Department of Ophthalmology, Saarland University Medical Center, Homburg/Saar, Germany.
Institute of Pathology, Saarland University Medical Center, Homburg/Saar, Germany.
Klin Monbl Augenheilkd. 2024 Sep;241(9):1081-1090. doi: 10.1055/a-1862-8403. Epub 2022 Aug 4.
To report a case of severe verrucous posterior polymorphous corneal dystrophy (PPCD) and cataract, which was treated with Descemet membrane endothelial keratoplasty (DMEK) and simultaneous cataract surgery as a triple procedure (Triple-DMEK).
A 62-year-old female patient presented to our department for co-evaluation of advanced PPCD with cataract and progressive light sensitivity in both eyes. The clinical examination demonstrated unusual clinical findings with prominent verrucous lesions on the posterior surface of the cornea without corneal decompensation. We performed a Triple-DMEK in case of simultaneous cataract. The corneal tissue was examined by light and transmission electron microscopy.
Intraoperatively, it was difficult to remove the verrucous structures completely after classical descemetorhexis. Light microscopic examination demonstrated epithelium-like transformation of the corneal endothelium by immunostaining (cytokeratin AE1/3 staining). Transmission electron microscopy revealed thickening of Descemet's membrane (18.5 to 30.0 µm). The anterior banded layer had a normal structure and was slightly thickened (3.5 to 5.5 µm). A normal posterior non-banded layer (PNBL) was observed but thinned (2.5 to 4.0 µm) or missing. It was followed by an altered PNBL with abnormal fibrillary inclusions, which was strongly and variably thickened (11.0 to 24.5 µm). The corneal endothelium was degenerated, partially absent, and epithelial-like altered. The nodular lesions were found to consist of a few degenerated cells that were embedded in an amorphous extracellular matrix interspersed with collagen fibers, which were not arranged in regular lamellae, forming the corneal stroma. The occurrence of pigment granules among the cellular debris suggested that the cells were endothelial cells. The corrected distance visual acuity improved from 20/50 to 20/30 in the right eye (+ 0.00/- 1.75/157°) and from 20/60 to 20/30 in the left eye (+ 0.00/- 1.75/33°), with significant improvement in light sensitivity.
The clinical and ultrastructural findings seem to be an unusual variant of the typical characteristic appearance of a PPCD. This case demonstrates that Triple-DMEK is feasible even in very advanced dystrophic changes of the posterior corneal surface, with good morphological and functional results.
报告一例严重的后多形性角膜后部疣状营养不良（PPCD）合并白内障，采用 Descemet 膜内皮角膜移植术（DMEK）联合白内障手术作为三联手术（Triple-DMEK）进行治疗。
一名 62 岁女性患者因双眼晚期 PPCD 合并白内障和进行性光敏感度下降就诊于我院。临床检查显示出不寻常的临床表现，角膜后表面有明显的疣状病变，但无角膜失代偿。我们对同时合并白内障的患者进行了 Triple-DMEK 手术。对角膜组织进行了光镜和透射电镜检查。
术中行经典的撕囊后，疣状结构难以完全清除。光镜检查显示角膜内皮免疫染色（角蛋白 AE1/3 染色）呈上皮样转化。透射电镜显示 Descemet 膜增厚（18.5 至 30.0 μm）。前带状层结构正常，轻度增厚（3.5 至 5.5 μm）。可见正常的后非带状层（PNBL），但变薄（2.5 至 4.0 μm）或缺失。其后是异常的 PNBL，伴异常纤维状内含物，显著且多变性增厚（11.0 至 24.5 μm）。角膜内皮变性，部分缺失，呈上皮样改变。结节性病变由少数变性细胞组成，嵌入无定形细胞外基质中，其间散布有胶原纤维，这些纤维未排列成规则的层状，形成角膜基质。细胞碎片中存在色素颗粒提示这些细胞为内皮细胞。右眼矫正视力从 20/50 提高到 20/30（+0.00/-1.75/157°），左眼从 20/60 提高到 20/30（+0.00/-1.75/33°），光敏感度显著提高。
临床和超微结构表现似乎是一种不典型的 PPCD 典型特征表现的变体。本例表明，即使在后角膜表面严重营养不良改变的情况下，Triple-DMEK 也是可行的，具有良好的形态和功能结果。
