Shimazaki Jun, Maeda Naoyuki, Hieda Osamu, Ohashi Yuichi, Murakami Akira, Nishida Kohji, Tsubota Kazuo
Department of Ophthalmology, Tokyo Dental College, 5-11-13 Sugano, Ichikawa, Chiba, 272-8513, Japan.
Department of Ophthalmology, Keio University School of Medicine, Tokyo, Japan.
Jpn J Ophthalmol. 2016 Sep;60(5):341-8. doi: 10.1007/s10384-016-0462-0. Epub 2016 Jul 11.
A national survey was conducted in Japan to analyze the clinical characteristics of pellucid marginal corneal degeneration (PMD).
A questionnaire was sent to all members of the Japan Cornea Society requesting demographic and clinical findings on PMD patients who had been examined between 2008 and 2010. The presence of band-shaped peripheral corneal thinning, detected by slit-lamp biomicroscopy or by a pachymetric map, was set as the diagnostic criterion of PMD.
Most of the participating facilities relied on either slit-lamp biomicroscopy or corneal topography as diagnostic tools for the detection of PMD. Data on 347 patients (579 eyes) were returned from the participating facilities for analysis, among which 162 eyes in 84 men and 24 women, with a median onset age of 37 years, met the PMD criteria. Of the 108 patients (162 eyes) with classical PMD, unilateral involvement was found in 25 % of patients (27 eyes). In 17 of the 27 eyes for which topographic findings for the contralateral eye were available, seven eyes were either keratoconus or had a suspected diagnosis of keratoconus. An association with allergic disease was found in 24 eyes (22.2 % of patients). Approximately half of the patients had 1.0 or better spectacle-corrected distance visual acuity, and only 5 % showed 0.1 or worse. The mean keratometric value was 45.7 diopters. On corneal topography, 100 eyes (61.7 %) showed the topographic "crab-claw pattern," and the remaining eyes showed other patterns, including inferior steepening (22.2 %) and an irregular pattern (10.5 %).
We conducted the largest clinical survey of PMD to date and found a male predominance, a relatively high prevalence of unilateral involvement, and associated allergic diseases.
在日本开展一项全国性调查，以分析透明边缘角膜变性（PMD）的临床特征。
向日本角膜学会的所有成员发送问卷，询问2008年至2010年间接受检查的PMD患者的人口统计学和临床检查结果。通过裂隙灯生物显微镜检查或角膜厚度测量图检测到的带状周边角膜变薄被设定为PMD的诊断标准。
大多数参与机构依靠裂隙灯生物显微镜检查或角膜地形图作为检测PMD的诊断工具。参与机构返回了347例患者（579只眼）的数据用于分析，其中84名男性和24名女性的162只眼符合PMD标准，发病年龄中位数为37岁。在108例（162只眼）典型PMD患者中，25%的患者（27只眼）为单侧受累。在对侧眼有地形图检查结果的27只眼中的17只眼中，7只眼为圆锥角膜或疑似圆锥角膜诊断。24只眼（占患者的22.2%）发现与过敏性疾病有关。大约一半的患者最佳矫正远视力为1.0或更好，只有5%的患者视力低于0.1。平均角膜曲率值为45.7屈光度。在角膜地形图上，100只眼（61.7%）表现为地形图上的“蟹爪样”，其余眼睛表现为其他模式，包括下方陡峭（22.2%）和不规则模式（10.5%）。
我们进行了迄今为止最大规模的PMD临床调查，发现男性居多、单侧受累患病率相对较高以及存在相关过敏性疾病。
