ArticlesCONGENITAL X-LINKED RETINOSCHISIS CLASSIFICATION SYSTEMPRENNER, JONATHAN L. MD*; CAPONE, ANTONIO JR MD*; CIACCIA, STEFANO MD*; TAKADA, YUICHIRO MD†; SIEVING, PAUL A. MD†; TRESE, MICHAEL T. MD*Author Information From *Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan; and †NEI and NIDCD, National Institutes of Health, Bethesda, Maryland. Dr. Prenner is supported by grants from the Ronald G. Michels and Heed Foundations. No proprietary interests. Reprint requests: Michael T. Trese, MD, 632 Wm. Beaumont Medical Building, 3535 West 13 Mile Road, Royal Oak, MI 48073; e-mail: [email protected] Retina: September 2006 - Volume 26 - Issue 7 - p S61-S64 doi: 10.1097/01.iae.0000244290.09499.c1 Buy Metrics Abstract Purpose: To establish a classification system for congenital X-linked retinoschisis (CXLRS) using clinical examination and optical coherence tomography (OCT). Methods: Thirty-eight eyes of 19 patients who carried a clinical diagnosis of CXLRS were examined with OCT and clinical examination. Eyes were classified into one of four types based on a combination of clinical examination and OCT. Results: All patients had bilateral OCT scanning performed at an average age of 8.64 years (range 2.24–17.4 years). Review of OCT scans revealed that 37 of 38 eyes had foveal schisis (97%) while 31 of 38 (82%) eyes had macular schisis deeper than the nerve fiber layer in areas of ophthalmoscopically normal macular retina. The authors termed this flat schisis phenomenon lamellar schisis. Thirty of 38 (79%) had peripheral bullous schisis cavities present. Conclusions: OCT examinations of patients with CXLRS reveal lamellar schisis in areas of ophthalmoscopically normal macular retina in 82% of eyes with the clinical diagnosis of CXLRS. Using both clinical examination and OCT, the authors were able to identify foveal, lamellar, and peripheral schisis, lamellar schisis only identifiable by OCT. These findings allow the authors to propose a classification system. The authors hope this classification system will allow a better understanding of the natural history of CXLRS disease and allow testing of therapeutic options. © The Ophthalmic Communications Society, Inc.