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.

CENTRAL SEROUS CHORIORETINOPATHY ASSOCIATED WITH STEROID ENEMA.

BACKGROUND/PURPOSE: To report a case of acute recurrent central serous chorioretinopathy that developed after a regimen of corticosteroid enemas and suppositories. METHODS: Observational case report. Fluorescein angiography and spectral domain optical coherence tomography. RESULTS: A 47-year-old male patient with ulcerative colitis managed through hydrocortisone enemas presented to clinic with a 1-day history of blurry vision of his left eye. Posterior segment examination revealed subretinal fluid in the superotemporal macula of the left eye extending centrally. After diagnosis of acute central serous chorioretinopathy, the patient was advised to taper steroid enemas and his visual symptoms and subretinal fluid resolved within the month. Seven years later, several months after using steroid suppositories for the first time since the original central serous chorioretinopathy episode, asymptomatic subretinal fluid accumulation with foveal sparing was found on routine ophthalmic examination. Three months later, most of this fluid had resolved with minimal residual subretinal fluid on clinical examination. CONCLUSION: Acute central serous chorioretinopathy may develop after corticosteroid enema or suppository use, a route of administration that has not been previously reported in association with the disease.

Comparison of SDOCT Scan Types for Grading Disorganization of Retinal Inner Layers and Other Morphologic Features of Diabetic Macular Edema.

Purpose: To assess grading reproducibility of disorganization of the retinal inner layers (DRIL) and other morphologic features of diabetic macular edema (DME) across spectral domain optical coherence tomography (SDOCT) instruments and scan types. Methods: A cross-sectional study enrolled participants with current or recent center-involved DME. In group A (27 eyes), we obtained two Cirrus scans (512 × 128 macular cube [Cube] and high-definition five-line raster [HD 5-Line]) and two Spectralis scans (high-resolution [HR] and high-speed [HS]). In group B, 26 eyes underwent HR scans and Optovue AngioVue (OP) 3 × 3-mm scans. All scans were graded for type and extent of DRIL, intraretinal cysts, cone outer segment tip visibility, and subretinal fluid (SRF). Results: In the total cohort, mean central subfield thickness was 342.9 ± 83.4 µm. Intraclass correlations were high for DRIL extent across the four different imaging settings (HR vs. HS, r = 0.93; HR vs. Cube, r = 0.84, HR vs. HD 5-Line, r = 0.76, HR vs. OP, r = 0.87) and ranged from good to excellent for intraretinal cyst and SRF area. There were significantly smaller mean normalized differences between HR/HS scans versus HR and all other scan modalities (HR/HS vs. HR/Cube, P = 0.02; HR/HD 5-Line, P = 0.0005; HR/OP, P < 0.0001). Conclusions: Our data suggest that the reproducibility for SDOCT parameters of DRIL and intraretinal cysts was high across all five SDOCT scan types; thus, evaluation of DRIL is feasible using multiple SDOCT models in eyes with DME. Translational Relevance: DME morphological changes can be evaluated on multiple SDOCT devices with good reproducibility, allowing clinicians and researchers flexibility in DME assessment for clinical care and research.

Congenital muscular dystrophy-dystroglycanopathy, type A, featuring bilateral retinal dysplasia and vertical angle kappa.

Muscular dystrophy-dystroglycanopathy type A (MDDGA3), one of a group of diseases collectively known as congenital muscular dystrophies, is an alpha-dystroglycanopathy with characteristic brain and ocular abnormalities. We report the case of a 9-month-old boy with developmental delay whose family sought evaluation for esotropia. Subsequent examination, imaging, and testing revealed significant motor and cognitive delay, marked weakness with appendicular spasticity, and a diffuse brain malformation. In addition, the patient had poor visual acuity, nystagmus, optic nerve hypoplasia, bilateral retinal dysplasia and retinal dragging with a large vertical angle kappa, and an avascular peripheral retina. Genetic testing revealed two known heterozygous mutations in the POMGnT1 gene confirming MDDGA3. He was treated with botulinum toxin injections for his strabismus and continues to be followed, with planned laser ablation of the peripheral avascular retina.