Tomioka Yasufumi, Tanaka Hiroshi, Sotozono Chie, Kinoshita Shigeru
Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Department of Frontier Medical Science and Technology for Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Am J Ophthalmol Case Rep. 2024 Aug 22;36:102146. doi: 10.1016/j.ajoc.2024.102146. eCollection 2024 Dec.
This report aims to document a long-term follow-up of a rare case of Brown-McLean syndrome where high-density central corneal endothelial cells (CECs) were maintained, yet the peripheral area developed corneal edema.
A 72-year-old Japanese male was referred to our hospital. He had a history of three instances of rhegmatogenous retinal detachment in the right eye, requiring three intraocular surgeries. While the circumferential corneal peripheral regions, predominantly in the nasal and temporal areas, exhibited corneal edema, the central region remained clear. The central corneal thickness was measured at 541 μm, and the thickness in the nasal and temporal regions exceeded 700 μm. CEC density at the center was determined to be 2499 cells/mm using a non-contact specular microscope, but imaging of the nasal and temporal cornea was obscure due to corneal edema. Panoramic images of the corneal endothelial layer, captured via slit-scanning wide-field contact specular microscopy, showed a high-density and uniform distribution of cells across the clear corneal region, close to the border between edematous and non-edematous areas. Twelve years after the initial visit, the central cornea maintained its transparency and a CEC density of 2456 cells/mm. Despite the entire peripheral corneal edema, there was no noted deterioration from twelve years prior. The annual reduction in central cornea CECs was a mere 0.09 %.
This case implies the unintended formation of a biological barrier at the border or a possible lack of CEC affinity of centrifugal movement, among other unidentified factors. Such factors may hinder the migration of CECs from the central to the peripheral regions of the cornea, a discovery that is crucial for advancing our knowledge of corneal endothelial biology and exploring new treatment options.
本报告旨在记录一例罕见的布朗 - 麦克林综合征的长期随访情况，该病例中高密度的中央角膜内皮细胞（CECs）得以维持，但周边区域出现了角膜水肿。
一名72岁的日本男性被转诊至我院。他有右眼三次孔源性视网膜脱离病史，需进行三次眼内手术。虽然角膜周边圆周区域，主要是鼻侧和颞侧区域出现了角膜水肿，但中央区域保持清晰。中央角膜厚度测量为541μm，鼻侧和颞侧区域的厚度超过700μm。使用非接触式镜面显微镜测定中央CEC密度为2499个细胞/mm，但由于角膜水肿，鼻侧和颞侧角膜的成像模糊。通过裂隙扫描宽视野接触式镜面显微镜拍摄的角膜内皮层全景图像显示，在靠近水肿和非水肿区域边界的透明角膜区域，细胞呈高密度且均匀分布。初诊12年后，中央角膜保持透明，CEC密度为2456个细胞/mm。尽管整个周边角膜水肿，但与12年前相比没有明显恶化。中央角膜CECs的年减少率仅为0.09%。
该病例提示在边界处意外形成了生物屏障，或者可能存在离心运动的CEC亲和力缺乏等其他未明确的因素。这些因素可能会阻碍CECs从角膜中央向周边区域的迁移，这一发现对于增进我们对角膜内皮生物学的认识和探索新的治疗选择至关重要。
