Anesthetic considerations in medium-chain acyl-CoA dehydrogenase deficiency

Abstract

In the 1980?s, medium-chain acyl-CoA dehydrogenase deficiency (MCADD) was first described in the literature as three children who presented with coma, hypoglycemia, hyperammonemia, and fatty liver while fasting. These symptoms while similar to Reye?s Syndrome, were found to be due to an inability to metabolize medium chain fatty acids during fasting periods. Fatty acids are utilized by the body asessential fuel for skeletal and cardiac muscle, and as an important source of energy during fasting periods. Medium-chain acyl-CoA dehydrogenase is a mitochondrial enzyme required for the beta oxidation of medium chain fatty acids (C4-14), which is deficient in this syndrome. Anesthesiologists may come across these patients in their practice, as MCADD is the most common inhertited disorder of mitochondrial fatty acid oxidation. In addition to determining NPO timing and IV fluid selection, other preoperative issues anesthesiologists must consider are medication management to avoid metabolic decompensation. This manuscript will consider a pediatric patient with MCADD who presented to our pediatric hospital and received a general anesthetic following the guidelines created by ourmultidisciplinary perioperative team.