Supplementary Material for: A CASE REPORT OF SUCCESSFUL CATARACT SURGERY IN THEIL-BEHNKE CORNEAL DYSTROPHY: A VISUAL REHABILITATION FOR THE PATIENT

Introduction Thiel-Behnke corneal dystrophy is a rare inherited condition characterized by symmetrical subepithelial corneal opacities that gradually reduce vision. It is an autosomal dominant inherited epithelial stromal TGFB1 dystrophy that mainly causes visual impairment. Case Presentation This case report describes case of a woman in her early 50s with progressive vision loss in her left eye over the past three months with a history of recurrent corneal erosions in childhood. Examination on a slit lamp showed corneal dystrophy affecting both eyes, matching the features of Thiel-Behnke dystrophy. Diagnosis was confirmed with Anterior Segment - Optical Coherence Tomography (AS-OCT), revealing a distinctive sawtooth pattern in Bowman's layer. The patient was managed conservatively for her dystrophy and was also surgically treated for her cataract in the left eye which showed a significant improvement in her vision. Conclusion Despite its rarity, Thiel-Behnke dystrophy along with cataract can severely impair vision, making early diagnosis and continuous monitoring crucial for preventing further visual decline.

引言 蒂尔-贝克角膜营养不良（Thiel-Behnke corneal dystrophy）是一种罕见的遗传性疾病，以对称性上皮下角膜混浊为典型特征，可导致视力进行性减退。该疾病属于常染色体显性遗传的上皮基质型TGFB1营养不良，主要造成视力损害。病例报告 本病例报告描述了一位50岁出头的女性患者，其近3个月来左眼出现进行性视力下降，既往童年时期有复发性角膜糜烂病史。裂隙灯检查显示双眼罹患角膜营养不良，表现符合蒂尔-贝克角膜营养不良的特征。通过前节光学相干断层扫描（Anterior Segment-Optical Coherence Tomography，AS-OCT）确诊，可见鲍曼层（Bowman's layer）呈现特征性锯齿样改变。患者针对角膜营养不良接受保守治疗，同时因左眼白内障接受手术治疗，术后视力获得显著改善。结论 尽管蒂尔-贝克角膜营养不良较为罕见，但该疾病与白内障合并存在时可严重损害视力，因此早期诊断与持续监测对预防视力进一步下降至关重要。