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Birdshot retinochoroidopathy
Clinical Eye and Vision Care 7 (1995) 99-102
Special article
Birdshot retinochoroidopathy Connie L. Beaulieu”, Shawna M . Feeley, Jeffrey K. Austin, W illiam L. Jones V A Medical Center, Albuquerque, NM, USA
ReceivedSeptember1994;revisionreceivedNovember1994;acceptedDecember1994
Abstract Birdshot retinochoroidopathy is a rare bilateral ocular disorder which typically affects healthy, middle-age Caucasians.The typical symptoms are recent onset of bilateral floaters with decreased vision in a white and quiet eye. Minimal anterior chamber reaction, vitreous infiltration without snowballs, and multifocal depigmented lesions in the outer retina and choroid are characteristic of birdshot retinochoroidopathy. Other associated findings include retinal vasculitis, paravascular hemorrhage, vitreous hemorrhage, cystoid macular edema, cellophane maculopathy, posterior subcapsular cataract, optic disc edema, vascular attenuation and tortuosity, optic atrophy and retinal or choroidal neovascularization. Management involves Amsler grid self-assessment and routine funduscopic examinations. A recently documented case of treatment with aromatic retinoids showed improvement while many other commonly prescribed medications are equivocal in effect. Laser photoablation or panretinal photocoagulation are recommended for treatable choroidal or retinal neovascularization, respectively. Keywords: Birdshot retinochoroidopathy; Vitiliginous chorioretinitis; Vitritis
appearance (see Table 1)
1. Introduction
2. Clinical
Birdshot retinochoroidopathy or vitiliginous chorioretinitis is a rare bilateral ocular disorder which affects primarily healthy Caucasian individuals. Gass described the disease as vitiliginous chorioretinitis because of the similarity of the fundus lesions to the appearance and evolution of the cutaneous lesiok seen in vitiligo [l]. Since there appears to be no association between birdshot retinochoroidopathy and vitiligo, the term vitiliginous choroidopathy is not commonly used. The disease has been termed birdshot retinochoroidopathy because the fundus appearance resembles that of birdshot scatter from a shotgun-
Birdshot retinochoroidopathy is essentially selflim iting with a course of exacerbations and remissions [2]. There is no gender predilection and the mean age of onset is approximately 50 years of age [3-51. This disease is rare with Priem and Oosterhuis reporting only 102 cases over a 7-year period in 14 major European clinics [4]. Typical symptoms include recent onset of bilateral floaters and a decrease in vision [5]. Additionally, some patients complain of photopsia and nyctalopia [l]. Externally, the eye appears white and quiet with m inimal if any anterior chamber inflammatory reaction. The vitreous is diffusely infiltrated with the greatest involvement posteriorly. Characteristic multifocal depigmented lesions localized in the outer retina, pigment epithelium and choroid are scattered throughout the fundus posterior
* Correspondingauthor.
0953~4431/95/$09.50 8 1995Elsevier ScienceIreland Ltd. All rights reserved. SSDI 0953-4431(95)00116-K
loo
C.L. Beaulieu et al. /Clinical
Eye and Vision Care 7 (1995) W-102
Table 1 Clinical presentation of birdshot retinochoroidopathy Floaters Decreased vision Anterior segment quiet; possible mild anterior chamber reaction Vitritis with absence of exudates or snowballs over pars plana Flat cream-colored lesions in outer retina to choroid” Possible associated findings: cystoid macular edema, cellophane maculopathy, posterior subcapsular cataract, optic disc edema or atrophy, vasculitis, paravascular hemorrhages, vascular attenuation and tortuosity, retinal or choroidal neovascularization aNote: May initially present without fundus lesions Fig. 1. Posterior pole appearance in birdshot retinochoroidopathy.
to the equator [6]. Findings ultimately common to all reported cases of birdshot retinochoroidopathy are the characteristic depigmented spots or patches, vitreous infiltration with absence of exudate or snowballs overlying the pars plana, and minimal to absent anterior segment inflammation. Other reported findings include: generalized retinal vasculitis, paravascular hemorrhage, vitreous hemorrhage, cystoid macular edema (CME), cellophane maculopathy, posterior subcapsular cataract, optic disc edema, vascular attenuation and tortuosity, optic atrophy and retinal or choroidal neovascularization [2,7]. Several authors have reported cases with absence of the patches on initial presentation, however, all eventually developed the characteristic fundus appearance [4,8]. The flat, indistinct, cream-colored fundus patches seen initially gradually evolve into white, well-defined lesions [3,9]. Throughout the disease course and following resolution, the spots lack any pigment aggregation. Early in the disease, most patches range from 0.25-1.0 disc diameter in size often becoming larger and more confluent with time [3,5]. The fundus appearance is symmetrical and although the general pattern of distribution varies, the spots consistently appear to radiate from the optic disc following the distribution of the large choroidal vessels (Fig. 1). The most common appearance is an aggregation of spots posterior to the equator with greatest density inferior nasal to the optic disc. Alternatively, the patches may involve one quadrant exclusively [lo]. The macular area is generally spared, however, some cases show a few scattered patches within the vascular arcades. During exacerbations, a diffuse retinal vasculitis is invariably present, often resulting in CME. Optic disc edema may occur in conjunction with the presence of CME [91. Following resolution of optic disc edema, mild optic atrophy with associated vascular attenuation is often present. Peripapillary or macular choroidal neovascularization may occur in birdshot retinochoroidopathy and is believed to be directly related to the disease process creating breaks in
Bruch’s membrane [7]. Chronic intraocular inflammation frequently produces cellophane maculopathy and posterior subcapsular cataracts [4]. The extent of macular and lenticular compromise determines the ultimate visual acuity. In mild cases, the final visual acuity is often 20/40 or better. Severe vision reduction of 20/200 or worse may result from chronic CME, macular epiretinal membrane or hole, macular scarring, optic atrophy, choroidal neovascularization or posterior subcapsular cataract [2,7]. Most patients retain good central vision in at least one eye for years following onset [ll]. 3. Diagnostic testing Electroretinograms (ERG) obtained from patients with birdshot retinochoroidopathy typically demonstrate a negative type response with a decreased b:awave amplitude showing greater effect on the b-wave, This ERG pattern, although not unique to birdshot retinochoroidopathy, is not characteristic of any other uveitis or chorioretinitis. These results indicate that functionally, the inner neural layers of the retina are more affected than the outer layers where the lesions are actually located [3]. Electrooculogram (EGG) measurements vary from normal to slightly depressed. During fluorescein angiography, the characteristic spots show normal or hypo-fluorescence through the early stages with occasional late-stage hyperfluorescence. Overall, the ophthalmoscopic appearance of the lesions is more diagnostic than the angiographic appearance [6,7]. However, fluorescein angiography may be useful in diagnosing choroidal neovascularization, vascular leakage, or cystoid macular edema [12]. There appears to be no association of birdshot retinochoroidopathy with any particular systemic disease and it is uncertain whether this disease is the result of a single or multiple causative agents. A genetic basis for the disease has been suggested because of the association between birdshot
C.L. Beaulieu et al. /Clinical Eye and Viiim Care 7 (1995) W-102
retinochoroidopathy and the HLAA29 antigen [9,13,14]. This association is one of the strongest between HLA and any disease process [2,6], with 95.8% of patients with birdshot retinochoroidopathy testing positive for HLAA29 [4,7,10]. In the general Caucasianpopulation, the antigen occurs with a frequency of 2.9% [12]. LeHoang et al. reported increased frequency of the I&A-A29 antigen in blacks, however, none of the black subjects in their study manifested birdshot retinochoroidopathy. 4. Differential diagnosis (see Table 2) Differential diagnosis of birdshot retinochoroidopathy includes pars planitis, Behcet’s disease, sarcoidosis, Vogt-Koyanagi-Harada’s syndrome (VKH), Harada’s disease, sympathetic ophthalmia, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white-dot syndrome (MEWDS), serpiginous choroiditis, leukemia, Whipple’s disease and presumed ocular histoplasmosis [4,6,8,15].If the hypopigmented spots are present, the funduscopic appearance alone differentiates birdshot retinochoroidopathy from all of the above entities with the exception of presumed ocular histoplasmosis, APMPPE, MEWDS, VKH and Harada’s Disease. Presumed ocular histoplasmosis is characterized by atrophic lesions with marginal pigment accummulation and associated peripapillary atrophy. This condition also shows m inimal to absent vitreous and anterior chamber inflammation. APMPPE and MEWDS may be differentiated by both the disease course and the funduscopic appearance. APMPPE, which occurs in young adults, is characterized by a rapid decrease in vision followed by spontaneous resolution over weeks to months with visual acuity usually returning to 20/30 or better. Furthermore, the acute grey white lesions, found primarily in the posterior pole, become irregularly pigmented over a period of months with rapid changes in appearance [ll]. MEWDS occurs Table 2 Differential diagnosis of birdshot retinochoroidopathy Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) Bechet’s disease Harada’s disease Leukemia Multiple evanescent white-dot syndrome (MEWDS) Pars planitis Presumed ocular histoplasmosis Sarcoidosis Sympathetic ophthalmia Serpiginous choroiditis Vogt-Koyanagi-Harada syndrome WKH) Whipple’s disease
101
unilaterally with a similar disease course. VKH and Harada’s disease typically exhibit an exudative retinal detachment without CME or retinal vasculitis [l]. Prior to the development of the characteristic patches in birdshot retinochoroidopathy, differential diagnosis may be established through the funduscopic appearance and extent of anterior segment involvement. Pars planitis characteristically exhibits an intermediate uveitis with exudate and vitreous opacities overlying the pars plana and peripheral retina [5]. Elevated yellow-white choroidal and retinal lesions along with anemic retinopathy is typical of leukemia [16]. Sympathetic ophthalmia exhibits greater anterior segment and uveal inflammation with secondary exudative retinal detachment [l]. Extensive anterior segment involvement with hypopyon is found in Behcet’s disease. Funduscopically, sarcoid may show perivascular candle-wax drippings. Serpiginous choroiditis is typified by white atrophic serpentine lesions radiating from the optic disc. VKH, Behcet’s disease, sarcoidosis, Whipple’s disease and leukemia may be further delineated by their associated systemic involvement. VKH commonly occurs in darkly-pigmented individuals with resultant alopecia, poliosis and vitiligo. Behcet’s primarily affects males of Mediterranean origin with characteristic aphthous ulcers of the mouth and genitalia. Pulmonary disease and elevated serum levels of angiotensin converting enzyme represent sarcoidosis. Whipple’s disease presents as a multisystem granulomatous disorder while leukemia may be differentiated by a systemic preponderance of white blood cells. 5. Management and treatment (see Table 3) Therapeutic agents commonly prescribed for the treatment of birdshot retinochoroidopathy include: oral corticosteroids (prednisone), nonsteroidal anti-inflammatory drugs (indomethacin), immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporin A), antibiotics, antimetabolites and radiation to the posterior segment [4,5,9,10,12].To date, systemic corticosteroids have been the most commonly prescribed medication. Although patients have occassionally reported improvement in symptoms, improvement of ocular signs with the use of oral corticosteroids has been equivocal. These commonly administered therapeutic agents appear to be essentially ineffective. However, Hesse et al. recently reported subjective and objective improvement in active birdshot retinochoroidopathy when treating concurrent psoriasis with aromatic retinoids (etretinate, acitretin). This is the first report of a possible ocular therapeutic effect from aromatic retinoids, suggesting a need for further investigation 1141.
102
C.L. Beaulieu et al. /Clinical Eye and Vision Care 7 (1995) 99-102
Table 3 Management and treatment of birdshot retinochoroidopathy Amsler grid self-assessment Laser photoablation; when choroidal neovascultiation is present within the arcades Panretinal photocoagulation; when necessary for retinal neovascularization Therapeutic agents (controversial): oral corticosteroids,~ nonsteroidal anti-inflammatory drugs, immunosuppressive drugs, antibiotics, antimetabolites and radiation to the posterior segment.
Management of birdshot retinochoroidopathy involves periodic funduscopic examination and Amsler grid self-assessment for the detection of choroidal neovascularization. Laser photoablation is advocated in the presence of treatable choroidal neovascularization [7]. Retinal neovascularization, secondary to retinal non-perfusion, is treated with panretinal photocoagulation. 6. Conclusion Birdshot retinochoroidopathy is a rare bilateral ocular disorder with a chronic course of exacerbations and remissions. The anterior segment generally appears quiet with occasional m ild anterior chamber reaction. Characteristic flat cream-colored fundus lesions are generally scattered throughout the postequatorial fundus. Common ocular sequelae include vitritis, retinal vasculopathy, CME and optic disc edema or atrophy. Although the etiology remains unclear, the strong association between the HLAA29 antigen and birdshot retinochoroidopathy suggests a genetic predispositon. Currently, various therapeutic regimens are routinely administered but none have proven significantly effective. Recently, aromatic retinoids were found in one case to yield objective and subjective improvement providing a new direction for future research in the treatment of birdshot retinochoroidopathy. Patients with birdshot retinochoroidopathy require routine evaluation for the development of retinal or choroidal neovascularization.
References [ll Gass JDM. Vitiliginous chorioretinitis. Arch Ophthalmol 1981;99:1778-1787.
121 Baarsma GS, Priem HA, Kijlstra A. Association of birdshot retinochoroidopathy and HLA-A29 antigen. Gun Eye Res 1990$63-68. [31 Hirose T, Katsumi 0, Pruett RC, Sakaue H, Mehta M. Retinal function in birdshot retinochoroidopathy. Acta Ophthalmol 1991;69:327-337. 141 Priem HA, Oosterhuis JA. Birdshot chorioretinopathy: clinical characteristics and evolution. Br J Ophthalmol 1988;72:646-659. 151 Ryan SJ, Maumenee AE. Birdshot retinochoroidopathy. Am J Ophthahnol 1980,89:31-45. 161 LeHoang P, Ozdemir N, Benhamou A, Tabary T, Edelson C, Betuel H, Semiglia R, Cohen JHM. HLL429.2 subtype associated with birdshot retinochoroidopathy. Am J Ophthalmol1992;113:33-35. [71 Brucker AJ, Deglin EA, Bene C, Hoffman ME. Subretinal choroidal neovascularization in birdshot retinochoroidopathy. Am J Ophthahnol 1985;9940-44. IsI Soubrane G, Bokobza R, Coscas G. Late-developing lesions in birdshot retinochoroidopathy. Am J Ophthalmol 1990,109:204-210. 191 Fuerst DJ, Tessler HH, Fishman GA, Yokoyama MM, Wyhinny GJ, Vygantas CM. Birdshot retinochoroidopathy. Arch Ophthalmol 1984;102:214-219. [lOI Suttorp-Schulten MSA, Luyendijk L, Van Dam AP, deKeizer RJW, Baarsma GS, Bos PJM, Rothova A. Birdshot chorioretinopathy and lyme borreliosis. Am J Ophthalmol 1993;115:149-153. [ill Gass JDM. Stereoscopic atlas of macular diseases: diagnosis and treatment. St. Louis MO: The C.V. Mosby Company, 1987. WI Fich M, Rosenberg T. Birdshot retinochoroidopathy in monozygotic twins. Acta Ophthahnol 1992;70:693-697. [131 Bloch-Michel E, Frau E. Birdshot retinochoroidopathy and J-&4-A29+ and HLA-A29idiopathic retinal vasculitis: comparative study of 56 cases. Can J Ophthalmol 1991;26(7):361-366. [I41 Hesse S, Berbis PH, Chemila JF, Privat Y. Psoriasis and birdshot chorioretinopathy: Response to aromatic retinoids. Dermatology 1993;187:137-139. I151 Brod RD. Presumed sarcoid choroidopathy mimicking birdshot retinochoroidopathy. Am J Ophthalmol 1990;109:357-358. tt61 Kaplan HJ, Aaberg TM. Birdshot retinochoroidopathy. Am J Ophthalmol 1980; 90~773-782.
Special article
Birdshot retinochoroidopathy Connie L. Beaulieu”, Shawna M . Feeley, Jeffrey K. Austin, W illiam L. Jones V A Medical Center, Albuquerque, NM, USA
ReceivedSeptember1994;revisionreceivedNovember1994;acceptedDecember1994
Abstract Birdshot retinochoroidopathy is a rare bilateral ocular disorder which typically affects healthy, middle-age Caucasians.The typical symptoms are recent onset of bilateral floaters with decreased vision in a white and quiet eye. Minimal anterior chamber reaction, vitreous infiltration without snowballs, and multifocal depigmented lesions in the outer retina and choroid are characteristic of birdshot retinochoroidopathy. Other associated findings include retinal vasculitis, paravascular hemorrhage, vitreous hemorrhage, cystoid macular edema, cellophane maculopathy, posterior subcapsular cataract, optic disc edema, vascular attenuation and tortuosity, optic atrophy and retinal or choroidal neovascularization. Management involves Amsler grid self-assessment and routine funduscopic examinations. A recently documented case of treatment with aromatic retinoids showed improvement while many other commonly prescribed medications are equivocal in effect. Laser photoablation or panretinal photocoagulation are recommended for treatable choroidal or retinal neovascularization, respectively. Keywords: Birdshot retinochoroidopathy; Vitiliginous chorioretinitis; Vitritis
appearance (see Table 1)
1. Introduction
2. Clinical
Birdshot retinochoroidopathy or vitiliginous chorioretinitis is a rare bilateral ocular disorder which affects primarily healthy Caucasian individuals. Gass described the disease as vitiliginous chorioretinitis because of the similarity of the fundus lesions to the appearance and evolution of the cutaneous lesiok seen in vitiligo [l]. Since there appears to be no association between birdshot retinochoroidopathy and vitiligo, the term vitiliginous choroidopathy is not commonly used. The disease has been termed birdshot retinochoroidopathy because the fundus appearance resembles that of birdshot scatter from a shotgun-
Birdshot retinochoroidopathy is essentially selflim iting with a course of exacerbations and remissions [2]. There is no gender predilection and the mean age of onset is approximately 50 years of age [3-51. This disease is rare with Priem and Oosterhuis reporting only 102 cases over a 7-year period in 14 major European clinics [4]. Typical symptoms include recent onset of bilateral floaters and a decrease in vision [5]. Additionally, some patients complain of photopsia and nyctalopia [l]. Externally, the eye appears white and quiet with m inimal if any anterior chamber inflammatory reaction. The vitreous is diffusely infiltrated with the greatest involvement posteriorly. Characteristic multifocal depigmented lesions localized in the outer retina, pigment epithelium and choroid are scattered throughout the fundus posterior
* Correspondingauthor.
0953~4431/95/$09.50 8 1995Elsevier ScienceIreland Ltd. All rights reserved. SSDI 0953-4431(95)00116-K
loo
C.L. Beaulieu et al. /Clinical
Eye and Vision Care 7 (1995) W-102
Table 1 Clinical presentation of birdshot retinochoroidopathy Floaters Decreased vision Anterior segment quiet; possible mild anterior chamber reaction Vitritis with absence of exudates or snowballs over pars plana Flat cream-colored lesions in outer retina to choroid” Possible associated findings: cystoid macular edema, cellophane maculopathy, posterior subcapsular cataract, optic disc edema or atrophy, vasculitis, paravascular hemorrhages, vascular attenuation and tortuosity, retinal or choroidal neovascularization aNote: May initially present without fundus lesions Fig. 1. Posterior pole appearance in birdshot retinochoroidopathy.
to the equator [6]. Findings ultimately common to all reported cases of birdshot retinochoroidopathy are the characteristic depigmented spots or patches, vitreous infiltration with absence of exudate or snowballs overlying the pars plana, and minimal to absent anterior segment inflammation. Other reported findings include: generalized retinal vasculitis, paravascular hemorrhage, vitreous hemorrhage, cystoid macular edema (CME), cellophane maculopathy, posterior subcapsular cataract, optic disc edema, vascular attenuation and tortuosity, optic atrophy and retinal or choroidal neovascularization [2,7]. Several authors have reported cases with absence of the patches on initial presentation, however, all eventually developed the characteristic fundus appearance [4,8]. The flat, indistinct, cream-colored fundus patches seen initially gradually evolve into white, well-defined lesions [3,9]. Throughout the disease course and following resolution, the spots lack any pigment aggregation. Early in the disease, most patches range from 0.25-1.0 disc diameter in size often becoming larger and more confluent with time [3,5]. The fundus appearance is symmetrical and although the general pattern of distribution varies, the spots consistently appear to radiate from the optic disc following the distribution of the large choroidal vessels (Fig. 1). The most common appearance is an aggregation of spots posterior to the equator with greatest density inferior nasal to the optic disc. Alternatively, the patches may involve one quadrant exclusively [lo]. The macular area is generally spared, however, some cases show a few scattered patches within the vascular arcades. During exacerbations, a diffuse retinal vasculitis is invariably present, often resulting in CME. Optic disc edema may occur in conjunction with the presence of CME [91. Following resolution of optic disc edema, mild optic atrophy with associated vascular attenuation is often present. Peripapillary or macular choroidal neovascularization may occur in birdshot retinochoroidopathy and is believed to be directly related to the disease process creating breaks in
Bruch’s membrane [7]. Chronic intraocular inflammation frequently produces cellophane maculopathy and posterior subcapsular cataracts [4]. The extent of macular and lenticular compromise determines the ultimate visual acuity. In mild cases, the final visual acuity is often 20/40 or better. Severe vision reduction of 20/200 or worse may result from chronic CME, macular epiretinal membrane or hole, macular scarring, optic atrophy, choroidal neovascularization or posterior subcapsular cataract [2,7]. Most patients retain good central vision in at least one eye for years following onset [ll]. 3. Diagnostic testing Electroretinograms (ERG) obtained from patients with birdshot retinochoroidopathy typically demonstrate a negative type response with a decreased b:awave amplitude showing greater effect on the b-wave, This ERG pattern, although not unique to birdshot retinochoroidopathy, is not characteristic of any other uveitis or chorioretinitis. These results indicate that functionally, the inner neural layers of the retina are more affected than the outer layers where the lesions are actually located [3]. Electrooculogram (EGG) measurements vary from normal to slightly depressed. During fluorescein angiography, the characteristic spots show normal or hypo-fluorescence through the early stages with occasional late-stage hyperfluorescence. Overall, the ophthalmoscopic appearance of the lesions is more diagnostic than the angiographic appearance [6,7]. However, fluorescein angiography may be useful in diagnosing choroidal neovascularization, vascular leakage, or cystoid macular edema [12]. There appears to be no association of birdshot retinochoroidopathy with any particular systemic disease and it is uncertain whether this disease is the result of a single or multiple causative agents. A genetic basis for the disease has been suggested because of the association between birdshot
C.L. Beaulieu et al. /Clinical Eye and Viiim Care 7 (1995) W-102
retinochoroidopathy and the HLAA29 antigen [9,13,14]. This association is one of the strongest between HLA and any disease process [2,6], with 95.8% of patients with birdshot retinochoroidopathy testing positive for HLAA29 [4,7,10]. In the general Caucasianpopulation, the antigen occurs with a frequency of 2.9% [12]. LeHoang et al. reported increased frequency of the I&A-A29 antigen in blacks, however, none of the black subjects in their study manifested birdshot retinochoroidopathy. 4. Differential diagnosis (see Table 2) Differential diagnosis of birdshot retinochoroidopathy includes pars planitis, Behcet’s disease, sarcoidosis, Vogt-Koyanagi-Harada’s syndrome (VKH), Harada’s disease, sympathetic ophthalmia, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white-dot syndrome (MEWDS), serpiginous choroiditis, leukemia, Whipple’s disease and presumed ocular histoplasmosis [4,6,8,15].If the hypopigmented spots are present, the funduscopic appearance alone differentiates birdshot retinochoroidopathy from all of the above entities with the exception of presumed ocular histoplasmosis, APMPPE, MEWDS, VKH and Harada’s Disease. Presumed ocular histoplasmosis is characterized by atrophic lesions with marginal pigment accummulation and associated peripapillary atrophy. This condition also shows m inimal to absent vitreous and anterior chamber inflammation. APMPPE and MEWDS may be differentiated by both the disease course and the funduscopic appearance. APMPPE, which occurs in young adults, is characterized by a rapid decrease in vision followed by spontaneous resolution over weeks to months with visual acuity usually returning to 20/30 or better. Furthermore, the acute grey white lesions, found primarily in the posterior pole, become irregularly pigmented over a period of months with rapid changes in appearance [ll]. MEWDS occurs Table 2 Differential diagnosis of birdshot retinochoroidopathy Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) Bechet’s disease Harada’s disease Leukemia Multiple evanescent white-dot syndrome (MEWDS) Pars planitis Presumed ocular histoplasmosis Sarcoidosis Sympathetic ophthalmia Serpiginous choroiditis Vogt-Koyanagi-Harada syndrome WKH) Whipple’s disease
101
unilaterally with a similar disease course. VKH and Harada’s disease typically exhibit an exudative retinal detachment without CME or retinal vasculitis [l]. Prior to the development of the characteristic patches in birdshot retinochoroidopathy, differential diagnosis may be established through the funduscopic appearance and extent of anterior segment involvement. Pars planitis characteristically exhibits an intermediate uveitis with exudate and vitreous opacities overlying the pars plana and peripheral retina [5]. Elevated yellow-white choroidal and retinal lesions along with anemic retinopathy is typical of leukemia [16]. Sympathetic ophthalmia exhibits greater anterior segment and uveal inflammation with secondary exudative retinal detachment [l]. Extensive anterior segment involvement with hypopyon is found in Behcet’s disease. Funduscopically, sarcoid may show perivascular candle-wax drippings. Serpiginous choroiditis is typified by white atrophic serpentine lesions radiating from the optic disc. VKH, Behcet’s disease, sarcoidosis, Whipple’s disease and leukemia may be further delineated by their associated systemic involvement. VKH commonly occurs in darkly-pigmented individuals with resultant alopecia, poliosis and vitiligo. Behcet’s primarily affects males of Mediterranean origin with characteristic aphthous ulcers of the mouth and genitalia. Pulmonary disease and elevated serum levels of angiotensin converting enzyme represent sarcoidosis. Whipple’s disease presents as a multisystem granulomatous disorder while leukemia may be differentiated by a systemic preponderance of white blood cells. 5. Management and treatment (see Table 3) Therapeutic agents commonly prescribed for the treatment of birdshot retinochoroidopathy include: oral corticosteroids (prednisone), nonsteroidal anti-inflammatory drugs (indomethacin), immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporin A), antibiotics, antimetabolites and radiation to the posterior segment [4,5,9,10,12].To date, systemic corticosteroids have been the most commonly prescribed medication. Although patients have occassionally reported improvement in symptoms, improvement of ocular signs with the use of oral corticosteroids has been equivocal. These commonly administered therapeutic agents appear to be essentially ineffective. However, Hesse et al. recently reported subjective and objective improvement in active birdshot retinochoroidopathy when treating concurrent psoriasis with aromatic retinoids (etretinate, acitretin). This is the first report of a possible ocular therapeutic effect from aromatic retinoids, suggesting a need for further investigation 1141.
102
C.L. Beaulieu et al. /Clinical Eye and Vision Care 7 (1995) 99-102
Table 3 Management and treatment of birdshot retinochoroidopathy Amsler grid self-assessment Laser photoablation; when choroidal neovascultiation is present within the arcades Panretinal photocoagulation; when necessary for retinal neovascularization Therapeutic agents (controversial): oral corticosteroids,~ nonsteroidal anti-inflammatory drugs, immunosuppressive drugs, antibiotics, antimetabolites and radiation to the posterior segment.
Management of birdshot retinochoroidopathy involves periodic funduscopic examination and Amsler grid self-assessment for the detection of choroidal neovascularization. Laser photoablation is advocated in the presence of treatable choroidal neovascularization [7]. Retinal neovascularization, secondary to retinal non-perfusion, is treated with panretinal photocoagulation. 6. Conclusion Birdshot retinochoroidopathy is a rare bilateral ocular disorder with a chronic course of exacerbations and remissions. The anterior segment generally appears quiet with occasional m ild anterior chamber reaction. Characteristic flat cream-colored fundus lesions are generally scattered throughout the postequatorial fundus. Common ocular sequelae include vitritis, retinal vasculopathy, CME and optic disc edema or atrophy. Although the etiology remains unclear, the strong association between the HLAA29 antigen and birdshot retinochoroidopathy suggests a genetic predispositon. Currently, various therapeutic regimens are routinely administered but none have proven significantly effective. Recently, aromatic retinoids were found in one case to yield objective and subjective improvement providing a new direction for future research in the treatment of birdshot retinochoroidopathy. Patients with birdshot retinochoroidopathy require routine evaluation for the development of retinal or choroidal neovascularization.
References [ll Gass JDM. Vitiliginous chorioretinitis. Arch Ophthalmol 1981;99:1778-1787.
121 Baarsma GS, Priem HA, Kijlstra A. Association of birdshot retinochoroidopathy and HLA-A29 antigen. Gun Eye Res 1990$63-68. [31 Hirose T, Katsumi 0, Pruett RC, Sakaue H, Mehta M. Retinal function in birdshot retinochoroidopathy. Acta Ophthalmol 1991;69:327-337. 141 Priem HA, Oosterhuis JA. Birdshot chorioretinopathy: clinical characteristics and evolution. Br J Ophthalmol 1988;72:646-659. 151 Ryan SJ, Maumenee AE. Birdshot retinochoroidopathy. Am J Ophthahnol 1980,89:31-45. 161 LeHoang P, Ozdemir N, Benhamou A, Tabary T, Edelson C, Betuel H, Semiglia R, Cohen JHM. HLL429.2 subtype associated with birdshot retinochoroidopathy. Am J Ophthalmol1992;113:33-35. [71 Brucker AJ, Deglin EA, Bene C, Hoffman ME. Subretinal choroidal neovascularization in birdshot retinochoroidopathy. Am J Ophthahnol 1985;9940-44. IsI Soubrane G, Bokobza R, Coscas G. Late-developing lesions in birdshot retinochoroidopathy. Am J Ophthalmol 1990,109:204-210. 191 Fuerst DJ, Tessler HH, Fishman GA, Yokoyama MM, Wyhinny GJ, Vygantas CM. Birdshot retinochoroidopathy. Arch Ophthalmol 1984;102:214-219. [lOI Suttorp-Schulten MSA, Luyendijk L, Van Dam AP, deKeizer RJW, Baarsma GS, Bos PJM, Rothova A. Birdshot chorioretinopathy and lyme borreliosis. Am J Ophthalmol 1993;115:149-153. [ill Gass JDM. Stereoscopic atlas of macular diseases: diagnosis and treatment. St. Louis MO: The C.V. Mosby Company, 1987. WI Fich M, Rosenberg T. Birdshot retinochoroidopathy in monozygotic twins. Acta Ophthahnol 1992;70:693-697. [131 Bloch-Michel E, Frau E. Birdshot retinochoroidopathy and J-&4-A29+ and HLA-A29idiopathic retinal vasculitis: comparative study of 56 cases. Can J Ophthalmol 1991;26(7):361-366. [I41 Hesse S, Berbis PH, Chemila JF, Privat Y. Psoriasis and birdshot chorioretinopathy: Response to aromatic retinoids. Dermatology 1993;187:137-139. I151 Brod RD. Presumed sarcoid choroidopathy mimicking birdshot retinochoroidopathy. Am J Ophthalmol 1990;109:357-358. tt61 Kaplan HJ, Aaberg TM. Birdshot retinochoroidopathy. Am J Ophthalmol 1980; 90~773-782.