Arindam Basu @arinbasu@qoto.org

Background and Objective: Amongst ophthalmologists, familial exudative vitreoretinopathy (FEVR) is agreed to be a rare disease. However, its incidence is currently unknown. The goal of this study is to identify the incidence of FEVR in Michigan. Study Design/Materials and Methods: This retrospective chart review evaluated new patient encounters from the largest retinal specialty practice within the State of Michigan from over a 6 year period. These identified encounters were queried for the international classification of disease 10 (ICD-10) code (H35.023): exudative retinopathy, not diabetes. Review of both the individual patient encounters and the appropriate clinical imaging resulted in the identification of our FEVR cohort which was subsequently compared with the live birthrate in the State of Michigan over the same 6 years to determine an incidence rate. Results: This study revealed 44 new FEVR diagnosis and 666, 102 livebirths over the 6 year period of interest. Thus, the incidence of FEVR in the state of Michigan was estimated to be 0.007%. (95% CI[0.005%-0.009%) Conclusion: Familial Exudative Vitreoretinopathy is indeed a rare diagnosis with an incidence rate of 0.007% within the State of Michigan. ### Competing Interest Statement The authors have declared no competing interest. ### Funding Statement None ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: WCG IRB of Associated Retinal Consultants gave ethical approval for this work I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines and uploaded the relevant EQUATOR Network research reporting checklist(s) and other pertinent material as supplementary files, if applicable. Yes All data produced in the present work are contained in the manuscript