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.
