后极部中心性脉络膜营养不良2例

Posterior polar central choroidal dystrophy: 2 cases report

后极部中心性脉络膜营养不良是一种罕见疾病。本文对2例患者进行了荧光素眼底血管造影、眼底自发荧光、相干光层析成像及视觉电生理等检查。眼底检查可见双侧后极部血管弓范围视网膜色素上皮(retinal pigment epithelium,RPE)及脉络膜毛细血管的对称性萎缩。自发荧光可见相应区域的对称性低荧光。光学相干断层成像术(optical coherence tomography,OCT)显示后极部外层视网膜和脉络膜毛细血管的萎缩变薄。

Posterior polar central choroidal dystrophy is a rare disease.Clinical data of two patients with posterior polar central choroidal dystrophy fluorescein angiography,fundus autofluorescence(FAF)imaging,optical coherence tomography(OCT),and electrophysiological tests were reported.Fundus examination showed bilateral symmetric atrophy of the retinal pigment epithelium and choriocapillaris in the posterior polar areas between vascular arcades.FAF imaging showed clearly defined hypoautofluorescent areas that corresponded to the aforementioned lesions.Atrophy of the choriocapillaris and outer retinal layer were detected by optical coherence tomography.