Welty Linda D
Neonatal Netw. 2006 Mar-Apr;25(2):89-98. doi: 10.1891/0730-0832.25.2.89.
Sturge-Weber syndrome (SWS) is a rare, sporadic, progressive, congenital syndrome. In its complete trisymptomatic form, SWS is physically characterized by port-wine stains over the trigeminal area, leptomeningeal angiomas usually over the parieto-occipital region, and eye abnormalities. Clinical manifestation for infants with SWS depends on the affected organs, but can include seizures, mental retardation, and glaucoma. This article begins with a case presentation of an infant with SWS and then presents the etiology, embryology, pathophysiology, clinical presentation, management, and prognosis of SWS.
斯特奇-韦伯综合征(SWS)是一种罕见的、散发性的、进行性的先天性综合征。在其完全三症状形式中,SWS的身体特征为三叉神经区域的葡萄酒色斑、通常位于顶枕区域的软脑膜血管瘤以及眼部异常。患有SWS的婴儿的临床表现取决于受影响的器官,但可能包括癫痫发作、智力迟钝和青光眼。本文首先介绍了一名患有SWS的婴儿的病例,然后阐述了SWS的病因、胚胎学、病理生理学、临床表现、治疗及预后。