Case ReportPOSTERIOR POLAR ANNULAR CHOROIDAL DYSTROPHY: A CASE SERIESLenis, Tamara L. MD*; Klufas, Michael A. MD*; Randhawa, Sandeep MD†; Sharma, Mithlesh MD‡; Sarraf, David MD*,§Author Information *Stein Eye Institute, University of California Los Angeles, Los Angeles, California; †Associated Retina Consultants, Oakland University William Beaumont School of Medicine, Michigan, Royal Oak, Michigan; ‡Department of Ophthalmology, The Permanente Medical Group, Roseville, California; and §Greater Los Angeles VA Healthcare Center, Los Angeles, California. Reprint requests: David Sarraf, MD, Retinal Disorders and Genetics Division, Stein Eye Institute, 100 Stein Plaza, University of California Los Angeles, Los Angeles, CA 90095-7000; e-mail: [email protected] D. Sarraf has received research grants from Allergan, Regeneron, Genentech, and Optovue. He is a consultant for Optovue and Genentech, and he is a speaker for Optovue. The remaining authors have no financial/conflicting interests to disclose. Presented at the Pacific Retina Club Meeting 2016, Stein and Doheny Eye Institute, Los Angeles, CA, March 18, 2016. Retinal Cases & Brief Reports: Winter 2017 - Volume 11 - Issue - p S24-S27 doi: 10.1097/ICB.0000000000000392 Buy Metrics AbstractIn Brief Purpose: To describe the multimodal imaging findings of posterior polar annular choroidal dystrophy (PPACD). Methods: Retrospective case series of 2 patients diagnosed with PPACD. Spectral-domain optical coherence tomography, fundus autofluorescence, and fluorescein angiography findings of PPACD are described. Electroretinography results are also presented. Results: Both patients presented with bilateral peripapillary atrophy extending to involve the temporal arcades in an annular, foveal sparing pattern. Both cases demonstrated outer retinal atrophy with foveal sparing on spectral-domain optical coherence tomography, and fluorescein angiography illustrated corresponding window defects with late staining. Fundus autofluorescence showed hypoautofluorescence in the center of atrophic areas but hyperautofluorescence at the leading edge. Electroretinography findings included cone loss with rod preservation. Inflammatory and infectious workup was unremarkable in both cases. Conclusion: This report describes the multimodal imaging findings of a rare and poorly described chorioretinal disorder, PPACD, and will serve to guide clinicians regarding the evaluation and management of this elusive condition. This case series describes two patients with posterior polar annular choroidal dystrophy. Multimodal imaging reveals features of disease that highlight both pathophysiology and clinical spectrum.