Birdshot Retinochoroidopathy (Grand Rounds)

From EyeWiki
Original article contributed by: Saradha Iragavarapu, MD
All contributors: Jennifer I Lim MD and Vinay A. Shah M.D.
Assigned editor: Pooja Bhat MD
Review: Assigned status Update Pending by Pooja Bhat MD on January 20, 2015.


Financial Disclosure

Saradha Iragavarapu, MD PGY-3

UNIVERSITY OF SOUTH FLORIDA

  • Dr. Iragavarapu states that she has no financial interest, affiliation, or other relationship with the manufacture of any commercial project discussed or with the manufacture of any competing commercial project.

Chief Complaint

  • "Foggy vision, difficulty with depth perception"
  • 72 y/o WF referred by outside ophthalmologist for "retinal dystrophy"
  • Sx began 10 years ago with blurred vision > difficulty with depth perception > dec color vision
  • Diagnosed with "retinal problem' in 2005 and had 3 steroid injections


History

POH/PMH

  • CE/PCIOL
  • "retinal problem"
  • HTN
  • Hypothyroidism

Fam Hx

  • Grandmother- "legally blind"
  • Aunt- "corneal problem"

Meds

  • "water pill"
  • Synthroid

Social history

  • Ex smoker, social drinker
  • Born and raised in Tampa, FL
  • No travel outside US
  • No pets
  • No recent URI's or illness

Examination

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OCT OD

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OCT OS

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Autoflouresence OD

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Autoflouresence OS

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FA OD

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FA OS

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Differential Diagnosis

Final Diagnosis

Birdshot Retinochoroidopathy

Other names:

  • Vitiliginous chorioretinitis
  • Salmon Patch Choroidopathy

Risk factors

  • HLA A29-type 2

Epidemiology

  • 4th-6th decade of life
  • Females affected slightly more than males

Symptoms

  • floaters, blurred vision, peripheral photopsias, nyctalopia, and loss of color vision

Fundus findings

  • Creamy yellow-white lesions at level of rpe/choroid
  • 500-1500um in size
  • Typically extend radially from nerve to cover most of the nasal retina.
  • 100% of patients with active disease have vitritis.
  • Variable degree of disc edema, CME, retinal vasculitis
  • Late stage optic atrophy

Diagnosis

  • Labs: ACE, lysozyme, PPD, CXR, FTA, HLA panel
  • ERG
  • FA
  • ICG
  • OCT
  • AF

Findings

  • ERG findings
    • Diminished 'b' wave in comparison to 'a' wave.
    • Delayed 30Hz flicker time.
  • FA findings
    • "quenching" phenomenon where dye leaves retinal circulation quickly.
    • Block early stain late
    • Vasculitis/vascular leakage
  • Inodcyanine Green findings
    • Hypofluorescent lesions
  • OCT
    • Decreased reflectivity of photoreceptor layer
    • CME
  • Autofluorescence
    • Round, hypofluorescent lesions
  • Histopathology
    • Lymphocytic infiltrate in the choroid, optic nerve head, retinal vessels

Complications

  • CME
  • Optic atrophy
  • Macular CNV (very rare)

Management

Treatment

  • 20% of patients achieve remission without treatment.
  • Corticosteroids
  • Cyclosporine 2.5-5mg/kg/day
  • Azathioprine 1.5-2mg/kg/day
  • Methotrexate 7.5-25mg/week
  • Mycophenolate mofetil
  • Daclizumab (not used anymore)
  • IV-lg

Additional Resources

  • G E Holder, A G Robson, C Pavesio, E M Graham. Electrophysiological characterisation and monitoring in the management of birdshot chorioretinopathy Br J Ophthalmol. 2005 June; 89(6):709-718.
  • Ryan B, Rush, Debra A. Goldstein, David G. Callanan, Beeran Meghpara, William J. Feuer, Janet L. Davis. Outcomes of Birdshot Chorioretinopathy Treated With an Intravitreal Sustained-Release Fluocinolone Acetonide—Containing Device American Journal of Ophthalmology, Volume 151, Issue 4, Pages 630-636.
  • Giuliari G, Hinkle DM, Foster CS. The spectrum of fundus autofluorescence findings birdshot chorioretinopathy Journal of Ophthalmology Epub, Volume 2008, 5 pages. Feb 8 2010.
  • Rothova, Aniki MD, PhD, Ossewaarde-van Norel, Annette MD, Los, Leonoor I MD, PhD, Berendschot, Tos T J M PhD. Efficacy of low-dose Methotrexate in the treatment of Birdshot Chorioretinopathy. Retina. 31(6):1150-1155, June 2011.
  • Birdshot retinochoroidopathy. Basic and Clinical Science Course. 2011.
  • Kayur H. Shah, MD, Ralph D. Levinson, et al. Birdshot Chorioretinopathy A Major Review. Surv Ophthalmol. 2006 Jul-Aug;51(4):446-7

Discussion

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