Intestinal lymphangiectasia
Last reviewed: 23.04.2024
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Intestinal lymphangiectasia is the obstruction or malformation of the intralucosal lymphatic vessels of the small intestine. This is primarily observed in children and at a young age. Symptoms of intestinal lymphangiectasia include malabsorption with growth retardation and edema. The diagnosis is based on a small bowel 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 people (the average age of the first manifestations of the disease is 11 years). Men and women get sick the same way. In the case of acquired malformation, the lesion may be secondary as a consequence of retroperitoneal fibrosis, pericarditis, pancreatitis, neoplastic processes and infiltrative lesions that block lymphatic vessels.
Disturbance of lymphatic drainage leads to an increase in pressure in the lymphatic system and the release of lymph into the lumen of the intestine. The violation of absorption of chylomicrons and lipoproteins leads to malabsorption of fats and protein. 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 moderate or small steatorrhea. There may be a chyleous fluid accumulation in the pleural cavity (chylothorax) and chylous ascites. There is a delay in growth in the case of the development of the disease during the first 10 years of life.
Diagnosis of intestinal lymphangiectasia
For the diagnosis of "Intestinal lymphangiectasia", an endoscopic biopsy of the small intestine is usually necessary, in which characteristic extensions and ectasies of the lymphatic vessels of the submucosal layer and mucous membrane are revealed. As an alternative, it is possible to perform lymphangiography (the introduction of contrast material into the distal part of the foot), which allows visualizing changes in the lymphatic vessels of the intestine.
Changes in laboratory parameters include lymphocytopenia and low levels of serum albumin, cholesterol, IgA, IgM, IgG, transferrin and ceruloplasmin. Radiocontrast studies with barium can demonstrate thickened, nodular folds of the mucous membrane reminiscent of folded coins. Absorption of D-xylose is normal. The loss of protein in the intestine can be detected using albumin labeled with chromium 51.
What do need to examine?
How to examine?
Treatment of intestinal lymphangiectasia
Changes in lymphatic vessels can not be corrected. Symptomatic treatment of intestinal lymphangiectasia includes a defatted (less than 30 g / day), protein-rich diet, which additionally contains medium chain triglycerides. In addition, Ca and fat-soluble vitamins are prescribed. The resection of part of the intestine or the anastomosing of altered lymphatic vessels with venous trunks may be effective. Pleural effusion should be drained by thoracocentesis.