Maintenance of ambulation in Duchenne muscular dystrophy. The role of the orthopedic surgeon.

As the processes of pseudohypertrophic muscular dystrophy advance, contractures about joints in the lower extremities progress until an unstable base of support secondary to equinovarus and weakened pelvic balance produced by hip flexion prohibit ambulation. Properly timed surgery and bracing have helped some patients to extend significantly their ability to ambulate. Because severe weakness can result from prolonged restraint, operative procedures must permit immediate mobilization without fear of excessive pain or wound dehiscence. The primary aims of surgery are to maintain standing balance and prolong independent ambulation. Conditions requiring surgical correction are lower extremity flexion contracture, metatarsus adductus, and rigid forefoot equinocavovarus. Techniques designed to improve these deformities and permit early postoperative mobilization include subcutaneous release of contracted tendons and percutaneous removal of cancellous bone with corrective manipulation of the feet. Postoperative plastic bracing enhances balance through mild knee flexion and ischial seating. Maintenance of the upright posture extends the ability of these patients to attend to their tasks of daily living. In addition, these techniques offer a method of prolonging ambulation in the patient with pseudohypertrophic muscular dystrophy. In so doing, confinement to a wheelchair with its inevitable downhill course may be significantly postponed.