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Intestinal lymphangiectasia.
Medical expert of the article
Last reviewed: 12.07.2025
Intestinal lymphangiectasia is an obstruction or malformation of the intramucosal lymphatic vessels of the small intestine. It is primarily seen in children and young adults. Symptoms of intestinal lymphangiectasia include malabsorption with growth retardation and edema. Diagnosis is based on small intestinal biopsy. Treatment of intestinal lymphangiectasia is usually symptomatic.
[ What causes intestinal lymphangiectasia?
Malformation of the lymphatic system is a congenital or acquired pathology. Congenital cases are usually observed in children and young adults (the average age of the first manifestations of the disease is 11 years). Men and women are affected equally often. In the case of acquired malformation, the lesion may be secondary as a consequence of retroperitoneal fibrosis, compressive pericarditis, pancreatitis, neoplastic processes and infiltrative lesions that block the lymphatic vessels.
Impaired lymphatic drainage leads to increased pressure in the lymphatic system and lymph discharge into the intestinal lumen. Impaired absorption of chylomicrons and lipoproteins leads to malabsorption of fats and proteins. Since carbohydrates are not absorbed through the lymphatic system, their absorption is not impaired.
Symptoms of intestinal lymphangiectasia
Early symptoms of intestinal lymphangiectasia include marked, often asymmetric, peripheral edema, chronic diarrhea, nausea, vomiting, and abdominal pain. Some patients have mild to moderate steatorrhea. Chylous fluid collection in the pleural space (chylothorax) and chylous ascites may be present. Growth retardation is noted if the disease develops during the first 10 years of life.
Diagnosis of intestinal lymphangiectasia
Diagnosis of intestinal lymphangiectasia usually requires endoscopic biopsy of the small intestine, which reveals characteristic dilations and ectasias of the lymphatic vessels of the submucosa and mucosa. Alternatively, lymphangiography (injection of a contrast agent into the distal part of the foot) can be performed, allowing visualization of changes in the intestinal lymphatic vessels.
Laboratory abnormalities include lymphocytopenia and low levels of serum albumin, cholesterol, IgA, IgM, IgG, transferrin, and ceruloplasmin. Barium contrast studies may show thickened, nodular mucosal folds resembling stacked coins. D-xylose absorption is normal. Intestinal protein losses can be demonstrated using chromium-51-labeled albumin.
What do need to examine?
How to examine?
Treatment of intestinal lymphangiectasia
Changes in the lymphatic vessels cannot be corrected. Symptomatic treatment of intestinal lymphangiectasia includes a low-fat (less than 30 g/day), protein-rich diet, additionally containing medium-chain triglycerides. Ca and fat-soluble vitamins are additionally prescribed. Resection of a part of the intestine or anastomosis of the changed lymphatic vessels with venous trunks may be effective. Pleural effusion should be drained by thoracentesis.