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Subretinal Neovascularization with Geographic (Serpiginous) Choroiditis
SUBRETINAL NEOVASCULARIZATION WITH G E O G R A P H I C (SERPIGINOUS) C H O R O I D I T I S L E E M. J A M P O L , M.D.,
D A V I D O R T H , M.D.,
AND M A U R I C E F. R A B B , Chicago, 1-5
Geographic (serpiginous) choroiditis is a chronic recurring disease of unknown origin characterized by destruction of the retinal pigment epithelium and choroidal vasculature with disruption of the overly ing retina. The disease, usually bilateral, begins in the peripapillary area but may extend to the macula and more peripheral portions of the fundus. Although once classified as a choroidal atrophy, 6 the oc casional presence of anterior uveitis, 7 ret inal vasculitis, 3 ' 7 ' 8 and vitreous cellular reaction 3 ' 7,8 has suggested an inflamma tory cause. Also consistent with this the ory is the absence of familial cases and the asymmetry noted in many patients. We describe herein three patients with geographic choroiditis complicated by the development of subretinal neovascular membranes with hemorrhaging and serous detachment of the retina. Argon laser photocoagulation successfully oblit erated the membrane in two of these patients with preservation of central vi sion.
M A R K J. D A I L Y ,
M.D.,
M.D.
Illinois tion at that time showed his visual acuity to be R.E.: 6/60 (20/200) and L.E.: 6/12 (20/40). The geographic peripapillary scarring had extended to involve the right macula. In August 1978, the patient lost central vision in his left eye.. His visual acuity at that time was R.E.: 6/60 (20/200) and L.E.: 6/30 (20/100). Slit-lamp biomicroscopy and applanation tonometry examinations showed normal findings. Oph thalmologic examination disclosed the peripapillary scarring with clumping, loss of retinal pigment epithelial pigment, and destruction of the choroidal vasculature. Two foci of active choroiditis were seen in the right eye (Figs. 1-3), although this eye was asymptomatic. 8 Lipid exudation was noted around one of these foci (Fig. 1). In the left eye an active focus of geographic choroiditis surrounded by subretinal hemorrhage and edema was present at the leading edge of the scar adjacent to the macula (Fig. 4). Fluorescein angiography revealed a subret inal neovascular membrane in this area (Fig. 5). No definite neovascular membranes could be identified in the two lesions in the right eye (Fig. 2). Despite therapy with systemic corticosteroids, vision in the left eye remained 6/60 (20/200). The subretinal neovascularization could not be treated with laser pho tocoagulation because of its proximity to the fovea.
CASE REPORTS Case 1—A 65-year-old man was first seen in December 1975. His visual acuity at that time was R.E.: 6/15 (20/50) and L.E.: 6/12 (20/40). He had had multiple episodes of visual loss in both eyes since 1955. Treatment on several occasions consist ed of systemic corticosteroids. Examination in 1975 showed peripapillary scarring in both eyes with no angioid streaks or peripheral retinal lesions; a diag nosis of Jensen's choroiditis (peripapillary choroid itis) was made. In July 1977, the patient complained of a decrease in vision in his right eye. Ophthalmologic examinaFrom the Departments of Ophthalmology, Uni versity of Illinois Eye and Ear Infirmary. Chicago. Illinois (Drs. Jampol, Orth, and Rabb); and Loyola University, Maywood, Illinois (Dr. Daily). Reprint requests to Lee M. Jampol, VI.D., 1855 VV. Taylor St., Chicago, II, 60612.
Fig. I (Jampol and associates). Case 1. Right eye, extensive peripapillary scarring is present with pig ment (Jumping and loss of choroidal vasculature. Focus of active geographic choroiditis is seen (ar rows) with surrounding exudation.
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Fig. 2 (Jampol and associates). Case 1. Right eye, second focus of active choroiditis is seen along superotemporal vessels (arrows).
Case 2—A 58-year-old woman noticed distortion and double vision in her right eye in 1972. She was first seen initially at another hospital; an examina tion included a negative tuberculin skin test result. Treatment with systemic corticosteroids failed to restore central vision. In November 1975 she noted bright flashes of light and distortion of vision in her
Fig. 4 (Jampol and associates). Case 1. Left eye, peripapillary scarring is visible with focus of activi ty present (arrows). Some subretinal hemorrhage is seen. A flat serous detachment extends to involve the fovea.
OCTOBER, 1979
Fig. 3 (Jampol and associates). Case 1. Right eye, late phase fluorescein angiogram showns staining of both active foci. No neovascular membrane is shown.
left eye. She was seen by an ophthalmologist who prescribed a course of systemic corticosteroids. The patient was first seen by us Jan. 5, 1976. Examination at that time revealed visual acuity to be R.E.: 6/120 (20/400) and L.E.: 6/9-2 (20/30-2). Exter nal examination, slit-lamp examination, and applanation tonometry were unremarkable. Examination of the right fundus revealed multiple areas of atrophy of the pigment epithelium and choriocapillaris within the posterior pole with a large subretinal scar that extended between the macula and the optic disk. No peripheral retinal lesions were present. In the left eye, similar large inactive scars were present throughout the posterior pole with a loss of pigment epithelial pigment and choriocapillaris (Fig. 6). Additionally, there was a yellow, creamy appearing lesion emanating from the superotem poral aspect of the left disk and extending toward the foveal area (Fig. 6). Overlying this lesion was a serous detachment of the sensory retina. Fluorescein angiography revealed a subretinal neovascular membrane in this area (Fig. 7). The diagnosis at that time was geographic choroiditis with a subretinal neovascular membrane in the left eye. The patient was seen again three days later, at which time vision in her left eye had deteriorated to 6/24 (20/80). In creased serous detachment of the retina and two small subretinal hemorrhages were noted. The patient was treated with argon laser photocoagulation. Using retrobulbar anesthesia, 167 spots were placed (50 to 100 u. spot size, 0.1- to 0.5-second duration, and 400 to 700 mW). No complications of treatment were noted. Visual acuity in the left eye returned to 6/7.5 (20/25) during the next several
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Fig. 5 (Jampol and associates). Case 1. Left eye, venous phase fluorescein angiogram shows welldeveloped neovascular net in area of active choroiditis. Subretinal blood blocks fluorescence (left). Late phase angiogram shows intense staining of active lesion and a lesser degree of staining of inactive scar (right).
weeks. There was evidence of regression of the neovascular membrane (Fig. 8). The subretinal hemorrhage and serous elevation of the retina grad ually cleared. When the patient was last seen Oct.
Fig. 6 (Jampol and associates). Case 2. Left eye, multiple areas of old geographic choroiditis are seen. A yellow subretinal membrane extends from the superior margin of the disk toward the fovea (arrows).
12, 1978, visual acuity was 6/9+ (20/30+) in the left eye. No evidence of reactivation of the choroiditis or reperfusion of the subretinal neovascular membrane was noted. Case 3—A 48-year-old man first noted blurring of vision in his left eye in March 1977. His medical history revealed pulmonary tuberculosis at age 14 vears and hypothyroidism. Visual acuity was 6/6 (20/20) in both eyes (R.E.: - 1 . 7 5 Sph.; L.E.: - 1 . 5 0 Sph.) but examination of the fundi revealed multiple geographic areas of chorioretinal atrophy in the posterior poles (Figs. 9 and 10). Peripapillary scar ring was present, but there were no peripheral chorioretinal lesions. Inferior to the left macula, a subretinal neovascular membrane was present with subretinal hemorrhage (Fig. 11). Visual field testing showed paracentral scotomas in both eyes that cor responded to the areas of chorioretinal atrophy. The patient was seen in consultation by several addition al retinal specialists who agreed that the patient had geographic (serpiginous) choroiditis. The patient was treated initially with corticosteroids and in April 1977 had intense argon laser photocoagulation to the neovascular membrane in the left eye (200 \i. spot size, 0.5 seconds, 1.2 W). Subsequently, this area showed total regression of the neovascular membrane (Fig. 12). The patient had several small flare-ups of geographic choroid itis in both eyes during the next year with extension of one area of scarring toward the right fovea. These episodes were treated with systemic corticosteroids. A high intraocular pressure believed to be secondary to the corticosteroid therapy was noted on several occasions. This was treated with topical epineph-
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OCTOBER, 1979
Fig. 7 (Jampol and associates). Case 2. Left eye, fluorescein angiography confirms the presence of a subretinal neovascular membrane (arrows). Venous phase (left). Late view showing leakage from membrane (right).
DISCUSSION
Geographic (serpiginous) choroiditis usually appears between the ages of 30 and 70 years with the patient having unilateral visual loss secondary to in volvement of the macula of one eye. Dis
ease in the peripapillary area of both eyes is evident. Successive attacks result in a pseudopodial extension of the process peripherally. The acute lesions have a gray opaque appearance that seems local ized at the level of the retinal pigment
Fig. 8 (Jampol and associates). Case 2. Left eye, several months posttreatment, angiogram confirms obliteration of the subretinal neovascularization.
Fig. 9 (Jampol and associates). Case 3. Right eye, geographic areas of chorioretinal atrophy are visible along the inferotemporal vessels.
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Fig. 10 (Jampol and associates). Case 3. Left eye, changes similar to right eye are seen; additionally, a subretinal neovascular membrane is seen near inferotemporal vessels. Subretinal hemorrhage is also present.
epithelium. Overlying retinal edema and, rarely, serous detachment of the retina or retinal vasculitis 3 ' 7 ' 8 may be seen. Fluorescein angiography of the acute lesions shows blockage of underlying
Fig. 12 (Jampol and associates). Case 3. Left eye, after laser treatment, the neovascular membrane is obliterated.
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Fig. 11 (Jampol and associates). Case 3. Left eye, fluorescein angiogram confirms presence of subret inal neovascularization.
choroidal fluorescence with a gradual flu orescein staining of the lesion. Leaking of fluorescein from choriocapillaris at the margins of the lesions may be seen. Over a period of weeks or months, the gray lesions evolve into atrophic scars charac terized by mottling and loss of retinal pigment epithelial pigment, the appear ance of subretinal scar tissue, and a varia ble and sometimes marked destruction of the underlying choriocapillaris and larger choroidal vessels. Recurrent episodes are frequently adjacent to the areas of previ ous involvement, although isolated foci occasionally may be separated from old scars. The pathogenesis of geographic choroiditis remains unknown. No systemic involvement is found. The presence of anterior uveitis, retinal vasculitis, and vit reous cells in some cases has suggested an inflammatory cause. 3 ' 7,8 Consequently, many patients have been treated with systemic corticosteroids or even cytotoxic immunosuppressive agents. 3 No evidence of an infective or toxic agent has been found. Prominent choroidal vascular
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obliteration initially led to the inclusion of geographic choroiditis in the group of choroidal atrophies. 6 With long-standing disease, the diffuse choroidal and retinal atrophy may suggest a hereditary dystrophic process. 4 However, the late onset, the acuteness of visual loss, the asymme try between eyes, and the absence of familial cases are contrary to this cause. Subretinal neovascularization associat ed with classic geographic choroiditis has not been previously described. Several authors have specifically commented on the absence of this complication. 2 - 4 ' 9 ' 10 Gass 1 0 described one patient with "an atypical case of serpiginous choroiditis" characterized by subretinal neovasculari zation with hemorrhaging and exudation. Our three cases show that subretinal neovascular membranes can occur in as sociation with geographic choroiditis. In Case 1 the membrane was impinging on the foveal avascular zone, with serous detachment of the retina and subretinal hemorrhage. The lesion could not be photocoagulated because of its location. Cen tral visual acuity remained 6/60 (20/200) despite systemic corticosteroid therapy. The neovascular membrane in Case 2 was destroyed by intense argon laser photoco agulation. The serous detachment and subretinal hemorrhage resolved with an improvement in visual acuity from 6/24 (20/80) to 6/9 (20/30) in the left eye. Argon laser photocoagulation also successfully destroyed the neovascular membrane in Case 3 and visual acuity remained 6/6 (20/20) in both eyes. In the normal eye, Bruch's membrane serves as a barrier separating the chor oidal circulation from the retina. 11 De struction of this barrier by trauma, photocoagulation, surgery, tumors, or degener ative diseases (for example, myopia, drusen, angioid streaks) may result in subretinal neovascular membranes fed by the choroidal circulation. Recently, some patients with dystrophic diseases such as
OCTOBER, 1979
Best's disease 12 and fundus flavimaculatus 1 3 have also developed subretinal neo vascularization. Another important cause of subretinal neovascularization is in flammatory chorioretinal disease (for ex ample, presumed ocular histoplasmosis, rubella 14 ). These diseases disrupt Bruch's membrane and allow choroidal vascular ingrowth. A destructive process such as geographic choroiditis is characterized by only occasional development of a subreti nal neovascular membrane. Possibly, the obliteration of the underlying choroidal vessels that characterizes geographic cho roiditis may account for the rarity of chor oidal vasculature ingrowth and subretinal neovascular membranes in this disease. Clinical experience has shown that in tense photocoagulation can destroy sub retinal neovascular membranes. In a simi lar manner destruction of the choroidal vessels by geographic choroiditis may discourage choroidal vascular growth through Bruch's membrane. ACKNOWLEDGMENT
Theodore Schlaegel, Jr., M.D., examined the pa tient described in Case 3 and administered the photocoagulation treatment. SUMMARY
Three patients with geographic cho roiditis developed subretinal neovascular membranes associated with subretinal hemorrhage and serous elevation of the retina. Proximity of the membrane to the foveal avascular zone prevented photoco agulation of the neovascular membrane in one patient. Central visual acuity re mained 6/60 (20/200) despite systemic corticosteroid therapy. The neovascular membranes in the other two patients were obliterated by argon laser photocoagula tion with preservation of central vision. The inflammatory process of geographic choroiditis can disrupt Bruch's mem brane, allowing occasional choroidal vas cular growth. Concomitant destruction of
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choroidal vessels may account for rarity of subretinal neovascularization in geo graphic choroiditis. REFERENCES 1. Schatz, H., Maumenee, A. E., and Patz, A.: Geographic helicoid peripapillary choroidopathy. Trans. Am. Acad. Ophthalmol. Otolaryngol. 78:747, 1974. 2. Hamilton, A. M., and Bird, A. C : Geographic choroidopathy. Br. J. Ophthalmol. 58:784, 1974. 3. Laatikainen, L., and Erkkila, H.: Serpiginous choroiditis. Br. J. Ophthalmol. 58:777, 1974. 4. Chisholm, I. H., Gass, J. D. M., and Hutton, W. L.: The late stage of serpiginous (geographic) choroiditis. Am. J. Ophthalmol. 82:343, 1976. 5. Baarsma, G. S., and Deutman, A. F.: Serpigi nous (geographic) choroiditis. Doc. Ophthalmol. 40:269, 1976. 6. Krill, A. E., and Archer, D.: Classification of the choroidal atrophies. Am. J. Ophthalmol. 72:562, 1971.
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7. Masi, R. J., O'Connor, G. R., and Kimura, S. J.: Anterior uveitis in geographic or serpiginous cho roiditis. Am. J. Ophthalmol. 86:228, 1978. 8. Weiss, H., Annesley, W. H., Jr., Shields, J. A., Tomer, T., and Christopherson, K.: The clinical course of serpiginous choroidopathy. Am. J. Oph thalmol. 87:133, 1979. 9. Schatz, H.: Other retinal pigment epithelial diseases. Int. Ophthalmol. Clin. 15:181, 1975. 10. Gass, J. D. M.: Steroscopic Atlas of Macular Diseases. Diagnosis and Treatment, 2nd ed. St. Louis, C. V. Mosby, 1977, p p . 114-117. 11. Goldberg, M. F.: Bruch's membrane and vas cular growth. Invest. Ophthalmol. 15:443, 1976. 12. Miller, S. A., Bresnick, G. H., and Chandra, S. R.: Choroidal neovascular membrane in Best's vitelliform macular dystrophy. Am. J. Ophthalmol. 82:252, 1976. 13. Klein, R., Lewis, R. A., Meyers, S. M., and Myers, F. L.: Subretinal neovascularization associ ated with fundus flavimaculatus. Arch. Ophthalmol. 96:2054, 1978. 14. Frank, K. E., and Purnell, E. W.: Subretinal neovascularization following rubella retinoDathy. Am. J. Ophthalmol. 86:462, 1978.
D A V I D O R T H , M.D.,
AND M A U R I C E F. R A B B , Chicago, 1-5
Geographic (serpiginous) choroiditis is a chronic recurring disease of unknown origin characterized by destruction of the retinal pigment epithelium and choroidal vasculature with disruption of the overly ing retina. The disease, usually bilateral, begins in the peripapillary area but may extend to the macula and more peripheral portions of the fundus. Although once classified as a choroidal atrophy, 6 the oc casional presence of anterior uveitis, 7 ret inal vasculitis, 3 ' 7 ' 8 and vitreous cellular reaction 3 ' 7,8 has suggested an inflamma tory cause. Also consistent with this the ory is the absence of familial cases and the asymmetry noted in many patients. We describe herein three patients with geographic choroiditis complicated by the development of subretinal neovascular membranes with hemorrhaging and serous detachment of the retina. Argon laser photocoagulation successfully oblit erated the membrane in two of these patients with preservation of central vi sion.
M A R K J. D A I L Y ,
M.D.,
M.D.
Illinois tion at that time showed his visual acuity to be R.E.: 6/60 (20/200) and L.E.: 6/12 (20/40). The geographic peripapillary scarring had extended to involve the right macula. In August 1978, the patient lost central vision in his left eye.. His visual acuity at that time was R.E.: 6/60 (20/200) and L.E.: 6/30 (20/100). Slit-lamp biomicroscopy and applanation tonometry examinations showed normal findings. Oph thalmologic examination disclosed the peripapillary scarring with clumping, loss of retinal pigment epithelial pigment, and destruction of the choroidal vasculature. Two foci of active choroiditis were seen in the right eye (Figs. 1-3), although this eye was asymptomatic. 8 Lipid exudation was noted around one of these foci (Fig. 1). In the left eye an active focus of geographic choroiditis surrounded by subretinal hemorrhage and edema was present at the leading edge of the scar adjacent to the macula (Fig. 4). Fluorescein angiography revealed a subret inal neovascular membrane in this area (Fig. 5). No definite neovascular membranes could be identified in the two lesions in the right eye (Fig. 2). Despite therapy with systemic corticosteroids, vision in the left eye remained 6/60 (20/200). The subretinal neovascularization could not be treated with laser pho tocoagulation because of its proximity to the fovea.
CASE REPORTS Case 1—A 65-year-old man was first seen in December 1975. His visual acuity at that time was R.E.: 6/15 (20/50) and L.E.: 6/12 (20/40). He had had multiple episodes of visual loss in both eyes since 1955. Treatment on several occasions consist ed of systemic corticosteroids. Examination in 1975 showed peripapillary scarring in both eyes with no angioid streaks or peripheral retinal lesions; a diag nosis of Jensen's choroiditis (peripapillary choroid itis) was made. In July 1977, the patient complained of a decrease in vision in his right eye. Ophthalmologic examinaFrom the Departments of Ophthalmology, Uni versity of Illinois Eye and Ear Infirmary. Chicago. Illinois (Drs. Jampol, Orth, and Rabb); and Loyola University, Maywood, Illinois (Dr. Daily). Reprint requests to Lee M. Jampol, VI.D., 1855 VV. Taylor St., Chicago, II, 60612.
Fig. I (Jampol and associates). Case 1. Right eye, extensive peripapillary scarring is present with pig ment (Jumping and loss of choroidal vasculature. Focus of active geographic choroiditis is seen (ar rows) with surrounding exudation.
AMERICAN JOURNAL O F OPHTHALMOLOGY 88:683-689, 1979
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Fig. 2 (Jampol and associates). Case 1. Right eye, second focus of active choroiditis is seen along superotemporal vessels (arrows).
Case 2—A 58-year-old woman noticed distortion and double vision in her right eye in 1972. She was first seen initially at another hospital; an examina tion included a negative tuberculin skin test result. Treatment with systemic corticosteroids failed to restore central vision. In November 1975 she noted bright flashes of light and distortion of vision in her
Fig. 4 (Jampol and associates). Case 1. Left eye, peripapillary scarring is visible with focus of activi ty present (arrows). Some subretinal hemorrhage is seen. A flat serous detachment extends to involve the fovea.
OCTOBER, 1979
Fig. 3 (Jampol and associates). Case 1. Right eye, late phase fluorescein angiogram showns staining of both active foci. No neovascular membrane is shown.
left eye. She was seen by an ophthalmologist who prescribed a course of systemic corticosteroids. The patient was first seen by us Jan. 5, 1976. Examination at that time revealed visual acuity to be R.E.: 6/120 (20/400) and L.E.: 6/9-2 (20/30-2). Exter nal examination, slit-lamp examination, and applanation tonometry were unremarkable. Examination of the right fundus revealed multiple areas of atrophy of the pigment epithelium and choriocapillaris within the posterior pole with a large subretinal scar that extended between the macula and the optic disk. No peripheral retinal lesions were present. In the left eye, similar large inactive scars were present throughout the posterior pole with a loss of pigment epithelial pigment and choriocapillaris (Fig. 6). Additionally, there was a yellow, creamy appearing lesion emanating from the superotem poral aspect of the left disk and extending toward the foveal area (Fig. 6). Overlying this lesion was a serous detachment of the sensory retina. Fluorescein angiography revealed a subretinal neovascular membrane in this area (Fig. 7). The diagnosis at that time was geographic choroiditis with a subretinal neovascular membrane in the left eye. The patient was seen again three days later, at which time vision in her left eye had deteriorated to 6/24 (20/80). In creased serous detachment of the retina and two small subretinal hemorrhages were noted. The patient was treated with argon laser photocoagulation. Using retrobulbar anesthesia, 167 spots were placed (50 to 100 u. spot size, 0.1- to 0.5-second duration, and 400 to 700 mW). No complications of treatment were noted. Visual acuity in the left eye returned to 6/7.5 (20/25) during the next several
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Fig. 5 (Jampol and associates). Case 1. Left eye, venous phase fluorescein angiogram shows welldeveloped neovascular net in area of active choroiditis. Subretinal blood blocks fluorescence (left). Late phase angiogram shows intense staining of active lesion and a lesser degree of staining of inactive scar (right).
weeks. There was evidence of regression of the neovascular membrane (Fig. 8). The subretinal hemorrhage and serous elevation of the retina grad ually cleared. When the patient was last seen Oct.
Fig. 6 (Jampol and associates). Case 2. Left eye, multiple areas of old geographic choroiditis are seen. A yellow subretinal membrane extends from the superior margin of the disk toward the fovea (arrows).
12, 1978, visual acuity was 6/9+ (20/30+) in the left eye. No evidence of reactivation of the choroiditis or reperfusion of the subretinal neovascular membrane was noted. Case 3—A 48-year-old man first noted blurring of vision in his left eye in March 1977. His medical history revealed pulmonary tuberculosis at age 14 vears and hypothyroidism. Visual acuity was 6/6 (20/20) in both eyes (R.E.: - 1 . 7 5 Sph.; L.E.: - 1 . 5 0 Sph.) but examination of the fundi revealed multiple geographic areas of chorioretinal atrophy in the posterior poles (Figs. 9 and 10). Peripapillary scar ring was present, but there were no peripheral chorioretinal lesions. Inferior to the left macula, a subretinal neovascular membrane was present with subretinal hemorrhage (Fig. 11). Visual field testing showed paracentral scotomas in both eyes that cor responded to the areas of chorioretinal atrophy. The patient was seen in consultation by several addition al retinal specialists who agreed that the patient had geographic (serpiginous) choroiditis. The patient was treated initially with corticosteroids and in April 1977 had intense argon laser photocoagulation to the neovascular membrane in the left eye (200 \i. spot size, 0.5 seconds, 1.2 W). Subsequently, this area showed total regression of the neovascular membrane (Fig. 12). The patient had several small flare-ups of geographic choroid itis in both eyes during the next year with extension of one area of scarring toward the right fovea. These episodes were treated with systemic corticosteroids. A high intraocular pressure believed to be secondary to the corticosteroid therapy was noted on several occasions. This was treated with topical epineph-
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OCTOBER, 1979
Fig. 7 (Jampol and associates). Case 2. Left eye, fluorescein angiography confirms the presence of a subretinal neovascular membrane (arrows). Venous phase (left). Late view showing leakage from membrane (right).
DISCUSSION
Geographic (serpiginous) choroiditis usually appears between the ages of 30 and 70 years with the patient having unilateral visual loss secondary to in volvement of the macula of one eye. Dis
ease in the peripapillary area of both eyes is evident. Successive attacks result in a pseudopodial extension of the process peripherally. The acute lesions have a gray opaque appearance that seems local ized at the level of the retinal pigment
Fig. 8 (Jampol and associates). Case 2. Left eye, several months posttreatment, angiogram confirms obliteration of the subretinal neovascularization.
Fig. 9 (Jampol and associates). Case 3. Right eye, geographic areas of chorioretinal atrophy are visible along the inferotemporal vessels.
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Fig. 10 (Jampol and associates). Case 3. Left eye, changes similar to right eye are seen; additionally, a subretinal neovascular membrane is seen near inferotemporal vessels. Subretinal hemorrhage is also present.
epithelium. Overlying retinal edema and, rarely, serous detachment of the retina or retinal vasculitis 3 ' 7 ' 8 may be seen. Fluorescein angiography of the acute lesions shows blockage of underlying
Fig. 12 (Jampol and associates). Case 3. Left eye, after laser treatment, the neovascular membrane is obliterated.
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Fig. 11 (Jampol and associates). Case 3. Left eye, fluorescein angiogram confirms presence of subret inal neovascularization.
choroidal fluorescence with a gradual flu orescein staining of the lesion. Leaking of fluorescein from choriocapillaris at the margins of the lesions may be seen. Over a period of weeks or months, the gray lesions evolve into atrophic scars charac terized by mottling and loss of retinal pigment epithelial pigment, the appear ance of subretinal scar tissue, and a varia ble and sometimes marked destruction of the underlying choriocapillaris and larger choroidal vessels. Recurrent episodes are frequently adjacent to the areas of previ ous involvement, although isolated foci occasionally may be separated from old scars. The pathogenesis of geographic choroiditis remains unknown. No systemic involvement is found. The presence of anterior uveitis, retinal vasculitis, and vit reous cells in some cases has suggested an inflammatory cause. 3 ' 7,8 Consequently, many patients have been treated with systemic corticosteroids or even cytotoxic immunosuppressive agents. 3 No evidence of an infective or toxic agent has been found. Prominent choroidal vascular
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obliteration initially led to the inclusion of geographic choroiditis in the group of choroidal atrophies. 6 With long-standing disease, the diffuse choroidal and retinal atrophy may suggest a hereditary dystrophic process. 4 However, the late onset, the acuteness of visual loss, the asymme try between eyes, and the absence of familial cases are contrary to this cause. Subretinal neovascularization associat ed with classic geographic choroiditis has not been previously described. Several authors have specifically commented on the absence of this complication. 2 - 4 ' 9 ' 10 Gass 1 0 described one patient with "an atypical case of serpiginous choroiditis" characterized by subretinal neovasculari zation with hemorrhaging and exudation. Our three cases show that subretinal neovascular membranes can occur in as sociation with geographic choroiditis. In Case 1 the membrane was impinging on the foveal avascular zone, with serous detachment of the retina and subretinal hemorrhage. The lesion could not be photocoagulated because of its location. Cen tral visual acuity remained 6/60 (20/200) despite systemic corticosteroid therapy. The neovascular membrane in Case 2 was destroyed by intense argon laser photoco agulation. The serous detachment and subretinal hemorrhage resolved with an improvement in visual acuity from 6/24 (20/80) to 6/9 (20/30) in the left eye. Argon laser photocoagulation also successfully destroyed the neovascular membrane in Case 3 and visual acuity remained 6/6 (20/20) in both eyes. In the normal eye, Bruch's membrane serves as a barrier separating the chor oidal circulation from the retina. 11 De struction of this barrier by trauma, photocoagulation, surgery, tumors, or degener ative diseases (for example, myopia, drusen, angioid streaks) may result in subretinal neovascular membranes fed by the choroidal circulation. Recently, some patients with dystrophic diseases such as
OCTOBER, 1979
Best's disease 12 and fundus flavimaculatus 1 3 have also developed subretinal neo vascularization. Another important cause of subretinal neovascularization is in flammatory chorioretinal disease (for ex ample, presumed ocular histoplasmosis, rubella 14 ). These diseases disrupt Bruch's membrane and allow choroidal vascular ingrowth. A destructive process such as geographic choroiditis is characterized by only occasional development of a subreti nal neovascular membrane. Possibly, the obliteration of the underlying choroidal vessels that characterizes geographic cho roiditis may account for the rarity of chor oidal vasculature ingrowth and subretinal neovascular membranes in this disease. Clinical experience has shown that in tense photocoagulation can destroy sub retinal neovascular membranes. In a simi lar manner destruction of the choroidal vessels by geographic choroiditis may discourage choroidal vascular growth through Bruch's membrane. ACKNOWLEDGMENT
Theodore Schlaegel, Jr., M.D., examined the pa tient described in Case 3 and administered the photocoagulation treatment. SUMMARY
Three patients with geographic cho roiditis developed subretinal neovascular membranes associated with subretinal hemorrhage and serous elevation of the retina. Proximity of the membrane to the foveal avascular zone prevented photoco agulation of the neovascular membrane in one patient. Central visual acuity re mained 6/60 (20/200) despite systemic corticosteroid therapy. The neovascular membranes in the other two patients were obliterated by argon laser photocoagula tion with preservation of central vision. The inflammatory process of geographic choroiditis can disrupt Bruch's mem brane, allowing occasional choroidal vas cular growth. Concomitant destruction of
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GEOGRAPHIC CHOROIDITIS
choroidal vessels may account for rarity of subretinal neovascularization in geo graphic choroiditis. REFERENCES 1. Schatz, H., Maumenee, A. E., and Patz, A.: Geographic helicoid peripapillary choroidopathy. Trans. Am. Acad. Ophthalmol. Otolaryngol. 78:747, 1974. 2. Hamilton, A. M., and Bird, A. C : Geographic choroidopathy. Br. J. Ophthalmol. 58:784, 1974. 3. Laatikainen, L., and Erkkila, H.: Serpiginous choroiditis. Br. J. Ophthalmol. 58:777, 1974. 4. Chisholm, I. H., Gass, J. D. M., and Hutton, W. L.: The late stage of serpiginous (geographic) choroiditis. Am. J. Ophthalmol. 82:343, 1976. 5. Baarsma, G. S., and Deutman, A. F.: Serpigi nous (geographic) choroiditis. Doc. Ophthalmol. 40:269, 1976. 6. Krill, A. E., and Archer, D.: Classification of the choroidal atrophies. Am. J. Ophthalmol. 72:562, 1971.
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7. Masi, R. J., O'Connor, G. R., and Kimura, S. J.: Anterior uveitis in geographic or serpiginous cho roiditis. Am. J. Ophthalmol. 86:228, 1978. 8. Weiss, H., Annesley, W. H., Jr., Shields, J. A., Tomer, T., and Christopherson, K.: The clinical course of serpiginous choroidopathy. Am. J. Oph thalmol. 87:133, 1979. 9. Schatz, H.: Other retinal pigment epithelial diseases. Int. Ophthalmol. Clin. 15:181, 1975. 10. Gass, J. D. M.: Steroscopic Atlas of Macular Diseases. Diagnosis and Treatment, 2nd ed. St. Louis, C. V. Mosby, 1977, p p . 114-117. 11. Goldberg, M. F.: Bruch's membrane and vas cular growth. Invest. Ophthalmol. 15:443, 1976. 12. Miller, S. A., Bresnick, G. H., and Chandra, S. R.: Choroidal neovascular membrane in Best's vitelliform macular dystrophy. Am. J. Ophthalmol. 82:252, 1976. 13. Klein, R., Lewis, R. A., Meyers, S. M., and Myers, F. L.: Subretinal neovascularization associ ated with fundus flavimaculatus. Arch. Ophthalmol. 96:2054, 1978. 14. Frank, K. E., and Purnell, E. W.: Subretinal neovascularization following rubella retinoDathy. Am. J. Ophthalmol. 86:462, 1978.