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Pediatric liver transplantations are becoming increasingly more common. Recent advances in the surgical and anesthetic management of these cases have greatly improved the survival rate. In order to successfully manage the anesthesia in these patients, one needs to have a thorough understanding of the pathophysiology of end-stage liver disease and the subsequent anesthetic implications. It is also necessary to appreciate the stages of the surgical procedure, as each stage presents different dilemmas to the anesthesiologist. This article will review the pathophysiology of liver failure in pediatric patients and outline the particular issues related to each stage of liver transplantation, allowing for the anticipation and management of the derangements that occur during surgery.

Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case.

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