Intestinal lymphangiectasia (idiopathic hypoproteinemia) is a rare disorder in which the lymph vessels supplying the lining of the small intestine become 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 blockage of the flow of the lymph fluid. Less commonly, these lymph vessels may become blocked 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). In both cases, pressure builds up in the abnormal lymph vessels. The increased pressure causes lymph fluid to leak back into the intestine, preventing fat and proteins from being absorbed into the bloodstream.

Symptoms and Diagnosis

A person with intestinal lymphangiectasia has swelling of one or both legs and diarrhea. Nausea, vomiting, fatty stools, and abdominal pain may also develop. Children have delayed growth.

Doctors usually make the diagnosis by removing tissue (biopsy) from the small intestine using an endoscope (a flexible viewing tube equipped with a light source and a camera through which a small clipper can be inserted). The tissue that is removed is then examined under a microscope. Sometimes doctors inject a dye that can be seen on x-rays into lymph vessels in the foot. The dye travels to the abdomen and chest and can show the abnormal intestinal lymph vessels.

Other blood tests are done to look for complications of the disorder. People may have low levels of protein, cholesterol, and white blood cells in the blood. The low protein levels result in tissue swelling.

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 January 2013 by Atenodoro R. Ruiz, Jr., MD