Adult Presentation of PHACES Syndrome

作者：Arora S S; Plato B M; Sattenberg R J; Downs R K; Remmel K S; Heidenreich J O^*

来源：Interventional Neuroradiology, 2011, 17(2): 137-146.

• stroke
• cerebrovascular
• congenital
• facial hemangioma
• posterior fossa
• moya-moya

A significant percentage of children with hemangiomas may have PHACES syndrome which refers to the association of posterior fossa malformations, facial hemangiomas, arterial cerebrovascular abnormalities, cardiovascular anomalies, eye abnormalities and ventral defects like sternal clefting or supraumbilical raphe. A variety of factors have led to under diagnosis of PHACES syndrome in the past including lack of awareness and limited imaging modalities. Also, patients with PHACES syndrome with arterial cerebro vascular abnormalities can present with acute ischemic stroke. However, these patients usually present before one year of age. We describe a 29-year-old woman with no history of cerebro vascular disease who initially presented with symptoms of a stroke and was subsequently diagnosed to have PHACES syndrome exhibiting an array of multiple unusual imaging findings. We also discuss the current literature and recommendations about PHACES syndrome.

• 出版日期2011-6