Imagine being constipated for two decades. For a 22-year-old Chinese man this was a reality until Shanghai doctors removed about 76cm of his colon. It weighed nearly 13kg and was filled with faeces that had accumulated since birth.
The man, who wishes to remain anonymous, said he’d suffered from constipation for his entire life and laxatives offered only mild relief. Dr Yin Lu from 10th People’s Hospital of Shanghai told The Paper, a Chinese news site, that his stomach looked as though he was more than nine months pregnant.
During the three-hour surgery, Dr Lu discovered that the man had years of faeces built up in his colon. He said it “looked like it could explode at any time”.
So how does this happen? Unfortunately the man's family did not pay any attention to his chronic constipation, so he thought it "was not a big deal". But the man is also believed to have Hirschsprung's disease, a condition that affects one in every 5 000 live births.
(Don't ignore constipation – read more about when it becomes an emergency here.)
About Hirschsprung disease
According to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) “Hirschsprung disease is a birth defect in which nerve cells are missing at the end of a child’s bowel.”
In a healthy digestive tract nerve cells control how the bowel works by letting it know when to move stool. But, when these nerve cells are missing, in the case of Hirschsprung's disease, the bowel does not work as it should.
Instead, the stool moves through the bowel until it reaches the area without nerve cells, where it either slows down or stops entirely, which can result in a blockage or constipation.
The NIDDK says the rectum and sigmoid colon are areas that are most often missing nerve cells. Some people, however, might have missing nerve cells in whole colon or part of the small intestine.
Causes of Hirschsprung's disease
According to Stanford Children’s Health, a foetus develops nerve cells in the digestive tract between the fourth and 12th week of pregnancy. But, for unknown reasons, “nerve cells do not grow past a certain point in the intestine in babies with Hirschsprung's disease.”
Males are five times more likely than females to develop Hirschsprung's disease. The NIDDK adds that children with Down’s syndrome and other medical conditions, for example congenital heart defects, are at a greater risk of developing Hirschsprung's disease.
The condition is congenital (i.e, present at birth), but symptoms are not always obvious when a child is born.
Signs and symptoms
Although symptoms may vary, how they vary will not depend on how much of the digestive tract is missing nerve cells. The NIKKD says that no matter where the nerve cells are missing, once faeces reach this section, a blockage forms and symptoms develop.
These symptoms include:
- constipation or intestinal obstruction
- stunted growth
- swollen abdomen
- unexplained fever
An early symptom in newborns is no bowel movement within 48 hours following birth. Other symptoms in newborns include:
- green or brown vomit
- explosive stools following a rectal examination
- swollen abdomen
- diarrhoea, often bloody
Once Hirschsprung's disease is diagnosed it needs to be corrected with surgery. It can be fatal if left untreated.
A common procedure is the "pull-through" operation. The NHS explains that the affected area of the bowel is removed and the healthy sections are joined together. This operation is usually performed when babies are about three months old.
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