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

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 4 weeks 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.