Von Hippel-Lindau Syndrome Phenotype With Prominent Vitreoretinal Neovascularization Treated With Early PPV: A Case Series and Literature Review

• Published in: Ophthalmic surgery, lasers & imaging Vol. 51; no. 2; pp. 109 - 115
• Main Authors: Zubair, Talhah, Callaway, Natalia F., Ludwig, Cassie Ann, Tang, Peter H., Shields, Ryan A., Ji, Marco H., Vail, Daniel, Powers, Matthew A., Moshfeghi, Darius M.
• Format: Journal Article
• Language: English
• Published: United States Slack, Inc 01.02.2020; SLACK INCORPORATED
• Subjects: Adolescent; Adult; Age; Edema; Eye; Female; Genetic aspects; Genotype & phenotype; Hemangioma; Hemangioma, Capillary - diagnosis; Hemangioma, Capillary - etiology; Humans; Intervention; Lasers; Literature reviews; Male; Neovascularization; Patients; Pay-per-view television; Pedigree; Phenotype; Respiratory distress syndrome; Retinal Detachment - etiology; Retinal Detachment - surgery; Retinal Neoplasms - diagnosis; Retinal Neoplasms - etiology; Retinal Neovascularization - etiology; Retinal Neovascularization - surgery; Spinal cord; Surgery; Vascular endothelial growth factor; Visual acuity; Visual Acuity - physiology; Vitrectomy; Vitreous Body - pathology; Vitreous Body - surgery; von Hippel-Lindau Disease - complications
• ISSN: 2325-8160; 2325-8179; 2325-8179
• DOI: 10.3928/23258160-20200129-07

To describe a case series of three patients in one family with Von Hippel-Lindau (VHL) disease who presented with vitreoretinal neovascularization and resulting tractional retinal detachments (TRDs). This vitreoretinal phenotype of VHL may benefit from early surgical intervention. Descriptive case series of three patients in one family with VHL disease. A review of the literature regarding surgical intervention for VHL was performed. All three patients developed prominent intravitreal neovascularization with fibrovascular growth within the vitreous secondary to a retinal capillary hemangioma. Two subjects with intravitreal neovascularization were treated with laser and cryotherapy but eventually developed a TRD. The final vision in these two patients was light perception and 20/300. The eye that was preemptively treated with vitrectomy to remove the vitreous sustaining the neovascularization had visual acuity of 20/50 after surgery. Intravitreal neovascularization with fibrovascular proliferation may be an indication for vitrectomy prior to the development of retinal detachment. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:109-115.].