Morphologic photoreceptor abnormality in occult macular dystrophy on spectral-domain optical coherence tomography

• Published in: Investigative ophthalmology & visual science 2010-07, Vol.51 (7), p.3673-3679
• Main Authors: Park, Sang Jun, Woo, Se Joon, Park, Kyu Hyung, Hwang, Jeong-Min, Chung, Hum
• Format: Article
• Language: English
• Subjects: Adult; Disease Progression; Electroretinography; Female; Fluorescein Angiography; Humans; Macular Degeneration - diagnosis; Macular Degeneration - physiopathology; Male; Middle Aged; Photoreceptor Cells, Vertebrate - pathology; Retrospective Studies; Tomography, Optical Coherence; Vision Disorders - diagnosis; Vision Disorders - physiopathology; Visual Acuity - physiology; Visual Field Tests; Visual Fields - physiology; Young Adult
• ISSN: 1552-5783; 1552-5783
• DOI: 10.1167/iovs.09-4169

PURPOSE. To investigate morphologic photoreceptor layer abnormalities and their correlation with visual function in occult macular dystrophy (OMD), by using spectral-domain optical coherence tomography (SD-OCT). METHODS. This observational case series included 18 eyes of 9 patients with OMD. All patients underwent an ophthalmic evaluation, which included a fundus examination, fluorescein angiography, full-field electroretinography (ERG), multifocal ERG, time-domain optical coherence tomography (TD-OCT), and visual field testing. Morphologic photoreceptor layer abnormalities of the retinal layers were investigated with SD-OCT. The structure-function relationship was investigated regarding visual acuity, symptom duration, and multifocal ERG RESULTS: RESULTS. Best corrected visual acuity ranged from 20/200 to 20/20. Four patients had a symmetric decline of acuity in both eyes (20/200-20/100), and five had unilateral vision impairment (20/200-20/50). TD-OCT showed foveal thinning in all patients, but revealed no other retinal layer abnormality. In 15 eyes of 8 patients, SD-OCT demonstrated a well-defined disruption of the inner segment-outer segment (IS-OS) junction of the photoreceptors and of the Verhoeff membrane (cone outer segment tips). SD-OCT showed that three of five patients with presumed unilateral OMD had bilateral OMD after initial or follow-up examinations. Degrees of abnormality in the photoreceptor layer varied and correlated with visual acuity and symptom duration. CONCLUSIONS. SD-OCT can demonstrate the disruption of photoreceptors in most patients with OMD and the morphologic changes on SD-OCT correlate with visual function and disease progression. These morphologic abnormalities can be an important feature and cause of vision loss in patients with OMD.