eMedicine Specialties > Ophthalmology > Retina

Retinopathy, Birdshot: Follow-up

Author: C Michael Samson, MD, Associate Professor, Department of Ophthalmology, New York Medical College; Consulting Staff, Co-director of Uveitis Service, Faculty in Residency Training Program, Director of Uveitis Fellowship Training Program, Department of Ophthalmology, New York Eye and Ear Infirmary; Private Practice, Vitreous Retina Macula Consultants of New York
Coauthor(s): Amro Mohamed Mohamoud Ali, MB, ChB, Consulting Staff, New York Eye and Ear Infirmary; C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution
Contributor Information and Disclosures

Updated: Sep 25, 2008

Follow-up

Further Outpatient Care

  • Patients should be observed every 4-6 weeks. The patient is queried about visual quality, including color perception and vision at nighttime, and about symptoms of the potential adverse effects from the medications. The patient is examined, and blood tests and blood pressure measurement are performed. If the patient describes change in the quality of vision, despite a change in the visual acuity or evidence of active inflammation by examination, fluorescein angiography and indocyanine green angiography are performed to detect inflammation not seen readily on funduscopy, looking in particular for disk leakage or leakage from vessels. The use of serial ERGs as a tool to detect subclinical inflammation is being investigated.
  • This author believes in a zero tolerance for even minimal inflammation. When inflammation is not controlled, the dosage of the medication is increased; this is continued until the inflammation is controlled, the patient reaches the maximal tolerated dose, or the patient shows signs of drug toxicity. Although most cases can be controlled with this strategy, a small number of patients will have persistent inflammation despite regional steroids and maximally tolerated cyclosporine therapy. In these cases, combination immunosuppressive therapy may be indicated and will require management by a physician experienced in their use.

Complications

  • Chronic cystoid macular edema – 50%; the most common cause of reduced central visual acuity 
  • Epiretinal membrane - 10% 
  • Macular pucker 
  • Choroidal neovascularization 
  • Peripapillary subretinal neovascularization - 6% 
  • Retinal neovascularization located on the optic disc 
  • Peripheral retinal neovascularization with capillary nonperfusion 
  • Optic nerve atrophy 
  • Other complications, such as cataract, glaucoma, and rhegmatogenous retinal detachment

Prognosis

  • Birdshot retinochoroidopathy is a chronic disease that is characterized by multiple exacerbations and remissions. Birdshot retinochoroidopathy tends to stabilize over a 3- to 4-year period. However, greater than one third of patients reach a visual acuity of 20/200 or worse. Visual loss is most commonly the result of cystoid macular edema and optic nerve atrophy.  
  • One series described deterioration on ERG and visual field or significant visual morbidity in 10 of 15 patients during follow-up. Of note, most patients in the series either had no treatment or treatment with steroids alone (ie, no immunomodulatory therapy).
  • Rothova and Schooneveld described a man with birdshot retinochoroidopathy for 20 years, undergoing alternative therapy (low-voltage therapy and multivitamins) as his only treatment. His end-stage picture consisted of multiple birdshot lesions, attenuated vessels, disk pallor, and pigmentary deposits similar to those seen in retinitis pigmentosa. He was legally blind. It is quite clear that, if uncontrolled, birdshot retinochoroidopathy usually has a progressive course, with significant ocular morbidity as the consequences.

Miscellaneous

Medicolegal Pitfalls

  • Birdshot retinochoroidopathy is a potentially blinding disorder. Early referral to a uveitis specialist before significant vision is lost is recommended.
 


More on Retinopathy, Birdshot

Overview: Retinopathy, Birdshot
Differential Diagnoses & Workup: Retinopathy, Birdshot
Treatment & Medication: Retinopathy, Birdshot
Follow-up: Retinopathy, Birdshot
References
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References

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  2. LeHoang P, Ozdemir N, Benhamou A, et al. HLA-A29.2 subtype associated with birdshot retinochoroidopathy. Am J Ophthalmol. Jan 15 1992;113(1):33-5. [Medline].

  3. Levinson RD, Rajalingam R, Park MS, et al. Human leukocyte antigen A29 subtypes associated with birdshot retinochoroidopathy. Am J Ophthalmol. Oct 2004;138(4):631-4. [Medline].

  4. Nussenblatt RB, Mittal KK, Ryan S, et al. Birdshot retinochoroidopathy associated with HLA-A29 antigen and immune responsiveness to retinal S-antigen. Am J Ophthalmol. Aug 1982;94(2):147-58. [Medline].

  5. Fuerst DJ, Tessler HH, Fishman GA, et al. Birdshot retinochoroidopathy. Arch Ophthalmol. Feb 1984;102(2):214-9. [Medline].

  6. Gaudio PA, Kaye DB, Crawford JB. Histopathology of birdshot retinochoroidopathy. Br J Ophthalmol. Dec 2002;86(12):1439-41. [Medline].

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  8. Kiss S, Ahmed M, Letko E, et al. Long-term follow-up of patients with birdshot retinochoroidopathy treated with corticosteroid-sparing systemic immunomodulatory therapy. Ophthalmology. Jun 2005;112(6):1066-71. [Medline].

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  13. de Smet MD, Yamamoto JH, Mochizuki M, et al. Cellular immune responses of patients with uveitis to retinal antigens and their fragments. Am J Ophthalmol. Aug 15 1990;110(2):135-42. [Medline].

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  15. Hirose T, Katsumi O, Pruett RC, et al. Retinal function in birdshot retinochoroidopathy. Acta Ophthalmol (Copenh). Jun 1991;69(3):327-37. [Medline].

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  20. Oh KT, Christmas NJ, Folk JC. Birdshot retinochoroiditis: long term follow-up of a chronically progressive disease. Am J Ophthalmol. May 2002;133(5):622-9. [Medline].

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Further Reading

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Keywords

birdshot retinopathy, birdshot retinochoroidopathy, BSRC, vitiliginous chorioretinitis

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Contributor Information and Disclosures

Author

C Michael Samson, MD, Associate Professor, Department of Ophthalmology, New York Medical College; Consulting Staff, Co-director of Uveitis Service, Faculty in Residency Training Program, Director of Uveitis Fellowship Training Program, Department of Ophthalmology, New York Eye and Ear Infirmary; Private Practice, Vitreous Retina Macula Consultants of New York
C Michael Samson, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Uveitis Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Coauthor(s)

Amro Mohamed Mohamoud Ali, MB, ChB, Consulting Staff, New York Eye and Ear Infirmary
Amro Mohamed Mohamoud Ali, MB, ChB is a member of the following medical societies: American Academy of Ophthalmology
Disclosure: Nothing to disclose.

C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution
C Stephen Foster, MD, FACS, FACR, FAAO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Russell P Jayne, MD, Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas
Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, and American Society of Retina Specialists
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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