Total Intravenous Anesthesia With Remimazolam and Remifentanil for Intraoperative Care of a Child With Venezuelan Heritage, at Risk for a Mitochondrial Disorder.

Recent clinical communications have alerted healthcare providers to potential concerns related to a previously undiagnosed disorder of mitochondrial function in children with maternal Venezuelan heritage. These patients have developed severe neurological damage following the administration of an apparently uneventful anesthetic that included the volatile anesthetic agent, sevoflurane. Subsequent analysis of family members and patients has identified a point mutation of the NADH dehydrogenase 4 (ND4) gene (mtND4 m.11232T>C), a subunit of complex I of the mitochondrial electron transport chain. Current recommendations include avoidance of volatile anesthetic agents and propofol. We present a 6-year-old child with Venezuelan heritage who presented for anesthetic care for reduction and percutaneous pinning of a supracondylar fracture. Total intravenous anesthesia (TIVA) was provided with remimazolam and remifentanil as the primary agents. Information regarding the current investigation of this novel mitochondrial defect is presented, options for anesthetic care reviewed, and the novel use of remimazolam and remifentanil as the primary agents for TIVA discussed.