EVALUATION OF PARAVASCULAR INNER RETINAL DEFECTS USING EN FACE OPTICAL COHERENCE TOMOGRAPHY.

PURPOSE: To evaluate the prevalence and risk factors for development of paravascular inner retinal defects (PIRDs) using en face optical coherence tomography. METHODS: This is a retrospective cross-sectional study. En face and cross-sectional optical coherence tomography images were reviewed (9 × 9 mm or 12 × 12 mm). Paravascular inner retinal defects were classified as either Grade 1 (i.e., paravascular inner retinal cysts) when the lesion was confined within the nerve fiber layer without any communication to the vitreous cavity or Grade 2 (i.e., paravascular lamellar hole) when the defects communicated to the vitreous. Paravascular inner retinal defect grading was correlated with presence of high myopia, stage of posterior vitreous detachment, and presence of epiretinal membrane and retinoschisis. RESULTS: Of 1,074 patients (2,148 eyes), PIRDs were detected in 261 eyes with a prevalence of 261 per 2,148 eyes (12.2%) and 176 per 1,074 patients (16.4%). A total of 116 eyes (44.4%) displayed Grade 2 PIRDs while 145 eyes (55.6%) were Grade 1. In the multivariate logistic regression model, the presence of partial/complete posterior vitreous detachment, retinoschisis, and epiretinal membrane was significantly correlated with PIRDs (OR = 2.78 [1.7-4.4], P < 0.001; OR = 2.93 [1.7-5], P < 0.001; and OR = 25.9 [2.8-242.5], P < 0.001, respectively). The presence of partial/complete posterior vitreous detachment and epiretinal membrane was also significantly associated with Grade 2 PIRDs versus Grade 1 PIRDs ( P = 0.03 and P < 0.001). CONCLUSION: Our results indicate that wide-field en face optical coherence tomography facilitates the identification of PIRDs over a large area of retina with a single capture. The presence of PIRDs was significantly associated with posterior vitreous detachment, epiretinal membrane, and retinoschisis, confirming the role of vitreoretinal traction in the pathogenesis of PIRDs.

A MIDDLE-AGED PATIENT WITH BILATERAL VISION LOSS AND NYCTALOPIA.

PURPOSE: To report a case of nonparaneoplastic autoimmune retinopathy in a patient with a diagnosis of Lambert-Eaton myasthenic syndrome. METHODS: Case report. Main outcome measures included findings on retinal examination and analysis of fundus autofluorescence, spectral-domain optical coherence tomography, and full-field electroretinogram. Vitamin A levels and results of antiretinal antibody testing and paraneoplastic workup are also presented. RESULTS: A 47-year-old male presented with a 1-year history of bilateral vision loss and nyctalopia. Past medical history was significant for Lambert-Eaton myasthenic syndrome, confirmed by positive voltage-gated calcium channel antibodies, and thymectomy reported as thymic follicular hyperplasia. Optical coherence tomography showed bilateral diffuse outer retinal atrophy and ellipsoid zone loss. Fundus autofluorescence displayed a bull's pattern of hyperautofluorescence around each fovea. Full-field electroretinogram showed an extinguished rod response and a severely depressed cone response in each eye. CONCLUSION: We describe a case of nonparaneoplastic autoimmune retinopathy in a patient with Lambert-Eaton myasthenic syndrome. Multimodal retinal imaging and electroretinogram confirmed the presence of autoimmune retinopathy with severe rod-cone degeneration. The association of this myasthenic syndrome with AIR is novel.