Evidence and Consensus Based Imaging Guidelines in Multiple Evanescent White Dot Syndrome Multimodal imaging in Uveitis (MUV) Taskforce Report 6
Marion R. Munk, Richard Stillenmunkes, Anne Tillmann, Lee M. Jampol, Maria Vittoria Cicinelli, Phoebe Lin, Kathryn L. Pepple, K. Bailey Freund, İlknur Tuğal-Tutkun, Zohar Habot-Wilner, Aniruddha Agarwal, Sapna Gangaputra, Rupesh Agrawal, Douglas A. Jabs, Srinivas R. Sadda, David Sarraf, Vishali Gupta, Vishali Gupta, Vishali Gupta, Alejandra de-la-Torre, Alejandro Fonollosa, Alessandro Invernizzi, Amani Fawzi, Andrew Dick, Aniruddha Agarwal, Anita Agarwal, Antoine Brezin, Ariel Schlaen, Bahram Bodaghi, Claudia Fabiani, David Sarraf, Debra A. Goldstein, Dilraj Grewal, Douglas A. Jabs, Edmund Tsui, Elisabetta Miserocchi, Ester Carreño, Francesco Pichi, Ilknur Tugal-Tutkun, Inês Leal, Janet L. Davis, Jeannette Ossewaarde- van Norel, Jennifer E. Thorne, Jessica G. Shantha, Jose S. Pulido, Justine R. Smith, Jyotirmay Biswas, K. Bailey Freund, Kathryn Pepple, Lee M. Jampol, Luca Cimino, Marc De Smet, Maria Vittoria Cicinelli, Marion R. Munk, Marion R. Munk, Massimo Accorinti, Maura Di Nicola, Meghan Berkenstock, Michael Altaweel, Phoebe Lin, Quan Dong Nguyen, Richard Spaide, Rupesh Agrawal, Sapna S. Gangaputra, Soon Phaik Chee, Srinivas Sadda, Sumit Sharma, Timothy M. Janetos, Vishali Gupta, Vishali Gupta, Zohar Habot-Wilner
Abstract
PURPOSE: To develop imaging and consensus-based guidelines on the application of multimodal imaging in multiple evanescent white dot syndrome (MEWDS). DESIGN: Consensus agreement guided by literature, and an expert committee using a nominal group technique (NGT). METHODS: The expert committee employed a structured NGT with multiple rounds of discussion, conflict resolution, and anonymous voting to: (1) establish imaging criteria for diagnosing and monitoring MEWDS using color fundus photography (CFP), optical coherence tomography (OCT), fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography (OCTA); and (2) develop consensus-based recommendations for assessing specific characteristics in patients with MEWDS. These formal recommendations were derived from a structured NGT using illustrative cases of MEWDS and were further voted upon by the entire task force. RESULTS: The diagnosis of acute MEWDS is supported by distinct multimodal features on CFP, multi-focal disruption of the ellipsoid/interdigitation zone with overlying outer retinal hyper-reflectivity with OCT, and hyper-autofluorescent spots with FAF (short-wave blue/green). In complex cases, wreath-like lesions on FFA and the absence of early hypofluorescence on ICGA help differentiate MEWDS from other chorioretinopathies. The lack of specific choroidal changes on OCT and preserved signal on OCTA on retinal and inner choroidal slabs also aid in diagnosis. CONCLUSIONS: Multimodal imaging is essential for diagnosing MEWDS and differentiating it from other non-infectious uveitis types, extending the Standardization of Uveitis Nomenclature (SUN) classification. These imaging criteria enable detailed assessment of disease activity and offer valuable insights into MEWDS pathogenesis.