In 1949, Sorsby described a familial fundus disease with progressive visual loss and bilateral hemorrhages and exudates of the posterior pole. The dystrophy, whose inheritance was apparently autosomal dominant, was called pseudo-inflammatory macular dystrophy because of extensive macular lesions that could suggest a post-inflammatory change. The ophthalmoscopic signs include the presence of diffuse drusen-like deposits with extensive changes of retinal pigment epithelium and focal atrophy of the choriocapillaris, particularly in the posterior pole. Some eyes grow subretinal neovascularization, which appears associated to retinal edema, deep hemorrhages and hard exudates. These cases ultimately result in a disciform macular scar. Atrophy of peripheral fundus is typical of advanced stages. We present the case of three sisters whose fundus lesions resembled Sorsby's pseudo-inflammatory macular dystrophy. In two of them, where there was a great suspicion of macular subretinal neovascularization, the laser treatment seemed to positively condition the course of the disease. In our opinion, even though a generalized atrophy of the choroid and retina cannot probably be avoidable, laser treatment can delay the loss of central vision, by blocking the capillaries from leaking, thus preventing secondary destruction from bleeding and fibrosis.

Sorsby's pseudo-inflammatory macular dystrophy: laser treatment]

PIERMAROCCHI, STEFANO;MIDENA, EDOARDO;
1989

Abstract

In 1949, Sorsby described a familial fundus disease with progressive visual loss and bilateral hemorrhages and exudates of the posterior pole. The dystrophy, whose inheritance was apparently autosomal dominant, was called pseudo-inflammatory macular dystrophy because of extensive macular lesions that could suggest a post-inflammatory change. The ophthalmoscopic signs include the presence of diffuse drusen-like deposits with extensive changes of retinal pigment epithelium and focal atrophy of the choriocapillaris, particularly in the posterior pole. Some eyes grow subretinal neovascularization, which appears associated to retinal edema, deep hemorrhages and hard exudates. These cases ultimately result in a disciform macular scar. Atrophy of peripheral fundus is typical of advanced stages. We present the case of three sisters whose fundus lesions resembled Sorsby's pseudo-inflammatory macular dystrophy. In two of them, where there was a great suspicion of macular subretinal neovascularization, the laser treatment seemed to positively condition the course of the disease. In our opinion, even though a generalized atrophy of the choroid and retina cannot probably be avoidable, laser treatment can delay the loss of central vision, by blocking the capillaries from leaking, thus preventing secondary destruction from bleeding and fibrosis.
1989
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11577/2473157
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