Butterfly--Shaped Pigment Dystrophy of the Fovea

Butterfly-shaped pigment dystrophy of the fovea associated with subretinal neovascularization

“…"Butterfly-shaped pigment dystrophy of the fovea" was described for the first time by Deutman et aL [11] in 1970. The characteristic symmetric bilateral pigmenta tion in the deeper layers of the central retina, a markedly subnormal EOG, normal or only very slightly diminished visual acuity, and an apparent autosomal dominant in heritance pattern are fundamental for the diagnosis [11].…”

“…The characteristic symmetric bilateral pigmenta tion in the deeper layers of the central retina, a markedly subnormal EOG, normal or only very slightly diminished visual acuity, and an apparent autosomal dominant in heritance pattern are fundamental for the diagnosis [11]. Because of their similar clinical appearance, butterfly shaped dystrophy [11,16,20], reticular dystrophy [7,10,18,24], macroreticular dystrophy [17], fundus pulverulentus [25], and adult-on set foveo macular vitelliform dystrophy [12] could be grouped together in a single pathologic entity: pattern dystrophy of the retinal pig ment epithelium [27]. The concomitant presence of mor phologically different pattern dystrophies either in the same affected family [13,14,16,19] or in various stages of the disease in a single patient [6] suggests that they might be different expressions of the same disorder.…”

Electroretinogram in Hereditary Retinal Disorders

“…The visual prognosis is generally good with a mild disturbance of central vision typically ranging from 20/20 to 20/70 and, metamorphopsia. Pattern dystrophies are divided into morphological subtypes including butterfly dystrophy, fundus pulverulentus, multifocal pattern dystrophy resembling Stargardt disease, and adult-onset vitelliform macular dystrophy (Deutman & Rümke, 1969;Deutman et al, 1970;Hsieh et al, 1972;Slezak & Hommer, 1969). However, some individual patients may not be precisely categorized into one of these groups with respect to fundus abnormalities.…”

Distrofia em forma-de-borboleta: relato de caso

“…O diagnóstico é freqüentemente estabelecido na segunda ou terceira décadas de vida, a partir de exame oftalmológico de rotina. Os pacientes são geralmente descritos como assintomáticos, podendo manter visão normal ou discretamente diminuída durante toda a vida (6) . Apesar de os achados de Deutman et al (6) sugerirem que a distrofia em forma-de-borboleta apresenta curso relativamente benigno, estudos recentes descreveram esta doença como crônica e progressiva, podendo estar associada a manifestações como atrofia do EPR macular e neovascularização coroidea, com possível deterioração visual (8)(9) .…”