星状非遗传性特发性黄斑劈裂研究现状

Research progress of stellate nonhereditary idiopathic foveomacular retinoschisis

摘要:

星状非遗传性特发性黄斑劈裂(SNIFR)是一类新型的黄斑劈裂，其眼底改变同X-性连锁视网膜劈裂类似，但目前尚未发现相关基因改变，女性好发，常单眼发病。该类黄斑劈裂主要位于外丛状层，可合并内层视网膜劈裂或周边视网膜劈裂，诊断需排除其他眼底疾病导致的黄斑劈裂。通过多模式眼底影像分析SNIFR特征性改变，增加对该疾病的认识。SNIFR目前发病机制尚不清楚，无明确治疗方案，碳酸酐酶抑制剂或玻璃体手术可能提高患者视力，减轻视网膜劈裂程度。

Abstract:

Stellate nonhereditary idiopathic foveomacular retinoschisis(SNIFR)is a new catagory of foveomacular retinoschisis, defined by spokelike appearance on fundus which is similar to X-linked congenital retinoschisis, and without agenetic predisposition. SNIFR is most commonly unilateral and female predominance. The retinoschisis in SNIFR occurs primarily in outer plexus split(OPL), and coexists with inner retinal or peripheral retinoschisis in some cases. The characteristics of SNIFR on multimodal fundus imaging are summarized in the review. The pathogenesis of SNIFR is not yet elucidated. At present, there is not practicable systematic treatment for this disease. Topical dorzolamide therapy or vitrectomy may improve the vision and foveomacular retinoschisis.