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L. Carlomusto, S. Lupo, D. Domanico, S. Putano, E.M. Vingolo; Three Years Follow–up in a Family With Butterfly–Shaped Pattern Dystrophy . Invest. Ophthalmol. Vis. Sci. 2005;46(13):5309.
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Purpose: to identify the mode of inheritance, the clinical evolution of the retinal lesions and the relationship between the different clinical forms of pattern dystrophy we studied 5 members of the same family presented with butterfly–shaped pattern dystrophy. They were examined every 6 months over a follow–up of 3 years. Methods: three members of the family affected by BSPD underwent to a complete ophthalmological examination, with the best correct visual acuity, examination of anterior segments, fundus evaluation. Fluorescein Angiography and HRT were performed in order to evaluate the progression of the retinal disease and electroretinogram. We also examined two relatives to discover the mode of inheritance in our patients. Results:two members of the family of 43 and 46 yrs of age showed a BCVA of 20/20 at least in both eyes the first, while 20/20 and 20/25 the second. The fundus examination revealed the yellow–appearing butterfly–shape macular lesion in both eyes, which remained stable over the follow–up of three years. The oldest member of the family of 65 yrs old showed a BCVA of 20/200 in the right eye and 20/400 in the left eye, and posterior subcapsular lens opacities in the left eye. At the fundus examination we observed a progression of the macular lesion and choriocapillaris atrophy. The mode of inheritance was autosomal dominant and the ERG results were normal only in the 43 yrs old member. Conclusions: an increasing severity of the macular dystrophy can be related to the age of the patient, with a secondary involvement of the photoreceptors. Fluorescein Angiography and HRT were good methods to evaluate the morphology and range of macular changes, the ERG abnormalities in two members of the family suggest a secondary involvement of the photoreceptors.
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