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JAMA Ophthalmology Clinical Challenge |

Retinal Vasculitis, Aneurysms, and Neovascularization in Blau Syndrome—Quiz Case

Sejal R. Amin, MD; Jose S. Pulido, MD
JAMA Ophthalmol. 2013;131(5):677-679. doi:10.1001/jamaophthalmol.2013.413a.
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Extract

A 6-year-old white boy presented to our office for evaluation after failing 3 school vision screenings. On initial presentation, the patient's visual acuity was 20/800 OD and 20/20 OS with a right afferent pupillary defect. He saw 0/14 Ishihara color plates with his right eye and 13/13 with his left eye. A slitlamp examination was notable for rare cells in the anterior chamber of both eyes, in addition to 3+ cells and hemorrhage within the right vitreous, and rare vitreous cells were found in the left eye. A fundoscopic examination of the right eye revealed indistinct disc margins, neovascularization, extensive vascular sheathing, and obliteration of peripheral vessels. The left fundus had distinct disc margins, but there was marked disc neovascularization and arteriolar aneurysms.

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Figure 1. Spectralis optical coherence tomography revealing cystoid macular edema in the right eye (A) and normal foveal contour and retinal integrity in the left eye (B).

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Figure 2. Fluorescein angiogram (left) and indocyanine green angiogram (right) of the right eye at 21 seconds (A) and at 5 minutes, 44 seconds (B), highlighting neuroretinitis, capillary dropout, and aneurysms at arteriolar bifurcations. I indicates inferior; L, left; R, right; and S, superior.

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Figure 3. Fluorescein angiogram (left) and indocyanine green angiogram (right) of the periphery of the left eye at 2 minutes, 58 seconds, exhibiting extensive capillary dropout. I indicates inferior; L, left; R, right; and S, superior.

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Figure 4. Fluorescein angiogram (left) and indocyanine green angiogram (right) of the left eye centered over the disc at 8 minutes, 45 seconds, highlighting neuroretinitis and aneurysmal dilatation. I indicates inferior; L, left; R, right; and S, superior.

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The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
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