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K. V. Miller, A. W. Eller, T. R. Friberg; Peripheral Retinal Vascular Leakage in Retinitis Pigmentosa Evaluated With Optos Widefield Fluorescein Angiography. Invest. Ophthalmol. Vis. Sci. 2010;51(13):4044. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
Analysis of the retinal vasculature in patients with retinitis pigmentosa (RP) utilizing the Optos P200A (Dunfermline, Scotland) widefield fluorescein angiographic (FA) system.
Patients with the diagnosis of RP were identified through a search of billing records. Medical records were then reviewed to identify patients with RP who had been evaluated with Optos widefield FA and Cirrus OCT. Images were analyzed for cystic changes or retinal thinning on OCT and correlated with the FA for leakage in the macula, posterior pole, and peripheral retina.
Sixteen patients were identified with RP who had undergone Optos widefield FA, Cirrus OCT, and clinical examination. No patients were found to have peripheral retinal exudates (Coats’-like response) on examination. Ten of 16 patients (63%) had FA and OCT findings consistent with cystoid macular edema (CME). Five patients (31%) showed peripheral vascular leakage on widefield FA. Of these five, three were identified as having CME. One patient had a history of telangiectasis and had been treated with laser. The vascular leakage was predominant in the temporal periphery and was associated with capillary nonperfusion. There was no evidence of retinal neovascularization in any patient.Discussion: Coats’-like response has been reported in RP. However, the peripheral retinal vasculature has not been previously studied in RP patients without telangiectasis or exudates. The advent of the Optos widefield FA has made this possible. Abnormal peripheral retinal vasculature in RP may represent a forme-fruste of Coats’-response, which may be more common than previously reported. The cause of this vascular damage is unknown, and may or may not reflect an inflammatory etiology. Interestingly, the peripheral vascular leakage was associated with CME in some (3/5) but not all cases. The high percentage (31%) of patients with peripheral vascular leakage in this study may be related to selection bias, as those patients with a history of CME were more likely referred for testing.
A significant percentage of RP patients may have peripheral retinal vascular leakage identified with Optos widefield FA. Further studies are needed to identify the prevalence, mechanism, treatment, and effect on prognosis.
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