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Protein-losing gastroenteropathy is a well-recognized entity in systemic sclerosis, for which several mechanisms have been postulated. Acquired intestinal lymphangiectasia as a cause of increased intestinal protein loss has not previously been described in the literature. We report the first case of acquired intestinal lymphangiectasia in systemic sclerosis.
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Abnormal size and/or number of the pulmonary and bronchial vessels and lymphatics may mimic pulmonary parenchymal lesions (pseudofibrosis). Abnormalities of the central pulmonary artery, pulmonary vein, thoracic aorta, and brachiocephalic branches may mimic intrathoracic mass lesions (pseudomass lesions). Radiologists should interpret these radiographic findings in the light of other accompanying radiographic findings and the clinical presentation.
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Intestinal lymphangiectasia with lipogranulomatous lymphangitis was diagnosed at necropsy in a 6.5-year-old Maltese dog that had a history of bouts of vomiting, abdominal distention, and diarrhea. The condition was attributed to trauma to the pleural and peritoneal cavities received from bite wounds inflicted one year previously.
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The dietary management of a child with intestinal lymphangiectasia complicated by short gut syndrome following surgery at the age of 3 weeks is described. Diets containing amino acids and peptides were not tolerated due to high osmolality. Satisfactory nutrition was achieved by using a feed with low osmolality and containing whole whey protein, maltodextrin, medium-chain triglycerides (MCT) and a small amount of long-chain triglycerides (LCT) to supply essential fatty acids (RD231).
A unifying concept linking disorders of lymphatic dysplasia, hyperplasia and neoplasia is presented. The central role of disturbed lymphangiogenesis is illustrated in a wide variety of lymphologic syndromes characterized by lymphedema, lymphangiectasia, lymphangioma, and lymphangiosarcoma.
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The case of a 60 year old man in whom diarrhea, steatorrhea and protein losing enteropathy developed one year after the diagnosis of Waldenström's macroglobulinemia is reported. Histological study of multilevel peroral biopsies of the small bowel mucosa showed the following features: a) lymphangiectasia, b) intralymphatic and interstitial deposits composed of lipids and immunoglobulin M of the kappa type (as demonstrated by specific histoimmunofluorescence), c) a mild lymphoplasmocytoid infiltrate, corresponding to the location of Waldenström's macroglobulinemia. The patient died six months later, in spite of improvement of his digestive tract functional abnormalities by a low-fat diet. Sixteen cases of small intestinal involvement have been reported during the course of Waldenström's macroglobulinemia, of which only one showed features similar to those found in our case. Lymphangiectasia appear to be due to mesenteric lymph node involvement by the tumoral process, and/or to increased blood viscosity; in turn the lymphangiectasia seem to be responsible for steatorrhea and plasma protein losses, since in our case both lipids and the immunoglobulin M-kappa were shown to cross the villous epithelium by immunohistochemistry.
We have evaluated the immunological abnormalities present in a 6 year old patient with primary intestinal and generalized lymphangiectasia confirmed by intestinal, lung and lymph node biopsies. Lymphocyte loss through the gut was confirmed by the detection of lymphocytes in her stool. An increased enteric protein loss was suggested by hypoproteinaemia, peripheral oedema, and a very short half-life for i.v. immune serum globulin (3 days). Lymphocyte subpopulation analysis revealed a selective loss of T lymphocytes, with a proportionally increased loss of the OKT4 positive helper/inducer subpopulation. Functionally, there was a decrease in proliferative responses to some mitogens and to allogeneic cells, and a lack of T cell help for in vitro B lymphocyte differentiation into immunoglobulin secreting cells. Natural killer function was normal. In this patient, a concomitant thymic deficiency was documented by failure to identify thymic tissue on a thymus biopsy and by an absence or decrease of the serum thymic factor (thymulin) and thymosin alpha 1. No compensatory lymphopoiesis was detected in the bone marrow. In an attempt to increase T lymphocyte development, the patient was treated with thymosin fraction 5. Daily treatment with this preparation resulted in a transient clinical improvement which could not be sustained on a weekly thymosin treatment schedule. However, lymphocyte numbers did not increase during this treatment. The findings in this patient support the notion that T lymphocytes are needed to stimulate thymic epithelium. In situations of excessive loss of long lived T lymphocytes a secondary thymic atrophy may occur and further contribute to the development of a deficiency in cell-mediated immunity.
The authors report a case of localised intestinal lymphangiectases in a segment of jejunum with the unusual presentation of an acute perforation. The anatomical findings did not provide a satisfactory aetiopathogenic explanation, although the most likely hypothesis is a past history of abdominal irradiation. The mechanism of the perforation also remains unclear and the presence of microscopic lesions in the wall of the remaining small intestine raises the problem of the long-term prognosis.
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We present two patients with biopsy proven primary intestinal lymphangiectasia in whom CT demonstrated diffuse nodular thickening of small bowel without adenopathy or hepatosplenomegaly. One patient had extensive ascites. Although the CT findings are not specific to lymphangiectasia, they may allow one to suggest the diagnosis in the patient with protein losing enteropathy and help distinguish it from inflammatory bowel disease, lymphoma, celiac disease, or Whipple disease.
The article deals with life threatening anomalies oft he lung in newborns which can only be cured by urgent surgical intervention. The clinical symptomatology is characterized in nearly all cases by intrathoracic displacement of healthy lung tissue. Own observations in connection with literature are dealt with.