Hirschsprung's disease

Overview

Hirschsprung's disease is a rare condition that causes poo to become stuck in the bowels. It's mainly diagnosed in babies and young children.

Symptoms

Symptoms of Hirschsprung's disease are usually noticeable soon after a baby is born, although sometimes they're not obvious until a child is older. Signs of the condition in a baby include: failing to pass meconium within 48 hours – the dark, tar-like poo that healthy babies pass soon after being born (although some babies later diagnosed with Hirschsprung's disease do pass meconium)a swollen bellyvomiting green or yellow-green fluid (bile) Signs in older infants and children include: a swollen bellyvomiting or a stomach achepersistent constipation that does not get better with the usual treatmentsnot feeding well or not gaining much weight

Causes

The muscles of the bowel are controlled by nerve cells called ganglion cells. In Hirschsprung's disease, ganglion cells are missing from a section at the end of bowel, extending up from the anus (the opening in the bottom that poo passes through). For some reason, the cells did not 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 with Hirschsprung's disease and it does sometimes run in families. If you have had a child with it before, you're more likely to have another child with it. The condition is occasionally part of a wider genetic condition, such asDown's syndrome, but most cases are not.

Treatment

All children with Hirschsprung's disease will need surgery. As they wait for surgery, they may need to: stop having milk feeds and instead be given fluids directly into a veinhave a tube passed through their nose and into their stomach to drain away any fluid and air collecting in ithave regular bowel washouts, where a thin tube is inserted into their bottom and warm salt water is used to soften and flush out the trapped stoolstakeantibioticsif they have enterocolitis Your child may need to stay in hospital during this time, or you may be able to look after them at home. Your doctor will advise you about this.