Inherited Retinal Diseases

Widefield fluorescein angiography beneficial in evaluation of X-linked retinoschisis

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Widefield fluorescein angiography (FA) should be considered when evaluating pediatric patients with X-linked retinoschisis (XLRS), according to a study that found consistent vascular changes in patients with XLRS using widefield FA.

In this retrospective case series, FA images from 29 eyes of 15 patients were analyzed. The most common findings were capillary dropout/ischemia in 72.4% of eyes, terminal supernumerary vessels in 72.4% of eyes, abnormal vascular loops in 69% of eyes, and vascular leakage in 58.6%.

Zone 1 and zone 2 were the most posterior zone of involvement accounting for 64.7% and 35.3%, respectively, of eyes with leakage.

All eyes had ≥1 vascular anomaly on FA, with 79.3% demonstrating peripheral bullous schisis or retinal detachment (RD), which was associated with the incidence of capillary dropout

More involvement in eyes with RD or bullous schisis correlated with more severe capillary dropout.

Al-Khersan H, Sengillo J, Fan KC, et al. Widefield Fluorescein Angiography Findings in Pediatric Patients with X-Linked Retinoschisis. Ophthalmol Retina. 2023;S2468-6530(23)00058-1. doi: 10.1016/j.oret.2023.02.005. Epub ahead of print. PMID: 36805350.