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Many eyes with von Hippel-Lindau disease maintain anatomic, functional stability
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Patients with ocular von Hippel-Lindau disease are able to maintain anatomic and functional stability, according to a study.
The retrospective analysis of a longitudinal, observational case series included 498 eyes of 249 participants with von Hippel-Lindau (VHL) disease who had at least 2 years of ophthalmic follow-up. Mean follow-up was 8.2 years.
Visual acuity, germline mutation in the VHL gene, and features of ocular VHL disease, including presence, location, number and extent of retinal capillary hemangioblastomas, were recorded in all patients.
Two hundred sixty-two eyes did not have ocular VHL at baseline, and 204 of these eyes did not have ocular VHL at final follow-up
Of the 236 eyes with ocular VHL at baseline, 88% did not have hemangioblastomas in a new retinal location at final follow-up. The number of hemangioblastomas was stable in 70% of eyes, and the extent of hemangioblastoma involvement was stable in 79% of eyes.
During follow-up, mean visual acuity decreased by 5.1 ± 0.6 letters in all study eyes. A decrease of more than 10 letters of visual acuity was observed in 16.1% of all study eyes.
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