Posterior pole and peripheral retinal fibrovascular proliferation in von hippel-lindau disease

Purpose: To report the occurrence of fibrovascular proliferation (FVP) in the retina in von Hippel-Lindau (VHL) patients and its association with prior treatment. Design: Aretrospective study.Methods: Aretrospective study of 101 VHLpatients. Fundus photos were available for 28 patients. FVP was classified into peripheral and posterior pole. Results: All 28 patients had retinal capillary hemangioblastomas (RCH) in 1 or both eyes; 15 patients were found to have FVP (group A), whereas 13 patients did not (group B). Mean age of patients in group Awas 35 ± 11.3 years and 36.6 ± 13.8 in group B (P = 0.74). In group A, 27 eyes had RCH; 21 (77.77%) had FVP. In group B, 19 eyes had RCH. The number of treated eyes was significantly higher in group A(81.48%) than group B (42.1%) (P = 0.007). In group A, FVP was noted in the posterior pole in 9 eyes, in the periphery in 5 eyes, and 7 eyes developed both posterior pole and peripheral FVP. Conclusions: FVP can occur in the peripheral retina and in the posterior pole. There is a significant association between prior treatment of RCH and the occurrence of FVP.