Abstract
Retinal hemangioblastomas are the most common manifestation of Von Hippel-Lindau (VHL) disease [1-3]. While peripheral retinal hemangioblastomas may be treated by thermal laser treatment or cryotherapy, optic nerve and macular lesions are more difficult to treat [4, 5]. Based on the theoretical benefit of administering anti-VEGF treatment, intra-vitreally administered bevacizumab (Avastin, a general pan-VEGF inhibitor) is attractive [6, 7]. Several short-term case series using ranibizumab (Lucentis, mAb fragment of bevacizumab with stronger affinity for VEGF-A) have shown it has promising but minimal success on most VHL-related hemangioblastomas [8, 9]. A comprehensive study by Wong et al. examined 5 patients over a period up to 61 weeks (47 ± 14 weeks) while Michels et al. examined one patient over a period of 4 months. Due to the short-term nature of these studies, we attempted long-term bevacizumab treatment over 60 months in a monocular subject with progressive visual loss due to a VHL associated macular and optic nerve hemangioblastoma. Over the treatment regimen of 15 injections, visual acuity improved 25 letters, OCT thickness improved from 646 um to 424 um, and structural lesions stabilized while exudates and edema resolved.
Highlights
A 33-year-old male with a long history of Von Hippel-Lindau (VHL) associated tumors including benign cysts on liver, kidneys and cerebellum presented with multiple large retinal hemangioblastomas in his remaining right eye
Visual acuity (VA), appearance on fluorescein angiogram (FA), and optical coherence tomography (OCT) measurements were used as indicators of treatment success
Wong et al examined the effects of ranibizumab on five patients with VHL associated hemangioblastomas in a short
Summary
A 33-year-old male with a long history of VHL associated tumors including benign cysts on liver, kidneys and cerebellum presented with multiple large retinal hemangioblastomas in his remaining right eye. Significant macular thickening with moderate hard exudates were present due to an optic nerve lesion with extension towards the fovea (Fig. 1A). We treated the lesion with a total of 15 bevacizumab injections over a period of 60 months.
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