先天性视网膜劈裂症自然病程的临床观察

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1、524ChinOphthalmicRes，June2010，Vo1．28，No．6·临床研究·先天性视网膜劈裂症自然病程的临床观察辛梅张关霞张军军圄ClinicalobservationofnaturalcourseofcongenitalretinoschisisX／NMei，ZHANGMei—xia，ZHANGJun-jun，{DepartmentofOphthalmology，WestChinaHospital，SichuanUniversity，Chengdu610041，ChinaAbstractBackgrou

2、ndCongenitalretinoschsisisrareX-linkedinheriteddisorder，mainlyaffectsbilateralretinaandisseldominclinic．Someseverecomplications，suchasretinaldetachmentandvitreousbleeding，remaindificuhtotreat．0bjectiveTheaimofthisstudywastoobservethenaturalcourseofclinicaldevelopm

3、entinpatientswithcongenitalretinoschsis．MethodsThiswasaretrospectivecase—observationstudy．Theclinicaldataandfunduscharacteristicsof32eyesfrom16caseswithcongenitalretinoschsiswereanalyzed．ThesepatientswerediagnosedinWestChinaHospitalthroughfluorescencefundusfluores

4、ceinangiography(FFA)andopticalcoherencetomography(OCT)．ResultsAllofthe16casesofcongenitalretinoschesiswerebilateralwiththefoveoschisis．Peripheralretinoschisiswasfoundin12eyesof6cases．Thepatientsagedfrom6to29yearsoldwiththeaverageage14．2years．Duringthefollow—upperi

5、od，3eyesdevelopedmacularhole，and3eyesprogressedretinaldetachment，and1eyedevelopedvitreoushemorrhage．Themaculawasinvolvedin1eyewithperipheralretinoschisis．Twenty-foureyespresentedthestablecourse．ConclusionThestateofillnessinmajorityofpatientswithcongenitalretinosch

6、isiscanbestabilizedforalongduration．Butsomecomplicationsandillnessprogressarefoundinpartialpatients．Regularfollow—upshouldbeperformedforthepatientswithcongenitalretinoschsis．OCTisausefultoolforthemonitoringthemanifestationoffundusincongenitalretinoschsis．Keywordsc

7、ongenitalretinoschisis；naturalcourse；opticalcoherencetomography摘要目的观察先天性视网膜劈裂症的自然病程。方法回顾性病例研究。对确诊为先天性视网膜劈裂症患者16例32眼的临床资料、眼底检查、荧光素眼底血管造影(FFA)和光学相干断层扫描(OCT)等进行分析，随访0．5～6a。结果l6例患者均为双眼发病，均有黄斑中心凹劈裂，6例患者合并周边部视网膜劈裂，年龄6～29岁，平均14．2岁。随访中，3眼中心凹劈裂进展为黄斑裂孔，3眼进展为视网膜脱离，1眼玻璃体积血，1眼

8、周边视网膜劈裂向赤道部进展，24眼未见明显改变。结论先天性视网膜劈裂症虽可长期保持稳定，但部分患者病情可进展，应定期进行随访。OCT可作为随访检查的主要工具。关键词先天性视网膜劈裂症；自然病程；光学相干断层扫描中图分类号R774．1文献标志码A先天性视网膜劈裂症为双眼发病的性连锁隐性遗1资料与方法传疾病