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Rossi S., Di Iorio V., De Benedictis A., Attanasio M., Testa F.
Dipartimento di Oftalmologia della Seconda Università di Napoli, Napoli, Italia
Aim. Usher Syndrome defines a group of rare hereditary diseases characterized by sensorineural deafness and retinitis pigmentosa (RP). The main ocular complications associated with Usher Syndrome are cataract and cystoid macular edema (CME).
The purpose of the present study is to determine the incidence of CME in Usher Syndrome.
Methods. 223 patients with clinical diagnosis of Usher Syndrome have been recruited by the “Centro Studi Retinopatie Ereditarie” of the Department of Ophthalmology of the Second University of Naples. Ophthalmic exam was performed on all the patients, including visual acuity measurement, fundus examination, visual field exam, electroretinography and optical coherence tomography (OCT).
Results. 220 of 223 recruited patients presented a typical form of RP characterized by RPE dystrophy with accumulation of pigment type osteoblasts, while three patients had an atypical RP. In 8% of the patients the OCT exam showed CME, bilaterally present in 15 patients and monolaterally in three.
Conclusion. Currently, few studies concerning the prevalence of CME in Usher Syndrome are reported in literature. Hence, further studies to define the percentage of incidence could be useful.