June 2011

Norrie Disease vs Familial Exudative Vitreoretinopathy

Author Affiliations

Author Affiliations: Retina Associates Southwest, Tucson, Arizona.


Copyright 2011 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2011

Arch Ophthalmol. 2011;129(6):805-820. doi:10.1001/archophthalmol.2011.131

We thank Kiernan and colleagues1 for their interest in and comments on our article.2 We have read their related article3 and would respectfully like to argue that their patient has stage 2 familial exudative vitreoretinopathy (FEVR), not Norrie disease. Norrie disease, FEVR, and bilateral persistent fetal vasculature share many clinical features, but with careful consideration of the clinical phenotype and with the aid of genetic testing, these distinct diseases can usually be parsed.