Central Retinal Vein Occlusion in Bird-Shot Retinochoroidopathy FREE

Visual loss in bird-shot retinochoroidopathy is caused by cystoid macular edema, optic neuropathy and atrophy, vitreous opacities, epiretinal membranes, and subretinal neovascularization. We describe a patient with this syndrome whose visual loss was caused by a central retinal vein occlusion. This may occur as a rare vascular complication of bird-shot retinochoroidopathy.

A 62-year-old woman was evaluated for a 2-day history of blurred vision and floaters in both eyes. Her ocular history was unremarkable, and her medical history was notable for systemic hypertension. Blood pressure was well controlled during treatment. On initial ocular examination, visual acuity was 20/25 OU. Slitlamp examination findings revealed no evidence of anterior chamber inflammation in either eye. Intraocular pressure was 14 mm Hg OU. Fundus examination results revealed bilateral mild vitreitis, retinal periphlebitis, and disc edema in association with depigmented cream-colored spots located predominantly in the nasal area of the fundus (Figure 1). These findings in association with the presence of HLA-A29 led to the diagnosis of birdshot retinochoroidopathy. Fluorescein angiography demonstrated diffuse retinal vasculitis with disc edema (Figure 2). As no visual loss occurred, the patient received no treatment and was put under medical control. Spontaneous remission was noted 2 years after onset, and visual acuity improved to 20/20 OU. Peripheral retinal periphlebitis and disc edema decreased.

After 4 months, the patient experienced a recurrence of uveitis. Disc edema and periphlebitis were exacerbated, and central retinal vein occlusion developed (Figure 3). Visual acuity decreased to 20/100 OD. Afferent pupillary defect was present. No etiologic factor, except very mild systemic hypertension, was found during the follow-up evaluation. A regimen of 60 mg daily of prednisone was administered, with no amelioration in the right eye. Capillary closure occurred. A panretinal laser photocoagulation was performed. Despite this treatment, preretinal and prepapillary neovascularization occurred 4 years after the onset of the vein occlusion. When last seen, 6 years after the first examination, visual acuity was reduced in the right eye to hand motions.

Diffuse and bilateral retinal vasculitis, particularly along the major retinal vessels, and retinal vascular leakage are frequent in birdshot retinochoroidopathy. Vasculopathy leads to cystoid macular edema and papilloedema.^1- 2

Vascular occlusion is known to occur in the presence of vasculitis (up to 5% of branch vein occlusion is owing to vasculitis),³ and vascular occlusion has been described in systemic disease associated with vasculitis.^4- 6 However, cross-referencing "central retinal vein occlusion" and "bird-shot retinochoroidopathy" on the MEDLINE database brings up no items. A 1988 study by Priem and Oosterhuis² of 102 patients with bird-shot retinochoroidopathy reports such an association: 1 central retinal vein occlusion and 2 branch retinal vein occlusions. The authors note that this incidence is higher than that found in a healthy population of the same age.

Two hypotheses may be advanced concerning central retinal vein occlusion associated with birdshot retinochoroidopathy. The obstruction of a small peripheral retinal venule is well documented in vasculitis. Although less common, larger retinal vessels may also become inflamed and subsequently occluded, as occurred in our reported clinical case. Furthermore, in bird-shot retinochoroidopathy, pathological features include granulomatous inflammation in the retina around and under retinal veins and in the underlying choroid.¹

Priem and Oosterhuis have developed another hypothesis: patients with bird-shot retinochoroidopathy have a high incidence of cardiovascular disease (systemic hypertension, coronary artery disease, strokes). Central retinal vein occlusion would then be one of the manifestations of cardiovascular risk.² The risk for this patient of developing vein occlusion was increased by the association of disc edema and absence of an optic cup, which is known to be a risk factor for nonarteritic optic neuropathy.

In conclusion, we believe that as observed in our clinical case, central retinal vein occlusion may occur as a rare vascular complication of bird-shot retinochoroidopathy.

None of the authors has any financial interest in the subject matter discussed in this article.

Corresponding author and reprints: Gilles Chaine, MD, Service d'Ophtalmologie, Hôpital Avicenne, 125 route de Stalingrad, 93009 Bobigny, CEDEX, France (e-mail: gilles.chaine@avc.ap-hop-paris.fr).