Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis).
نویسندگان
چکیده
Generalised lymphatic anomaly (GLA), also known as lymphangiomatosis, is a rare disease caused by congenital abnormalities of lymphatic development. It usually presents in childhood but can also be diagnosed in adults. GLA encompasses a wide spectrum of clinical manifestations ranging from single-organ involvement to generalised disease. Given the rarity of the disease, most of the information regarding it comes from case reports. To date, no clinical trials concerning treatment are available. This review focuses on thoracic GLA and summarises possible diagnostic and therapeutic approaches.
منابع مشابه
Lymphangiomatosis presenting with bronchial cast formation.
Lymphangiomatosis is a rare disorder of the lymphatic system that is known to cause chylothorax. Chyloptysis may occur but chylous bronchial cast formation is rare. A case is reported of lymphangiomatosis in a 34 year old woman whose initial manifestation was cough productive of bronchial casts. Two years later the patient developed a chylothorax. Ligation of the thoracic duct through a low tho...
متن کاملPulmonary lymphangiomatosis: a report of two cases.
Lymphangiomatosis, a rare diseases of controversial origin, occurs in individuals of any age, regardless of gender, but is predominantly seen in younger individuals. It often presents with thoracic involvement, although, the bones, spleen and liver can also be affected. Histologically, the pulmonary involvement includes proliferation, complex anastomoses and secondary dilatation of the lymphati...
متن کاملGorham-Stout Disease Management during Pregnancy
Gorham-Stout Disease (GSD) is a rare lymphatic disorder affecting children or young adults with no predilection of sex. It is generally associated with vanishing bone osteolytic lesions, thoracic and abdominal involvement, and diffuse pulmonary lymphangiomatosis. Chylous effusions and chylothorax, consequent to the abnormal proliferation of lymphatic vessels, may induce respiratory failure with...
متن کاملDisseminated lymphangiomatosis with skeletal involvement: detection with magnetic resonance lymphangiography.
Disseminated lymphangiomatosis is an uncommon disorder characterized by diffuse or multifocal proliferation of complex, irregular lymphatic channels involving soft tissue, viscera, retroperitoneum, eyes and the skeletal system. Currently, magnetic resonance imaging, computed tomography, ultrasonography, and conventional radiography are the favored radiologic imaging modalities in assessing the ...
متن کاملGeneralized Lymphangiomatosis: A Case Report
Generalized lymphangiomatosis is the term used for diffuse involvement of soft tissue, viscera (especially lungs and spleen) or bone by a lymphangiomatous proliferation. The presentation, as in our case, is often with a chylothorax. It is categorized as a subtype of lymphangiectasia, which is owing to dilatation, and proliferation of lymphatics (1). Lymphangiomatosis generally presents in the f...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- European respiratory review : an official journal of the European Respiratory Society
دوره 25 140 شماره
صفحات -
تاریخ انتشار 2016