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Management of Ptosis in Kearns-Sayre Syndrome: A Case Report and Literature Review

  • Moulay O. Moustaine (Department of Ophthalmology, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University) ;
  • Zakaria Azemour (Department of Ophthalmology, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University) ;
  • Frarchi Mohammed (Department of Ophthalmology, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University) ;
  • Othman Benlanda (Department of Anesthesia Resuscitation, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University) ;
  • Hicham Nassik (Department of Anesthesia Resuscitation, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University) ;
  • Mehdi Karkouri (General Laboratory of Pathological Anatomy, CHU Ibn Rochd, Faculty of Medicine and Pharmacy, Hassan II University)
  • Received : 2023.03.30
  • Accepted : 2023.10.25
  • Published : 2024.03.15

Abstract

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease that affects young adults, due to a deletion of mitochondrial DNA and characterized by the triad: age of onset lower than 20 years, chronic progressive external ophthalmoplegia, and an atypical pigmentary retinopathy. It is also characterized by other endocrine, neurological, and especially cardiac impairment with a very high risk of cardiac complications during surgical procedures under all types of anesthesia. We report a case of KSS revealed by severe bilateral ptosis and confirmed by a muscle biopsy with "ragged red fibers." The ptosis was surgically managed by cautious Frontal suspension under local anesthesia "Frontal nerve block." Through this case, we discuss challenges in the management of KSS patients.

Keywords

References

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