Sunir J. Garg, MD, FACS



• Chronic, bilateral, posterior uveitis

• The characteristic lesions are yellow-white spots at the level of choroid that “radiate” from the disk.

• Over 90% of patients are HLA-A29 positive.



Very rare eye disease


• In the population, it affects <1/200,000.

• It affects 1% of patients in uveitis clinics and 5–7% of all patients with posterior uveitis.


• Most common in Caucasian patients

• Equal male/female distribution

• Mean age of onset ∼50 years old


• No strong familial association (although disease has been reported in monozygotic twins)

• Very strong association with HLA-A29 allele (birdshot has the highest association between HLA class I alleles and any disease)

• 90% of patients with disease have HLA-A29 compared with 7% of general population.




• Likely autoimmune process

• Some similarities between birdshot and murine models of retinal S-antigen-induced uveitis


Autoimmunity, likely involving type IV hypersensitivity


None known



• Gradual, painless vision loss

• Floaters are common.

• Symptoms are usually bilateral.

• Nyctalopia, peripheral visual field constriction, abnormal color vision, photopsias, and photophobia may also occur.


• The eyes are usually painless and appear white and noninflamed.

• The anterior segment has no to mild inflammation.

• A mild to moderate diffuse vitreitis is common.

• The characteristic lesions are cream colored hypopigmented spots in the choroid with relatively indistinct borders.

• These lesions are suggestive of scattering of “birdshot” from a shotgun.

• The choroidal spots occasionally may not appear until later in the disease.

• Cystoid macular edema (CME) is a common cause of decreased visual acuity.

• Retinal vasculitis involving veins and capillaries is sometimes seen, as is optic disk edema.



• None are absolutely required for diagnosis; however, HLA-A29 is a reliable marker of disease (∼95% specificity and sensitivity).

• Must rule out other treatable causes of posterior uveitis, including syphilis, tuberculosis, lyme disease, ocular lymphoma, and sarcoidosis.


Initial approach

• Fluorescein angiography (FA) shows early hypofluorescence and late hyperfluorescence of choroidal lesions, as well as hyperfluorescence of the disk, vascular leakage, and CME (when present).

• Indocyanine green angiography (ICGA) often demonstrates more lesions than seen on either clinical exam or FA. ICG shows early hypofluorescence of choroidal lesions.

• Optical coherence tomography (OCT) can be useful to detect macular edema.

• Electroretinograms (ERG) are often abnormal, typically showing a decrease in both photopic and scotopic amplitudes; B-wave may be affected more than A-wave initially.

• Peripheral visual field testing may show constriction.

Follow-up & special considerations

• FA useful to monitor amount of inflammation and to follow response to therapy

• OCT useful to evaluate patients for macular edema, and for evaluating response to treatment

Pathological Findings

There are limited reports in the literature; however, lymphocytic aggregates in the choroid and retinal vasculature have been noted.


• White dot syndromes (acute posterior multifocal placoid pigment epitheliopathy, multifocal choroiditis and panuveitis, multiple evanescent white dot syndrome)

• Infectious posterior uveitis (tuberculosis, syphilis, and presumed ocular histoplasmosis syndrome)

• Masquerade syndromes (leukemia and ocular lymphoma)

• Sarcoidosis



First Line

• Ocular and systemic steroids have been the mainstay of therapy; however, the typical chronicity of this disease warrants consideration of nonsteroidal immunomodulatory therapy early in the disease course.

• Dramatic improvement of ocular inflammation can often be seen with systemic steroids (prednisone 1 mg/kg per day); however, long-term toxicity of steroids limits this approach.

• Cyclosporine, mycophenolate mofetil, azathioprine, cyclophosphamide, and methotrexate have all been used as nonsteroidal immunomodulatory therapies.

Second Line

• Periocular and intravitreal triamcinolone acetate has been used to treat severe inflammation and/or macular edema.

• More recently, use of antitumor necrosis factor alpha drugs and intravenous immunoglobulin has been reported.


Issues for Referral

• Management of birdshot retinochoroidopathy often requires referral to a uveitis and/or retina specialist.

• Consultation with a rheumatologist or hematologist may be required when immunomodulatory agents are used.


• As with other types of ocular inflammation, cataract formation may be accelerated in patients with birdshot; cataract surgery is best delayed until the patient is free of inflammation for at least 3 months.

• Glaucoma filtration or shunting surgery may be required in some patients with uncontrolled glaucoma.

• In the near future, implantable drug delivery devices may play a major role in the treatment of this disease.


Initial Stabilization

Pulse intravenous steroids may be considered in severe cases with profound bilateral vision loss.



The course of birdshot is often progressive over a number of years, with intermittent exacerbations of disease activity; therefore, patients should be monitored closely by a specialist familiar with the treatment of the disease.

Patient Monitoring

• Visual acuity may be normal until late in the disease course.

• Careful biomicroscopic examination should be undertaken at regular intervals.

• Ancillary testing such as FA, ICGA, OCT, peripheral visual field testing and ERG testing should also be taken at regular intervals to monitor disease activity.


• Patients should be aware of the chronic progressive nature of birdshot chorioretinopathy, and the associated need for close monitoring and long-term treatment of the disease.

• Patients should also be aware that a variety of immunomodulatory treatments are available, and not be discouraged if one treatment modality is insufficient to treat their disease.


• Because of the chronic progressive nature of the disease, visual prognosis is guarded over time.

• Patients often need to be treated with immunomodulatory agents that have side effects.

• Visual function can often be impaired to a greater extent than the visual acuity indicates.


• CME is the most common cause of decreased visual acuity.

• Epiretinal membranes (ERM) are also common.

• Glaucoma may be seen in up to 20% of patients and is likely a secondary effect from systemic and ocular steroid treatments.

• Choroidal neovascular membranes (CNVM) can occur in areas of chronic choroidal inflammation.

• Retinal neovascularization is uncommon, but can result from retinal vascular inflammation and secondary retinal ischemia.

• Optic nerve atrophy is rare, but has been reported.


• Ryan SJ, Maumenee AE. Birdshot retinochoroido-pathy. Am J Ophthalmol 1980;89(1):31–45.

• Jap A, Chee SP. Immunosuppressive therapy for ocular diseases. Curr Opin Ophthalmol 2008;19(6):535–540.

• Monnet D, Brézin AP. Birdshot chorioretinopathy. Curr Opin Ophthalmol 2006;17(6):545–550.

• Kiss S, Anzaar F, Foster SC. Birdshot retinochoroido-pathy. Int Ophthalmol Clin 2006;46(2):39–55.

• Shah KH, Levinson RD, Yu F, et al. Birdshot chorioretinopathy. Surv Ophthalmol 2005;50(6):519–541.



363.20 Chorioretinitis, unspecified


• Chronic, progressive, bilateral posterior uveitis

• Characteristic peripapillary choroidal lesions and associated vitreitis are usually present.

• Very strong association with HLA-A29

• Common cause of decreased visual acuity is cystoid macular edema.

• Electroretinograms are usually abnormal.

• Patients are often initially responsive to steroids but often require maintenance with nonsteroidal immunomodulatory agents.

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Nov 9, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on Chorioretinopathy

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