Abstract
Abstract: :
Purpose: To describe five cases of genetically screened Malattia Leventinese. Malattia leventinese is an autosomal dominant macular dystrophy. Here, the purpose is to describe imaging of Malattia Leventinese. Methods: A complete examination of five patients with Malattia Leventinese was performed. All patients underwent color photographs, autofluorescence, fluorescein angiography, infracyanin angiography and Optical Coherence Tomography on both eyes. Results: Color photographs showed multiple drüsen in the macular area, following a radiate pattern in their disposition, and in their size. Fluorescein angiography and infracyanin angiography showed hypofluorescence during early–phases. In late–phases of angiograms, drüsen were caracterized by hyperfluorescent center surrounded by a dark area. With Optical Coherence Tomography, drüsen of Malattia Leventinese appeared as hyperreflective and localized below the complex of Bruch's membrane and pigmentary epithelium. In spite of underlying drüsen, retinal thickness appeared relatively preserved on Optical Coherence Tomography scans. Conclusions: This serie of five cases is one of the first study about infracyanin angiography and Optical Coherence Tomography in Malattia Leventinese. We showed some pecularities of infracyanin angiography observed in this disease. These results showed that drüsen of Malattia leventinese are probably different in their composition of those observed in Age–related macular degeneration.
Keywords: retina • drusen • imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound)