Anesthetic management of an 8-month-old infant with osteogenesis imperfecta undergoing liver transplantation: a case report

Korean Journal of Anesthesiology 2014³â 66±Ç 6È£ p.472 ~ p.475

ÀÌÁö¿ø(Lee Ji-Won) - Seoul National University Hospital Department of Anesthesiology and Pain Medicine
·ùÈ£°É(Ryu Ho-Geol) - Seoul National University Hospital Department of Anesthesiology and Pain Medicine
À¯¼®ÇÏ(Yoo Seok-Ha) - Seoul National University Hospital Department of Anesthesiology and Pain Medicine
½Å½Â¿¬(Shin Seung-Yeon) - Seoul National University Hospital Department of Anesthesiology and Pain Medicine
(Kang Sun-Hye) - Seoul National University Hospital Department of Anesthesiology and Pain Medicine
Á¤Áø¿µ(Jeong Jin-Young) - Seoul National University Hospital Department of Anesthesiology and Pain Medicine
À¯¿ëÀç(Yoo Yong-Jae) - Seoul National University Hospital Department of Anesthesiology and Pain Medicine
±è¾È³ª(Kim An-Na) - Seoul National University Hospital Department of Anesthesiology and Pain Medicine

Abstract

Anesthetic management of pediatric liver transplantation in a patient with osteogenesis imperfecta (OI) requires tough decisions and comprehensive considerations of the cascade of effects that may arise and the required monitoring. Total intravenous anesthesia (TIVA) with propofol and remifentanil was chosen as the main anesthetic strategy. Malignant hyperthermia (MH), skeletal fragility, anhepatic phase during liver transplantation, uncertainties of TIVA in children, and propofol infusion syndrome were considered and monitored. There were no adverse events during the operation. Despite meticulous precautions with regard to the risk of MH, there was an episode of high fever (40¡É) in the ICU a few hours after the operation, which was initially feared as MH. Fortunately, MH was ruled out as the fever subsided soon after hydration and antipyretics were given. Although the delivery of supportive care and the administration of dantrolene are the core principles in the management of MH, perioperative fever does not always mean a MH in patients at risk for MH, and other common causes of fever should also be considered.

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Intravenous anesthesia, Liver transplantation, Osteogenesis imperfecta, Pediatrics, Pharmacokinetics, Malignant hyperthermia
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