Download PDF
CC BY 4.0 · Arch Plast Surg 2024; 51(02): 182-186
DOI: 10.1055/a-2207-7587
Pediatric/Craniomilofacial/Head & Neck
Case Report

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

Moulay O. Moustaine 
1   Department of Ophthalmology, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University, Agadir, Morocco
,
Zakaria Azemour 
1   Department of Ophthalmology, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University, Agadir, Morocco
,
Frarchi Mohammed 
1   Department of Ophthalmology, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University, Agadir, Morocco
,
Othman Benlanda 
2   Department of Anesthesia Resuscitation, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University, Agadir, Morocco
,
Hicham Nassik 
2   Department of Anesthesia Resuscitation, CHU Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr University, Agadir, Morocco
,
Mehdi Karkouri 
3   General Laboratory of Pathological Anatomy, CHU Ibn Rochd, Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco
› Author Affiliations
› Further Information
   Permissions and Reprints

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

Kearns–Sayre syndrome - ptosis - chronic progressive external ophthalmoplegia - Frontal suspension

Authors' Contributions

Conceptualization: M.O.M., Z.A. Data curation: M.O.M., Z. A. Writing - original draft: M.O.M., Z.A. Writing - review & editing: M.O.M., Z.A., F.M., B.O., N.H., K.M.


Ethical Approval

The study was approved by the Biomedical Research Ethics Committee of the Faculty of Medicine and Pharmacy of Casablanca (IRB no. 00002504).


Patient Consent

The patient and his family have given their explicit consent for their data and photos to be used for scientific purposes.




Publication History

Received: 30 March 2023

Accepted: 25 October 2023

Accepted Manuscript online:
09 November 2023

Article published online:
08 April 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 

  • References

  • 1 Zeviani M, Moraes CT, DiMauro S. et al. Deletions of mitochondrial DNA in Kearns-Sayre syndrome. Neurology 1988; 38 (09) 1339-1346
    CrossrefPubMedGoogle Scholar
  • 2 Berardo A, DiMauro S, Hirano M. A diagnostic algorithm for metabolic myopathies. Curr Neurol Neurosci Rep 2010; 10 (02) 118-126
    CrossrefPubMedGoogle Scholar
  • 3 Shemesh A, Margolin E. Kearns-Sayre Syndrome. In: StatPearls. StatPearls Publishing; Treasure Island (FL): 2022
    Google Scholar
  • 4 Kearns TP, Sayre GP. Retinitis pigmentosa, external ophthalmophegia, and complete heart block: unusual syndrome with histologic study in one of two cases. AMA Arch Opthalmol 1958; 60 (02) 280-289
    CrossrefGoogle Scholar
  • 5 Tsang SH, Aycinena ARP, Sharma T. Mitochondrial disorder: Kearns-Sayre syndrome. Adv Exp Med Biol 2018; 1085: 161-162
    CrossrefPubMedGoogle Scholar
  • 6 Capková M, Tesarová M, Wenchich L. et al. [Disorders of mitochondrial energy metabolism in patients with the Kearns-Sayre syndrome]. Cas Lek Cesk 2002; 141 (02) 51-54
    PubMedGoogle Scholar
  • 7 Khambatta S, Nguyen DL, Beckman TJ, Wittich CM. Kearns-Sayre syndrome: a case series of 35 adults and children. Int J Gen Med 2014; 7: 325-332
    PubMedGoogle Scholar
  • 8 Gaboune L, Baha Ali T, Benfdil N. et al. [Kearns-Sayre syndrome: a case report]. J Fr Ophtalmol 2012; 35 (09) 718.e1-718.e4
    CrossrefPubMedGoogle Scholar
  • 9 Isashiki Y, Nakagawa M, Ohba N. Retinal manifestations in mitochondrial diseases. Acta Ophthalmol Scand 1998; 76: 43-49
    CrossrefPubMedGoogle Scholar
  • 10 Laloi-Michelin M, Virally M, Jardel C. et al. Kearns Sayre syndrome: an unusual form of mitochondrial diabetes. Diabetes Metab 2006; 32 (02) 182-186
    CrossrefPubMedGoogle Scholar
  • 11 Moraes CT, Ricci E, Petruzzella V. et al. Molecular analysis of the muscle pathology associated with mitochondrial DNA deletions. Nat Genet 1992; 1 (05) 359-367
    CrossrefPubMedGoogle Scholar
  • 12 Kitoh T, Mizuno K, Otagiri T, Ichinose A, Sasao J, Goto H. Anesthetic management for a patient with Kearns-Sayre syndrome. Anesth Analg 1995; 80 (06) 1240-1242
    PubMedGoogle Scholar
  • 13 Calzavacca P, Schmidt W, Guzzi M. General anaesthesia for laparoscopic cholecystectomy in a patient with the Kearns-Sayre syndrome. Case Rep Anesthesiol 2011; 2011: 806086
    PubMedGoogle Scholar
  • 14 Çakmak BM, Akpek EA, Arslan G. Anesthesia management of a patient with Kearn's-Sayre syndrome. J Clin Anal Med 2013; 4 (02) 155-157
    Google Scholar
  • 15 Talaván Serna J, Belmonte Bayo L, Gutiérrez Rueda E, Rodríguez Martínez S. Anesthetic management of Kearns-Sayre syndrome. Case report. Rev Esp Anestesiol Reanim (Engl Ed) 2021; 68 (04) 232-234
    Google Scholar
  • 16 Choi C, Sunwoo IN, Kim HS, Kim DI. Transient improvement of pyruvate metabolism after coenzyme Q therapy in Kearns-Sayre syndrome: MRS study. Yonsei Med J 2000; 41 (05) 676-679
    CrossrefPubMedGoogle Scholar
  • 17 Weitgasser L, Wechselberger G, Ensat F, Kaplan R, Hladik M. Treatment of eyelid ptosis due to Kearns-Sayre syndrome using frontalis suspension. Arch Plast Surg 2015; 42 (02) 214-217
    Article in Thieme ConnectPubMedGoogle Scholar