Reappraisal of birdshot retinochoroiditis (BRC): a global approach

  • Marina Papadia
  • Carl P. Herbort
Inflammatory Disorders



This study aimed to readjust the appraisal of birdshot retinochoroiditis (BRC) in light of a global approach, including the full array of investigational procedures.

Patients and methods

This retrospective study reviewed charts of BRC cases treated in the uveitis clinic of our center between 1995 and 2011. We identified 25 patients with BRC; of these, 19 had sufficient data for inclusion in the study. Patients were examined with a standard clinical approach for inflammatory disorders, including dual fluorescence angiography with fluorescein and indocyanine green, perimetry, and laser flare photometry, both at presentation and during follow-up. Spectral optical coherence tomography (OCT) was performed when available. Disease characteristics and evolutionary patterns were reported.


Human leucocyte antigen was positive for the A29 allele in all patients. The mean age at presentation was 49.6 ± 10.0 years, the mean diagnostic delay was 21.5 ± 18 months, and the mean follow-up was 85 ± 60 months. Out of 19 patients, three presented with mutton-fat keratic precipitates (KPs), three had no depigmented lesions at presentation, and eight did not fulfill the recommended criterion of three depigmented peripapillar lesions. Cystoid macular edema (CMO) at entry was present in 8/19 cases. Perimetric anomalies were noted in all patients at presentation. In 92 % of cases, fluorescein findings included disc hyperfluorescence, retinal vasculitis of large vessels, and leakage from medium-sized and small vessels. In all patients, a (pseudo)-delay was noted in the arterio-venous circulation time (mean venous dye appearance = 42.1 ± 13.1 s), which reflected massive capillary leakage. At presentation, all patients exhibited indocyanine green angiographic signs, including hypofluorescent dark dots, vessel fuzziness, and areas of diffuse late hyperfluorescence. This allowed early diagnosis in 3/19 patients (16 %) without birdshot fundus lesions at presentation.


BRC is a granulomatous uveitis, and mutton-fat KPs do not exclude the disease. When BRC is suspected, indocyanine green angiography is crucial to allow early diagnosis and to monitor the evolution of choroiditis. Perimetry is an obligate investigation for diagnosis and follow-up. CMO is less frequent than stated earlier. Scores of fluorescein and indocyanine green angiographic signs indicated that choroiditis responded readily to therapy, but retinitis was relatively resistant to therapy.


Birdshot retinochoroiditis Indocyanine green angiography Choroidal granuloma 


  1. 1.
    LeHoang P, Ozdemir N, Benhamou A, Tabary T, Edelson C, Betuel H, Semiglia R, Cohen JH (1992) HLA-A29.2 subtype associated with birdshot retinochoroidopathy. Am J Ophthalmol 113:32–35Google Scholar
  2. 2.
    Shah KH, Levinson RD, Yu F, Goldhardt R, Gordon LK, Gonzales CR, Heckenlively JR, Kappel PJ, Holland GN (2005) Birdshot chorioretinopathy. Surv Ophthalmol 6:519–541CrossRefGoogle Scholar
  3. 3.
    Herbort CP, Probst K, Cimino L, Tran VT (2004) Differential inflammatory involvement in retina and choroid in birdshot chorioretinopathy. Klin Monatsbl Augenheilkd 221:351–356PubMedCrossRefGoogle Scholar
  4. 4.
    de Courten C, Herbort CP (1998) Potential role of computerized visual field testing for the appraisal and follow-up of birdshot chorioretinopathy. Arch Ophthalmol 116(10):1389–1391PubMedGoogle Scholar
  5. 5.
    Thorne JE, Jabs DA, Kedhar SR, Peters GB, Dunn JP (2008) Loss of visual field among patients with birdshot chorioretinopathy. Am J Ophthalmol 145(1):23–28, Epub 2007 Nov 12PubMedCrossRefGoogle Scholar
  6. 6.
    Gordon LK, Goldhardt R, Holland GN, Yu F, Levinson RD (2006) Standardized visual field assessment for patients with birdshot chorioretinopathy. Ocul Immunol Inflamm 14(6):325–332PubMedCrossRefGoogle Scholar
  7. 7.
    Levinson RD, Brezin A, Rothova A, Accorinti M, Holland GN (2006) Research criteria for the diagnosis of birdshot chorioretinopathy: results of an international consensus conference. Am J Ophthalmol 141(1):185–187PubMedCrossRefGoogle Scholar
  8. 8.
    Herbort CP, LeHoang P, Guex-Crosier Y (1998) Schematic interpretation of indocyanine green angiography in posterior uveitis using a standard angiographic protocol. Ophthalmology 105(3):432–440PubMedCrossRefGoogle Scholar
  9. 9.
    Monnet D, Brézin AP, Holland GN, Yu F, Mahr A, Gordon LK, Levinson RD (2006) Longitudinal cohort study of patients with birdshot chorioretinopathy. I. Baseline clinical characteristics. Am J Ophthalmol 141(1):135–142PubMedCrossRefGoogle Scholar
  10. 10.
    Rothova A, Suttorp-van Schulten MS, Frits Treffers W, Kijlstra A (1996) Causes and frequency of blindness in patients with intraocular inflammatory disease. Br J Ophthalmol 80(4):332–336PubMedCrossRefGoogle Scholar
  11. 11.
    Rothova A, Berendschot TT, Probst K, van Kooij B, Baarsma GS (2004) Birdshot chorioretinopathy: long-term manifestations and visual prognosis. Ophthalmology 111(5):954–959PubMedCrossRefGoogle Scholar
  12. 12.
    Gass JD (1981) Vitiliginous chorioretinitis. Arch Ophthalmol 99(10):1778–1787PubMedCrossRefGoogle Scholar
  13. 13.
    Guex-Crosier Y, Herbort CP (1997) Prolonged retinal arterio-venous circulation time by fluorescein but not by indocyanine green angiography in birdshot chorioretinopathy. Ocul Immunol Inflamm 5(3):203–206PubMedCrossRefGoogle Scholar
  14. 14.
    Bouchenaki N, Herbort CP (2001) The contribution of indocyanine green angiography to the appraisal and management of Vogt–Koyanagi–Harada disease. Ophthalmology 108(108):54–64PubMedCrossRefGoogle Scholar
  15. 15.
    Herbort CP, Mantovani A, Bouchenaki N (2007) Indocyanine green angiography in Vogt–Koyanagi–Harada disease: angiographic signs and utility in patient follow-up. Int Ophthalmol 27:173–182PubMedCrossRefGoogle Scholar
  16. 16.
    Kawaguchi T, Horie S, Bouchenaki N, Ohno-Matsui K, Mochizuki M, Herbort CP (2010) Suboptimal therapy controls clinically apparent disease but not subclinical progression of Vogt–Koyanagi–Harada disease. Int Ophthalmol 30:41–50PubMedCrossRefGoogle Scholar
  17. 17.
    Wolfensberger TJ, Herbort CP (1999) Indocyanine green angiographic features in ocular sarcoidosis. Ophthalmology 106:285–289PubMedCrossRefGoogle Scholar
  18. 18.
    Wolfensberger TJ, Piguet B, Herbort CP (1999) Indocyanine green angiographic (ICGA) features in tuberculous chorioretinitis. Am J Ophthalmol 127:350–353PubMedCrossRefGoogle Scholar
  19. 19.
    Ryan S, Maumenee AE (1980) Birdshot retinochoroidopathy. Am J Ophthalmol 89:31–45PubMedGoogle Scholar
  20. 20.
    Cimino L, Auer C, Herbort CP (2000) Sensitivity of indocyanine green angiography for the follow-up of active inflammatory choriocapillaropathies. Ocul Immunol Inflamm 8:275–283PubMedCrossRefGoogle Scholar
  21. 21.
    Herbort CP (1998) Posterior uveitis: new insights provided by indocyanine green angiography. Eye 12:757–759PubMedCrossRefGoogle Scholar
  22. 22.
    Fardeau C, Herbort CP, Kullmann N, Quentel G, LeHoang P (1999) Indocyanine green angiography in Birdshot chorioretinopathy. Ophthalmology 106:1928–1934PubMedCrossRefGoogle Scholar
  23. 23.
    Herbort CP, Bodaghi B, LeHoang P (2001) Angiographie au vert d’indocyanine au cours des maladies oculaires inflammatoires: principes, interprétation schématique, sémiologie et intérêt clinique. J Fr Ophtalmol 24:423–447PubMedGoogle Scholar
  24. 24.
    Thorne JE, Jabs DA, Peters GB, Hair D, Dunn JP, Kempen JH (2005) Birdshot retinochoroidopathy: ocular complications and visual impairment. Am J Ophthalmol 140(1):45–51PubMedCrossRefGoogle Scholar
  25. 25.
    Cimino L, Tran VT, Herbort CP (2002) Importance of visual field testing in the functional evaluation and follow-up of birdshot chorioretinopathy. Ophthalmic Res 34(S1):141Google Scholar
  26. 26.
    Lim L, Harper A, Guymer R (2006) Choroidal lesions preceding symptom onset in birdshot chorioretinopathy. Arch Ophthalmol 124(7):1057–1058PubMedCrossRefGoogle Scholar
  27. 27.
    Machida S, Tanaka M, Murai K, Takahashi T, Tazawa Y (2004) Choroidal circulatory disturbance in ocular sarcoidosis without the appearance of retinal lesions or loss of visual function. Jpn J Ophthalmol 48(4):392–396PubMedCrossRefGoogle Scholar
  28. 28.
    Howe LJ, Stanford MR, Graham EM, Marshall J (1997) Choroidal abnormalities in birdshot chorioretinopathy: an indocyanine green angiography study. Eye 11(4):554–559PubMedCrossRefGoogle Scholar
  29. 29.
    Gaudio PA, Kaye DB, Crawford JB (2002) Histopathology of birdshot retinochoroidopathy. Br J Ophthalmol 86(12):1439–1441PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Marina Papadia
    • 1
    • 2
  • Carl P. Herbort
    • 1
    • 3
  1. 1.Inflammatory and Retinal Eye DiseasesCentre for Ophthalmic Specialised Care (COS)LausanneSwitzerland
  2. 2.Eye Clinic, Department of Neurosciences, Ophthalmology and GeneticsUniversity of GenovaGenoaItaly
  3. 3.University of LausanneLausanneSwitzerland

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