Congenital Myopathies POSNA

Description: Objectives
1. Describe clinical features which accompany congenital myopathies
2. Discuss pertinent anesthetic risk factors when considering surgery for congenital myopathies
Discussion
The congenital myopathies comprise a group of disorders characterized by hypotonia and weakness. Central core disease is a nonprogressive autosomal dominant with muscle weakness similar to Duchenne muscular dystrophy, except that weakness accompanying central core disease is easily detectable in infancy and motor development is delayed. Diagnosis is established by muscle biopsy. Scoliosis, soft tissue contractures, clubfeet, and hip dislocation have been associated with central core disease.