Division of Dermatology, Department of Pediatrics, University of Washington School of Medicine and Seattle Children's Hospital, Seattle, Washington.
Division of Neurology, University of Washington School of Medicine and Seattle Children's Hospital, Seattle, Washington.
J Am Acad Dermatol. 2020 Oct;83(4):1110-1117. doi: 10.1016/j.jaad.2020.05.017. Epub 2020 May 12.
Children with forehead port-wine stains (PWSs) are at risk of Sturge-Weber syndrome (SWS). However, most will not develop neurologic manifestations.
To identify children at greatest risk of SWS.
In this retrospective cohort study of children with a forehead PWS, PWSs were classified as "large segmental" (half or more of a contiguous area of the hemiforehead or median pattern) or "trace/small segmental" (less than half of the hemiforehead). The outcome measure was a diagnosis of SWS.
Ninety-six children had a forehead PWS. Fifty-one had a large segmental PWS, and 45 had a trace/small segmental PWS. All 21 children with SWS had large segmental forehead PWSs. Large segmental forehead PWSs had a higher specificity (0.71 vs 0.27, P < .0001) and a higher positive predictive value (0.41 vs 0.22, P < .0001) for SWS than any forehead involvement by a PWS.
Retrospective study at a referral center.
Children with large segmental forehead PWSs are at highest risk of SWS.
患有额部葡萄酒色斑（PWS）的儿童存在罹患 Sturge-Weber 综合征（SWS）的风险。然而，大多数儿童不会出现神经系统表现。
确定具有最高罹患 SWS 风险的儿童。
在这项回顾性队列研究中，纳入患有额部 PWS 的儿童，根据 PWS 的类型将其分为“大片段型”（半侧或更多额部的连续区域或中线模式）或“痕量/小片段型”（小于半侧额部）。结局指标为 SWS 的诊断。
96 例儿童患有额部 PWS。51 例为大片段型 PWS，45 例为痕量/小片段型 PWS。所有 21 例患有 SWS 的儿童均存在大片段型额部 PWS。大片段型额部 PWS 对 SWS 的特异性（0.71 比 0.27，P<.0001）和阳性预测值（0.41 比 0.22，P<.0001）均高于任何 PWS 累及额部的情况。
回顾性研究，且在转诊中心开展。
存在大片段型额部 PWS 的儿童罹患 SWS 的风险最高。
