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Brian C. Toy, Elvira Agrón, Divya Nigam, Emily Y. Chew, Wai T. Wong; Longitudinal Study of Retinal Angiomatosis and Visual Function in Ocular von Hippel-Lindau (VHL) Disease. Invest. Ophthalmol. Vis. Sci. 2011;52(14):4544.
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We aimed to characterize the long-term anatomic and visual function changes associated with ocular VHL disease in patients examined at the National Eye Institute/National Institutes of Health. The results of this study may be useful in providing clinical guidance for the management of persons with ocular VHL disease.
Patients with clinical or genetic evidence of VHL disease and at least 2 years of ophthalmic follow-up were identified and their ophthalmic records retrospectively analyzed. Univariate statistical analysis and logistic regression were performed using custom Matlab scripts and SAS.
Of 249 patients at study baseline, 154 (62%) had evidence of ocular VHL in at least one eye (29% unilateral, 33% bilateral). Of 498 eyes analyzed, 236 eyes (47%) were affected by VHL disease at baseline. Longitudinal analyses over a mean follow up period of 8.2±4.0 years revealed that 50 out of 167 at-risk participants (30%) developed new ocular VHL in a previously unaffected eye during follow-up. Of 262 eyes without ocular involvement at baseline, 58 eyes (22%) developed new ocular involvement. In eyes with existing ocular involvement at baseline, 6% developed angiomas in a new retinal location (juxtapapillary or peripheral), 20% increased in peripheral angioma number (from ≤3 to >3), 19% increased in the extent of peripheral retinal involvement (from ≤1 to >1 quadrant), and 7% progressed to end-stage disease. Significant decreases in mean ETDRS visual acuity were found in eyes developing angiomas in a new retinal location (-6.1 letters; p=0.04, paired t-test), developing new juxtapapillary angiomas (-11.4 letters; p=0.005), developing new peripheral angiomas (-4.3 letters; p=0.02), increasing in peripheral angioma number (-12.5 letters; p=0.002), or increasing in peripheral involvement (-7.6 letters; p=0.004).
The overall rate of developing new ocular VHL disease in patients with VHL disease was relatively slow. A majority of eyes that were without ocular VHL at baseline did not acquire ocular involvement during follow-up. Eyes with existing ocular VHL disease also demonstrated relative stability with a minority of eyes showing increases in measures of angioma burden during follow up. Increasing angioma burden, particularly the development of new juxtapapillary angiomas, was associated with a significant long-term worsening of visual acuity.
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