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Intestinal Lymphangiectasia

(Idiopathic Hypoproteinemia)

by Atenodoro R. Ruiz, Jr., MD

Intestinal lymphangiectasia is a rare disorder in which the lymph vessels supplying the lining of the small intestine are blocked.

  • This disorder is the result of improperly formed intestinal lymph vessels or blockage of lymph flow from the intestines.

  • Diarrhea and swelling of the legs are the main symptoms.

  • 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 (see Overview of the Lymphatic System) from the digestive tract carry digested fats that were absorbed by the small intestine. Sometimes, children are born with intestinal wall lymph vessels that are improperly formed, which causes blockage of the flow of lymph fluid. These children are typically diagnosed before 3 years of age. Less commonly, the flow of lymph fluid from the digestive tract becomes blocked later in life as a result of such conditions as inflammation of the pancreas (pancreatitis), tumors, or stiffening of the sac that envelops the heart (constrictive pericarditis). Blockage of the lymph flow causes lymph fluid to leak back into the intestine, preventing fat and proteins from being absorbed.

Symptoms of Intestinal Lymphangiectasia

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.

Diagnosis of Intestinal Lymphangiectasia

  • Biopsy

  • Sometimes contrast lymphangiography

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 (called contrast lymphangiography). 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 of Intestinal Lymphangiectasia

  • Treatment of cause

  • Supplements and changes in diet

  • Sometimes surgery

When intestinal lymphangiectasia is caused by a specific condition, that 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.

Sometimes surgery on the intestines or the blocked lymph vessels can help.