WHAT IS IT?

Hirschsprung disease is a congenital disease in which the nerves that control bowel movement are missing. This means that faeces can build up and form a blockage. This leads to chronic constipation and can cause enterocolitis if not treated soon. The most common form of treatment is surgery.

 

CAUSES

It is not yet completely understood why this disease occurs, but there is thought to be a genetic component associated with it. The ganglion (nerve) cells do not develop in the end section of the baby’s bowel when it is growing in the womb. This means the bowel muscles cannot be controlled normally.

 

SYMPTOMS

The symptoms are usually noticeable as soon as a baby is born, and they are:

• In inability to pass meconium within 48 hours

• A swollen belly

• Vomiting bile

 

If these symptoms are missed then it may not be picked up until the child gets older, in which case the symptoms:

• A swollen belly

• Constipation that does not improve with normal treatments

• Lack of hunger or gaining the appropriate about of weight

 

TREATMENT

In most cases the unhealthy section of bowel is removed in a ‘pull through’ operation and the remaining healthy sections of bowel are joined together. This begins by the surgeon performing a colostomy operation on the patient to divert the bowel while it heals. Once this has healed it can usually then be reversed by closing the stoma and joining the healthy sections of bowels together.

 

DISEASE OUTLOOK

After the operations are complete usually a child will have a normal functioning bowel.

 

EPIDEMIOLOGY

It’s a rare condition that mainly affects babies and young children as it is present at birth. It only affects one in every 5000 babies.