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KMID : 1155520110060030290
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Anesthesia and Pain Medicine
2011 Volume.6 No. 3 p.290 ~ p.293
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Clinical manifestations and anesthetic management of Kearns-Sayre syndrome -A case report-
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Lee Hae-Mi
Heo Su-Jeong
Jee Dae-Lim
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Abstract
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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.
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KEYWORD
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General anesthesia, Kearns-Sayre syndrome, Mitochondrial disease
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