Growth Charts
Possible Newborn Health Problems
Heart Defects
Gastro-intestinal Tract Disorders
Duodenal Atresia
Hirschsprung's Disease
Imperforate Anus
Tracheo-oesophageal Fistula
Congenital Cataracts
Unusual Blood Results

Hirschsprung's Disease

Hirschsprung’s disease occurs when some of the large intestine does not have the usual nerve supply. The medical term for this is aganglionic.

The part of the intestine without a nerve supply cannot relax. It remains collapsed, shut tight and is unable to squeeze, so faeces cannot be passed.

Eventually the pressure of faeces is so high that some are forced through the obstruction.

Hirschsprung’s disease occurs in the last part of the intestine (often the rectum and sigmoid colon) and always ends at the anus. In some children, the length of intestine involved is shorter or longer.

Babies with Hirschsprung's disease may not pass meconium in the first two days of life.

They usually develop abdominal swelling and vomiting in the first few days after birth and may become very unwell with enterocolitis, a severe inflammatory condition of the intestine.

These babies need
• a drip

• a tube into the stomach

• a heart echo

• to be in a paediatric surgery centre

A barium enema x-ray of the large intestine;

• may show a swollen part of the intestine in front of a narrow collapsed part of the intestine

• may look normal if a very short or very long part of bowel is affected

A biopsy a tiny sample of the rectum is pinched away and looked at under a microscope; the baby has Hirschsprung's disease if no nerve cells are seen.

Surgery for Hirschsprung’s disease is needed within the newborn period.

This may be
one "pull-through" operation;
• to remove the aganglionic bowel
•bring the healthy intestine down into the rectum, and join it to the rectal wall

two or three operations;
• to create a temporary colostomy; the colon is brought out to the surface of the abdomen so that faeces can be expelled. The baby is allowed to grow for several months
• a pull-through operation brings the healthy intestine down into the rectum, and joins it to the rectal wall; the rectum is "pulled down"
colostomy may be closed
• to close the colostomy if not closed in second operation

After the operation, the baby will need a few days in the neo-natal intensive care unit, then a few weeks in hospital until feeding is going well and there are no other problems.

Further operations will be in the paediatric department of the hospital.

After surgery the child will continue to have check-ups with the paediatric surgeon to check that the child has no intestinal problems and when older to check the child is gaining good bowel control.