Intestinal pseudo-obstruction

Also known as: Enteric neuropathy

Intestinal pseudo-obstruction is a rare condition with symptoms that resemble those caused by a blockage, or obstruction, of the intestines, also called the bowel. However, when a health care provider examines the intestines, no blockage exists. Instead, the symptoms are due to nerve or muscle problems that affect the movement of food, fluid, and air through the intestines.

What causes intestinal pseudo-obstruction?

Problems with nerves, muscles, or interstitial cells of Cajal prevent normal contractions of the intestines and cause problems with the movement of food, fluid, and air through the intestines. Interstitial cells of Cajal are called “pacemaker” cells because they set the pace of intestinal contractions. These cells convey messages from nerves to muscles.

Primary or idiopathic intestinal pseudo-obstruction is intestinal pseudo-obstruction that occurs by itself. In some people with primary intestinal pseudo-obstruction, mutations, or changes, in genes—traits passed from parent to child—cause the condition. However, health care providers do not typically order genetic testing for an intestinal pseudo-obstruction, as they don’t commonly recognize gene mutations as a cause.

Some people have duplications or deletions of genetic material in the FLNA gene. Researchers believe that these genetic changes may impair the function of a protein, causing problems with the nerve cells in the intestines. As a result, the nerves cannot work with the intestinal muscles to produce normal contractions that move food, fluid, and air through the digestive tract. Also, these genetic changes may account for some of the other signs and symptoms that can occur with intestinal pseudo-obstruction, such as bladder symptoms and muscle weakness.

A condition called mitochondrial neurogastrointestinal encephalopathy may also cause primary intestinal pseudo-obstruction. In people with this condition, mitochondria—structures in cells that produce energy—do not function normally. Mitochondrial neurogastrointestinal encephalopathy can also cause other symptoms, such as problems with nerves in the limbs and changes in the brain.

Secondary intestinal pseudo-obstruction develops as a complication of another medical condition. Causes of secondary intestinal pseudo-obstruction include

  • abdominal or pelvic surgery
  • diseases that affect muscles and nerves, such as lupus erythematosus, scleroderma, and Parkinson’s disease
  • infections
  • medications, such as opiates and antidepressants, that affect muscles and nerves
  • radiation to the abdomen
  • certain cancers, including lung cancer

What are the symptoms of intestinal pseudo-obstruction?

Intestinal pseudo-obstruction symptoms may include

  • abdominal swelling or bloating, also called distension
  • abdominal pain
  • nausea
  • vomiting
  • constipation
  • diarrhea

Over time, the condition can cause malnutrition, bacterial overgrowth in the intestines, and weight loss. Malnutrition is a condition that develops when the body does not get the right amount of the vitamins, minerals, and other nutrients it needs to maintain healthy tissues and organ function.

Some people develop problems with their esophagus, stomach, or bladder.

How is intestinal pseudo-obstruction diagnosed?

To diagnose intestinal pseudo-obstruction, a health care provider may suggest the person consult a gastroenterologist—a doctor who specializes in digestive diseases. A health care provider will perform a physical exam; take a complete medical history, imaging studies, and a biopsy; and perform blood tests. A health care provider may order other tests to confirm the diagnosis. The health care provider also will look for the cause of the condition, such as an underlying illness.

Intestinal pseudo-obstruction can be difficult to diagnose, especially primary intestinal pseudo-obstruction. As a result, a correct diagnosis may take a long time.

How is intestinal pseudo-obstruction treated?

A health care provider will treat intestinal pseudo-obstruction with nutritional support, medications, and, in some cases, decompression. Rarely, a person will need surgery. If an illness, a medication, or both cause intestinal pseudo-obstruction, a health care provider will treat the underlying illness, stop the medication, or do both.

Nutritional Support

People with intestinal pseudo-obstruction often need nutritional support to prevent malnutrition and weight loss. Enteral nutrition provides liquid food through a feeding tube inserted through the nose into the stomach or placed directly into the stomach or small intestine. A health care provider inserts the feeding tube, sometimes using x ray or endoscopy for guidance, and teaches the person how to care for the tube after returning home. Enteral nutrition is sufficient for most people with intestinal pseudo-obstruction. In a severe case, a person may need IV feeding, also called parenteral nutrition, which provides liquid food through a tube placed in a vein.

Enteral nutrition is possible because the intestinal lining is normal in most people with intestinal pseudo-obstruction. Enteral nutrition is preferred over parenteral nutrition because it has a much lower risk of complications.

Medications

A health care provider prescribes medications to treat the different symptoms and complications of intestinal pseudo-obstruction, such as

  • antibiotics to treat bacterial infections
  • pain medication, which should be used sparingly, if at all, because most pain medications delay intestinal transit
  • medication to make intestinal muscles contract
  • antinausea medications
  • antidiarrheal medications
  • laxatives

Decompression

A person with acute colonic pseudo-obstruction and a greatly enlarged colon who does not respond to medications may need a procedure, called decompression, to remove gas from the colon. A gastroenterologist can perform the procedure in a hospital or an outpatient center. The gastroenterologist may choose to decompress the colon by using colonoscopy. During colonoscopy, the gastroenterologist inserts a flexible tube into the colon through the anus. A health care provider gives the person a light sedative, and possibly pain medication, to relax. If the person requires long-term decompression, the gastroenterologist also can decompress the colon through a surgical opening in the cecum. In this case, the health care provider gives the person local anesthesia.

Surgery

In severe cases of intestinal pseudo-obstruction, a person may need surgery to remove part of the intestine. However, surgery should be performed rarely, if at all, because intestinal pseudo-obstruction is a generalized disorder that typically affects the entire intestine. Removing part of the intestine cannot cure the disease.

A surgeon will perform the surgery at a hospital; a person will need general anesthesia. A few highly specialized treatment centers offer small intestine transplantation. A health care provider may recommend small intestine transplantation when all other treatments have failed.

Eating, Diet, and Nutrition

Researchers have not found that eating, diet, and nutrition play a role in causing or preventing intestinal pseudo-obstruction. Following special diets usually does not help improve the disorder. However, eating frequent, small meals with pureed foods or liquids may ease digestion. Vitamin and trace mineral supplements may help a person who is malnourished.

Points to Remember

  • Intestinal pseudo-obstruction is a rare condition with symptoms that resemble those caused by a blockage, or obstruction, of the intestines, also called the bowel. However, when a health care provider examines the intestines, no blockage exists. Instead, the symptoms are due to nerve or muscle problems that affect the movement of food, fluid, and air through the intestines.
  • Intestinal pseudo-obstruction symptoms may include abdominal swelling or bloating, also called distension; abdominal pain; nausea; vomiting; constipation; and diarrhea. Over time, the condition can cause malnutrition, bacterial overgrowth in the intestines, and weight loss.
  • To diagnose intestinal pseudo-obstruction, a health care provider may suggest the person consult a gastroenterologist—a doctor who specializes in digestive diseases. A health care provider will perform a physical exam; take a complete medical history, imaging studies, and a biopsy; and perform blood tests. A health care provider may order other tests to confirm the diagnosis.
  • A health care provider will treat intestinal pseudo-obstruction with nutritional support, medications, and, in some cases, decompression. Rarely, a person will need surgery. If an illness, a medication, or both cause intestinal pseudo-obstruction, a health care provider will treat the underlying illness, stop the medication, or do both. A health care provider may recommend small intestine transplantation when all other treatments have failed.