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Retinal Arterial Tortuosity in Moyamoya Disease

Diana Katsman, MD, PhD1; Michael A. Klufas, MD1; David Sarraf, MD2; SriniVas Sadda, MD3
[+] Author Affiliations
1Retina Division, Stein Eye Institute, University of California, Los Angeles
2Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California, Los Angeles
3Doheny Eye Institute, University of California, Los Angeles
JAMA Ophthalmol. 2016;134(1):111-114. doi:10.1001/jamaophthalmol.2015.4645.
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This case report describes retinal arterial tortuosity associated with moyamoya disease.

Moyamoya disease is a cerebrovascular disorder of unknown etiology that is characterized by bilateral progressive stenosis of the distal internal carotid artery and proximal anterior and middle cerebral arteries, which may result in transient ischemic attacks or strokes.1 Although it is not primarily an eye disorder, multiple ocular conditions including morning glory optic disc anomaly, chorioretinal coloboma, anterior ischemic optic neuropathy, ocular ischemic syndrome, and retinal vascular occlusions among others have been associated with the disease. We report a case of moyamoya disease in a young woman with unique retinal arterial vascular changes.

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Figure 1.
Multimodal Retinal Imaging

A and B, Fundus photographs demonstrate abnormal retinal arteries. C and D, Fluorescein angiography shows normal vascular filling. E and F, Spectral-domain optical coherence tomography (SD-OCT) reveals focal elevations at the vitreoretinal interface consistent with corkscrew vessels. G and H, Angioflow optical coherence tomography (OCT) and en face OCT (8 × 8 mm) confirm the multilevel twisting of retinal arteries.

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Figure 2.
Magnetic Resonance Imaging and Angiography

A, Axial T2-weighted magnetic resonance imaging reveals hyperintensity (arrowheads) consistent with chronic ischemia. A indicates anterior; P, posterior. B, Magnetic resonance angiography demonstrates stenosis of the right internal carotid artery (white arrowhead) and no flow in the right vertebral artery (black arrowhead).

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