Clinical manifestations and anesthetic management of Kearns-Sayre syndrome: A case report

Lee, Hae Mi; Heo, Su Jeong; Jee, Dae Lim

Case Report

Pediatric Anesthesia

Anesthesia and Pain Medicine 2011;6(3):290-293.
Published online July 30, 2011.

Clinical manifestations and anesthetic management of Kearns-Sayre syndrome: A case report

Department of Anesthesiology and Pain Medicine, School of Medicine, Yeungnam University, Daegu, Korea. adjee@medical.yeungnam.ac.kr

Abstract

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder resulting in multi-system dysfunction. A 14-year-old boy with KSS underwent external levator muscle resection for correction of ptosis. There were no abnormalities on the pre-operative evaluation, except for low-grade heart block and ocular problems. General anesthesia was conducted with a minimum dose of thiopental sodium and sevoflurane under close monitoring, and a laryngeal mask was inserted without muscle relaxation. The surgery was uneventful; however, a careful approach was required during anesthesia because life-threatening complications may occur in patients with KSS.

Key Words: General anesthesia, Kearns-Sayre syndrome, Mitochondrial disease