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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
Hae Mi Lee, Su Jeong Heo, Dae Lim Jee
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


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