White Dot Syndromes Series Part 4: Birdshot Chorioretinopathy

Mohammad Rafieetary, OD, details clinical findings, disease prognosis, and management strategies for birdshot chorioretinopathy.

Birdshot chorioretinopathy may not be common, but failure to detect and treat the disease may lead to permanent vision loss. In this fourth installment of Optometry Advisor’s White Dot Syndromes Series, Mohammad Rafieetary, OD, details the clinical characteristics, prognostic outcomes, and management strategies associated with this condition. For the previous installment please click here.

Birdshot retinochoroidopathy, also known as birdshot chorioretinopathy (BSCR) or birdshot uveitis, is a bilateral, posterior uveitic disease. This particular white dot syndrome can be identified by the multiple cream- or yellowish-white-colored lesions observed on clinical examination and color fundus photography and confirmed by the presence of HLA-A29 — a human leukocyte antigen (HLA) present in 85 to 95% of individuals with BSCR.1  

Approximately 1 to 2% of uveitis cases involve BCSR and the condition is more prevalent among women who are middle-aged (mean age, 53 years).2 

Clinical Characteristics

In the subacute phase, patients with BSCR present with complaints of floaters, light flashes, and scotomas and experience poor night and color vision and contrast sensitivity.3,4 Vision loss is often progressive. Clinical signs include bilateral, oval-shaped, cream-colored lesions at the retinal pigment epithelium (RPE) and choroid (Figure 1).5  

Credit: Mohammad Rafieetary, OD
Figure 1. Multiple cream-colored lesions are seen here and marked by the arrows. These are images from a man (age, 51 years; White) with vague visual complaints. His diagnosis was confirmed by a positive HLA-A29. 

Anterior uveitis is not commonly seen, but some degree of vitritis is often present.2 Optical coherence tomography (OCT), which shows reduced reflectivity and RPE alterations, can also identify posterior vitreous cells (Figure 2).    

Credit: Mohammad Rafieetary, OD
Figure 2. An OCT of the same patient presented in figure 1 reveals RPE alteration and cells in the vitreous near the macular surface.

Fluorescein angiography (FA) and indocyanine green (ICG) angiography can reveal diffuse choroiditis and retinal vasculitis. ICG can distinctly show the lesions that resemble a pattern created by small shotgun pellets or birdshot (Figure 3).

Credit: Mohammad Rafieetary, OD
Figure 3. The top 4 images reveal retinal vasculitis and multiple choroidal lesions, the latter of which are more evident in the bottom 4 images. 


Systemic corticosteroids are preferred in acute cases of BSCR.2,4 However chronic cases may require immunomodulation with agents such as cyclosporine, methotrexate, or azathioprine.2,4 


Although the scientific community has not reached a consensus on the long-term prognostic outcomes associated with BSCR, research shows that 20% of cases are self-limiting and waxing and waning of the inflammatory response can result in irreversible chorioretinal damage leading to poor visual outcomes.5 Potential long-term complications include macular edema, retinal and choroidal neovascularization, epiretinal membrane, and optic atrophy (Figure 4).6 

Credit: Mohammad Rafieetary, OD, Jeff McMillan, OD
Figure 4. A man (age, 37 years; White) with advance BSCR and positive HLA-A29, (bottom images) shows diffuse pigment mottling OD (bottom left), while OS (bottom right) is remarkable for diffuse RPE atrophy and attenuation of retinal vasculature. Loss of RPE and outer retina is notable on the OCT scans. Visual fields demarcate the loss of visual function. Diffuse pigment mottling (bottom left) and diffuse RPE atrophy and attenuation of retinal vascular can be observed in advanced birdshot chorioretinopathy. 

Case Report

A 53-year-old woman was seen in October of 2020 for evaluation of a choroidal lesion in her left eye. The patient was asymptomatic and in relatively good health. Her visual acuities were 20/20 in each eye and all other findings were unremarkable except for trace nuclear sclerosis cataracts in both eyes and a large amelanotic choroidal nevus in the left eye (Figure 5).

Credit: Mohammad Rafieetary, OD
Figure 5. Fundus photograph and OCT of the right eye demonstrate normal findings. While the left eye disc and macula are normal, a 6.1 mm flat amelanotic choroidal nevus with overlying drusen is seen in the supratemporal retina. The OCT of the lesion (top right) shows no elevation. FAF (bottom right) does not reveal the lesion, except for the overlying drusenoid lesions.

The patient attended a 6-month follow-up visit based on risks associated with the lesion, and all of the findings, including the nevus, remained stable. The patient was asked to follow up with the referring optometrist and return to our clinic in 1 year. The patient returned 16 months later, indicating progressive vision loss for several months which had been attributed to cataract progression. Her best corrected visual acuities were now 20/30 (OD) and 20/40 (OS). While her lens status remained trace nuclear sclerosis, significant changes were noted upon posterior segment examination. Multiple scattered cream-colored choroidal lesions were noted on fundus examination, with OCT scans of both eyes revealing significant outer retinal disease. FA was remarkable for diffuse choroiditis and retinal vasculitis (Figure 6).

Credit: Mohammad Rafieetary, OD
Figure 6: Fundus photos (top) reveal scattered cream-colored ovoid lesions. OCT (middle) RPE and outer retinal changes are shown compared with the previous normal scans. FA (bottom) reveals diffuse choroidal leakage and retinal vessel staining.  

The patient was diagnosed with posterior uveitis with suspicion of BSCR due to the clinical presentation. Oral prednisone (40 mg daily) was prescribed, and the patient was sent for a systemic work-up which confirmed a positive HLA-A29 presence. Vision improved to 20/25 (OD) and 20/20 (OS) at a 2-week follow-up visit and there was a remarkable improvement in posterior segment OCT findings. The patient was referred to a rheumatologist for immunomodulatory therapy and continues to be monitored for ocular symptoms (Figure 7). 

Credit: Mohammad Rafieetary, OD
Figure 7: Here, fundus and OCT images reveal the remarkable improvement following initial systemic steroid therapy.

BSCR is not a commonly encountered clinical finding and failure to provide early intervention can result in poor visual outcomes. Clinicians who are familiar with the disease’s acute phase presentation and serology can provide a speedy diagnosis and timely management strategies to avoid permanent vision loss in their patients. 


  1. Zucchiatti I, Miserocchi E, Sacconi R, Bandello F, Modorati G. HLA-A29-positive uveitis: birdshot chorioretinopathy, what else? Case Rep Ophthalmol. 2013;4(3):287-293. doi:10.1159/000357276
  2. Shah KH, Levinson RD, Yu F, et al. Birdshot chorioretinopathy. Surv Ophthalmol. 2005;50(6):519-541. doi:10.1016/j.survophthal.2005.08.004
  3. Ladas JG, Arnold AC, Holland GN. Control of visual symptoms in two men with birdshot retinochoroidopathy using low-dose oral corticosteroid therapy. Am J Ophthalmol. 1999;128(1):116-118. doi:10.1016/s0002-9394(99)00092-6 
  4. Vitale AT. Birdshot retinochoroidopathy. J Ophthalmic Vis Res. 2014;9(3):350-361. doi:10.4103/2008-322X.143376
  5. Gasch AT, Smith JA, Whitcup SM. Birdshot retinochoroidopathy. Br J Ophthalmol. 1999;83(2):241-249. doi:10.1136/bjo.83.2.241
  6. Priem HA, Oosterhuis JA. Birdshot chorioretinopathy: clinical characteristics and evolution. Br J Ophthalmol. 1988;72(9):646-659. doi:10.1136/bjo.72.9.646