Metabolic Disorders and Anesthesia.

Abstract

Metabolic disorders encompass a group of inherited inborn errors of metabolism that are uncommonly encountered but can pose challenges when encountered during the perioperative period. Hence, it is paramount that anesthesiologists are experienced and familiar with management of these conditions. Hundreds of inborn errors of metabolism have already been identified, yet new metabolic disorders continue to be discovered with advancements in genomic science. In our general review, we define the more common metabolic disorders encountered in perioperative medicine and discuss the perioperative anesthetic considerations and challenges associated with each disorder. The following disorders are covered in our review: disorders of carbohydrate metabolism, disorders of amino acid metabolism, disorders of branched-chain amino acid metabolism, organic acidemias, mitochondrial disorders, lysosomal storage disorders, metal metabolism disorders, and urea cycle disorders.