What is chronic intestinal pseudo-obstruction?

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About chronic intestinal pseudo-obstruction

This is a disorder in which the patient shows symptoms of bowel obstruction — including abdominal distension, pain, vomiting and chronic constipation — even when there’s no evidence of a blockage.

Causes of chronic intestinal pseudo-obstruction

Patients with this condition have a problem with bowel motility. When the bowel is working normally, the stomach churns up food that is then slowly emptied into the small bowel. The small bowel pushes it down the entire bowel so that the food can be digested and absorbed. This process is regulated by neurons in the intestinal wall. When the nerves or muscles don’t work properly, the food just sits there instead of working its way through the intestinal tract.

Hirshsprung’s disease is a motility disorder that usually affects the last part of the large bowel and can be treated surgically. With pseudo-obstruction, the problem can affect the stomach and small and large bowel, so there’s not always an easy surgical repair.

Chronic intestinal pseudo-obstruction diagnosis

This is a very rare disorder. There are about 200 new cases diagnosed every year, according to the International Foundation for Functional Gastrointestinal Disorders. Children’s Wisconsin treats about 25 to 30 cases a year.

Development of chronic intestinal pseudo-obstruction

Most patients with this condition develop a problem with the nerve or muscle lining of their bowel in utero, though some children develop the condition later in life. In those cases, doctors aren’t always sure what damaged the bowel lining.

Symptoms of chronic intestinal pseudo-obstruction

Symptoms vary but can include:

Developing this condition

There are very rare reports of chronic intestinal pseudo-obstruction running in families, but in most cases doctors don’t yet know the underlying cause or risk factors.

This is a chronic condition that can have a serious impact on a child’s quality of life. These patients often require tube-feeding support in order to stay properly nourished, and many may need surgery to remove the affected part of the intestines. In severe cases, the child may need a bowel or liver transplant. This condition can be fatal.

Evaluation and diagnosis of chronic intestinal pseudo-obstruction

Doctors will evaluate your child using several tests, including an upper GI study,  gastric emptying study, and manometry studies, which measure the pressure inside the gastrointestinal tract.

Treatment for chronic intestinal pseudo-obstruction

Although there are medicines that can improve bowel motility, most only work short-term. Gastrostomy (also known as a G-tube) can be used to feed patients as well as remove secretions from the stomach. Some patients require IV nutrition. In some cases doctors may use ileostomy, which is a surgical opening in the small intestine, to relieve distension.

Chronic intestinal pseudo-obstruction requires ongoing treatment to maintain proper nutrition.

Contacting a physician

Contact your pediatrician if your child is having any symptoms that are persistent or that come and go without explanation, particularly if it’s affecting your child’s nutrition. In most cases this condition is usually diagnosed within the first two months of life. The first step is to rule out any anatomical problem, and that might require a surgical opinion. If there’s no anatomical reason for your child’s symptoms, your next step would be to consult a motility expert.

Long-term outlook for chronic intestinal pseudo-obstruction

This is a chronic condition that has a lifelong effect. While most children with this condition go to school and engage in normal activities, they usually require daily support for nutrition and medication.

Living with chronic intestinal pseudo-obstruction

Children’s staff can train parents to handle many of the treatments, such as tube or IV nutrition, at home. Families will likely need at-home nursing support.

For more information about this condition, visit the International Foundation for Functional Gastrointestinal Disorders.

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