title: Anesthetic management of an 8-month-old infant with osteogenesis imperfecta undergoing liver transplantation: a case report.
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℃) 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.
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Jiwon
authors.initials: J
authors.lastname: Lee
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Ho-Geol
authors.initials: HG
authors.lastname: Ryu
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Anna
authors.initials: A
authors.lastname: Kim
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Seokha
authors.initials: S
authors.lastname: Yoo
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Seung-Yeon
authors.initials: SY
authors.lastname: Shin
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Sun-Hye
authors.initials: SH
authors.lastname: Kang
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Jinyoung
authors.initials: J
authors.lastname: Jeong
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Yongjae
authors.initials: Y
authors.lastname: Yoo
keywords: Intravenous anesthesia; Liver transplantation; Malignant hyperthermia; Osteogenesis imperfecta; Pediatrics; Pharmacokinetics