Estimating the Incidence of Familial Exudative Vitreoretinopathy (FEVR) in The State of Michigan

Background and ObjectiveAmongst 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 MethodsThis 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. ResultsThis 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%) ConclusionFamilial Exudative Vitreoretinopathy is indeed a rare diagnosis with an incidence rate of 0.007% within the State of Michigan.