Early identification of retinal angiomas in a large kindred von Hippel-Lindau disease

Am J Ophthalmol. 1980 Apr;89(4):540-5. doi: 10.1016/0002-9394(80)90063-x.


We studied prospectively a large kindred afflicted with von Hippel-Lindau disease. Of 111 members examined ophthalmoscopically, 14 definite retinal angiomas were found and three lesions were thought to be angiomas. The lesions found could be divided into the following three groups: (1) classic angiomas, (2) atypical angiomas, and (3) lesions indicative of early angiomas. A correlation between increasing age, systemic evidence of disease, and size and appearance of angiomas was found.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Angiomatosis / genetics*
  • Child
  • Eye Neoplasms / diagnosis
  • Eye Neoplasms / genetics*
  • Female
  • Fluorescein Angiography
  • Genetic Counseling
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Radiography
  • Retina* / blood supply
  • Retinal Vessels / diagnostic imaging
  • von Hippel-Lindau Disease / diagnosis
  • von Hippel-Lindau Disease / genetics*