Foveal optical sectioning with the retinal thickness analyzer in patients with juvenile x-linked retinoschisis

Purpose. The nearly pathognomonic foveal abnormality present in 98 to !00% of patients with juvenile X-linked retinoschisis (XLRS) has not been demonstrated histopathologically. The retinal thickness analyzer (RTA) is a non-contact imaging technique that generates multiple optical cross-sections of the macula. A commercial prototype (Talia Technology Ltd.) was utilized to evaluate foveal abnormalities in patients with XLRS. Methods. The RTA scans an obliquely oriented slit laser beam across the macula to rapidly obtain a series of optical cross-sections which are digitized. The RTA was used to examine five eyes of four patients with XLRS. Results. The RTA demonstrated foveal schisis in all eyes examined. In two eyes of two patients, a single optically empty schisis cavity with an inner leaf in a dome-shaped configuration was present in the region of the fovea. In one patient, two separate schisis cavities were present in the region of the fovea. In the fellow eye of the same patient, the schisis cavity had a rippled appearance. Fine strands, some partially, and others completely, bridging the schisis cavity were also present in both eyes of this patient. In another patient, the inner leaf of the schisis cavity was very thin, and only slightly elevated. Similar fine strands bridging the schisis cavity were present. Copclusion. Scanning retinal thickness analysis is a novel noninvasive imaging modality capable of producing optical cross-sections that demonstrate foveal schisis in patients with XLRS. This information may be valuable in detecting this disease and in following patients with XLRS for progression and may lead to a better understanding of the correlation of the foveal schisis characteristics with visual function in these patients.