Combined General and Regional Anesthesia for a Patient With Duchenne Muscle Dystrophy With an Implanted Left Ventricular Assisted Device Undergoing Orthopedic Surgery

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked inherited dystrophinopathy, with an incidence of 1 in 3,600 -5,000 male liveborn infants. The leading cause of death is often cardiomyopathyrelated heart failure. Given the progressive nature of the disorder with involvement of skeletal muscle, respiratory and cardiac function, perioperative care remains challenging with an increased incidence of perioperative morbidity and mortality. Perioperative care can be challenging due to life-threatening perioperative adverse events related to associated end-organ effects, as well as sensitivity to various anesthetic agents, rhabdomyolysis, hyperkalemia, hyperthermia, and cardiac arrest. We present a 22-year-old DMD patient with left ventricular assisted device (LVAD), who presented for repair of both left distal femur and tibial diaphysis fractures. Anesthetic care included the unique combination of total intravenous anesthesia with dexmedetomidine and remimazolam combined with regional anesthesia including a supra-inguinal fascia iliaca block, saphenous nerve block, and popliteal nerve block. The basics of dystrophinopathies are presented, perioperative concerns discussed, and previous reports of the use of regional anesthesia as an adjunct to general anesthesia in adult and pediatric patients with DMD are reviewed.