Purpose
To describe the standard and wide field angiographic features of patients with congenital X-linked retinoschisis (XLRS) and propose a pathophysiologic mechanism for these findings.
Methods
This is a retrospective case series of 18 patients (36 eyes) with congenital X linked retinoschisis from 2008-2014. Color fundus photographs, optical coherence tomography, and standard and wide field angiographic images were separately reviewed. Patients were classified into either exudative or serous retinoschisis based on the presence or absence of lipid. Clinical features were then categorized.
Results
Eleven eyes exhibited exudative retinoschisis (30%). Twelve eyes (33%) had peripheral retinoschisis. The most common location of exudation was the macula (n=9/11). Subretinal hemorrhage was present in 4 eyes (11%). Macular findings included an atypical foveal avascular zone in 7 eyes (19%) and submacular fibrosis or retinal folds in 6 eyes (16.7%). Peripheral characteristics included retinal fibrosis or folds in 4 eyes (11%), bridging vessels in 3 eyes (8%), and frosted branch-like angiitis in 3 eyes (8%). Thirteen of 22 eyes (59%) demonstrated leakage on fluorescein angiography (FA), nine of which were serous XLRS.
Conclusions
The wide spectrum of exudative and serous angiographic features of congenital XLRS suggests that schisis cavity formation may be associated with increased vascular permeability. The presence of exudation with concurrent leakage on FA indicates that exudation may not solely be secondary to intra-schisis hemorrhage, as has been classically described.