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Sorsby Pseudoinflammatory Fundus Dystrophy

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

Abstract

This dominantly inherited disease begins with fine, pale, drusen-like deposits or confluent faint yellow material or sheets beneath the retinal pigment epithelium (RPE), but it eventually progresses to either geographic atrophy with pigmentary clumps or scar due to choroidal neovascular membrane (at about 40 years of age) (Figs. 20.1, 20.2 and 20.3). The patient usually becomes symptomatic, with loss of central vision (about 20/200 or less), in the fourth to sixth decade of life. When neovascular membrane develops, it mimics age-related macular degeneration (AMD), but the age of onset is much earlier.

Keywords

Autosomal dominant Sorsby fundus dystrophy 

Suggested Reading

  1. Polkinghorne PJ, Capon MR, Berninger T, Lyness AL, Sehmi K, Bird AC. Sorsby’s fundus dystrophy. A clinical study. Ophthalmology. 1989;96:1763–8.CrossRefGoogle Scholar
  2. Sorsby A, Mason ME. A fundus dystrophy with unusual features. Br J Ophthalmol. 1949;33:67–97.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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