Aganglionosis
What causes Hirschsprung's disease?
The muscles of the bowel are controlled by nerve cells called ganglion cells. In Hirschsprung's disease, these ganglion cells are missing from a section at the end of bowel, extending up from the anus, the opening in the bottom thatpoo passes through.
For some reason, the cells didn't develop in that area when the baby was growing in the womb. It's not clear why this happens, but it's not thought to be caused by anything the mother did while she was pregnant.
A number of genes are associated withHirschsprung's disease and it does sometimes run in families. If you've had a child with it before, you're more likely to have another child with it.
The conditionis occasionally part of a wider genetic condition, such as Down's syndrome , but most cases aren't.
Medically Reviewed by a doctor on 21 Dec 2018
Introduction
Hirschsprung's disease is a rare bowel condition that mainly affects babies and young children. Read about the symptoms, causes and treatments.
Symptoms of Hirschsprung's disease
Symptoms of Hirschsprung's disease are usually noticeablefrom soon after a baby is born, although occasionallythey're not obvious until a child is a year or two old. Signs of the condition in a baby
When to get medical advice
Visit your GP if your child develops the symptoms described above. Hirschsprung's disease can be serious if left untreated, so it's important to get help as soon as possible. If your GP suspects the
How Hirschsprung's disease is diagnosed
Your child's tummy will usually be examined and sometimes a rectal examination may be carried out.This is where a doctor or nurse inserts a finger into the back passage (rectum) to feel for abnormali
What causes Hirschsprung's disease?
The muscles of the bowel are controlled by nerve cells called ganglion cells. In Hirschsprung's disease, these ganglion cells are missing from a section at the end of bowel, extending up from the anus
Treatments for Hirschsprung's disease
All children with Hirschsprung's disease will need surgery. As they wait for surgery, theymay need to: stop having milk feeds and instead be given fluids directly into a vein havea tube passed thr
Outlook for Hirschsprung's disease
Most children are able to pass stools normally and have a normal functioning bowel after surgery, althoughthey may take a bit longer to toilet train. Some may experience persistent constipation and n
Information about your child
If your child has been affected byHirschsprung's disease, your clinical team will pass information about him or her on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDR
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