Size of Facial Port-Wine Birthmark May Predict Neurologic Outcome in Sturge-Weber Syndrome - 24/08/17
, Bernard A. Cohen, MD 2, *Abstract |
Objective |
To determine whether the size of the birthmark in patients with Sturge-Weber syndrome (SWS) who have brain involvement can help predict neurologic disability.
Study design |
Fifty-one patients with SWS with facial birthmarks and brain involvement documented on magnetic resonance imaging were included in this retrospective chart review. A neuroradiologist, blinded to all clinical information, assigned a previously validated SWS neuroimaging score. A pediatric neurologist prospectively assigned previously validated neurologic severity scores, based on seizures, hemiparesis, visual field cut, and cognitive impairments. Three raters, blinded to clinical scores, independently graded the size of facial birthmark in each patient based on photographs. Their scores were averaged. Birthmark scores were compared with the imaging and neurologic severity results using nonparametric correlation analysis.
Results |
Size of facial port-wine birthmark correlates with magnetic resonance imaging scores on the left and right sides (ρ = 0.57 and 0.66 [P < .001], respectively). Size is also positively associated with the neurologic severity rating for patients age 6 years and above (1-sided Fisher exact, P = .032).
Conclusions |
The size of facial port-wine birthmark in SWS brain involvement can be developed as a tool to predict neurologic severity of the disease.
Le texte complet de cet article est disponible en PDF.Keyword : capillary malformation
Abbreviations : MRI, PWB, SWS
Plan
| Supported by the National Institute of Neurological Disorders and Stroke (NINDS) (National Institutes of Health [NIH] U54NS065705) (PI: Lawton; Sturge-Weber syndrome project to A.C.) and from Celebrate Cure Foundation (to A.C.). The Brain Vascular Malformation Consortium (U54NS065705) is a part of the NIH Rare Disease Clinical Research Network, supported through the collaboration between the NIH Office of Rare Diseases Research at the National Center for Advancing Translational Science and the NINDS. The authors declare no conflicts of interest. |
Vol 188
P. 205 - septembre 2017 Retour au numéro
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