Successful perioperative management of a pediatric patient with medium-chain acyl-CoA dehydrogenase deficiency using a continuous tissue glucose monitoring device: A case report

Abstract

Medium-chain acyl-CoA dehydrogenase deficiency can cause symptoms resulting from β-oxidation disorder during preoperative fasting. Tight perioperative glucose monitoring is needed to avoid these symptoms. We report the first pediatric case using continuous tissue glucose monitoring devices. The patient was a 9-year-old boy with medium-chain acyl-CoA dehydrogenase deficiency for whom femoral osteotomy and selective muscle release of the hip and knee was planned to treat hip dislocation and joint contracture. To monitor tissue glucose levels continuously during preoperative fasting, a percutaneous sensor was attached to the right upper extremity, 2 days before the operation. Anesthetic management using Ringer's acetate containing 5% glucose, an inhalational agent, and epidural anesthesia without a muscle relaxant or propofol was performed without complications. The device achieved tight perioperative glucose monitoring. Continuous tissue glucose monitoring devices helped perioperative glucose monitoring of the pediatric patient with medium-chain acyl-CoA dehydrogenase deficiency.

Keywords

• acylcarnitines; continuous tissue glucose monitoring; fasting; hypoglycemia; medium-chain acyl-coa dehydrogenase deficiency