Stargardt′s病的临床观察(附69例报道)  

Clinical observation on Stargardt′s diseases:69 cases report

作　　者：唐朝珍[1] 姜德咏[1] 聂爱光[1] 曹丹[1] 蒋群[1] 

机构地区：[1]中南大学湘雅二医院眼科,410011

出　　处：《中国实用眼科杂志》2002年第9期691-693,共3页Chinese Journal of Practical Ophthalmology

摘　　要：目的 :探讨Stargardt′s病的临床表现。方法 :回顾性分析 69例 13 8眼Stargardt′s病的临床和眼底荧光素血管造影 (fundusfluoresceinangiographyFFA)资料 ,分析其视力损害程度、年龄分布状态及病灶表现形态。结果 :视力 0 0 2～ 0 1者 115眼 (83 3 3 % ) ,0 2～ 0 5者 2 3眼 (16 66% ) ;近视力最高Jr =2、最低Jr =0。其中 10岁前起病者3 3例、 11～ 2 0岁起病者 2 6例、 2 1～ 3 0岁起病 4例、 3 1～ 40岁起病 6例。病灶形态 :①单纯黄斑病灶型 2 4例 48眼 ;②黄斑病灶联合眼底黄色斑点型 2 9例 5 4眼 ;③黄斑病灶与后极部色素脱失相融合型 16例 3 2眼。第三型病灶形态与前两型病灶在起病年龄与病程长短方面差别无明显意义 (P >0 0 5 )。三种病灶形态的病变程度差别亦无意义 (P >0 0 5 )。结论 :①Stargardt′s病对视力的影响较大 ,但暂未见到视力全部丧失的病人 ;②Stargardt′s病主要出现于青少年时期 ,亦可在成年人后期发病 ;③黄斑病灶与后极部色素脱失融合为一体的特殊病灶可能是Stargardt′s病的另一种特殊的FFA变异形态。Objective:To study the clinical manifestations of Stargardt′s disease. Methods:The histories and FFA data of 69 cases(138 eyes)of Stargardt′s disease were analyzed retrospectively.Emphasis was put on the damage of visual acuity,age distribution and the lesion morphology. Results:5/6 of the eyes(115 eyes)had the visual acuities at 0 02～0 1,16 66% of the eyes(23 eyes)had the visual acuities better than 0 2(0 2～0 5).Best near vision was Jr.2 and worst Jr.0.The onset ages of most patients(33 cases)were younger than 10 years old,26 cases were between 11 to 20 years old,10 cases over 20 years old.There were 3 forms of lesion morphology:1)local macular lesions(48 eyes of 24 cases),2)combination of macular lesion and fundus xanthelasma(29 cases,54 eyes),3)macular lesion with de-pigmentation at posterior fundus(32 eyes of 16 cases). Conclusions:No patient with Stargardt′s disease was found with completely lost visual acuity.The onset age of Stargardt′s disease seems mainly in the teenager period of patients,but may occur at later.The unique manifestation of macular lesion combined with de-pigmentation at the posterior pole may be a special variation of FFA of Stargardt′s diseases.

关 键 词：Stargardt′s病 色素上皮 窗样缺损 临床表现 眼底荧光素血管造影 遗传性黄斑营养性疾病 

分 类 号：R774.5[医药卫生—眼科]