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Unilateral retinocephalic vascular malformation syndrome (Wyburn-Mason or BonnetDechaume-Blanc) – Case report and literature review Wyburn-Mason syndrome or Bonnet-Blanc-Dechaume synd rome represents a rare congenital neurocutaneous disease, nonhereditary group of phakomatoses characterized by vascular abnormalities affecting the ipsilateral face, the vision’s pathways and brain, was not mandatory the coexistence of three locations to con)rm the diagnosis. It is believed t hat the disorder is attributed to a defect in development of primitive vascular mesoderm. In 1937 and 1943, cases reported of patients with unilateral vascular disease affecting the eye, brain and face, with such wealth of detail, such as syndrome started to receive the name of those authors in the literature. The name of unilateral retinocephalic vascular malformation syndrome (MVR) was primarily used by in 1974. The Wyburn-Mason syndrome typically presents after the third decade of life and manifests itself in many ways. Ocular manifestations are the most common, while the facial skin manifestations cannot be found. In this paper, we present a rare case of unilateral retinocephalic vascular malformation syndrome in an adult woman, who underwent surgical resection of brain injuries and retinal supratentorial ipsilateral lesion at different times, with another infratentorial lesion, asymptomatic and underwent conservative treatment for a while. The pathology of the lesions con)rmed cavernous hemangio ma.

arquivos brasileiros de neurocirurgia

Síndrome de malformação vascular retinocefálica congênita unilateral (síndrome de Wyburn-Mason ou síndrome de Bonnet-Dechaume-Blanc) – Relato de caso raro e revisão de literatura