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8. Geographic (Serpiginous) Choroidopathy
Disorders of the Fundus Edited by Ronald E. Carr, MD
8. Geographic (Serpiginous) Choroidopathy
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RONALD E. CARR, MD, KENNETH G. NOBLE, MD
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Geographic (serpiginous) choroidopathy is a non-familial, progressive disease of the retinal pigment epithelium and choriocapillaris whose cause is unknown. The age of discovery is between the fourth and sixth decade although the age of onset may be earlier since these individuals are asymptomatic until the macula is affected. There is no obvious sex predilection but it has been most commonly reported in Caucasians. The diagnosis is made on the characteristic fundus picture which is present bilaterally, although marked asymmetry is not uncommon. The inactive healed lesions radiate from the peripapillary area in an irregular convoluted serpentine course and appear as scalloped, punched-out areas of pigment and choroidal atrophy (Fig lA). In this chronic disorder, active areas are continually arising and are identifiable as opaque yellowish to grayish lesions with hazy borders (Fig 2). They usually occur contiguous with an older healed lesion although occasional isolated focal areas of active lesions may arise. The general course is one of multiple recurrences with activity progressing in a centrifugal fashion from the disc outward toward the midperiphery. If the advancing borders do not involve the fovea, the prognosis for retain-
From the Department of Ophthalmology, the New York University Medical Center, 550 First Avenue, New York, NY 10016.
ing vision is good although the clinician should be alerted to those rare instances when centripetal progression may result in activity of areas previously bypassed. Visual acuity may also be compromised by the development of a subretinal choroidal vascular network arising at the border of a lesion (Fig 3). Fluorescein angiography in the early phase of the inactive healed lesion shows the larger choroidal vessels indicating atrophy of the choriocapillaris and pigment epithelium, while in the late phase there is diffuse hyperfluorescence due to staining of the sclera from adjacent normally leaking choriocapillaris (Figs lB, lC). The active lesions shows hypofluorescence initially (due to either edema of the pigment epithelium or choriocapillaris nonperfusion) with gradual staining and late hyperfluorescence. Visual function studies demonstrate that this disorder is focal or geographic in nature and does not represent a generalized retinal degeneration. Visual field scotoma corresponds to the visible retinal lesion and the amplitude of the electroretinogram is decreased roughly in proportion to the amount of retina involved. The cause of this disorder remains obscure. There is no associated vasculitis or papillitis, evidence of inflammation elsewhere in the eye is usually lacking, and these individuals are in good general health. There is no known treatment.
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Fig 1. The atrophic areas of retina and choroid are well-demarcated and run from the disc temporally to involve the macula. Most lesions have a small isthmus to an adjoining area although a few isolated areas are evident (Fig IA, top). The visibility of the larger choroidal vessels in the early angiogram signifies atrophy of both the retinal pigment epithelium and the choriocapillaris (Fig lB, bottom left). The "jigsaw puzzle" appearance of the lesion is made vivid by the hyperfluorescence of late scleral staining (Fig IC, bottom ri[?ht).
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Fig 2. Visual acuity in the left eye of this 50-year-old man was counting fingers due to macular involvement. The course of the serpiginous lesions in his right eye (vision 20/20) was of great concern. Although over a five-year period the patient was asymptomatic maintaining 20/20 vision, he developed an active lesion first at the inferior edge (Fig 2A, top left), then at the superior edge (Fig 2B, top right) both of which healed (Fig 2C, bottom right).
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Fig 3. The sudden dramatic visual loss is due to a choroidal neovascular network arising at the border of an inactive atrophic lesion and resulting in a hemorrhagic maculopathy (Fig 3A, top left). The early lacy, nodular hyperfluorescence and late leakage are the characteristic angiographic findings (Figs. 3B, top right; 3C, bottom right).
8. Geographic (Serpiginous) Choroidopathy
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RONALD E. CARR, MD, KENNETH G. NOBLE, MD
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Geographic (serpiginous) choroidopathy is a non-familial, progressive disease of the retinal pigment epithelium and choriocapillaris whose cause is unknown. The age of discovery is between the fourth and sixth decade although the age of onset may be earlier since these individuals are asymptomatic until the macula is affected. There is no obvious sex predilection but it has been most commonly reported in Caucasians. The diagnosis is made on the characteristic fundus picture which is present bilaterally, although marked asymmetry is not uncommon. The inactive healed lesions radiate from the peripapillary area in an irregular convoluted serpentine course and appear as scalloped, punched-out areas of pigment and choroidal atrophy (Fig lA). In this chronic disorder, active areas are continually arising and are identifiable as opaque yellowish to grayish lesions with hazy borders (Fig 2). They usually occur contiguous with an older healed lesion although occasional isolated focal areas of active lesions may arise. The general course is one of multiple recurrences with activity progressing in a centrifugal fashion from the disc outward toward the midperiphery. If the advancing borders do not involve the fovea, the prognosis for retain-
From the Department of Ophthalmology, the New York University Medical Center, 550 First Avenue, New York, NY 10016.
ing vision is good although the clinician should be alerted to those rare instances when centripetal progression may result in activity of areas previously bypassed. Visual acuity may also be compromised by the development of a subretinal choroidal vascular network arising at the border of a lesion (Fig 3). Fluorescein angiography in the early phase of the inactive healed lesion shows the larger choroidal vessels indicating atrophy of the choriocapillaris and pigment epithelium, while in the late phase there is diffuse hyperfluorescence due to staining of the sclera from adjacent normally leaking choriocapillaris (Figs lB, lC). The active lesions shows hypofluorescence initially (due to either edema of the pigment epithelium or choriocapillaris nonperfusion) with gradual staining and late hyperfluorescence. Visual function studies demonstrate that this disorder is focal or geographic in nature and does not represent a generalized retinal degeneration. Visual field scotoma corresponds to the visible retinal lesion and the amplitude of the electroretinogram is decreased roughly in proportion to the amount of retina involved. The cause of this disorder remains obscure. There is no associated vasculitis or papillitis, evidence of inflammation elsewhere in the eye is usually lacking, and these individuals are in good general health. There is no known treatment.
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Fig 1. The atrophic areas of retina and choroid are well-demarcated and run from the disc temporally to involve the macula. Most lesions have a small isthmus to an adjoining area although a few isolated areas are evident (Fig IA, top). The visibility of the larger choroidal vessels in the early angiogram signifies atrophy of both the retinal pigment epithelium and the choriocapillaris (Fig lB, bottom left). The "jigsaw puzzle" appearance of the lesion is made vivid by the hyperfluorescence of late scleral staining (Fig IC, bottom ri[?ht).
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Fig 2. Visual acuity in the left eye of this 50-year-old man was counting fingers due to macular involvement. The course of the serpiginous lesions in his right eye (vision 20/20) was of great concern. Although over a five-year period the patient was asymptomatic maintaining 20/20 vision, he developed an active lesion first at the inferior edge (Fig 2A, top left), then at the superior edge (Fig 2B, top right) both of which healed (Fig 2C, bottom right).
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Fig 3. The sudden dramatic visual loss is due to a choroidal neovascular network arising at the border of an inactive atrophic lesion and resulting in a hemorrhagic maculopathy (Fig 3A, top left). The early lacy, nodular hyperfluorescence and late leakage are the characteristic angiographic findings (Figs. 3B, top right; 3C, bottom right).