Management of a case of Enhanced S-cone syndrome with massive foveoschisis treated with pars plana vitrectomy with silicone oil tamponade

Ophthalmic Genet. 2021 Oct;42(5):615-618. doi: 10.1080/13816810.2021.1925927. Epub 2021 May 10.

Abstract

Introduction: Goldmann Favre Syndrome (GFS) is a vitreoretinal degenerative disease with macular retinoschisis. The current treatment of foveoschisis is topical and oral carbonic anhydrase inhibitors.Case: A 22-year-old male diagnosed with GFS presented a progressive decrease in vision of the right eye. The optical coherence tomography showed a significant macular schisis. A medical treatment with topical and oral carbonic anhydrase inhibitors was ineffective. We performed a pars plana vitrectomy and silicone oil placement which led to an improvement of the visual acuity and a reduction of the foveoschisis.Conclusion: We describe here the first case of surgical treatment for macular schisis in a patient with GFS.

Keywords: Enhanced S-cone syndrome; Goldmann Favre syndrome; foveoschisis; vitrectomy.

Publication types

  • Case Reports

MeSH terms

  • Endotamponade / methods*
  • Eye Diseases, Hereditary / diagnosis
  • Eye Diseases, Hereditary / surgery*
  • Humans
  • Male
  • Retinal Degeneration / diagnosis
  • Retinal Degeneration / surgery*
  • Retinoschisis / diagnosis
  • Retinoschisis / surgery*
  • Silicone Oils / administration & dosage*
  • Tomography, Optical Coherence
  • Vision Disorders / diagnosis
  • Vision Disorders / surgery*
  • Visual Acuity
  • Vitrectomy*
  • Young Adult

Substances

  • Silicone Oils

Supplementary concepts

  • Enhanced S-Cone Syndrome