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- W2079120004 abstract "A doença de Von Recklinghausen é uma facomatose de transmissão autossómica dominante. Apesar da heterogeneidade da expressão clínica, os estigmas cutâneos clássicos como manchas café au lait são frequentes. A arteriopatia é incomum, sendo a degenerescência aneurismática extremamente rara. Os autores apresentam o caso clínico de um homem de 63 anos com doença de Von Recklinghausen e degenerescência multianeurismática aorto‐ilíaca, femoral e poplítea, submetido com sucesso a tratamento cirúrgico convencional. A degenerescência aneurismática na doença de Von Recklinghausen encontra‐se associada a displasia muscular lisa e a invasão neurofibromatosa da parede vascular. Esta fragilidade arterial implica dissecção laboriosa e técnica cirúrgica meticulosa para limitar a hemorragia e a deiscência anastomótica. The Von Recklinghausen's disease is a facomatosis with autosomal dominant transmission. Despite the heterogeneity of clinical expression, the classic cutaneous stigmata such as café au lait spots are common. The arteriopathy is unusual, with rare aneurysmal degeneration. The authors disclose the clinical case of a 63 years old man with Von Recklinghausen's disease and multi‐aneurysmal degeneration of the aorto‐iliac, femoral and popliteal sectors, who successfully underwent open surgery. The aneurysmal degeneration in Von Recklinghausen's disease is associated with smooth muscle dysplasia and neurofibromatosis invasion of the vascular wall. This arterial fragility implies laborious and meticulous surgical dissection to limit the bleeding and anastomotic dehiscence." @default.
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- W2079120004 date "2014-12-01" @default.
- W2079120004 modified "2023-09-27" @default.
- W2079120004 title "Degenerescência aneurismática na doença de Von Recklinghausen" @default.
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- W2079120004 doi "https://doi.org/10.1016/j.ancv.2014.10.006" @default.
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