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.
Fundus photography (A, right eye;
B, left eye) on initial ocular examination showing disc edema and vascular
sheathing predominantly in the right eye in association with a depigmented
cream-colored area in the nasal area of the fundus.
Fluorescein fundus angiography
of the right eye on initial ocular examination. Late-phase fluorescein angiogram
showing marked diffuse leakage of fluorescein and disc edema.
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.
Late-phase fluorescein angiogram
(at 690 seconds, right eye), obtained 2 years and 4 months after onset of
the disease, showing numerous retinal hemorrhages, tortuosity of retinal veins,
diffuse leakage of fluorescein, and marked disc edema.
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
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
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
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
Fajnkuchen F, Giraud C, Gatinel D, Chaine G. Central Retinal Vein Occlusion in Bird-Shot Retinochoroidopathy. Arch Ophthalmol. 2002;120(7):987-989. doi: