Intestinal lymphangiomas are hamartomatous polypoid formations that mostly spread deep into the intestinal wall.
What it is?
There are three forms of lymphangiomatous intestinal lesions:
- lyfangiomas;
- primary intestinal lymphangiectasia;
- lymphatic cysts.
Lyphangiomas are extremely rare and are localized primarily in the duodenum .
Primary intestinal lymphangiectasia (Waldmann's disease) is an anomaly in the development of lymphatic vessels in the form of multiple cavernous formations. The latter are located predominantly in the submucosa. Lymphangiomas contain thick lymph with a high content of lymphocytes.
Lymphatic cysts are multiple cysts up to 1 cm in diameter in the submucosa of the intestinal wall or in the mesentery.
Unlike lymphangiectasias, they do not contain lymphocytes and are not accompanied by loss of protein in the intestinal lumen.
Symptoms
The disease appears in early childhood.
The main symptom is swelling. With obstructive changes in the lymphatic vessels, edema can be asymmetrical, and in severe cases, ascites (fluid accumulation in the abdominal cavity) and hydrothorax (fluid accumulation in the lungs), flesh to anasarca (large-scale swelling of the subcutaneous tissue and soft tissues) occurs.
Due to protein deficiency, children are delayed in development and growth, adults develop atrophy of the muscular system, and the distal phalanges of the fingers take on the appearance of drumsticks. Abdominal pain is rare. Cramps often occur due to a lack of calcium. The latter, combining with fats in the intestinal lumen, is not absorbed. 80% of patients have chronic diarrhea with steatorrhea .
Diagnostics
The diagnosis is established on the basis of x-ray and endoscopic examination of the intestine with histological examination of the mucous membrane.
With primary lymphangiectasia and lymphangiomas, the intestinal mucosa is swollen, its folds are sharply thickened, the intestinal wall may be rigid, the mucous membrane is loose, motley in appearance due to whitish spots 1-2 mm in diameter.
During histological examination, the villi of the mucous membrane of the small intestine are expanded due to impaired outflow of lymph and edema.
For more accurate topical diagnosis of the affected intestinal segment, radionuclide, electrophoretic and immunological studies of intestinal juice obtained from different parts of the small intestine are used.
Differential diagnosis is carried out with diseases in which intestinal lymph circulation is disrupted.
These include
retroperitoneal fibrosis,
fistula between the thoracic duct and the small intestine,
tumors with metastases,
adhesive pericarditis,
right ventricular failure,
thrombosis of the superior vena cava.
Treatment
Treatment is mainly symptomatic. To eliminate protein deficiency (hypoproteinemia), long-term intravenous infusion of protein-containing solutions, primarily albumin and γ-globulin, is used. Treatment is carried out in courses 2-3 times a year for 1 month. All patients are prescribed calcium and iron supplements.
The diet should contain an increased amount of protein (up to 130g/day). Long-chain refractory fats are replaced whenever possible with easily digestible medium-chain triglycerides.
In patients with primary lymphangiectasia, in cases where conservative treatment is ineffective, resection of the most affected small bowel loops can be discussed.
Forecast
With primary intestinal lymphangiectasia, the prognosis is unfavorable: children usually die before reaching adolescence.
With lymphangiomatosis and lymphocysts in the case of segmental lesions of the small intestine, resection of the affected segment can lead to improvement and even recovery.
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