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Birdshot Chorioretinopathy

Home / Ophthalmic Pathology Retina and Retinal Pigment Epithelium

Title: Birdshot Chorioretinopathy, Case Report

Author: Taylor Fields, Fourth Year Medical Student, Medical College of Georgia

Photographers: Cyrie Fry, CRA, and Paula Morris, CRA.

Date: 08/23/15

Secondary CORE Category: Retina and Vitreous / Focal and Diffuse Choroidal and Retinal Inflammation

Keywords/Main Subject: Posterior Uveitis; Chorioretinopathy; Birdshot; BSCR; BCR Diagnosis- Birdshot Chorioretinopathy

Brief Description of Case:

This case is a 52-year-old white female who presented complaining of “hundreds of floaters” that had been progressing for several months in both eyes. She noted a crescent shape to the floaters and a one month of a small blurry spot in the inferonasal visual field in her right eye. She also described nyctalopia, a less vivid quality to her vision and morning photopsias and black spots upon awakening that lasted about 30 minutes.

Visual acuity was 20/20 in each eye and intraocular pressure by tonopen was 21 and 19 in the right and left eyes respectively. Her pupillary exam, visual fields, color vision and motility were all normal. The Fundus exam revealed 1+ disc edema, bilateral epiretinal membranes, narrowing of her retinal vessels and numerous mid-peripheral white/yellow choroidal spots bilaterally (Pictures 1-4). ICG photos revealed areas of decreased dye perfusion within the choroid (Picture 3).

The differential diagnosis included inflammatory uveitis (birdshot chorioretinopathy, sarcoidosis), infectious causes (TB, syphilis, Lyme disease) and masquerade syndromes such as choroidal lymphoma. She was found to be HLA-A29 positive.  An extensive laboratory work-up was otherwise negative.  Full-field electroretinograms (ffERGs) showed attenuated scotopic dim blue flash ERGs to about 59% with slowed photopic b-wave time of 35 milliseconds.

Diagnosis

The patient was diagnosed with birdshot chorioretinopathy (BSCR). Her fundus exam and ffERG results are typical of early stages of BSCR. This disease is an idiopathic posterior uveitis that commonly presents in Caucasians in their sixth decade of life. It has a strong association with the human leukocyte antigen HLA-A29 (96% of patients are carriers).1,2 BSCR typically presents with a gradual centripetal loss of vision and presence of floaters.3

Treatment

The patient was started on a 60 mg prednisone taper as well as mycophenolate and cyclosporine. Systemic immunomodulatory therapy is the mainstay of treatment for BSCR. Concomitant therapy with an antimetabolite and a T-cell inhibitor can be beneficial for preserving vision, and decreasing the cumulative glucocorticoid dose.4,5

Prognosis

BSCR is considered a chronic and progressive isolated ocular disorder. A majority of patients develop retinal dysfunction; however, central vision is typically spared until late in the disease. Visual acuity at initial presentation is a prognostic factor for long-term visual outcomes with appropriate treatment.6

Images

 

Picture 1. Full color montage photos of the right (A) and left (B) eyes. These show characteristic pisiform lesions of BSCR within the periphery of the retina (black arrows).


Picture 2. These are color (A & C) and red free (B & D) photos of the left (A & B) and right (C & D) fundi. The periphery has areas of white/yellow choroidal spots (black arrows). There is also narrowing of vessels and disc edema present.


Picture 3. These are late ICG photos of the nasal portions of the Left (A) and Right (B) retinas. There is decreased choroidal perfusion, and thus dark spots, in the areas of inflammation (black arrows).


Picture 4. These are wide field fluorescein angiograms (FA) of the right (A) and left (B) fundi. This shows vasculature leakage, periphlebitis, and disc edema.

Summary of Case

This is a 52 year-old white female diagnosed with birdshot chorioretinopathy who presented with several months of worsening floaters and was found to have the characteristic midperipheral white/yellow choroidal lesions bilaterally.  She was found to be HLA-A29 positive, confirmatory of the diagnosis.

References

 Levinson RD, Brezin A, Rothova A, et al. Research criteria for the diagnosis of birdshot chorioretinopathy: results of an international consensus Am J Ophthalmol 2006; 141:185 – 187.

  1. Brézin AP, Monnet D, Cohen JH, Levinson HLA-A29 and birdshot chorioretinopathy.Ocul Immunol Inflamm. 2011;19(6):397-400.
  2. Shah KH, Levinson RD, Yu F, et al. Birdshot chorioretinopathy. Surv Ophthalmol 2005; 50:519 –
  3. Becker MD, Wertheim MS, Smith JR, Rosenbaum Long-term follow-up of patients with birdshot retinochoroidopathy treated with systemic immunosuppression. Ocul Immunol Inflamm 2005; 13:289–293.st
  4. Kiss, Szilard, Ahmed, Muna, Letko, Erik, & Foster, Stephen. (2005). Long-term Follow- up of Patients with Birdshot Retinochoroidopathy Treated with Corticosteroid-Sparing Systemic Immunomodulatory Therapy. Ophthalmology, 112(6), 1066-1071.e2.
  5. Tomkins-Netzer O, Taylor SR, Lightman Long-term clinical and anatomic outcome of birdshot chorioretinopathy. JAMA Ophthalmol 2014; 132:57 – 62.

Faculty Approval by: Griffin Jardine, MD

Identifier: Moran_CORE_23873