Fundus autofluorescence in maternally inherited diabetes and deafness

the gold standard for monitoring maculopathy?

Christopher A. Ovens*, Kate Ahmad, Clare L. Fraser

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

Maternally inherited diabetes and deafness (MIDD) is a mitochondrial disease associated with dysfunction of the retinal pigment epithelium and photoreceptor outer segments in a peri-foveal arrangement. If chorioretinal atrophy develops, patients risk losing vision. We retrospectively analysed three patients with genetically proven MIDD, assessing atrophy size and progression using overlay in photoshop. Patients showed increase in chorioretinal atrophy of 205%, 46% and 34%, respectively. We also found location-specific progression, where hyper-autofluorescent deposits evolved into areas of atrophy. These results support the use of fundus autofluorescence as a valuable tool in monitoring disease progression and providing prognostic information for clinicians and patients.

Original languageEnglish
Pages (from-to)168-173
Number of pages6
JournalNeuro-Ophthalmology
Volume44
Issue number3
Early online date24 Sep 2019
DOIs
Publication statusPublished - 2020

Keywords

  • Atrophy
  • fundus autofluorescence
  • maculopathy
  • MIDD

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