ABSTRACT
OBJETIVOS: Verificar a relação entre alterações anatômicas (drusas duras, drusas moles, hiperpigmentação, neovasos, descolamento do epitélio pigmentado da retina, hipopigmentação e atrofia coriorretiniana) e a sensibilidade à luz em pacientes com degeneração macular relacionada à idade (DMRI); analisar a sensibilidade macular em áreas com ausência de lesões anatômicas nos pacientes com DMRI comparando-as ao grupo de controles, para avaliar a existência ou não de lesão funcional em área sem lesão anatômica. MÉTODOS: Estudo comparativo, descritivo e analítico, de corte transversal. O grupo de casos foi formado por 31 indivíduos portadores de DMRI com idade entre 51 e 88 anos. O grupo de controles ficou composto por 31 indivíduos considerados "sadios", não portadores de DMRI com idade entre 61 e 80 anos. Os grupos foram pareados por sexo e idade. Realizou-se a perimetria macular estática, vermelho-vermelho, com o oftalmoscópio de rastreamento a laser (ORL). Os resultados da perimetria macular foram correlacionados à lesão anatômica identificada no local correspondente pelo laser infravermelho e fotografias coloridas. RESULTADOS: As áreas com neovasos ou atrofia apresentaram sensibilidade significantemente diferente em relação às áreas com ausência de lesões anatômicas nos pacientes com DMRI. Houve perda funcional significativa em áreas com ausência de lesões anatômicas nos pacientes com DMRI em relação ao grupo de controles. CONCLUSÕES: Áreas com neovasos ou atrofia podem ser fatores individuais de piora da sensibilidade macular localizada. Pode ocorrer perda funcional mesmo sem lesão anatômica aparente nos pacientes com DMRI.
PURPOSES: To evaluate the correlation between anatomical changes (hard druses, soft druses, hyperpigmentation, new vessels, detachment of retinal pigment epithelium, hypopigmentation and chorioretinal atrophy) and light sensitivity in patients with age-related macular degeneration (ARMD); analyze macular sensitivity in areas with no anatomical lesions in patients with ARMD and compared them to the control group in order to detect if there was any functional lesion in areas with no anatomical changes. METHODS: A cross-sectional, comparative, descriptive and analytic study was performed. The case group consisted of 31 subjects with ARMD aged between 51 and 88 years. The control group consisted of 31 "healthy" subjects, without ARMD aged between 61 and 80 years. The groups were matched for gender and age. We performed static macular perimetry, red-red, using a scanning laser ophthalmoscope (SLO). Results of macular perimetry were correlated with the anatomic lesion identified in the same site by infrared laser and color photographs. RESULTS: Areas with new vessels or atrophy showed a significantly different sensitivity in relation to areas without anatomical lesions in patients with ARMD. There was significant functional loss in areas with no anatomical lesions in patients with ARMD in relation to the control group. CONCLUSIONS: Areas with new vessels or atrophy could be distinct factors for worsening of the localized macular sensitivity. There might be functional loss even in areas with no apparent anatomical changes in ARMD patients.
Subject(s)
Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Contrast Sensitivity/physiology , Macular Degeneration/pathology , Macular Degeneration/physiopathology , Visual Field Tests , Age Factors , Atrophy/etiology , Atrophy/pathology , Atrophy/physiopathology , Case-Control Studies , Cross-Sectional Studies , Lasers , Macular Degeneration/complications , Neovascularization, Pathologic/etiology , Neovascularization, Pathologic/pathology , Neovascularization, Pathologic/physiopathology , Ophthalmoscopes , Pigment Epithelium of Eye/pathology , Pigment Epithelium of Eye/physiopathology , Retinal Drusen/etiology , Retinal Drusen/pathology , Retinal Drusen/physiopathology , Visual Field TestsABSTRACT
A doença de Erdheim-Chester (DEC) tem causa desconhecida e se caracteriza por ser granulomatosa e infiltrativa, com proliferação de histiócitos contendo colesterol e particular acometimento ósseo. À histologia, é semelhante à histiocitose de células Langerhans, salvo na análise imuno-histoquímica. Pela primeira vez é descrito o acometimento intra-ocular nesta doença. MPSG, 46 anos, sexo feminino, apresentou-se com proptose do olho direto. Referia úlceras no palato duro, osteoesclerose bilateral e simétrica na diáfise tibial e fibular e nódulo na mama direita (biópsia: infiltrado rico em histiócitos xantomatosos e perfil imuno-histoquímico com CD68 +, S-100 e CD1a negativos, compatível com DEC). À RNM, tumoração extraconal justa-bulbar temporal superior na órbita direita, hiperintensa em T1 e próxima da glândula lacrimal. Em cada olho, visão 20/20, com numerosas drusas nas arcadas e na região macular similares às drusas laminares basais, além de duas regiões placóides elevadas com infiltrado alaranjado sub-retiniano e hiperfluorescentes na angiografia na região peridiscal inferior e justamacular temporal do olho direito. Dois anos depois, surgiram membranas neovasculares sub-retinianas em ambos os olhos. O OD manteve visão 20/20 com pequenas alterações campimétricas e o OE evoluiu com grande cicatriz disciforme e visão de vultos. Esta descrição pioneira demonstra características in vivo dos granulomas histiocíticos da DEC, e alerta para possíveis complicações intra-oculares.
Erdheim-Chester disease (ECD) is a granulomatous and infiltrative disorder of unknown etiology with proliferation of cholesterol-containing histiocytes and peculiar bone involvement. It is very similar to Langerhans cell histiocytosis (LCH) on histology but with a different immunohistochemical profile. This is the first report of intraocular involvement in this disease. MPSG, a 46 y.o. woman, presented with proptosis of the OD. She referred ulcerated lesions on the hard palate, symmetrical and bilateral osteosclerosis of the fibulae and tibiae and a nodule in the right breast (biopsy: xantomatous histiocytic infiltrate CD68+, S-100 and CD1a negative on immunohistochemistry compatible with ECD). MRI studies demonstrated an extraconal tumor in the juxta-bulbar temporal portion of the right orbit close to the lacrimal gland and hyperintense on T1. Vision was 20/20 OU, with numerous drusen in the posterior pole, similar to basal laminar drusen. Two regions of orange subretinal infiltrates that showed progressive staining on the angiogram were seen in the peripapillary region and also close to the fovea in the OD. Choroidal neovascular membranes were seen 2 years later in OU leading to severe visual loss in the OS and to a slight visual field loss in the OD, which retained 20/20 vision. This pioneer report depicts in vivo characteristics of histiocytic granulomas in ECD. Caution should be taken with patients with ECD as potentially blinding intraocular complications may arise.
Subject(s)
Female , Humans , Middle Aged , Erdheim-Chester Disease/complications , Exophthalmos/etiology , Brazil/epidemiology , Choroidal Neovascularization/etiology , Choroidal Neovascularization/pathology , Erdheim-Chester Disease/epidemiology , Exophthalmos/pathology , Fluorescein Angiography , Orbital Neoplasms/etiology , Orbital Neoplasms/pathology , Retinal Drusen/etiology , Retinal Drusen/pathology , Retinal Neovascularization/etiology , Retinal Neovascularization/pathology , Tomography, Optical Coherence , Visual Acuity/physiologyABSTRACT
Lesão viteliforme da mácula é mais observada nas distrofias de Best e foveomacular do adulto. No presente estudo, os autores relatam caso raro de descolamento viteliforme macular associado a drusas da lâmina basal, acompanhado no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo há aproximadamente quatro anos, avaliado periodicamente com exames complementares. Os autores descrevem aspectos relevantes desta doença como etiopatogenia, evolução, diagnósticos diferenciais e tratamento.
Subject(s)
Aged , Humans , Male , Macular Degeneration/pathology , Retinal Detachment/pathology , Retinal Drusen/pathology , Atrophy , Fluorescein Angiography , Macular Degeneration/complications , Retinal Detachment/complications , Retinal Drusen/complicationsABSTRACT
PURPOSE: Age-related macular degeneration (ARMD) is clinically less common in India compared to the West. Therefore, clinicians are unfamiliar with histopathologic evidence of age-related macular changes in the Indian population. METHODS: Fifty consecutive human donor eyes removed for corneal grafting were studied for gross, microscopic and histochemical features of age-related changes in the macula in the Indian population. A horizontal block was cut from the globe including the optic disc, and the macula. Six sections, 6 microns thick, were cut from three levels in the macula at a distance of 140 microns. These were stained with haemotoxylin-eosin, periodic acid-Schiff, Mallory, Masson trichrome, alcian blue and von Kossa stains. The presence of basal laminar deposits, drusen and thickening and calcification of Bruch's membrane in the macula were assessed at 400 x magnification using a modified version of Sark's classification. RESULTS: Twenty-four donor eyes (48%) had some form of age-related macular change. These included basal laminar deposits, hard drusen, soft drusen, extensive retinal pigment epithelium atrophy of the macula, and disciform degeneration of macula. A combination of changes was often seen. Age-related changes were more common in the seventh and eighth decade. CONCLUSION: Our study shows that histological changes characteristic of the early stages of age-related macular degeneration are fairly common in the Indian population. However, advanced macular changes are significantly rare.