RESUMO
Epidermal nevus syndrome is a neurocutaneous disorder characterized by the association of epidermal nevi with central nervous system or skeletal abnormalities. Central nervous system abnormalities include hemimegalencephaly, hydrocephalus, various migration disorders, intraspinal lipomas, and enlarged spinal roots. Rarely, vascular anomalies cause neurologic signs. We report on a 30-month-old girl with epidermal nevus syndrome who also exhibited vascular malformations of the azygos anterior cerebral artery, a cortical aneurysm, and right internal carotid artery hypoplasia. This rare vascular abnormality was not previously reported in patients with epidermal nevus syndrome.
Assuntos
Anormalidades Múltiplas/patologia , Artéria Cerebral Anterior/anormalidades , Malformações Vasculares do Sistema Nervoso Central/patologia , Nevo Pigmentado/patologia , Neoplasias Cutâneas/patologia , Anormalidades Múltiplas/diagnóstico por imagem , Pré-Escolar , Feminino , Humanos , Síndrome , Tomografia Computadorizada por Raios X/métodosRESUMO
Distal vaginal agenesis is one of the rarest congenital malformations of female genital tract. We describe successfully operated teenaged monozygotic twins by combined abdominal and perineal approach. The imaging studies and surgical experience were presented.
