Garro Sarah J, Bradshaw Wanda T
Duke University School of Nursing, Durham, North Carolina.
Adv Neonatal Care. 2014 Apr;14(2):96-102. doi: 10.1097/ANC.0000000000000060.
Sturge-Weber syndrome (SWS) is a rare, congenital neurocutaneous condition (affecting both the skin and the brain) characterized by a facial port-wine stain located specifically on the ophthalmic branch of the trigeminal nerve, cerebral vascular abnormalities, and potential eye involvement. The incidence of SWS is approximately 1 in 20,000 to 50,000 infants, and therefore all clinical presentations are important to discuss. This article describes a case presentation of SWS and then discusses the etiology, pathophysiology, management, diagnosis, and prognosis of SWS.
斯特奇-韦伯综合征(SWS)是一种罕见的先天性神经皮肤疾病(影响皮肤和大脑),其特征为特定位于三叉神经眼支上的面部葡萄酒色斑、脑血管异常以及可能的眼部受累。SWS的发病率约为每20000至50000名婴儿中有1例,因此所有临床表现都值得探讨。本文描述了一例SWS的病例报告,然后讨论了SWS的病因、病理生理学、管理、诊断和预后。