Late-Stage Sjögren-Larsson Syndrome Maculopathy Imaged With OCT Angiography

Ophthalmic Surg Lasers Imaging Retina. 2018 Sep 1;49(9):e78-e82. doi: 10.3928/23258160-20180907-11.

Abstract

Three adult siblings with Sjögren-Larsson syndrome (SLS) demonstrated signs of late-stage SLS maculopathy, including intraretinal crystals, atrophic changes, and lipofuscin deposition. This first report of SLS maculopathy imaged with optical coherence tomography angiography revealed decreased retinal capillary density, vessel dilation, and increased flow voids in the superficial and deep capillary plexuses. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:e78-e82.].

Publication types

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

MeSH terms

  • Adult
  • Capillaries / pathology
  • Female
  • Fluorescein Angiography / methods*
  • Follow-Up Studies
  • Fundus Oculi
  • Humans
  • Macula Lutea / pathology*
  • Male
  • Retinal Diseases / diagnosis*
  • Retinal Diseases / etiology
  • Retinal Vessels / pathology*
  • Siblings*
  • Sjogren-Larsson Syndrome / complications*
  • Sjogren-Larsson Syndrome / diagnosis
  • Tomography, Optical Coherence / methods*