Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis.

ABSTRACT

Children with mitochondrial disorders represent a subset of patients who require unique anesthetic considerations. Routinely administered medications for general anesthesia, such as propofol, have been shown to increase the risk of developing metabolic acidosis. In addition, both depolarizing and nondepolarizing neuromuscular blockers are contraindicated due to the risk of hyperkalemic cardiac arrest and worsening of preexisting muscle weakness, respectively. These limitations pose challenges while choosing appropriate medications for induction of general anesthesia, especially when the risk of aspiration is high. We present a novel case of using inhaled sevoflurane and intravenous alfentanil to facilitate intubation in a 4-year-old girl with a complex 1 mitochondrial disorder suffering from severe gastroparesis and esophageal dysmotility.