Cases reported "Lymphangiectasis"

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1/4. Generalized lymphangiomatosis and chylothorax in the pediatric age group.

    Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly palliative. Three patients died within 1/2 to three years of presentation.
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ranking = 1
keywords = spleen
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2/4. Cystic lymphangioma of the spleen.

    Splenic cysts are uncommon, with fewer than 600 cases reported in the world literature. This report concerns a case of cystic lymphangioma of the spleen, consisting of a large splenic cyst with associated multiple, small, subcapsular cysts and lymphangiectasia. The cysts are lined by endothelium, and they conform to the characteristics of dilated lymphatic channels. lymphangioma of the spleen is thought to arise from congenital obstruction of lymphatic flow, with subsequent dilatation of lymphatic channels. The diagnosis and treatment of splenic cysts are discussed and a new classification of splenic cysts is proposed.
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ranking = 6
keywords = spleen
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3/4. Dysplasia of the lymphatics with lymphoedema, generalized lymphangiectasis, chylothorax and "pseudo-storage-disease".

    A patient showing an unusual association of various abnormalities of the lymphatic vascular system is reported. These abnormalities became first evident in early childhood and consisted of lymphoedema of the left leg, lymphangiectasis in various organs and occlusion of the thoracic duct at its entrance into the venous angle. Chylous effusions and subcutaneous chyloedema appeared in adolescence. Diagnostic biopsies of spleen, liver and bone-marrow revealed the presence of multiple foamcells, suggesting the diagnoses of lipid storage disease. The patient died from severe honeycomb-lungs at the age of 20. This case cannot be attributed to one of the wellknown disease entities of the lymphatic system. The "pseudo"-storage disease is regarded as secondary to the backflow of chylus into the tissues.
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ranking = 1
keywords = spleen
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4/4. Lymphangiomatous hamartoma of the spleen.

    A 72 year old female was admitted for an operation of gastric cancer. At the operation, a 3 x 3 cm-sized, whitish and multilocular nodule was incidently found at the left side of the spleen. This nodule was adjoining the splenic capsule and was characterized with sparsely found multilocular lymphatic cysts with varying sizes. The walls of these cysts were composed of partly thin fibrous tissue, or partly thin to thick splenic trabeculae. Abnormally elongated, nodular or fragmentary trabeculae were also present in the nodule. White and red splenic pulp was found widely dispersed among the cysts. The surrounding splenic tissue was not compressed. We considered the essence of this tumor to be an abnormal mixture of normal splenic elements such as lymphatics, splenic trabeculae, and red and white pulp, and regarded this tumor as a focal malformation (lymphangiomatous hamartoma) rather than a true neoplasm such as a lymphangioma.
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ranking = 5
keywords = spleen
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