ABSTRACT
BACKGROUND: Especially during adolescence, hereditary retinal dystrophies can cause visual impairment. Because of their high mobility and working activities, these patients represent a great challenge for a Low-Vision Department. This study illustrates the spectrum of appropriate magnifying devices for them, as prescribed in our Low-Vision Department. PATIENTS AND METHODS: Between January 2003 and October 2004, we took care of 2,500 patients in our Low-Vision Department. 141 (5.6 %) of them suffered from a hereditary retinal dystrophy. After ascertaining the best corrected distance and near visual acuity as well as the magnification demand we prescribed the appropriate optic or electronic magnifying aids. RESULTS: The preferential age for the first visit was between 15 and 40 years. Within the retinal dystrophies we saw cone-rod dystrophy in 46 (33 %) patients, Stargardt's disease in 38 (27 %), retinitis pigmentosa in 37 (26 %), albinism in 15 (11 %) and achromatopsia in 5 (3 %) patients. The average of the best corrected distance visual acuities of the better eye was 0.142 (SD +/- 3.3 lines), the best corrected visual acuity for reading was 0.124 (SD +/- 6.1 lines). The average magnification demand was 5 x (SD +/- 6.7). The most prescribed optic magnifying aids were distance spectacles and magnifying glasses (each 20 %), monocular telescopes, contrast enhancing filters (each 13 %) and reading glasses (8 %). Electronic magnifying aids were prescribed in only 8 %. CONCLUSIONS: Fluent reading ability in hereditary retinal dystrophies could be achieved mostly by optic magnifying aids. The high proportion of prescribed distance glasses and magnifying glasses shows that often simple magnifying aids are suitable for these patients. Because of the high photophobia, contrast-enhancing filters should always be considered.
Subject(s)
Eyeglasses/statistics & numerical data , Retinal Dysplasia/epidemiology , Retinal Dysplasia/rehabilitation , Vision, Low/epidemiology , Vision, Low/rehabilitation , Adolescent , Adult , Comorbidity , Germany/epidemiology , Humans , Treatment Outcome , Vision, Low/diagnosis , Visual Acuity , Young AdultABSTRACT
Os autores apresentam um caso de distrofia macular em forma-de-borboleta, diagnosticado em paciente do sexo masculino, apresentando concomitante atrofia do epitélio pigmentado da retina e perda visual central em um dos olhos. Os achados relatados contrariam conceitos inicialmente disponíveis de curso sempre benigno da doença. A lesão característica e bem delimitada no pólo posterior e a angiofluoresceinografia, permitiram estabelecer o diagnóstico. Descreve-se ainda, pela primeira vez, os achados da distrofia em forma-de-borboleta à tomografia de coerência óptica.
The authors present a case of butterfly-shaped pattern dystrophy diagnosed in a male patient, with retinal pigmented epithelium atrophy and central visual acuity decrease in one of the eyes. The evolution of this case was not benign as described in previous reports. A well-defined lesion located in the posterior pole of both eyes associated with fluorescein angiography allowed the diagnosis of this pattern dystrophy. Optical coherence tomography was performed, showing the aspects of the pathology, for the first time.
