Long-Term Functional and Clinical Outcomes in Familial Exudative Vitreoretinopathy.

IF 2.3 2区医学 Q2 OPHTHALMOLOGY

Retina-The Journal of Retinal and Vitreous Diseases Pub Date : 2024-12-20 DOI:10.1097/IAE.0000000000004372

Hongan Chen, Anne Strong Caldwell, Jennifer L Patnaik, Eric G Feinstein, Maryam Ghiassi, Marc T Mathias, Scott C N Oliver

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Abstract

Purpose: To characterize long-term functional and anatomical outcomes in patients with familial exudative vitreoretinopathy (FEVR).

Methods: A retrospective chart review was conducted of all patients with a diagnosis of FEVR at a tertiary academic institution and its affiliated children's hospital treated from January 2003 through January 2024. Demographic and clinical data were collected. Functional vision was defined as at least one eye with Snellen visual acuity (SVA) ≥20/40 and ambulatory vision defined as at least one eye with SVA ≥20/200.

Results: A total of 186 eyes from 96 patients comprised the FEVR cohort, of which 90 eyes from 46 patients were included for analysis. Average age at presentation was 6.4 years (SD 8.6) and average duration of follow-up was 9.1 years (SD 7.3, range 0-40).At most recent exam, 77.3% of patients had functional vision and 88.6% had ambulatory vision. Globe salvage was achieved in 98.4% of eyes. Of the exam findings studied, only retinal detachment at presentation was significantly associated with worse vision in multivariable analysis.

Conclusions: Patients in our FEVR cohort show promising long-term functional and ambulatory vision outcomes. More than 75% of patients were 20/40 or better, and 89% of patients avoided legal blindness.

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家族性渗出性玻璃体视网膜病变的长期功能和临床结果。

目的：探讨家族性渗出性玻璃体视网膜病变（FEVR）患者的长期功能和解剖结果。方法：回顾性分析2003年1月至2024年1月在某高等教育机构及其附属儿童医院就诊的所有诊断为出血热的患者。收集了人口统计学和临床数据。功能视力定义为至少一只眼Snellen视敏度(SVA)≥20/40，动态视力定义为至少一只眼SVA≥20/200。结果：来自96例患者的186只眼睛组成了FEVR队列，其中46例患者的90只眼睛被纳入分析。平均发病年龄为6.4岁（SD 8.6），平均随访时间为9.1年（SD 7.3，范围0-40）。在最近的一次检查中，77.3%的患者具有功能性视力，88.6%的患者具有活动视力。98.4%的眼成功挽救眼球。在所研究的检查结果中，在多变量分析中，只有出现时的视网膜脱离与视力恶化显著相关。结论：我们的FEVR队列患者显示出有希望的长期功能和动态视力结果。超过75%的患者达到20/40或更好，89%的患者避免了法定失明。

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

Retina-The Journal of Retinal and Vitreous Diseases 医学-眼科学

CiteScore

5.70

自引率

9.10%

发文量

554

审稿时长

3-6 weeks

期刊介绍： RETINA® focuses exclusively on the growing specialty of vitreoretinal disorders. The Journal provides current information on diagnostic and therapeutic techniques. Its highly specialized and informative, peer-reviewed articles are easily applicable to clinical practice. In addition to regular reports from clinical and basic science investigators, RETINA® publishes special features including periodic review articles on pertinent topics, special articles dealing with surgical and other therapeutic techniques, and abstract cards. Issues are abundantly illustrated in vivid full color. Published 12 times per year, RETINA® is truly a “must have” publication for anyone connected to this field.