Touirssa Othmane, Maier Philip, Boehringer Daniel, Auw-Haedrich Claudia, Glegola Mateusz, Reinhard Thomas, Nuessle Simone
Eye Center, University Medical Center Freiburg, Albert-Ludwigs-University of Freiburg, Killianstrasse 5, 79106, Freiburg, Germany.
Eye Clinic, University Hospital Basel, Mittlere Str. 91, Basel, 4056, Switzerland.
J Epidemiol Glob Health. 2025 Aug 28;15(1):112. doi: 10.1007/s44197-025-00458-y.
Corneal dystrophies are inherited disorders that can lead to significant visual impairment and often require surgical intervention in advanced stages. Fuchs endothelial corneal dystrophy (FECD) is the most frequently diagnosed type in Western countries and remains a leading global indication for corneal transplantation. In contrast, non-Fuchs dystrophies represent a diverse group of less common entities, each with distinct clinical features, surgical considerations, and regional variations in incidence and management. Despite their relevance, long-term data on the full spectrum of corneal dystrophies remain scarce. This study aimed to evaluate the distribution and temporal trends in dystrophy types and associated surgical procedures over eight decades at a tertiary referral center in Germany.
This retrospective analysis included 3 827 histopathologically confirmed corneal dystrophy specimens identified from an archive of 58 150 ophthalmic specimens collected between 1945 and 2024. Extracted data included dystrophy type, patient age at surgery, sex assigned at birth and associated surgical procedures. Distribution and temporal trends were analyzed descriptively.
FECD accounted for 90.3% (n = 3 455) of all cases, with a more than 15-fold increase in annual cases between 2003 and 2024. Its surgical management transitioned from exclusive use of penetrating keratoplasty (PKP) to posterior lamellar keratoplasty in over 99% of cases by 2024. Among non-Fuchs dystrophies (n = 372), granular (21.2%), macular (17.5%), and lattice dystrophy (17.2%) were most frequent. These exhibited greater surgical variability, reflecting their heterogeneity across 21 non-Fuchs dystrophy types in this study. Stromal and epithelial-stromal dystrophies were predominantly managed with PKP, whereas superficial epithelial and basement membrane dystrophies were increasingly treated with phototherapeutic or manual superficial keratectomy. Limbo-keratoplasty was introduced in the early 2000s for recurrent subepithelial and epithelial-stromal types.
This study provides unique insights into the type distribution and surgical management of corneal dystrophies over eight decades in a German center, encompassing nearly all IC3D-classified entities. The marked increase in FECD specimen numbers and the shift toward lamellar keratoplasty reflect evolving clinical practices and rising demand on corneal transplantation services. The broader clinical spectrum and procedural diversity among non-Fuchs dystrophies underscore the ongoing need for pathology-specific management strategies tailored to population-specific needs.
角膜营养不良是遗传性疾病，可导致严重视力损害，在疾病晚期常需手术干预。富克斯内皮性角膜营养不良（FECD）是西方国家最常诊断出的类型，仍是全球角膜移植的主要适应症。相比之下，非富克斯角膜营养不良是一组不太常见的不同疾病，每种疾病都有独特的临床特征、手术考量以及发病率和治疗方法的地区差异。尽管它们很重要，但关于角膜营养不良全谱的长期数据仍然稀缺。本研究旨在评估德国一家三级转诊中心八十年来角膜营养不良类型及相关手术的分布情况和时间趋势。
这项回顾性分析纳入了3827份经组织病理学确诊的角膜营养不良标本，这些标本来自1945年至2024年间收集的58150份眼科标本档案。提取的数据包括角膜营养不良类型、手术时患者年龄、出生时指定的性别以及相关手术。对分布情况和时间趋势进行了描述性分析。
FECD占所有病例的90.3%（n = 3455），2003年至2024年间年病例数增加了15倍多。到2024年，其手术治疗方式从单纯的穿透性角膜移植术（PKP）转变为在超过99%的病例中采用后板层角膜移植术。在非富克斯角膜营养不良（n = 372）中，颗粒状（21.2%）、斑状（17.5%）和格子状角膜营养不良（17.2%）最为常见。这些疾病的手术方式差异更大，反映了本研究中21种非富克斯角膜营养不良类型的异质性。基质性和上皮 - 基质性角膜营养不良主要采用PKP治疗，而浅表上皮性和基底膜性角膜营养不良越来越多地采用光治疗或手动浅表角膜切除术治疗。21世纪初引入了角膜缘移植术用于复发性上皮下和上皮 - 基质性类型。
本研究为德国一家中心八十年来角膜营养不良的类型分布和手术治疗提供了独特见解，涵盖了几乎所有国际角膜病学会（IC3D）分类的疾病。FECD标本数量的显著增加以及向板层角膜移植术的转变反映了临床实践的演变和对角膜移植服务需求的增加。非富克斯角膜营养不良更广泛的临床谱和手术多样性凸显了持续需要针对特定人群需求制定的病理学特异性管理策略。
