【摘要】目的 探讨青年型视网膜劈裂症的临床特点、治疗及其预后。方法 对青年型视网膜劈裂症6例（12眼）进行常规检眼镜、Coldmann三面镜、荧光素眼底血管造影（FFA）临床检查，根据不同情况进行氪多波长激光光凝或巩膜扣带术联合氪激光光凝手术治疗，并随访1～5年。结果 6例（12眼），年龄18～36岁，均为双眼患病，病变位于颞下，双眼对称。5眼同时患有前部视网膜劈裂及视网膜脱离。7眼仅有视网膜劈裂。7眼行氪激光光凝治疗，2眼合并视网膜浅脱离采用包扎、药物联合氪激光光凝治疗，3眼采用巩膜扣带联合氪激光治疗。随访期间未见视网膜脱离，视力均有不同程度提高。结论 青年型视网膜劈裂合并视网膜脱离尽早发现，适宜治疗，预后良好。
Clinical analysis of juvenile retinoschisis
Zhang Li
Department of Ophthalmology，China-Japan Friendship Hospital，Beijing 100029.
【Abstract】ObjectiveTo investigate the clinical characteristics,treatment and prognosis of syndrome of juvenile retinoschisis.Methods6 cases （12 eyes）,with ophthalmoscope,Goldmann three-mirror lens combined with scleral indentation fundus,fluorescein angiography （FFA）.Based on clinical manifestations,the eyes were treated with krypton laser photocoagulation or scleral buckling combined with krypton laser photocoagulation and followed for 1～5 years.Results6 cases were involved in both eyes symmetrically located at inferior temporal with age of 18 to 36 years old.Anterior retinoschisis and retinal detachment was concurrent in 5 eyes.7 eyes were only anterior retinoschisis.7 eyes along with retinoschisis were treated with krypton laser photocoagulation to enclose the area of retinoschisis.2 eyes with superficial retinal detachment treated with wrap up,medicine and photocoagulation.3 eyes with anterior retinoschisis and retina detachment were treated with scleral buckling and photocoagulation.During following time retina detachment was not fund,and vision acuity was improved at various degrees.ConclusionThe juvenile retinoschsis has typical clinical characteristics.A better outcome will be obtained if it can be found as early as possible and treated with proper methods.
Key wordsretinoschisisretinal detachment
视网膜劈裂是指由视杯内层发育的视网膜神经上皮层本身的层间劈开，可分为先天性、退变性和继发性3种［1］。前者劈裂发生于视网膜神经纤维层，后两者劈裂部位为邻近内核层的外丛状层［2］。先天性视网膜劈裂症亦称遗传性视网膜劈裂，此病少见，绝大多数为男性发病，双侧性，主要为性连锁隐性遗传，又称为X连锁青年性视网膜劈裂症（X-linked juvenile retinoschisis，XLRS）。鉴于国内这方面资料甚少，笔者曾遇到6例，总结分析如下。
1临床资料
1.1一般资料6例（12眼）患者中，男2例、女4例；年龄18～36岁，均为双眼。6例患者均行检眼镜，眼底血管造影等临床检查，随访1～5年，视力：0.02～1.0之间。
1.2眼底情况12眼为黄斑部以外的视网膜劈裂，多从赤道部开始，发生于颞下方，呈球形改变或低平薄纱样隆起，劈裂处可见视网膜血管位于劈裂腔外层、内层或穿过劈裂腔。其中有些病例可见劈裂区的血管白鞘。7眼可见劈裂区视网膜内孔；5眼可见视网膜内外孔及视网膜脱离，其中1眼视网膜脱离波及黄斑区；2眼可见黄斑区色素变动；2眼可见少许玻璃体出血。
1.3眼底荧光血管造影黄斑部表现为色素紊乱的2眼，可见与眼底病变相对应的区域有斑状荧光，造影过程中形态无变化；颞下劈裂区域表现为视网膜血管迂曲、扩张、片状无灌注区、斑状荧光，内劈裂孔周围视网膜形成高荧光带，裂孔内可见不规则高荧光斑和色素沉着、出血所致荧光遮蔽；造影后期内劈裂孔内荧光斑减退，孔周高荧光斑略有减退。
1.4并发症5眼合并孔源性视网膜脱离，均为视网膜内外层出现裂孔；2眼劈裂腔可见少许出血。
1.5治疗7眼仅有视网膜劈裂内层孔，笔者于视网膜劈裂部的周围与正常视网膜的交界处行堤坝式氪激光光凝，其中2眼视网膜劈裂有内外层裂孔伴有视网膜浅脱离，先给予药物、包扎、卧床(注意体位)，待视网膜下积液吸收后，再行氪激光光凝。3眼视网膜劈裂并发孔源性视网膜脱离，无增殖性玻璃体视网膜病变，裂孔位于赤道前部，行巩膜扣带、放液术，术后1～2天行氪激光光凝封闭裂孔。
1.6随访观察6例经过1～5年随访，视力无变化或有不同程度的提高，劈裂区孔封闭，视网膜复位良好，未发现再脱离。
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