Anesthetic complications in patients with inborn errors of metabolism undergoing non-cardiac surgery
Abstract
Introduction: Inborn 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.
Objective: To describe the complications of anesthetic management in pediatric patients with inborn errors of metabolism undergoing non-cardiac surgery.
Methods: Retrospective 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.
Results: The 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 hyper-glycinemia, with seizures in one case and the need for mechanical ventilation in another.
Conclusions: Low-risk procedures probably explain why there were few complications. It was found that seizures and respiratory insufficiency are potential perioperative complications in nonketotic hyperglycinemia.
References
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