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S. R. Hannan, K. C. Madhusudhana, F. Shawkat, R. S. B. Newsom, A. J. Lotery; Pattern Dystrophy: A Functional and Morphological Study. Invest. Ophthalmol. Vis. Sci. 2007;48(13):4152.
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1. To investigate and integrate the tomographic, angiographic and electrodiagnostic findings in Pattern dystrophy. 2. To correlate visual acuity with central macular thickness (CMT) and Electro-oculogram findings.
This is a prospective observational study of patients with Pattern dystrophy. Clinical examination, Snellen visual acuity, fluorescein angiography (FA), Optical Coherence Tomography (OCT), electrodiagnostic tests (Electroretinogram: ERG, Electro-oculogram: EOG and Visual evoked potential: VEP) were performed.
There were 38 eyes (20 patients) with Pattern dystrophy (unilateral=2, bilateral=18). Of these, twenty-eight eyes had Adult-onset Foveomacular Vitelliform Dystrophy (AOFVD) and two eyes had Reticular Pattern Dystrophy. There were four eyes each with Multi-focal Pattern Dystrophy and Butterfly Pattern Dystrophy (BPD). The age at presentation ranged between 40 to 90 yrs. Mean visual acuity was 20/40. The mean CMT was 223µm. On OCT all eyes with AOFVD demonstrated a well-defined, fusiform thickening between the retinal pigment epithelium (RPE) and the photoreceptor layer. Twelve eyes showed additional thinning of the overlying neurosensory retina with one eye progressing to a full thickness macular hole. In all eyes FA demonstrated a central hypofluorescent area surrounded by a ring of hyperfluorescence and late staining of the lesion. In eyes with BPD, Reticular and Multifocal dystrophies, OCT verified focal thickening of the RPE with and without identifiable hyperreflective deposits between the RPE and the photoreceptor layer. FA in BPD showed hypofluorescence corresponding to the triradiate lesions outlined by hyperfluorescence. In reticular dystrophy, FA showed a reticular pattern of hypofluorescence in the posterior pole. Electrodiagnostic tests (N=35) revealed borderline or reduced EOG in 22 eyes (63%) and degraded pattern ERG and/or pattern VEP in 25 eyes (71%) consistent with macular dysfunction. OCT thickness and Arden ratio did not show any significant correlation with visual acuity.
Pattern dystrophies are frequently misdiagnosed for age-related macular degeneration. Although the pattern dystrophies show diversity in clinical presentation, relatively consistent findings on FA/OCT and electrodiagnostic tests help in making the accurate diagnosis, thus avoiding inappropriate treatment. The disease process in this condition may not be limited to the RPE as shown by abnormal pattern ERG/VEP results observed in our study.
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