Periocular Port Wine Stain: The Great Ormond Street Hospital Experience

Abstract

To identify the sensitivity and specificity of risk factors for the development of glaucoma in patients with port wine stain (PWS). A retrospective case-control study involving a large cohort of patients with PWS. A total of 216 patients (total of 252 eyes) with unilateral or bilateral PWS seen in the eye department in Great Ormond Street Hospital, London, United Kingdom. We studied the anatomic distribution of PWS and the incidence of choroidal hemangioma, episcleral hemangioma, iris heterochromia, and Sturge-Weber syndrome (SWS). We analyzed the sensitivity and specificity of these features as risk factors for glaucoma. Development of glaucoma. Mean age at presentation was 2.9 years (3 weeks to 18.8 years). Mean follow-up was 3.2 years (0-15 years). A total of 180 patients (83.3%) had unilateral lesion, and 36 patients (16.7%) had bilateral lesion. Thirty-one patients (14.3%) had isolated V1 lesion, 35 patients had V2 lesion only (16.2%), and 93 patients (43%) had both V1 and V2 involved. On the last visit, 46 eyes (18.3%) in 39 patients had glaucoma; their mean age was 3.25 years. Glaucoma was more common if PWS was bilateral (P=0.0001), both upper and lower lids were involved (P < 0.0001), and episcleral hemangioma (P < 0.0001), iris heterochromia (P=0.004), or choroidal hemangioma (P < 0.0001) was present. Twenty-four patients had SWS; this was significantly associated with upper lid PWS (P=0.001) and bilateral PWS (P=0.0003). Glaucoma was more common in patients with SWS compared with those without (66.7% vs. 18%, P=0.01). Combined upper and lower lid PWS, episcleral hemangioma, SWS, and iris heterochromia are sensitive prognosticators for the development of glaucoma. Iris heterochromia is associated with the development of early glaucoma in patients with PWS. Patients at high risk of glaucoma should be seen more often in clinic. Patients who do not have combined lid involvement or episcleral hemangioma have a lower risk and can therefore be seen less often in clinic. The author(s) have no proprietary or commercial interest in any materials discussed in this article.