Cases reported "Lymphangiectasis"

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1/7. An imaging evaluation of angiodysplasia syndromes.

    Current imaging techniques such as magnetic resonance, magnetic resonance angiography, computer tomography, ultrasound, plain x-rays, and lymphangioscintigraphy have enhanced the ability to define blood and lymph vascular malformations in more precise pathophysiologic terms. Not only can these imaging modalities distinguish arterial anomalies from lymphatic and venous angiodysplasia, but they also readily differentiate edema in the epifascial as opposed to the subfascial peripheral compartments. Moreover, visceral lymphangiectasia (e.g., chylous and non-chylous reflux), bone and muscle overgrowth, agenesis, and fat deposits can also be delineated. Clinical examples are provided including an algorithm for approaching these conditions.
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keywords = bone
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2/7. 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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keywords = bone
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3/7. Congenital lymphangiectatic elephantiasis.

    A case of a child with a rare giant congenital cavernous lymphangioma giving rise to elephantiasis of the left lower extremity is presented. Plain radiographs revealed underlying bone lesions and magnetic resonance (MR) imaging accurately defined extension of the process into the pelvis and retroperitoneum.
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keywords = bone
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4/7. Lymph-vessel embolism in a case of Whipple's disease.

    A case of Whipple's disease is described where the lymphatics in the regional lymph nodes appear to be obstructed by embolized macrophages, containing the characteristic PAS positive bacillary material. It is suggested that the regional lymphangiectasia in Whipple's disease may in part result from such cellular embolism.
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keywords = macrophage
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5/7. 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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keywords = bone
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6/7. Response of lymphangiectasis to radiotherapy.

    A 14-year-old girl with lymphangiectasis of the skull causing rapid extensive destruction of the left orbit, zygoma, mandible, sphenoid, and occiput underwent radiotherapy with 2000 rad to the entire skull, mandible, and upper cervical vertebrae. Three years later, progression of the disease has ceased, and the involved bone is slowly remineralizing.
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keywords = bone
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7/7. Rapidly fatal lymphangioma-like Kaposi's sarcoma.

    A 34-year-old black South African male was treated for lymphangioma-like clinical Kaposi's sarcoma. The disease followed a rapidly fatal course and was characterized by unexplained recurrent pleural effusions and severe refractory anaemia due to the presence of dilated vascular channels in the bone marrow. Definitive diagnosis was only made at autopsy. Lymphangiomatous changes in the skin without spindle cell formation caused diagnostic difficulties.
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keywords = bone
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