Surgical Correction of Scoliosis in Patients With Duchenne Muscular Dystrophy: 30-Year Experience.

Abstract

The natural history of scoliosis in Duchenne muscular dystrophy (DMD) is progressive and debilitating if neglected. The purpose of this study was to evaluate outcomes related to spinal deformity surgery in patients with DMD over a 30-year period. This was a single center retrospective study of all operatively treated scoliosis in DMD patients over 30 years. Minimum follow-up was 2 years. Owing to changes in instrumentation over time, patients were divided into 2 groups: Luque or pedicle screws (PS) constructs. Radiographic, perioperative variables, pulmonary function test (preoperatively and postoperatively), and complication data were evaluated. There were 60 subjects (Luque: 47, PS: 13). The Luque group was on average 13 years old, 53 kg, and had 7 years of follow-up. Coronal Cobb was 31±12 degrees preoperatively, 16±11 degrees at first postoperatively, and 21±15 degrees at final follow-up (P≤0.001). Pelvic obliquity was 7±6 degrees preoperatively, 5±5 degrees at first postoperatively (P=0.43), and 5±4 degrees at final follow-up (P=0.77). The majority of this group was fused to L5 (45%) or the sacrum (49%). The PS group was on average 14 years old, 65 kg, and had 4 years of follow-up. Coronal Cobb was 43±19 degrees preoperatively, 12±9 degrees at first postoperatively (P≤0.001), and 12±8 degrees at final follow-up. Pelvic obliquity was 6±5 degrees preoperatively, 3±3 degrees at first postoperatively (P=0.06), and 2±2 degrees at final follow-up (P=0.053). The majority were fused to the pelvis (92%). Both groups' pulmonary function declined with time. Both groups had high complication rates (Luque 68%; PS group 54%). The Luque group had more implant-related complications (26%); the PS group had a higher rate of early postoperative infections (23%). Over a 30-year period of operative treatment of scoliosis in DMD, both PS constructs and Luque instrumentation improved coronal Cobb. The PS group had improved and maintained pelvic obliquity. Both groups had high complication rates. Level IV-therapeutic.