Anesthesia for a child with complex I respiratory chain enzyme deficiency.

The mitochondrial myopathies are a rare group of conditions affecting the respiratory chain and oxidative phosphorylation. The anesthetic management of a 6-year-old girl with complex I respiratory chain deficiency requiring surgery for a fractured hip is presented and discussed. Potential problems were masseter spasm, tendency to develop lactate acidosis, and malignant hyperthermia susceptibility. These problems were avoided by the use of a laryngeal mask airway, allowing the patient to spontaneously ventilate; caudal analgesia; and maintenance of anesthesia with a proprofol infusion.