Perioperative General Anesthetic Management for a Patient with Congenital Myotonic Dystrophy Accompanied by Mental Retardation and Attention Deficit Hyperactivity Disorder

Abstract

Perioperative management of general anesthesia for a patient with myotonic dystrophy requires special consideration because they have a number of problems, including malignant hyperthermia and post-operative respiratory complications. It is also necessary to monitor carefully the patient especially in the intensive care unit after surgery. We report here the perioperative general anesthetic management of a patient with congenital myotonic dystrophy accompanied by mental retardation and attention deficit hyperactivity disorder, a combination which we anticipated would further complicate the anesthetic management.
The patient had previously undergone mandible movement surgery. This time, he was scheduled to undergo plate removal surgery under general anesthesia. We selected the total intravenous anesthesia （TIVA） technique using a combination of propofol and remifentanil hydrochloride, with rocuronium as a muscle relaxant. Intraoperative problems were not observed. Following the mandible movement surgery, we observed the patient’s condition and judged it possible to return him to the general ward. However, this time, the high risk of complications was a concern in the postoperative period, because his symptoms of myotonic dystrophy might deteriorate. Therefore, he was moved to the intensive care unit after extubation.
In the intensive care unit, the patient had to be exposed to the environment and to tolerate unpleasant things such as insertion of a urethral catheter and frequent suction of phlegm. Also, a decrease in SpO₂ by transient apnea was observed at the time of sleeping, so oxygen administration via a nasal cannula was necessary until the next morning. In order to overcome these issues, we utilized the identity preservation phenomenon through visual stimulation, by allowing the patient to watch his favorite DVD repeatedly. This method kept the patient on bed rest and anesthetic management was successful at ease.