Progressive macular ischemia in retinal vasculopathy with cerebral leukodystrophy

Author:

Kuo Che-Yuan1ORCID,Lin Po-Kang12,Soong Bin-Wen345,Chen Shih-Jen12ORCID

Affiliation:

1. Department of Ophthalmology, Taipei Veterans General Hospital, Taipei

2. Department of Ophthalmology, School of Medicine, National Yang Ming Chiao Tung University, Hsinchu

3. Taipei Neuroscience Institute, Taipei Medical University, Taipei

4. Department of Neurology, Taipei Medical University-Shuang Ho Hospital, New Taipei City

5. Department of Neurology, Taipei Veterans General Hospital, Taipei

Abstract

Purpose: We present a case of retinal vasculopathy with cerebral leukodystrophy and review the usefulness of optical coherence tomography angiography (OCT-A) in the assessment of long-term outcomes. Case description: A 31-year-old woman developed sudden-onset scotoma in her right eye. Fundus examination and fluorescein angiography showed a patch of soft exudate and capillary nonperfusion in the posterior pole and outside the vascular arcades. OCT-A revealed that the initial vessel density (VD) of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) of the right eye were 32% and 49.2%, respectively. Interestingly, over time, the VD of the SCP and DCP gradually decreased to 23.1% and 26.2%, respectively. In contrast, the initial VD of the SCP and DCP of the left eye were both stable at 44.3% and 56.2%, respectively, and only decreased slightly to 39.3% and 45.7%, respectively, over time. The average VD loss of the SCP and DCP, assessed over 1 year, was 8% and 13%, respectively, in the right eye, and 3% and 6%, respectively, in the left eye. Conclusion: Based on this case report, in which we demonstrated a long-term decline in VD of the macula in a young woman with mild retinal vasculopathy with cerebral leukodystrophy, we suggest that there is a potential and valuable role for OCT-A in this rare disease.

Publisher

SAGE Publications

Subject

Ophthalmology,General Medicine

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