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 ma nagement of a 6-year-old girl with complex I respiratory chain deficie ncy requiring surgery for a fractured hip is presented and discussed. Potential problems were masseter spasm, tendency to develop lactate ac idosis, and malignant hyperthermia susceptibility. These problem; were avoided by the use of a laryngeal mash airway, allowing the patient t o spontaneously ventilate; caudal analgesia; and maintenance of anesth esia with a proprofol infusion. (C) 1998 by Elsevier Science Inc.