Forehead location and large segmental pattern of facial port-wine stains predict risk of Sturge-Weber syndrome - 08/09/20
Abstract |
Background |
Children with forehead port-wine stains (PWSs) are at risk of Sturge-Weber syndrome (SWS). However, most will not develop neurologic manifestations.
Objective |
To identify children at greatest risk of SWS.
Method |
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.
Results |
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.
Limitations |
Retrospective study at a referral center.
Conclusion |
Children with large segmental forehead PWSs are at highest risk of SWS.
Le texte complet de cet article est disponible en PDF.Key words : leptomeningeal angiomatosis, port-wine stain, prediction, segmental, Sturge-Weber syndrome
Abbreviations used : ICD, MRI, PHACE, PWS, SWS
Plan
Current affiliation: Connie Chon's permanent address is Oakland University School of Medicine, Rochester, Michigan. |
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Funding sources: None. |
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Conflicts of interest: None disclosed. |
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IRB approval status: The Seattle Children's Hospital Institutional Review Board approved this study. |
Vol 83 - N° 4
P. 1110-1117 - octobre 2020 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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