Hemi-central Retinal Vein Occlusion in a Patient With Neurofibromatosis 1

Yeon Hee Lee; Kyoung Nam Kim; Kyoung Sup Shin; Jung Yeul Kim

doi:10.3341/jkos.2010.51.1.150

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Lee, Kim, Shin, and Kim: Hemi-central Retinal Vein Occlusion in a Patient With Neurofibromatosis 1

Original Article

J Korean Ophthalmol Soc 2010;51(1):150-154.

Published online: 7 September 2010

DOI: https://doi.org/10.3341/jkos.2010.51.1.150

Hemi-central Retinal Vein Occlusion in a Patient With Neurofibromatosis 1

Yeon Hee Lee, MD^1,², Kyoung Nam Kim, MD¹, Kyoung Sup Shin, MD¹, Jung Yeul Kim, MD^1,²

¹Department of Ophthalmology, College of Medicine, Chungnam National University, Daejeon, Korea

²Chungnam National University Research Institute for Medical Sciences, Daejeon, Korea

Address reprint requests to Jung Yeul Kim, MD Department of Ophthalmology, Chungnam National University Hospital #640 Daesa-dong, Jung-gu, Daejeon 301-721, Korea Tel: 82-42-280-8433, Fax: 82-42-255-3745, E-mail: kimjy@cnu.ac.kr

Received 29 April 2009 Accepted 1 September 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

We report on a case of hemi-central retinal vein occlusion (Hemi-CRVO) which occurred in a patient with Type 1 neurofibromatosis.

Case summary:

A 31-year-old woman presented with a chief complaint of a one-month history of decreased visual acuity in the left eye. The patient had Type 1 neurofibromatosis but had no other systemic diseases. The best corrected visual acuity (BCVA) was 0.8 in the right eye and 0.05 in the left eye. Multiple Lisch nodules were present in the irises of both eyes on slit lamp examination. Fundus examination revealed a hemi-central retinal artery occlusion in the left eye. Optical coherence tomograms showed that the central macular thickness was 192 microns in the right eye and 732 microns in the left eye. There was cystoid macular edema in the left eye. A fluorescein angiogram showed delayed filling in the superior branch retinal vein of the left eye. There was an extensive area of non-perfusion of the retinal capillary in the corresponding area, including the perifoveal area. Blood tests for hyper-coagulability, autoimmune disorders, connective tissue disorders and other tests for the brain and carotid artery and coronary artery disease showed no abnormal findings. The clinical course was monitored following an intravitreal injection of bevacizumab (Avastin^®). Eight months later, the BCVA had improved to 0.2.

Conclusions

Although it is rare, hemi-CRVO occurring in younger patients who have no underlying diseases could be related to neurofibromatosis.

Keywords: Hemi-central retinal vein occlusion, Neurofibromatosis 1

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Figure 1.

Anterior segment photograph shows multiple Lisch nodules on the iris. (A. right eye: B. left eye)

Figure 2.

(A) Fundus photograph demonstrating features of intraretinal hemorrhage, cotton wool spot and macular thickening of the left fundus. (B) The venous phase fluorescein angiogram demonstrating intraretinal hemorrhage and capillary nonperfusion in the superior two quadrants, including the parafoveal region.

Figure 3.

A. Optical coherence tomography demonstrating prominent retinal thickening in the macular region with retinal cysts and subretinal fluid accumulation. B. 8-month post injection of the Avastin^® demonstrating dramatic resolution of retinal thickening and return of normal foveal contour.

Figure 4.

8-month post injection of Avastin^® demonstrating improvement of intraretinal hemorrhage and cotton wool spot on the left fundus.

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