Photodynamic Therapy (PDT) for Retinal Hemangioblastoma: Treatment Outcomes in 17 Consecutive Patients.

PURPOSE: To report the efficacy of photodynamic therapy (PDT) for management of retinal hemangioblastoma.

DESIGN: Retrospective case series.

PARTICIPANTS: Seventeen patients with retinal hemangioblastoma treated with PDT.

METHODS: The medical records of 17 patients with retinal hemangioblastoma treated with PDT were reviewed and treatment outcomes were assessed. Photodynamic therapy was performed with verteporfin infusion intravenously at a dose of 6 mg/m2 body surface area over 10 minutes, followed by application of 50 J/cm2 laser light at 689 nm for 83 or 166 seconds.

MAIN OUTCOME MEASURES: Tumor control, subretinal/intraretinal fluid resolution, visual outcome.

RESULTS: There were 18 retinal hemangioblastomas in 17 eyes treated with PDT. Median patient age was 31 years (mean 36 years, range 7-66 years), and median follow up was 51 months (mean 61 months, range 2-144 months). Genetic testing confirmed von Hippel-Lindau disease in 8/17 (47%) patients, and ocular symptoms were present in 12/17 (71%) patients. The tumors were unilateral in all patients and unifocal in the majority of patients (n=13/17, 76%). The tumor median basal diameter was 3.5 mm (mean 3.4 mm, range 1.5-6.0 mm) and median thickness was 2.1 mm (mean 2.3 mm, range 1.0-5.0 mm). The tumor location was juxtapapillary in 9/18 cases (50%). Associated findings included exudation (n=14/17, 82%), subretinal fluid (n=14/17, 82%), and macular edema (n=12/17, 71 %). The median number of PDT sessions was 1.5 (mean 1.8, range 1.0-4.0). Standard duration PDT (83 seconds) was used in all cases except 2, in which double duration (166 seconds) was used. Post-PDT transient exudative response occurred in 4/17 (24%) eyes. Outcomes revealed tumor control in 13/18 (72%) tumors, partial or complete resolution of subretinal fluid in 10/14 (71%) and macular edema in 7/12 (58%) eyes, and visual acuity stable or improved in 12/17 (71%) eyes. Photodynamic therapy-related transient exudative response was noted in 4/17 (24%) eyes.

CONCLUSION: Photodynamic therapy is an effective treatment for both juxtapapillary and peripheral retinal hemangioblastomas, providing satisfactory rates of tumor control and visual stabilization/improvement. Patients should be monitored for PDT-related transient exudative response.