Anesthetic complications in patients with inborn errors of metabolism undergoing non-cardiac surgery

Abstract

IntroductionInborn errors of metabolism are alterations in one or more steps in a metabolic pathway. They are associated with multisystem complications and have a high impact on the quality of life of patients. Enzyme replacement has extended life, hence the importance of recognizing the most frequent anesthetic complications. ObjectiveTo describe the complications of anesthetic management in pediatric patients with inborn errors of metabolism undergoing non-cardiac surgery. MethodsRetrospective descriptive observational case series of pediatric patients with inborn errors of metabolism undergoing non-cardiac surgery at the Pablo Tobón Uribe Hospital between 2008 and 2011, and their anesthetic outcomes. ResultsThe most frequent inborn error of metabolism was glycogen storage disease type III in 7 (37%), followed by nonketotic hyperglycinemia in 4 (21%). There were 2 (6%) anesthetic complications in the immediate post-operative period in patients with nonketotic hyperglycinemia, with seizures in one case and the need for mechanical ventilation in another. ConclusionsLow-risk procedures probably explain why there were few complications. It was found that seizures and respiratory insufficiency are potential perioperative complications in nonketotic hyperglycinemia.