Posterior staphyloma in oculocutaneous albinism: another possible cause of reduced visual acuity

J AAPOS. 2015 Dec;19(6):562-4. doi: 10.1016/j.jaapos.2015.06.009.


Posterior staphyloma is typically associated with myopic degeneration and has not been recognized as a cause of reduced visual acuity in albinism. We report 3 cases of posterior staphyloma, each with oculocutaneous albinism (OCA) defined by phenotype and genotype. Two cases are biological sisters with OCA type 2; one was myopic and the other was hyperopic. The third case involves a man with OCA associated with Hermansky-Pudlak syndrome (HPS-5). Staphyloma may be another cause of reduced visual acuity in albinism, particularly with increasing age. It may occur in association with myopia or hyperopia.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged, 80 and over
  • Albinism, Oculocutaneous / complications*
  • Albinism, Oculocutaneous / diagnosis
  • Albinism, Oculocutaneous / genetics
  • Dilatation, Pathologic
  • Female
  • Hermanski-Pudlak Syndrome / complications*
  • Hermanski-Pudlak Syndrome / diagnosis
  • Hermanski-Pudlak Syndrome / genetics
  • Humans
  • Infant
  • Male
  • Posterior Eye Segment / pathology*
  • Scleral Diseases / complications*
  • Scleral Diseases / diagnosis
  • Scleral Diseases / genetics
  • Siblings
  • Vision Disorders / diagnosis
  • Vision Disorders / etiology*
  • Visual Acuity*

Supplementary concepts

  • Oculocutaneous albinism type 2