Wyburn-Mason Syndrome: A Case Report

Chang Hyun Shin; Su Na Lee

doi:10.3341/jkos.2010.51.1.145

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Shin and Lee: Wyburn-Mason Syndrome: A Case Report

Original Article

J Korean Ophthalmol Soc 2010;51(1):145-149.

Published online: 7 September 2010

DOI: https://doi.org/10.3341/jkos.2010.51.1.145

Wyburn-Mason Syndrome: A Case Report

Chang Hyun Shin, MD, Su Na Lee, MD

Department of Ophthalmology, Eulji University Hospital, College of Medicine, Daejeon, Korea

Address reprint requests to Su Na Lee, MD Department of Ophthalmology, Eulji University Hospital, College of Medicine #1306 Dunsandong, Seo-gu, Daejeon 302-799, Korea Tel: 82-42-611-3000, Fax: 82-42-611-3145, E-mail: snlee@eul.ji.ac.kr

Received 13 August 2009 Accepted 17 November 2009

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

To describe a case of Wyburn-Mason Syndrome, which is characterized by arteriovenous malformations in the central nervous system and the retina.

Case summary:

A 13-year-old girl who underwent neurosurgical intervention for intraventricular hemorrhage was referred to our ophthalmic clinic because brain angiogram and MRI finding revealed arteriovenous malformations (AVMs) that extended from the orbit and optic chiasm along the optic pathway. Fundus and fluorescein angiography of the left eye showed marked dilation and tortuosity of the retinal vessels. Fluorescein angiographic findings showed rapid filling of all vessels and no dye leakage.

Conclusions

Retinal racemose hemangioma should be considered for Wyburn-Mason syndrome, and the finding of retinal AVMs should warrant cerebral imaging studies including MRI and angiography.

Keywords: Wyburn Mason syndrome, Arteriovenous malformation, Retinal racemose hemangioma

References

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Figure 1.

Angiograms of the left internal carotid artery (A: Anteroposterior view, B: Right antero-oblique view, C & D: Lateral view). There are AVMs that extends from the orbit and optic chiasm along the optic pathway to the midbrain (o: Ophthalmic artery, i: Internal carotid artery, AVM: AV malformations)

Figure 2.

Magnetic resonance imaging shows aggregated enhancing structures (yellow arrow) in the left central orbital space and suprasellar cistern, and around the supraclinoid internal carotid artery.

Figure 3.

Color fundus photograph of the left eye shows marked dilation and tortuosity of the retinal vessels, “Bag of Worms”

Figure 4.

Fluorescein angiograms of the left eye. In the arterial phase, Rapid arterial filling and immediately emptying of fluorescein dye into the vein through the various A-V communications are seen. In the late phase, No leakage is evident.

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