2Ophthalmic Consultants of Boston, Boston MA
Methods: Retrospective single case report. Patient: A 27-year-old female presented with decreased vision in her left eye for the past year. On exam, her visual acuity for the left eye was 20/50. She has had trouble accessing medical care but denies any illness, infections or trauma.
Results: Upon ophthalmic examination, retinal abnormalities were seen such as the presence of peripheral vessel sheathing, peripheral non-perfusion and extensive neovascularization. Fluorescein angiography revealed significant late leakage of the retina vessels with greater severity in the left eye. Laboratory findings were unremarkable. After diagnosis of primary retinal vasculitis, the patient was treated with photocoagulation in both eyes. She also received two treatments of intravitreal bevacizumab for the neovascularization.
Conclusion: Primary retinal vasculitis is often hard to diagnose due to disease course variation. The cause of retinal vasculitis must first be established as either an isolated condition or due to an inflammatory systemic condition. A thorough review of the patient's medical history, review of systems, ophthalmic and physical examination, along with proper laboratory tests must be performed in order to ensure accurate diagnosis and treatment.
Keywords: Primary retinal vasculitis; Eales' disease; Perivascular sheathing,; Vascular leakage; Neovascularization
Clinically, patients with retinal vasculitis most commonly present with blurred vision, altered color perception, distortion of images, floaters and scotomas. However, sometimes patients may present with no visual symptoms. Retinal vasculitis can be detected using ophthalmologic examinations and fluorescein angiography, which can show perivascular sheathing, vascular leakage, inflammatory cells of the vitreous body, narrowing of the retinal blood vessels, and neovascularization. Late leakage or staining of retinal vessels is also evident on FA. Sometimes, mild anterior or posterior uveitis may be seen. The presence of choroidal inflammation may indicate sarcoidosis or birdshot retinochoroidopathy [1, 6] Active vasculitis causes the formation of exudates around retinal vessels, which results in white sheathing or cuffing of the vasculature. This can lead to vascular leakage, which causes retinal swelling and macular edema that can greatly affect visual acuity and lead to vision loss. Visual acuity prognosis is difficult to anticipate due to complications such as macular ischemia, branch and central retinal vein occlusion, persistent neovascularization and vitreous hemorrhages.
Secondary retinal vasculitis due to systemic diseases more often leads to retinal occlusion, rather than vascular sheathing. The retinal arterioles or branch retinal arteries can become occluded by microthrombosis without significant intraocular inflammation, a difference from active vasculitis [2, 3].
On exam, her vision was 20/20 OD and 20/50 OS with symmetrically reactive pupils and normal intraocular pressures. Anterior segment exam was normal. However, posterior segment exam of the right eye showed peripheral vessel sheathing with peripheral non-perfusion. The left eye showed extensive neovascularization of the optic disc, central and peripheral non-perfusion, along with intraretinal microvascular anomalies (Figure 1). Fluorescein angiography of both eyes revealed significant late leakage of the retina vessels with the left eye to a greater degree (Figure 2b). Spectral Domain OCT showed appropriate foveal contour with no macular edema or subretinal fluid. The following laboratory tests were performed: CBC with peripheral smear, PPD, Quantiferon, ACE, lysozyme, ESR, RPR, ANA, and a chest x-ray. All findings were negative. The patient was also found to be negative for diabetes, hypertension, HIV and TB.
Due to disease course variation, the poor outcome of primary retinal vasculitis is dependent on a number of factors. To control inflammation of the retinal vasculature, the main treatment option for primary retinal vasculitis often involves the use of corticosteroids with or without immunosuppressive therapy. Cyclosporine and azathioprine have shown to be effective in reducing inflammation. Anti-tuberculosis treatment is given for positive TB patients, which is more often seen in individuals with Eales' disease. Laser photocoagulation is often combined with bevacizumab intravitreal injections to treat neovascularization and prevent further retinal ischemia [2, 4]. Our patient responded well to panretinal photocoagulation of the left eye and scatter photocoagulation of the right eye. Additionally, the extensive neovascularization of the disc regressed with two treatments intravitreal bevacizumab over a 6-month period. The prognosis of patients with primary retinal vasculitis is variable due to the heterogenic nature of the disease. Some patients that undergo treatment have full preservation of their ocular function and vision. However, others may have permanent loss of function despite treatment. Current status of patient is unknown. Due to her return to Saudi Arabia, she has been lost to follow-up.
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