中国实用眼科杂志
中國實用眼科雜誌
중국실용안과잡지
CHINESE JOURNAL OF PRACTICAL OPHTHALMOLOGY
2014年
3期
289-292
,共4页
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
脈絡膜萎縮%無脈絡膜癥%迴鏇狀脈絡膜視網膜萎縮%中心暈輪狀脈絡膜萎縮%繞視乳頭型脈絡膜萎縮
맥락막위축%무맥락막증%회선상맥락막시망막위축%중심훈륜상맥락막위축%요시유두형맥락막위축
Choroidal vascular atrophy%Choroideremia%Gyrate atrophy of choroid and retina%Central areolar choroidal atrophy%Peripapillary choroidal atrophy
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2～4 PD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.
目的 探討原髮性脈絡膜萎縮的臨床特徵.方法 臨床病例繫列研究.迴顧分析于2001年1月至2013年5月在昆明醫科大學第四附屬醫院眼科診治的各類原髮性脈絡膜萎縮計56例(112隻眼)的眼底特徵、及眼底熒光血管造影,併複習文獻,對各類萎縮的遺傳特性、髮病機製、眼底萎縮特徵及視力預後等進行對比總結.結果 16例(32隻眼)無脈絡膜癥患者眼底錶現為瀰漫性視網膜色素上皮和脈絡膜毛細血管組織萎縮,脈絡膜大血管和鞏膜暴露;18例(36隻眼)迴鏇狀脈絡膜視網膜萎縮患者眼底錶現為視網膜週邊部或後極部扇形或花瓣樣脈絡膜全層萎縮區;9例(18隻眼)中心暈輪狀脈絡膜萎縮患者眼底錶現為黃斑區可見直徑約2～4 PD萎縮竈;13例(26隻眼)患者確診為繞視乳頭型脈絡膜萎縮,眼底錶現後極部視乳頭週圍環形灰白色病竈.所有萎縮竈眼底熒光血管造影均錶現為萎縮區域內的揹景熒光消失,僅見脈絡膜大血管顯影.結論 認識瞭解原髮性脈絡膜萎縮的各類病變,可為患者提供遺傳咨詢、診斷及鑒彆診斷、判斷視功能預後的診治意見和方法.
목적 탐토원발성맥락막위축적림상특정.방법 림상병례계렬연구.회고분석우2001년1월지2013년5월재곤명의과대학제사부속의원안과진치적각류원발성맥락막위축계56례(112지안)적안저특정、급안저형광혈관조영,병복습문헌,대각류위축적유전특성、발병궤제、안저위축특정급시력예후등진행대비총결.결과 16례(32지안)무맥락막증환자안저표현위미만성시망막색소상피화맥락막모세혈관조직위축,맥락막대혈관화공막폭로;18례(36지안)회선상맥락막시망막위축환자안저표현위시망막주변부혹후겁부선형혹화판양맥락막전층위축구;9례(18지안)중심훈륜상맥락막위축환자안저표현위황반구가견직경약2～4 PD위축조;13례(26지안)환자학진위요시유두형맥락막위축,안저표현후겁부시유두주위배형회백색병조.소유위축조안저형광혈관조영균표현위위축구역내적배경형광소실,부견맥락막대혈관현영.결론 인식료해원발성맥락막위축적각류병변,가위환자제공유전자순、진단급감별진단、판단시공능예후적진치의견화방법.
Objective To investigate clinical characteristics of primary choroidal vascular atrophies.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 (18 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 only large choroidal vascular imaging was observed.Conclusions Recognizing all kinds of lesions can provide patients with genetic counseling,diagnosis and differential diagnosis,determine the prognosis of visual function.
