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It is the job of certain muscles to absorb nutrients and push food through the intestine. You might hear doctors use the word "motility" to describe the ability of these muscles to contract. The process depends on coordination between muscles, nerves, and hormones in the digestive tract. Chronic intestinal pseudo-obstruction (CIP) is a disorder in which motility is compromised, and the contractions are uncoordinated and inefficient. When this happens, digestion is not normal, and the intestine may not absorb enough nutrition.
The term "pseudo-obstruction" refers to a group of gastrointestinal disorders that have similar symptoms but can have a variety of causes. The intestines react as if there is an obstruction or blockage, even though no physical evidence of blockage is found. The symptoms of chronic intestinal pseudo-obstruction are caused by a problem with how the muscles and nerves in the intestines work. When tests show that the dysfunction is caused by unsynchronized contractions, the disorder is classified as neurogenic (arising from the nerves). If the dysfunction is caused by weak or absent contractions, the disorder is classified as myogenic (arising from the muscles).
In some patients, chronic pseudo-obstruction may affect the esophagus, stomach, and even the bladder along with the intestines. Chronic pseudo-obstruction in children is usually congenital, or present at birth. It may also be acquired, such as after an illness.
Symptoms can vary, and range from mild to severe. The most common symptoms of chronic intestinal pseudo-obstruction in children are nausea, vomiting, abdominal distention and pain, and constipation. Diarrhea, a feeling of fullness even after a small snack, food aversion, and weight loss may also be present. These symptoms may be similar to those of other gastrointestinal disorders.
There is no single lab test to diagnose pseudo-obstruction; it is diagnosed based on symptoms, clinical findings, and tests to rule out the presence of a physical obstruction.
The doctor will take a complete medical history, do a physical exam, and take X-rays to see if there is evidence of physical blockage. Over time, chronic intestinal pseudo-obstruction can cause bacterial infections, malnutrition, and muscle problems in other parts of the body. Many children with congenital pseudo-obstruction also have bladder disease.
Further tests can be done to look at the underlying causes of the disorder. Tests may include manometry, which measures patterns and pressure within the gastrointestinal tract. Manometry can help confirm the diagnosis, help measure the extent of the disease, and help determine the proper treatment. Biopsies (tissue samples), which allow the study of both muscles and nerves under a microscope, may be obtained if a surgical procedure is performed.
The main treatment is nutritional support to prevent malnutrition and antibiotics to treat bacterial infections. Disorders that may coexist and worsen symptoms of chronic intestinal pseudo-obstruction – such as gastroparesis (delayed stomach emptying), gastroesophageal reflux, or bacterial overgrowth – need to be identified and treated.
The challenges of treating chronic pseudo-obstruction are often multifaceted and involve the patient and family as well as the physician. The physician may suggest a multidisciplinary approach to treatment. A management team might include the child's pediatric gastroenterologist, a pediatric pain management specialist, a behavioral specialist, and others.
Chronic abdominal pain or the fear of pain is a common complaint in children with chronic intestinal pseudo-obstruction and may be treated with behavioral or relaxation therapy as well as with non-narcotic medicines.
Some children benefit from small, frequent meals. Others are unable or unwilling to eat because of the severity of their symptoms. For those who cannot eat, nutritional support may be provided using predigested liquid diets that are fed through tubes placed into the stomach or intestines (enteral feeding). One method uses a nasogastric tube (NG-tube), which is placed through a nasal passageway into the stomach. Another method uses a gastrostomy (G-tube), in which a liquid diet is fed directly into the stomach through a tube that has been surgically introduced through the abdominal wall. Gastrostomy is not effective when the obstruction occurs in the stomach. In that case, a third method involves feeding through a jejunostomy (J-tube). A jejunostomy feeding tube is surgically placed in the small intestine (jejunum). Besides providing nutrition, both the gastrostomy and jejunostomy can act as an outlet if needed to decrease pressure and pain in the bowel.
Parenteral (i.e., not enteral) nutrition is considered if gastrostomy and jejunostomy prove ineffective. Parenteral nutrition is the slow infusion of a solution of nutrients into a vein through a catheter, which is surgically implanted. This may be partial, to supplement food or nutrients taken by mouth, or total parenteral nutrition (TPN), providing the only source of energy and nutrient intake for the child. Complications associated with long-term use of TPN include infections and liver problems, which can be difficult and life-threatening.
In severe cases, surgery to remove part of the intestines might be necessary. In some patients, when chronic intestinal pseudo-obstruction is limited to an isolated segment of the bowel, surgical bypass may be considered.
In the most severe cases, when patients receiving total parenteral nutrition experience life-threatening complications such as severe infection or liver failure, small bowel transplant may be considered.
One possibility for future treatment that is under investigation is gastric or intestinal "pacing". This process uses high frequency electric stimulation, and works in a way similar to that of a heart pacemaker. Pacing the stomach may be an effective way to reduce nausea and vomiting associated with CIP. However, much more research is needed before it is known whether pacing will be a good option.
Learn more about Intestinal Transplant Disease States.
Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:
Children’s Hospital of Pittsburgh of UPMC
One Children’s Hospital Way
4401 Penn Ave.
Pittsburgh, PA 15224
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