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Vikas Khetan, Gad Dotan, Jan D Marshall, Elizabeth Affel, Denise-Armiger George, Alex V Levin; Spectral-domain optical coherence tomography findings in Alström Syndrome. Invest. Ophthalmol. Vis. Sci. 2014;55(13):1136.
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To characterize morphological retinal changes in affected Alström patients using spectral-domain optical coherence tomography (SD-OCT, Bioptigen Inc, Research Triangle Park, NC).3
We studied volunteer patients attending the conference of Alström Syndrome International, a support group for affected families, using hand-held SD-OCT in an office setting. Patients also had clinical dilated retinal examination. Past medical records were reviewed
We studied 24 eyes of 22 patients (mean age 17.14 ± 11.14 years, range 2 - 38 years, 11 males). Central macular OCT changes are often mild during the first decade of life and gradually progress, demonstrating increased deepening of central foveal pit, disruption of normal retinal architecture, and progressive loss of photoreceptors and retinal pigment epithelium with increased prominence of choroidal vasculature. Other changes include hyperreflective echos in all retinal layers, severe retinal wrinkling, optic nerve drusen and vitreoretinal traction. Clinical retinal findings did not always show the OCT changes but also worsened with age.
SD-OCT in Alström syndrome may offer anatomic insight to help elucidate the pathophysiology of the disease.
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