[Anesthetic management of a child with myotubular myopathy who underwent two operations using only total intravenous anesthesia (TIVA)].

We present a case of anesthetic management in a child with myotubular myopathy. A 3-month-old, 3.0 kg, male patient, who had been suspected of a congenital myopathy, was scheduled for the muscle biopsy. He was intubated at birth in NICU. Anesthesia was induced with propofol and remifentanil, and maintained with propofol and remifentanil. The results of biopsy and gene analysis led to the diagnosis of myotubular myopathy. Five months later, this 8-month-old, 4.0 kg, patient was scheduled for the tracheostomy. Anesthesia was induced with propofol, fentanyl and rocuronium bromide, and maintained with propofol and fentanyl. The child underwent two operations under total intravenous anesthesia (TIVA) with propofol and fentanyl or remifentanil. These anesthetic courses were uneventful without symptoms of malignant hyperthermia nor propofol infusion syndrome. We did not use sugammadex, because there is still no evidence to the safe use of sugammadex in infants (aged 28 days-23 months). Congenital myopathy is related to malignant hyperthermia, and total intravenous anesthesia (TIVA) is a preferable and safe method for children with this disease.