Intestinal lymphangiectasia (idiopathic hypoproteinemia) is a disorder in which the lymph vessels supplying the lining of the small intestine become enlarged and obstructed.

• This disorder is the result of improperly formed lymph vessels.
• Diarrhea is the main symptom.
• The diagnosis is based on the results of a biopsy.
• Once the specific cause of the disorder is treated, following a low-fat, high-protein diet and taking supplements can help manage symptoms.

The lymph vessels from the digestive tract carry digested fats that were absorbed by the small intestine. Sometimes, these lymph vessels are improperly formed at birth, causing them to be enlarged. Less commonly, these lymph vessels may enlarge later in life as a result of such conditions as inflammation of the pancreas (pancreatitis) or stiffening of the sac that envelops the heart (constrictive pericarditis). The enlarged lymph vessels carry lymphatic fluid poorly, and the fluid leaks back into the intestine, preventing fat and proteins from being absorbed into the bloodstream.

Symptoms and Diagnosis

A person with intestinal lymphangiectasia has diarrhea. Nausea, vomiting, fatty stools, and abdominal pain may also develop. The person may also have swelling (edema) if lymph vessels elsewhere in the body are blocked.

Levels of protein in the blood are low. The low protein levels result in tissue swelling. The number of lymphocytes in the blood is decreased, and cholesterol levels in the blood may be normal or low.

The diagnosis is established by a biopsy of the small intestine showing enlargement of the lymph vessels. Measurement of a certain protein, called alpha₁-antitrypsin, in the stool can indicate the severity of protein loss into the intestines.

Treatment

When intestinal lymphangiectasia is caused by a specific condition, the underlying condition is treated. Symptoms can be helped by eating a low-fat, high-protein diet and taking supplements of calcium, vitamins, and certain triglycerides (medium-chain triglycerides), which are absorbed directly into the blood and not through the lymph vessels.

Last full review/revision December 2007 by Atenodoro R. Ruiz, Jr., MD