Chronic Intestinal Pseudo-Obstruction

Chronic intestinal pseudo-obstruction (CIP) is a rare disorder in which intestinal nerve or muscle problems prevent food, fluid, and air from moving through the stomach and intestines. The child experiences the symptoms of an intestinal blockage, though no actual physical blockage exists. Over time, children with CIP can become malnourished because their gastrointestinal tracts are unable to absorb food and get nutrition.

About 100 children are born with congenital CIP in the U.S. each year. CIP is a lifelong condition that can be managed with proper medical care.

Chronic intestinal pseudo-obstruction prevents fluids and food from moving though the stomach and intestines. Symptoms vary, depending on which portion of the intestinal tract is affected. Common symptoms include:

• Abdominal distention (a bloated or swollen belly)
• Vomiting
• Constipation
• Abdominal pain
• Diarrhea
• Feeling full after a small snack
• Food aversion
• Pain related to the distention or obstructive symptoms

The lack of intestinal function can lead to complications. It usually prevents the body from absorbing nutrients. As a result, many children suffer from malnutrition, failure to thrive, and weight loss. It is common for doctors to suspect and diagnose this condition soon after birth or before a child’s first birthday in the cases of primary pseudo-obstruction.

Chronic intestinal pseudo-obstruction is caused by nerve or muscle problems that prevent the intestines from contracting normally to move food, fluid, and air through the intestines. In many cases, the root cause is unknown. In children, the condition is typically congenital, meaning that most children who have it were born with it.

Chronic intestinal pseudo-obstruction can be caused by a problem with the nerves or the muscles in the stomach and intestines:

• Neuropathic: when the stomach and intestines contract, but the contractions are unsynchronized due to a problem with the nerves in the gastrointestinal tract
• Myogenic: when the stomach does not contract or the contractions are weak due to a muscular problem

At times the problem with the intestinal movement may be secondary to other underlying conditions like metabolic problems, mitochondrial diseases, and surgery. These problems are considered secondary causes of pseudo-obstruction. They can have the same problems as children with the primary form.

Because the symptoms of chronic intestinal pseudo-obstruction (CIP) are similar to those of other gastrointestinal conditions, your child's doctor may need to order several tests before making a formal diagnosis.

Your child's doctor may order one or more of the following tests and procedures:

• Imaging studies, such as X-rays, can rule out an intestinal obstruction.
• Antroduodenal and colonic manometry measure pressure in the bowel and can help diagnose CIP without more invasive testing. During an antroduodenal manometry test, a doctor feeds a small, flexible tube through the nose or an existing gastrostomy (feeding tube) into the stomach and small intestine to measure pressure. For a colonic manometry, the doctor does the same thing but feeds a tube into the intestine through the colon.
• Laparotomy is a surgical procedure that is rarely necessary. A surgeon makes an abdominal incision so they can examine and biopsy the intestines.

While there is no known cure for pseudo-obstruction, proper medical treatment can stabilize the condition, prevent complications, and improve your child’s quality of life so they can participate in typical childhood activities.

Treatment may include:

• Nutritional support therapy: Proper nutrition is a top priority for children with CIP. Children with CIP should eat several small meals throughout the day of easy-to-digest, pureed foods. Children who cannot eat even small meals may need enteral nutrition, which delivers a liquid diet into the stomach or intestines through a feeding tube. In more severe cases, a child’s doctor may recommend parenteral nutrition, which supplies nutrients directly into the veins through a catheter.
• Surgical decompression: To relieve pressure in the intestines, a surgeon may insert a feeding tube directly into the stomach or create an opening in the abdomen through which the intestines can release gas and empty stool.
• Medications: Prokinetic drugs may improve motility, the ability to move food through the intestines, in some children by aiding contractions. Depending on the child's symptoms, the child's clinician may also prescribe antibiotics, anti-nausea medications, anti-diarrheal medications, or laxatives.
• Pain management: There are several options to control and relieve pain and discomfort associated with CIP, including oral medications and even epidural anesthesia. However, pain medication can also slow digestion and should be used sparingly for children with CIP.
• Small bowel transplantation: In severe cases, the intestines and possibly other organs may need to be replaced with an intestinal and multivisceral transplant.

The Motility and Functional Gastrointestinal Disorders Center at Boston Children’s Hospital provides access to state-of-the-art specialized multi-disciplinary gastroenterology services including the most advanced testing, new therapy, and access to more options for your child’s treatment plan. Our expert team will work together and with your family to develop an individualized treatment plan that addresses the full spectrum of your child’s health needs and provides the best outcomes and quality of life.