原发性脉络膜萎缩临床观察  

Clinical observations of primary choroidal vascular atrophies

作　　者：李娟娟[1] 黎铧[1] 张利伟[1] 

机构地区：[1]昆明医科大学第四附属医院眼科,云南650021

出　　处：《中国实用眼科杂志》2014年第3期289-292,共4页Chinese Journal of Practical Ophthalmology

摘　　要：目的探讨原发性脉络膜萎缩的临床特征。方法临床病例系列研究。回顾分析于2001年1月至2013年5月在昆明医科大学第四附属医院眼科诊治的各类原发性脉络膜萎缩计56例（112只眼）的眼底特征、及眼底荧光血管造影，并复习文献，对各类萎缩的遗传特性、发病机制、眼底萎缩特征及视力预后等进行对比总结。结果16例（32只眼）无脉络膜症患者眼底表现为弥漫性视网膜色素上皮和脉络膜毛细血管组织萎缩，脉络膜大血管和巩膜暴露；18例（36只眼）回旋状脉络膜视网膜萎缩患者眼底表现为视网膜周边部或后极部扇形或花瓣样脉络膜全层萎缩区；9例（18只眼）中心晕轮状脉络膜萎缩患者眼底表现为黄斑区可见直径约2-4PD萎缩灶；13例（26只眼）患者确诊为绕视乳头型脉络膜萎缩，眼底表现后极部视乳头周围环形灰白色病灶。所有萎缩灶眼底荧光血管造影均表现为萎缩区域内的背景荧光消失，仅见脉络膜大血管显影。结论认识了解原发性脉络膜萎缩的各类病变，可为患者提供遗传咨询、诊断及鉴别诊断、判断视功能预后的诊治意见和方法。Objective To investigate clinical characteristics of primary choroidal vascular atro- phies. Methods Diagnosed as primary choroidal vascular atrophies of 56 cases were collected, the characteristics of fundus, fundus fluorescein angiography, visual conditions and other clinical data were analyzed. Results Among 56 cases, 16 （32 eyes） with choroideremia showed diffuse retinal pigment epithelium and choroidal capillaris atrophy, large vessels were exposed; 18 （36 eyes） with gyrate atrophy of choroid and retina showed fan-shaped or petal-like choroidal atrophy; 9 08 eyes） with central areolar choroidal atrophy showed macular atrophy whose diameter were about 2-4 PD; 13 （26 eyes） with peripapillary choroidal atrophy showed gray rings around the optic nerve lesion. Fundus fluorescein angiography of all atrophies showed background fluorescence disappeared and on- ly large choroidal vascular imaging was observed. Conclusions Recognizing all kinds of lesions can provide patients with genetic counseling, diagnosis and differential diagnosis, determine the prog- nosis of visual function.

关 键 词：脉络膜萎缩 无脉络膜症 回旋状脉络膜视网膜萎缩 中心晕轮状脉络膜萎缩 绕视乳头型脉络膜萎缩 

分 类 号：R773.4[医药卫生—眼科]