Objective To resarch the clinical features and in vivo confocal microscopic findings of posterior polymorphous corneal dystrophy (PPCD). Methods It was a retrospective consecutive case study.Ten patients with PPCD, attended at Optometry Department of Henan Eye Institute from March 2007 to August 2009, were analyzed. All the subjects were examined by slit-lamp, Orbscan Ⅱ , specular microscopy,HRT3/RCM confocal microscopy. Mann-Whitney U test was used to analysis the data. Results The age of the patients ranged from 8 to 35 years. Seven eyes of the 4 patients have the vesicular lesions, five eves of the 5 patients were band lesions and 1 patient had bilateral diffused opacities, this patient also had iridocorneal adhesions with associated papillary ectropion but without glaucoma. In total, 14 eyes of the 10 patients had PPCD. Two eyes had abnormal Orbscan Ⅱ topography, it showed both anterior and posterior surface protrusion. Specular microscopy exam indicated large cells in size and reduced endothelium density.The mean size of the affected eye was 584 μm2 ,the normal eye was 316 μm2. The difference was statistically significant( U =0.000,P =0.002). The density of the endothelium was 1746 cells/mm2 in affected eye and 3201 cells/mm2 in normal eye. The difference was also statistically significant( U =0.000,P =0.002). In vivo confocal microscopy showed endothelial polymorphism. Occasional bright endothelial nuclei were seen. A variety of curvilinear and vesicular abnormalities were imaged including orange or finger like lesion, round or oval dark area with hyper reflectivity border. Some large lesions may lose endothelium with rough surface have a dike appearance. Conclusions Careful exam by slit-lamp may help to diagnose PPCD and further specular microscopy and (or) in vivo confocal microscopy exam will confirm it. Some cases may have abnormal topography, or associated with high intraocular pressure.%目的 探讨后部多形性角膜营养不良的临床特点及其在共焦显微镜下的改变.方法 回顾性系列病例研究.对2007年3月至2009年8月河南省眼科研究所视光门诊就诊的10例后部多形性角膜营养不良患者行详细的裂隙灯显微镜检查及眼前节照相,并行共焦显微镜、角膜内皮镜及OrbscanⅡ等检查,分析其特点.采用Mann-Whitney U检验进行统计学分析.结果 10例患者年龄范围8～35岁,其中囊泡状后部多形性角膜营养不良4例(7只眼),带状后部多形性角膜营养不良5例(5只眼),弥漫性后部多形性角膜营养不良1例(2只眼),共计10例(14只眼).1例囊泡状病变者双眼合并高眼压症状,2例带状病变者(2只眼)伴有角膜地形图异常,表现为角膜后表面前突;1例弥漫性病变者双眼有虹膜周边前粘连,角膜基质水肿.角膜内皮镜检查显示病变角膜内皮细胞增大,平均大小为584μm2,而无病变眼为316 μm2,差异有统计学意义(U=0.000,P=0.002);内皮细胞数量减少,病变眼平均内皮细胞密度为1746个/mm2,无病变眼为3201个/mm2,差异有统计学意义(U=0.000,P=0.002).共焦显微镜检查可见角膜内皮细胞呈多形性改变;部分出现细胞核,部分皱缩呈橘皮样或指纹样外观,甚至内皮细胞缺失;囊泡状病变表现为圆形或椭圆形弹坑样或火山口样病灶;带状病变的病灶为宽带状,边缘呈堤状外观,病变区内皮细胞消失,可有纤维样组织增生,幅状粘连;弥漫性病变表现为内皮细胞大面积缺失,表面粗糙,呈沟壑状.结论 后部多形性角膜营养不良经裂隙灯显微镜检查可明确诊断,共焦显微镜检查可辅助确诊,部分患者早期可有角膜地形图异常或伴有高眼压症状.
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