A Case Report of Successful Cataract Surgery in Theil-Behnke Corneal Dystrophy: A Visual Rehabilitation for the Patient.

IF 0.5 Q4 OPHTHALMOLOGY

Case Reports in Ophthalmology Pub Date : 2025-03-28 eCollection Date: 2025-01-01 DOI:10.1159/000544846

Radhika Paranjpe, Varsha Manade, Preethi Abraham, Khushboo Goyal, Surbhi Chodvadiya

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引用次数: 0

Abstract

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 3 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, 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.

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角膜营养不良患者白内障手术成功1例：视力康复。

Thiel-Behnke角膜营养不良是一种罕见的遗传性疾病，其特征是对称的角膜上皮下混浊，逐渐降低视力。它是一种常染色体显性遗传的上皮间质TGFB1营养不良，主要导致视力障碍。病例介绍：本病例报告描述了一名50岁出头的女性，在过去的3个月内左眼进行性视力丧失，并有儿童期复发性角膜糜烂史。裂隙灯检查显示双眼角膜营养不良，符合Thiel-Behnke营养不良的特征。诊断通过前段光学相干断层扫描证实，在鲍曼层显示一个独特的锯齿状图案。患者因营养不良而接受了保守治疗，并因左眼白内障接受了手术治疗，视力有了显著改善。结论：尽管罕见，但Thiel-Behnke营养不良合并白内障可严重损害视力，早期诊断和持续监测对防止视力进一步下降至关重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Case Reports in Ophthalmology OPHTHALMOLOGY-

CiteScore

0.90

自引率

0.00%

发文量

129

审稿时长

12 weeks

期刊介绍： This peer-reviewed online-only journal publishes original case reports covering the entire spectrum of ophthalmology, including prevention, diagnosis, treatment, toxicities of therapy, supportive care, quality-of-life, and survivorship issues. The submission of negative results is strongly encouraged. The journal will also accept case reports dealing with the use of novel technologies, both in the arena of diagnosis and treatment. Supplementary material is welcomed. The intent of the journal is to provide clinicians and researchers with a tool to disseminate their personal experiences to a wider public as well as to review interesting cases encountered by colleagues all over the world. Universally used terms can be searched across the entire growing collection of case reports, further facilitating the retrieval of specific information. Following the open access principle, the entire contents can be retrieved at no charge, guaranteeing easy access to this valuable source of anecdotal information at all times.