History of Present Illness
A 32-year-old healthy female with no past medical history presents to the eye clinic complaining of painless blurred vision, especially in the left eye (OS). She has been noticing progressive changes of vision in the past year. She has a history of retinal laser for idiopathic aneurysms in the OS a few years ago at another eye center ( Figs. 64.1 and 64.2 ).
| OD | OS | |
|---|---|---|
| Visual acuity | 20/25 | 20/200 |
| Intraocular pressure (IOP) | 15 | 15 |
| Sclera/conjunctiva | White and quiet | White and quiet |
| Cornea | Clear | Clear |
| Anterior chamber (AC) | Deep and quiet | Deep and quiet |
| Iris | Unremarkable | Unremarkable |
| Lens | Clear | Clear |
| Anterior vitreous | 1+ white cells | 1+ white cells |
| Mild disc swelling, peripapillary flame hemorrhage | Leaking macroaneurysms surrounded by circinate exudates | |
| Fig. 64.1A, 64.2A | Fig 64.1B, 64.2B |
Questions to Ask
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Complete review of systems, particularly assessing for the presence of vascular disease risk factors, systemic vasculitis, and infectious etiologies.
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Were you ever diagnosed with deep venous thrombosis, stroke, miscarriage, or any other blood clotting issue?
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Is there any history of inflammatory conditions or coagulation disorders in the family?
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Were you ever treated with radiotherapy?
Assessment
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Bilateral mild optic disc edema with multiple leaking aneurysmal dilatations of retinal arterioles. Bilateral epiretinal membrane with exudation and intraretinal fluid at the macula OS. Peripheral capillary dropout bilaterally with neovascularization elsewhere (NVE) OS.
Differential Diagnosis
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Senile acquired macroaneurysms, Eales disease, Coat disease, radiation-induced retinopathy, Leber miliary aneurysm, collagen vascular disorders, antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis, hypertensive retinopathy
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Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN—a diagnosis of exclusion)
Working Diagnosis
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IRVAN syndrome
Testing
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Fluorescein angiography is a useful test that can highlight several findings in IRVAN:
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Aneurysmal arteriolar dilatations
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Late staining of the optic disc
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Areas of capillary dropout
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Neovascularization (at the optic disc or elsewhere in the retina)
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Capillary and arteriolar leakage
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Optical coherence tomography (OCT) of the optic disc and macula allows visualization of arteriolar aneurysms, macular exudation, and macular intraretinal ( Fig. 64.3 ).
