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Eur Rev Med Pharmacol Sci ; 20(3): 426-32, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26914115

RESUMO

Gorham-Stout disease or the so-called vanishing bone syndrome is a rare disorder characterized by intra-osseous proliferation of vascular channels resulting in destruction and resorption of the osseous matrix. The exact pathology of this disease showed no evidence of malignant, neuropathic, or infectious components involved in the causation of this disorder except for the culprit of lympho-vascular malformations in the bone. The mechanism of bone resorption is yet to be clarified. The clinical presentation of Gorham's disease varies according to the organ of involvement. Patients diagnosed with Gorham's disease in the bone may initially present with insidious onset of dull aching pain, progressive weakness, or pathologic fractures as the initial presentation. Gorham's disease is progressive in most patients; yet it can be self-limiting in a few reported cases. The axes of treating this disease as reported in the literature include the use of medical treatment, surgical intervention, radiotherapy and/or the combination of any them. However, there is no consensus about the most effective approach for treating this rare disease. The challenge in this disease lies in both: how to diagnose and how to treat. Our novel approach combined surgical intervention, medication and radiotherapy as a treatment of Graham-Stout disease in the humerus, and showed no progression of the disease our case.


Assuntos
Conservadores da Densidade Óssea/uso terapêutico , Fraturas do Úmero/terapia , Osteólise Essencial/terapia , Adolescente , Alendronato/uso terapêutico , Terapia Combinada , Fixação Interna de Fraturas , Fraturas Espontâneas/etiologia , Fraturas Espontâneas/cirurgia , Humanos , Fraturas do Úmero/cirurgia , Úmero/diagnóstico por imagem , Úmero/patologia , Úmero/cirurgia , Masculino , Osteólise Essencial/diagnóstico , Radiografia , Radioterapia Adjuvante , Síndrome , Resultado do Tratamento
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