more about other relative conditions:

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