Best Vitelliform Macular Dystrophy

  • Stephen H. Tsang
  • Tarun SharmaEmail author
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 1085)


Best vitelliform macular dystrophy (VMD or BVMD) is one of the most common macular dystrophies, affecting 1 in 10,000 individuals. The clinical presentation varies, depending on the stage of the disease at which the patient presents, usually one of these five stages:
  • Previtelliform

  • Vitelliform (Figs. 16.1, 16.2, 16.3 and 16.4)

  • Pseudohypopyon

  • Vitelliruptive (Fig. 16.5)

  • Atrophic


Autosomal dominant Best disease 

Suggested Reading

  1. Boon CJ, Klevering BJ, Leroy BP, Hoyng CB, Keunen JE, den Hollander AI. The spectrum of ocular phenotypes caused by mutations in the BEST1 gene. Prog Retin Eye Res. 2009;28:187–205.CrossRefGoogle Scholar
  2. Guerriero S, Preising MN, Ciccolella N, Causio F, Lorenz B, Fischetto R. Autosomal recessive bestrophinopathy: new observations on the retinal phenotype – clinical and molecular report of an Italian family. Ophthalmologica. 2011;225:228–35.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Jonas Children’s Vision Care, Bernard & Shirlee Brown Glaucoma Laboratory, Columbia Stem Cell Initiative-Departments of Ophthalmology, Biomedical Engineering, Pathology & Cell Biology, Institute of Human Nutrition, Vagelos College of Physicians and Surgeons, Columbia UniversityNew YorkUSA
  2. 2.Department of Ophthalmology, Columbia UniversityEdward S. Harkness Eye Institute, NewYork-Presbyterian HospitalNew YorkUSA

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