an 8-month - old male infant weighing 7.9 kg was scheduled for living donor liver transplantation due to progressive familial intrahepatic cholestasis . he was previously diagnosed with type 3 or 4 oi ( paternal col1a2 gene ) . the patient had suffered from fractures of the femur and humerus and had undergone four cycles of pamidronate treatment . apart from the elevated liver function tests ( alanine transaminase : 165 iu / l , aspartate transaminase : 211 iu / l and total bilirubin : 19.3 mg / dl ) due to cholestasis , other preoperative lab results were within the normal range . to minimize the risk of mh , after installation of a pediatric circuit and new co2 absorbent , ventilator washing ( ohmeda s/5 avance , madison , wi , usa , flow 10 l / min , tv 600 ml / fx , 10 /min , for 30 minutes ) was thoroughly performed and total intravenous anesthesia using propofol and remifentanil was planned and prepared . the patient was monitored using electrocardiogram , pulse oximetry , noninvasive blood pressure measurement , and bis . anesthesia was induced with 0.15 mg of atropine , 20 mg of propofol , and 5 mg of rocuronium . a 5-fr double lumen central venous catheter was placed in the right internal jugular vein under ultrasound guidance . when performing endotracheal intubation and central venous catheter insertion , we avoided excessive neck extension and rotation . anesthesia was maintained with total intravenous anesthesia using 2% propofol and remifentanil . during the operation , the average infusion rates of propofol and remifentanil were 100 g / kg / min and 0.1 g / kg / min respectively . body temperature and end - tidal co2 were carefully monitored and were within the normal range throughout the operation . arterial blood gas analyses were performed periodically to check for ph , pao2 , and paco2 . the patient received the lateral section of the left lobe of the liver from his mother . donor liver graft was preserved with the histidine - tryptophan - ketoglutarate ( htk ) solution ( custodiol , zenith pharm inc . , germany ) . just before anastomosis between the hepatic vein of the graft and reconstructed common orifice of the right , middle , and left hepatic vein , and the inferior vena cava of the recipient , the graft was perfused with 5% albumin through the portal vein . after portal vein anastomosis , the graft was reperfused by releasing the hepatic vein and portal vein clamps . during the reperfusion period , 50 mg of calcium chloride and 3 g of epinephrine were administered to treat the hypotension caused by the postreperfusion syndrome . apart from the reperfusion period , the patient 's vital signs were stable throughout the operation and the bis score was maintained between 45 and 65 . intraoperative laboratory data did not show features suggestive of propofol infusion syndrome ( table 1 ) . the anesthetic team worked in cooperation with the surgeon throughout the whole operation , and the surgery was cautiously carried out without a retractor . after the operation , vital signs were stable when the patient arrived at the picu and the axillary temperature was 36.2. three hours later , the patient had a sudden spiking fever of 40 ( fig . 1 ) and an increase in the abdominal circumference was observed . for assessment of the fever , blood and sputum cultures were performed . liver ultrasound was performed to rule out bowel congestion that can be associated with portal vein obstruction . electrolytes and arterial blood gas analysis were checked to investigate the possibility or mh but were normal ( table 1 ) . liver transplantation in this 8 month - old was performed using tiva because of the underlying disease of oi . the reported incidence range is 5 - 10 cases per 100,000 births and the disorder is commonly classified into 5 types . clinical manifestations of oi are brittle teeth , scoliosis , and bone fragility with fractures occurring with minimal trauma . however , mh can be prevented if tiva is used instead of inhaled anesthetics and triggering factors such as succinylcholine are avoided . it is not clear whether the adult bis algorithm and equipment can be applied to pediatric patients . the eeg of newborns is different from that of adults because brain maturation and synapse formation continues until the age of 5 . however , some have suggested the possibility of using bis in pediatric patients by showing the correlation of bis with end - tidal sevoflurane concentration in infants and children . therefore , although not optimal , we monitored the bis and maintained the bis score between 45 and 65 . what complicates tiva in pediatric liver transplantation even further are the pharmacokinetic changes that occur during the anhepatic phase . hepatic metabolism of propofol contributes about 60% of the total propofol clearance . in adult liver transplantation , apparent systemic clearance of propofol was decreased by up to 42% during the anhepatic phase compared with the liver dissection phase . in our case , the infusion rate of propofol was carefully titrated with the aid of the bis monitor so that it was not low enough to cause intraoperative awareness , but not high enough to trigger the side effects of propofol , especially the propofol infusion syndrome . intraoperative laboratory data may not be able to discriminate between the metabolic acidosis produced in the anhepatic period during liver transplantation and that caused by the propofol infusion syndrome . however , the degree of metabolic acidosis is expected to be much more severe when the propofol infusion syndrome coincides with the lactic acidosis that usually develops during liver transplantation . the average infusion rates of propofol and remifentanil were 100 - 150 g / kg / min and 0.1 - 0.15 g / kg / min respectively throughout the operation . volatile anesthetics and succinylcholine are the most commonly known triggers of mh in susceptible patients . there are reports of other mh triggering drugs in the literature and these include serotonergic drugs , phosphodiesterase type iii inhibitors , statins , tetracaine , methylene blue and ondansetron . we were unable to find reports of mh triggered by drugs or solutions used in liver transplantation such as htk solution for liver graft preservation , nafamostat mesilate ( futhan , sk chemicals , japan ) and basiliximab ( simulect , novartis , switzerland ) ; this lack of reports was yet another challenge when managing our case . given that most triggers of mh are related to anesthesia and that liver transplantation is the only viable option , we performed tiva to manage the oi patient receiving pediatric liver transplantation and we report this case . although cautious anesthesia was perforrmed with regard to mh , there was an episode of fever in the icu . however , the physicians considered that giving conservative treatment and performing a work - up for other surgical complications were more appropriate prior to using dantrolene . we closely monitored the patient , and as the fever subsided after hydration and antipyretics , the possibility of mh was considered insignificant . however , dehydration atelectasis and excessive temperature preservation are thought to be likely causes of the postoperative fever . in pediatric liver transplantation of an oi patient , anesthesiologists need to consider some issues . first , when monitoring the sedation depth using bis in pediatric patients , the difference in bis between pediatric patients and adults owing to eeg has to be taken into account . second , for the maintenance of tiva during the anhepatic phase , it should be kept in mind that changes in drug metabolism can occur due to the absence of the liver . third , close monitoring and frequent measurements of laboratory data such as arterial blood gas are required . if metabolic acidosis is more severe than usually occurs during the anhepatic phase , propofol infusion syndrome should be considered and ruled out . fourth , the potential for drugs used during organ transplantation to trigger mh should always be considered . lastly , when patients with a high risk of developing mh develop fever , conservative treatment such as hydration and antipyretics should be a priority . based on our experience , we believe that propofol based tiva is a safe alternative anesthetic strategy in oi patients undergoing liver transplantation . we would also like to stress that when managing a febrile event in a patient with a high risk of developing mh , other causes should be ruled out and conservative supportive management such as hydration and antipyretics should not be overlooked .
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 .