Thoracogenic spinal deformity: a rare cause of early-onset scoliosis

OBJECTIVE Surgery for severe congenital defects, such as congenital diaphragmatic hernia, congenital heart defects, and tracheoesophageal disorders, are life-saving treatments for many infants. However, the incidence of scoliosis following thoracoabdominal surgery has been reported to range from 8% to 50%. Little is known about severe scoliosis that occurs after chest wall procedures in infants. The authors sought to determine the prevalence of thoracogenic scoliosis, disease severity, and need for scoliosis surgery in patients who underwent chest wall procedures in early childhood. METHODS A multicenter database of patients with early-onset scoliosis was queried to identify patients with a history of thoracogenic or acquired iatrogenic scoliosis. Patients with significant congenital spine deformities were excluded. Forty-one patients (1.6%) were noted to have thoracogenic scoliosis. Of these patients, 14 patients were observed; 10 received casts and/or braces; and 17 underwent treatment with rib-based distraction rods, Shilla procedures, or spine-based growing rod devices. Radiographs, complications, and patent characteristics were reviewed. RESULTS The mean age at scoliosis diagnosis for the 41 patients was 6.0 years. The mean time to follow-up was 2.9 years (4.5 years in the 17 surgical patients). The mean preoperative coronal Cobb angle in the surgical group was 65 degrees and improved to 47 degrees postoperatively (p = 0.01). The mean Cobb angle for the nonoperative group was 31 degrees initially and 32 degrees at follow-up (p = 0.44). Among the 17 patients undergoing surgery for scoliosis, there were 13 complications in 7 patients, including a brachial plexus palsy following rib-based distraction rod placement. This resolved with revision of the rib hooks. There were no known complications in the nonoperative cohort. CONCLUSIONS Severe scoliosis can develop after thoracoabdominal surgeries during infancy. Further work is needed to understand the pathogenesis of scoliosis in this population so as to implement measures for prevention and early diagnosis and to guide appropriate treatment