Facial Port-Wine Stain Phenotypes Associated with Glaucoma Risk in Neonates

Abstract

To determine if the size and location of facial port-wine stains (PWS) can predict glaucoma risk in neonates. Retrospective cohort study. Children with facial PWS who had undergone ophthalmologic examination within 4weeks of their birth were included. Clinical information, including facial photographs, intraocular pressure, corneal diameter, optic disc cup-to-disc ratio, and Sturge-Weber syndrome (SWS) diagnoses were collected. Based on facial photographs, PWS distribution, eyelid involvement, and PWS scores according to degree of involvement in each embryonic facial vasculature distribution (segment [S]1, S2 and S3) were evaluated. Among the 34 patients, 7 (21%) had bilateral PWS lesions. Eighteen (53%) had diagnoses of glaucoma. The proportion of eyes showing PWS involving both S1 and S2 was the highest (n= 15, 37%), and the frequency of glaucoma diagnosis (n= 9, 60%) was also the greatest. In eyelid involvement analysis, among the 7 eyes with only lower-eyelid lesions, 5 (83%) had glaucoma. Among the 11 eyes with only upper-eyelid lesions, however, 2 (18%) had diagnoses of glaucoma. A logistic regression model showed that the significant factors associated with glaucoma risk were greater PWS scores in S2 (odds ratio [OR]: 3.604; 95% confidence interval: 1.078-12.050; P= .037) or lower-eyelid involvement (OR: 12.816; 95% CI: 1.698-96.744; P= .013). Among the newborns with facial PWS, 1) a greater extent of birthmarks involving the S2 area, and 2) lesions including the lower eyelid were associated with higher risk of glaucoma development within the neonatal period.