Piram Maryam, Lorette Gérard, Sirinelli Dominique, Herbreteau Denis, Giraudeau Bruno, Maruani Annabel
Department of Dermatology, Trousseau University Hospital, Tours, France.
Pediatr Dermatol. 2012 Jan-Feb;29(1):32-7. doi: 10.1111/j.1525-1470.2011.01485.x. Epub 2011 Sep 9.
Facial port-wine stain (PWS) may be associated with cerebrovascular abnormalities such as Sturge-Weber syndrome (SWS). In a large series, we aimed to assess which topography of facial PWS can predict SWS. This was a cross-sectional study of consecutive patients with facial PWS seen in pediatric dermatologic or angiodysplasia consultations from 1993 to 2005 at the University Hospital Center of Tours. A standardized form was used to collect data on clinical and imaging findings. Patients with and without SWS were compared in terms of topography of the cutaneous angioma and related ophthalmologic and neurologic features. Two hundred fifty-nine patients were included, 15 with a diagnosis of SWS. All patients with SWS showed involvement of the V1 trigeminal cutaneous area. SWS was significantly associated with bilateral topography of the PWS, its extension to another territory, and involvement of the upper eyelid. Knowledge of the topography of facial PWS with risk of associated neurological or ocular anomalies allows for early diagnosis of SWS and avoids unnecessary and costly radiography for patients with uncomplicated facial PWS.
面部葡萄酒色斑（PWS）可能与诸如斯特奇-韦伯综合征（SWS）等脑血管异常有关。在一个大型系列研究中，我们旨在评估面部PWS的哪些形态特征可以预测SWS。这是一项横断面研究，研究对象为1993年至2005年在图尔大学医院中心儿科皮肤科或血管发育异常门诊就诊的连续性面部PWS患者。使用标准化表格收集临床和影像学检查结果的数据。对有和没有SWS的患者在皮肤血管瘤的形态特征以及相关眼科和神经学特征方面进行比较。共纳入259例患者，其中15例诊断为SWS。所有SWS患者均表现出三叉神经第一支（V1）皮肤区域受累。SWS与PWS的双侧形态特征、其扩展至其他区域以及上睑受累显著相关。了解具有相关神经或眼部异常风险的面部PWS的形态特征有助于早期诊断SWS，并避免对单纯性面部PWS患者进行不必要且昂贵的影像学检查。
