Muscular Dystrophies and Mitochondrial Myopathies

 Children with neuromuscular diseases have a broad range of presentation and anesthesia considerations. Muscle strength is reduced by degenerative nerve supply and affected neuromuscular junctions or by weakening of muscle fibers directly. Muscular dystrophies are an inherited group of disorders characterized by progressive muscle weakness. These children pose specific challenges related to anesthetic care due to skeletal muscle, pulmonary, and cardiac involvement. This chapter discusses the perioperative management of children with Duchenne, Becker, limb-girdle, EmeryDreifuss, and myotonic muscular dystrophies. Also discussed are mitochondrial myopathies, a group of clinical conditions common to the pediatric population. Cerebral palsy (CP) is a nonspecific, descriptive term that encompasses a constellation of symptoms due to a neurologic lesion resulting from the insult to the developing brain sustained early in life. Although the neurologic lesion in CP is non-progressive, the motor dysfunction due to spasticity may be progressive, leading to spinal deformities, joint contractures, and dislocations requiring medical and surgical interventions. Anesthetic care for children with the above stated neuromuscular disorders requires understanding of their disease process and careful attention to all aspects of perioperative care. Thoughtful planning should include thorough preoperative assessment, attention to co-morbidities, management of chronic medications, and meticulous intraoperative care for these patients. Postoperative assessment of pain and its management are essential to facilitate recovery and uneventful perioperative course.

Keywords: Becker muscular dystrophy, Cerebral palsy, Coagulopathy, Duchenne muscular dystrophy, Emery-Dreifuss muscular dystrophy, Hyperkalemic cardiac arrest, Hypothermia, Intrathecal baclofen pump, Lactic acidosis, Limb-girdle muscular dystrophy, Malignant hyperthermia susceptibility, Mitochondrial encephalopathy, Mitochondrial myopathies, Myotonic dystrophy, Neuromuscular spine fusion, Rhabdomyolysis, Seizure disorder, Spasticity.