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马方综合征相关性青光眼,一种治疗挑战:病例报告。

Radius-Maumenee syndrome-associated glaucoma, a therapeutic challenge: A case report.

机构信息

Department of Ophthalmology, Moorfields Eye Hospital, London, UK.

Department of Ophthalmology, Great Ormond Street Hospital, London, UK.

出版信息

Eur J Ophthalmol. 2024 Sep;34(5):NP37-NP43. doi: 10.1177/11206721241261096. Epub 2024 Jun 11.

Abstract

INTRODUCTION

Radius-Maumenee syndrome is a rare cause of open-angle glaucoma, secondary to elevated episcleral venous pressure (EVP) without any orbital or systemic abnormalities.

CASE PRESENTATION

We present a case of a male patient in his mid-sixties, who presented with bilateral dilated episcleral vessels, bilateral glaucoma, chorioretinal folds in both maculae and choroidal effusion in his left eye. Our case highlights the differentials that should be considered and the systematic investigations that should be performed. We describe the clinical, optical coherence tomography and angiography findings of this patient and propose a potential pathophysiological mechanism leading to the propensity for perioperative complications.

CONCLUSION

Radius-Maumenee syndrome should be a diagnosis of exclusion. Secondary glaucoma can remain refractory to medical treatment and filtering surgery carries the risk intra-operative or post-operative uveal effusions.

摘要

简介

Radius-Maumenee 综合征是一种罕见的开角型青光眼病因,其特征为眼上静脉压(EVP)升高,而无眼眶或全身异常。

病例介绍

我们报告了一例 60 多岁的男性患者,他双侧出现扩张的球结膜血管、双侧青光眼、双眼黄斑部视网膜脉络膜皱褶和左眼脉络膜积液。本病例强调了应考虑的鉴别诊断和应进行的系统检查。我们描述了该患者的临床、光学相干断层扫描和血管造影结果,并提出了导致围手术期并发症易感性的潜在病理生理学机制。

结论

Radius-Maumenee 综合征应作为排除性诊断。继发性青光眼可能对药物治疗仍有抵抗,滤过性手术有术中或术后葡萄膜渗漏的风险。

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