A case report on type I sturge weber syndrome with bilateral port-wine stain

Nissi Chrysolite Gongati, V. Agrawal, Srinivasulu Poluru
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Abstract

Sturge-Weber syndrome (SWS) is a type of neuro-cutaneous diseases which is seen in 1 in 50,000 populations and affects both the sexes equally. It usually manifests with a facial Port Wine Stain (PWS). Facial port-wine stains are usually isolated findings, however, when associated with cerebral and ocular vascular malformations they form part of the classical triad of Sturge-weber syndrome (SWS). The prevalence of PWS is estimated at three to five children per 1000 live births; there are ~26 million people worldwide with PWS birthmarks. The majority of facial PWS (~90%) are unilateral in a trigeminal dermatomal distribution. Here, we report a case of 39 years old female with SWS who presented with classical triad of SWS i.e., bilateral port-wine stain, epilepsy and severe glaucoma.
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伴有双侧葡萄酒色斑的I型sturge weber综合征1例
斯特奇-韦伯综合征(SWS)是一种神经皮肤疾病,每5万人中就有1人患此病,男女患此病的几率相同。它通常表现为面部葡萄酒色斑(PWS)。面部酒斑通常是孤立的发现,然而,当与大脑和眼部血管畸形相关时,它们构成了斯特奇-韦伯综合征(SWS)经典三征的一部分。据估计,每1000名活产婴儿中有3至5名患有PWS;全世界约有2600万人患有PWS胎记。大多数面部PWS(约90%)是单侧三叉皮节分布。在此,我们报告一例39岁女性SWS患者,其表现为典型的SWS三征,即双侧葡萄酒色斑,癫痫和严重青光眼。
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