Dataset Information

Three-dimensional spectral domain optical coherence tomography in chronic exposure to welding arc.

ABSTRACT: Three-dimensional spectral domain optical coherence tomography was performed in a 26-year-old man with chronic exposure to welding arc. Advanced macular visualisation provided significant findings of inner segment-ellipsoid zone disruption with the presence of cystoid changes and hyper-reflective material in the area of disruption. The external limiting membrane was intact in both the eyes. C-scan retinal pigment epithelium fit map of the left eye revealed a well-delineated defect whereas the right eye showed a poorly delineated smaller defect. The hyper-reflective material can be hypothesised to originate from the disrupted photoreceptor layer. The hyper-reflective material was more evident in the left eye which could be correlated with more marked diminution of vision and a prominent yellow lesion at the fovea.

SUBMITTER: Saxena S

PROVIDER: S-EPMC4025256 | biostudies-other | 2014

REPOSITORIES: biostudies-other

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Three-dimensional spectral domain optical coherence tomography in chronic exposure to welding arc.

Saxena Sandeep S Mishra Nibha N Meyer Carsten H CH

BMJ case reports 20140515

Three-dimensional spectral domain optical coherence tomography was performed in a 26-year-old man with chronic exposure to welding arc. Advanced macular visualisation provided significant findings of inner segment-ellipsoid zone disruption with the presence of cystoid changes and hyper-reflective material in the area of disruption. The external limiting membrane was intact in both the eyes. C-scan retinal pigment epithelium fit map of the left eye revealed a well-delineated defect whereas the ri ...[more]

PMID: 24832707

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Project description:IMPORTANCE Evidence is mounting that achromatopsia is a progressive retinal degeneration, and treatments for this condition are on the horizon. OBJECTIVES To categorize achromatopsia into clinically identifiable stages using spectral-domain optical coherence tomography and to describe fundus autofluorescence imaging in this condition. DESIGN, SETTING, AND PARTICIPANTS A prospective observational study was performed between 2010 and 2012 at the Edward S. Harkness Eye Institute, New York-Presbyterian Hospital. Participants included 17 patients (aged 10-62 years) with full-field electroretinography-confirmed achromatopsia. MAIN OUTCOMES AND MEASURES Spectral-domain optical coherence tomography features and staging system, fundus autofluorescence and near-infrared reflectance features and their correlation to optical coherence tomography, and genetic mutations served as the outcomes and measures. RESULTS Achromatopsia was categorized into 5 stages on spectral-domain optical coherence tomography: stage 1 (2 patients [12%]), intact outer retina; stage 2 (2 patients [12%]), inner segment ellipsoid line disruption; stage 3 (5 patients [29%]), presence of an optically empty space; stage 4 (5 patients [29%]), optically empty space with partial retinal pigment epithelium disruption; and stage 5 (3 patients [18%]), complete retinal pigment epithelium disruption and/or loss of the outer nuclear layer. Stage 1 patients showed isolated hyperreflectivity of the external limiting membrane in the fovea, and the external limiting membrane was hyperreflective above each optically empty space. On near infrared reflectance imaging, the fovea was normal, hyporeflective, or showed both hyporeflective and hyperreflective features. All patients demonstrated autofluorescence abnormalities in the fovea and/or parafovea: 9 participants (53%) had reduced or absent autofluorescence surrounded by increased autofluorescence, 4 individuals (24%) showed only reduced or absent autofluorescence, 3 patients (18%) displayed only increased autofluorescence, and 1 individual (6%) exhibited decreased macular pigment contrast. Inner segment ellipsoid line loss generally correlated with the area of reduced autofluorescence, but hyperautofluorescence extended into this region in 2 patients (12%). Bilateral coloboma-like atrophic macular lesions were observed in 1 patient (6%). Five novel mutations were identified (4 in the CNGA3 gene and 1 in the CNGB3 gene). CONCLUSIONS AND RELEVANCE Achromatopsia often demonstrates hyperautofluorescence suggestive of progressive retinal degeneration. The proposed staging system facilitates classification of the disease into different phases of progression and may have therapeutic implications.

Dataset Information

Three-dimensional spectral domain optical coherence tomography in chronic exposure to welding arc.

Publications

Three-dimensional spectral domain optical coherence tomography in chronic exposure to welding arc.

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