OSLI Retina

February 2020

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February 2020 · Vol. 51, No. 2 109 ■ C L I N I C A L S C I E N C E ■ Von Hippel-Lindau Syndrome Phenotype With Prominent Vitreoretinal Neovascularization Treated With Early PPV: A Case Series and Literature Review Talhah Zubair, AB; Natalia F. Callaway, MD, MS; Cassie Ann Ludwig, MD; Peter H. Tang, MD, PhD; Ryan A. Shields, MD; Marco H. Ji, MD; Daniel Vail, BA; Matthew A. Powers, MD, MBA; Darius M. Moshfeghi, MD BACKGROUND AND OBJECTIVE: To describe a case series of three patients in one family with Von Hippel-Lindau (VHL) disease who presented with vitreoretinal neovascularization and resulting trac- tional retinal detachments (TRDs). This vitreoreti- nal phenotype of VHL may benefit from early surgi- cal intervention. PATIENTS AND METHODS: Descriptive case series of three patients in one family with VHL disease. A review of the literature regarding surgical interven- tion for VHL was performed. RESULTS: All three patients developed prominent intravitreal neovascularization with fibrovascular growth within the vitreous secondary to a retinal capillary hemangioma. Two subjects with intravit- real neovascularization were treated with laser and cryotherapy but eventually developed a TRD. The final vision in these two patients was light percep- tion and 20/300. The eye that was preemptively treated with vitrectomy to remove the vitreous sus- taining the neovascularization had visual acuity of 20/50 after surgery. CONCLUSION: Intravitreal neovascularization with fibrovascular proliferation may be an indication for vitrectomy prior to the development of retinal de- tachment. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:109-115.] INTRODUCTION Retinal capillary hemangiomas (RCHs) are the ear- liest and most common presenting manifestation of Von Hippel-Lindau (VHL) disease. 1,2 Although small RCHs can remain asymptomatic for years or even re- gress, most continue to progress. 3 Left untreated, these benign tumors often cause secondary effects including subretinal exudates, fibrovascular proliferation, and serous and tractional retinal detachment (RD), and this can ultimately result in permanent vision loss. 4 Various interventions have been attempted to treat large RCHs, defined as greater than 2 disc diameters (DD) in size. 7-13 Anti-vascular endothelial growth fac- tor (VEGF) agents have failed to show stable regression of large RCHs. 7 Brachytherapy, external beam therapy, transpupillary thermotherapy, and photodynamic therapy (PDT) have all been used to treat large RCHs with marginal success. 8-13 Traditionally, the final resort for large treatment-resistant RCHs or those evolving to RD is surgical intervention. Outcomes are often poor, and the timing of surgery is often at the discretion of the treatment physician. Here we describe a series of three VHL patients within a single family with a unique clinical pheno- type of VHL demonstrating as significant intravitreal neovascularization and fibrovascular proliferation. Two of these patients elected to be observed until they developed RDs, with poor visual outcomes. One patient underwent early pars plana vitrectomy (PPV) and was able to maintain moderate visual acuity (VA) until she declined further treatment. We review the literature for the timing of surgical intervention for VHL-associated RCH and further describe this unique phenotype of VHL. From Stanford University Byers Eye Institute, Department of Ophthalmology, Palo Alto, California (TZ, NFC, CAL, PHT, RAS, MHJ, DV, DMM); and the De- partment of Ophthalmology, University of Colorado, Denver, Colorado (MAP). Originally submitted June 25, 2019. Revision received June 25, 2019. Accepted for publication August 27, 2019. Supported by the Heed Ophthalmic Foundation Award (NFC) and the Stanford University Medical Scholars Research Program (TZ). The authors report no relevant financial disclosures. Dr. Moshfeghi did not participate in the editorial review of this manuscript. Address correspondence to Darius M. Moshfeghi, MD, Horngren Family Vitreoretinal Center, Byers Eye Institute, Department of Ophthalmology, Stanford University School of Medicine, 2452 Watson Court, Room 2277, Palo Alto, CA 94303; email: dariusm@stanford.edu. doi: 10.3928/23258160-20200129-07

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