Chest and spinal disease in patients with progressive neuromuscular disease.

The chest and spine deformity in neuromuscular disease (NMDz) can impact respiratory mechanics and pulmonary function by changing the orientation of the muscles and joints of the respiratory system and placing them in a mechanically unfavorable position. This increases mechanical load on weak respiratory muscles and eventually can cause respiratory failure. Therefore, chest and spine deformity in NMDz will both lead to increased respiratory "load" and decreased respiratory muscle "pump", an exceptionally bad combination. While the current pharmacotherapies used for progressive neuromuscular disease focus on slowing progression, a similar approach has been used for decades in managing chest and spine deformity in patients with NMDz. There are, however, variable approaches to doing so and a recognition that not all "neuromuscular scoliosis" is the same and that each patient type (i.e. hypotonic vs. hypertonic) requires a different approach. Figuring out what approach to use requires both an understanding of the underlying pathophysiology of a particular neuromuscular condition and considering available options for and timing of surgical interventions. The remaining discussion will focus on hypotonic neuromuscular scoliosis.