Ocular manifestations of facial port-wine stain, nevus of Ota, and phakomatosis pigmentovascularis in Asian patients

Abstract

© 2020 American Academy of Dermatology, Inc. Background: Heightened intraocular pressure resulting in glaucoma and impaired vision is treatable if detected early. It is therefore necessary to identify populations at risk for glaucoma for regular screening visits. Objective: To investigate the prevalence of glaucoma in patients with facial port-wine stains (PWSs), nevus of Ota, and phakomatosis pigmentovascularis (PPV) and to establish the association between facial vascular birthmarks and ocular complications. Methods: This study is a retrospective chart review of 166 patients with facial PWS, PPV, and nevus of Ota over a 10-year period. Results: Of the 166 cases, 76 patients were diagnosed with PWS, 83 with nevus of Ota, and 7 with PPV. The mean age of patients was 12.8 years, ranging from newborn to 63 years old. Fifteen patients were diagnosed with glaucoma. Of 15 patients, 11 presented with PWS, and 4 presented with both PWS and PPV. Of 83 patients with nevus of Ota, only 2 (2.4%) presented with increased ocular pressure. Limitations: The relatively short follow-up period is a limiting factor in this study. Conclusions: Early and periodic ophthalmic examinations in patients with PWS, PPV, and nevus of Ota are essential to minimizing the risk of developing glaucoma in these groups of patients.