Abstract

BERRIO VALENCIA, Marta Inés; MARTINEZ OCHOA, Catalina María  and  DURANGO GOMEZ, Heisa Catalina. Anesthetic complications in patients with inborn errors of metabolism undergoing non-cardiac surgery. Rev. colomb. anestesiol. [online]. 2013, vol.41, n.4, pp.257-260. ISSN 0120-3347.  https://doi.org/10.1016/j.rca.2013.05.010.

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 hyperglycinemia, with seizures in one case and the need for mechanical ventilation in another. Condusions: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.

Keywords : Metabolism, Inborn Errors; Anaesthesia; Intraoperative Complications; Infant; Newborn; Hypoglycemia.

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