Radius-Maumenee Syndrome Presenting with Ocular Pain and Conjunctival Injection: A Case Report

Byeong Soo Kang; Eun Ji Lee; Hee Kyung Yang

doi:10.3341/jkos.2015.56.12.1969

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Kang, Lee, and Yang: Radius-Maumenee Syndrome Presenting with Ocular Pain and Conjunctival Injection: A Case Report

Original Article

J Korean Ophthalmol Soc 2015;56(12):1969-1973.

Published online: 29 August 2015

DOI: https://doi.org/10.3341/jkos.2015.56.12.1969

Radius-Maumenee Syndrome Presenting with Ocular Pain and Conjunctival Injection: A Case Report

Byeong Soo Kang, M.D¹, Eun Ji Lee, M.D^1,², Hee Kyung Yang, M.D^1,^2,

¹Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea

²Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Korea

Address reprint requests to Hee Kyung Yang, MD Department of Ophthalmology, Seoul National University Bundang Hospital, #82 Gumi-ro 173beon-gil, Bundang-gu, Seongnam 13620, Korea Tel: 82-31-787-7370, Fax: 82-31-787-4057 E-mail: eye@snubh.org

Received 17 April 201516 July 2015 Accepted 22 September 2015

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

Radius-Maumenee syndrome (RMS) is characterized by idiopathic dilated episcleral vessels usually associated with glaucoma. The authors report a case of a 32-year-old Korean male with dilation of the episcleral vessels and glaucoma in both eyes.

Case summary

A 32-year-old Korean male presented with conjunctival injection and chronic pulsatile ocular pain in both eyes for 11 years. His best corrected visual acuity was 20/20 in both eyes. Slit lamp biomicroscopy showed episcleral venous en-gorgement in both eyes. Fundus photographs revealed superotemporal and inferotemporal retinal nerve fiber layer defects and Humphrey visual field testing showed superior nasal steps and paracentral scotomas in both eyes. He suffered from chronic eye pain in both eyes although he had applied glaucoma medication and his symptoms had worsened during the past year. Brain magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed no evidence of carotid cavernous fis-tula or other orbital lesions. Due to the presence of episcleral venous engorgement, glaucoma and negative tests for other possi-ble diseases, he was diagnosed with RMS.

Conclusions

RMS is an idiopathic disease with episcleral vessel dilation and frequently associated with glaucoma. Its diagnosis is confirmed by eliminating other possible causes of episcleral venous engorgement.

Keywords: Dilated episcleral vein, Open angle glaucoma, Radius-Maumenee syndrome

References

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3. Stock RA, Fernandes NL, Pastro NL. . Idiopathic dilated epis-cleral vessels (Radius-Maumenee syndrome): case report. Arq Bras Oftalmol. 2013; 76:45–7.

4. Parikh RS, Desai S, Kothari K. Dilated episcleral veins with secon-dary open angle glaucoma. Indian J Ophthalmol. 2011; 59:153–5.

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Figure 1.

Anterior segment photographs of both eyes. (A, B) Right eye shows mild episcleral vessel engorgement. (C, D) Left eye shows prominent episcleral vessel engorgement.

Figure 2.

Red-free retinal nerve fiber layer photographs of both eyes. (A) The right eye shows superotemporal and inferotemporal retinal nerve fiber layer defects. (B) The left eye shows superotemporal and inferotemporal retinal nerve fiber layer defects, tortuous retinal vessels, optociliary shunt vessels and disc hemorrhage.

Figure 3.

Optical coherence tomography of both eyes. Definite inferotemporal retinal nerve fiber layer defects and relative superior temporal retinal nerve fiber layer thinning are found in both eyes. OD = oculus dexter; OS = oculus sinister; NAS = nasal; INF = inferior; TMP = temporal; SUP = superior; T = temporal; S = superior; N = nasal; I = inferior; TS = superotemporal; NS = superonasal; G = general; TI = inferotemporal; NI = inferonasal; PMB = papillomacular bundle; N/T = nasal/temporal ratio.

Figure 4.

Humphrey visual field testing of both eyes. Superior nasal steps and paracentral scotomas are found in both eyes. (A) Right eye (B) Left eye. SITA = Swedish Interactive Threshold Algorithm; ASB = apostilb; GHT = glaucoma hemifield test; VFI = visu-al field index; MD = mean deviation; PSD = pattern standard deviation.

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