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Hirschsprung’s disease

What is Hirschsprung’s disease?

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

Normally, the bowel continuously squeezes and relaxes to push poo along, a process controlled by your nervous system.

In Hirschsprung’s disease, the nerves (called ganglion cells) that control this movement are missing from a section at the end of the bowel, which means poo can build up and form a blockage.

This can cause severe constipation and occasionally leads to a serious bowel infection called enterocolitis if it is not found and treated early on.

Illustration diagram of a normal intestines, bowel and colon, and a diagram of Hischsprung's disease enflamed bowel

What are the symptoms?

Hirschsprung’s disease is usually identified shortly after birth. Delay in the passage of meconium (the first sticky, greeny-black poo) is a common symptom. A bloated tummy or being sick may also happen.

Older babies and children with Hirschsprung’s may show symptoms later on, with chronic constipation which is resistant to the usual treatments and is often accompanied by poor weight gain, decreased appetite and a bloated tummy.

How do you test for Hirschsprung’s disease?

The best way to test for Hirschsprung’s disease is a rectal biopsy.

This means taking a sample of cells from the rectum and looking at them under a microscope. We will know it is Hirschsprung’s disease if there are no nerve cells in the sample.

We can also do an initial test for Hirschsprung’s disease by measuring the pressure inside the bottom in older children.

What is enterocolitis?

Enterocolitis is a severe form of bowel infection that is associated with Hirschsprung’s disease. Because of the slow bowel movements, an overgrowth of bacteria happens and causing an infection

This is often treated with antibiotics, fluids in a drip and a rectal washout.

How will you manage my baby with Hirschsprung’s disease?

Some babies can be treated with rectal washouts and introducing feeds gradually. But in some babies, this is not enough, and they need further management.

We will give them a stoma (taking part of the bowel and bringing it to the skin for the temporary passage of poo) until your baby is ready for  surgery called a pull-through operation.

Pull-through surgery

Pull-through surgery is a major operation. We make a cut on your child’s tummy and take samples (biopsies) from the bowel to identify where the good part of the bowel is.

We then remove the diseased part of the bowel where there are no nerve cells and join the healthy parts together This surgery will need general anaesthesia and will be done in the first few months of your baby’s life.

What are the risks of surgery?

Any operation carries a risk of infection or bleeding. We will give your child antibiotics to reduce the chance of infection and they may need a blood transfusion.

There is a risk of a leak where the bowel has been joined back together. If there is a leak this may need a further operation and a temporary stoma.

The bowel can also become twisted, narrowed or blocked and need further surgery.

There is a risk of damaging other things inside the tummy such as the bowel or the ureter which carries wee from the kidney to the bladder.

There is a long term risk of enterocolitis although this is usually smaller than the risk before surgery.

There is a risk of constipation and or incontinence (not being able to control the bowel). In about 1 in 6 children the bowel problems after surgery are severe and a further stoma, like a colostomy, may be needed.

All operations in the tummy also carry a risk of scar tissue forming which can cause a blockage that may need more surgery, although the risk of this is generally low.

What will happen after surgery?

Your child will need to stay in hospital after surgery. They will be given pain-relieving medicine to make them comfortable and fluids into a vein until they can manage food and drink.

No special diet is needed once your child is home, but it is important for them to drink plenty of fluids as they recover. Your child should recover well, and their bowels should function normally after surgery.

At first, your child will probably have a sore bottom when they poo. It can help to:

  • leave their bottom open to the air whenever possible
  • use baby oil to gently clean their bottom
  • use nappy cream after each change

Contact us immediately if your child develops problems such as a swollen belly, a fever or foul-smelling diarrhoea.

Further resources

Hirschsprung’s and motility disorders support network (HMDSN), is an organisation which helps support families of children with Hirschsprung’s disease.

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Please note: this is a generic information sheet relating to care at Sheffield Children’s NHS FT. These details may not reflect treatment at other hospitals. This information is not intended as a substitute for professional medical care. Always follow your healthcare professionals’ instructions. If this resource relates to medicines, please read it alongside the medicine manufacturer’s patient information leaflet. If this information has been translated into another language from English, efforts have been made to maintain accuracy, but there may still be some translation errors. If you are unsure about any of the guidance in this resource or have specific questions about how it relates to your child, always ask your healthcare professional for further advice.

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