Diagnosis and treatment of congenital scoliosis

Background: Congenital scoliosis is caused by anomalies of the vertebra, such as hemivertebra or unsegmented bar, which result in asymmetric growth of the spine. The disruption of vertebra development during embryogenesis may be accompanied by other congenital multi-organ anomalies. The progression of the scoliotic curve may also hinder the development of other organs.Current Concepts: Hemivertebra excision and short spinal fusion have demonstrated favorable outcomes. However, the need for spinal growth and lung development has led to new treatment modalities. Growth-friendly surgeries, such as with a growing rod or vertical expandable rib-based distraction device, have demonstrated good results with curve correction while maintaining spinal growth. Although the outcome of conservative treatment for congenital scoliosis is questionable, casting may be effective as a “time-buying strategy” to delay the need for surgery.Discussion and Conclusion: It is essential to decide on a treatment plan considering the progression of the curve and growth of the spine and lungs through an individualized approach.

Spring distraction system to correct early onset scoliosis: 2 year follow-up results from 24 patients

Current surgical treatment options for Early Onset Scoliosis (EOS), with distraction- or growth-guidance systems, show limited growth and high complication rates. We developed the Spring Distraction System (SDS), which does not have to be periodically lengthened and which provides continuous corrective force to stimulate spinal growth. This study aimed to assess curve correction and maintenance, spinal growth, and complication rate following SDS treatment. All primary- and revision patients (conversion from failed other systems) with SDS and ≥2 years follow-up were included. Outcome measures were coronal Cobb angle, sagittal parameters, spinal length measurements and complications and re-operations. Radiographic parameters were compared pre-operatively, post-operatively and at latest follow-up. Spinal length increase was expressed as mm/year. Twenty-four skeletally immature EOS patients (18 primary and 6 revision cases) were included. There were 5 idiopathic, 7 congenital, 3 syndromic and 9 neuromuscular EOS patients. Mean age at implantation was 9.1 years (primary: 8.4; conversion: 11.2). Major curve improved from 60.3° to 35.3°, and was maintained at 40.6° at latest follow-up. Mean spring length increase during follow-up was 10.4mm/year. T1-S1 length increased 13.6mm/year and the instrumented segment length showed a mean increase of 0.8mm/segment/year. In total, 17 re-operations were performed. Ten re-operations were performed to treat 9 implant-related complications. In addition, 7 patients showed spinal growth that exceeded expected growth velocity; their springs were re-tensioned during a small re-operation. Spring distraction may be feasible as an alternative to current growing spine solutions. Curve correction and growth could be maintained satisfactory without the need for repetitive lengthening procedures. Complications and re-operations could not be prevented, which emphasizes the need for further improvement.

Mid-term results of a modified self-growing rod technique for the treatment of early-onset scoliosis

Aims To report the mid-term results of a modified self-growing rod (SGR) technique for the treatment of idiopathic and neuromuscular early-onset scoliosis (EOS). Methods We carried out a retrospective analysis of 16 consecutive patients with EOS treated with an SGR construct at a single hospital between September 2008 and December 2014. General demographics and deformity variables (i.e. major Cobb angle, T1 to T12 length, T1 to S1 length, pelvic obliquity, shoulder obliquity, and C7 plumb line) were recorded preoperatively, and postoperatively at yearly follow-up. Complications and revision procedures were also recorded. Only patients with a minimum follow-up of five years after surgery were included. Results A total of 16 patients were included. Six patients had an idiopathic EOS while ten patients had a neuromuscular or syndromic EOS (seven spinal muscular atrophy (SMA) and three with cerebral palsy or a syndrome). Their mean ages at surgery were 7.1 years (SD 2.2) and 13.3 years (SD 2.6) respectively at final follow-up. The mean preoperative Cobb angle of the major curve was 66.1° (SD 8.5°) and had improved to 25.5° (SD 9.9°) at final follow-up. The T1 to S1 length increased from 289.7 mm (SD 24.9) before surgery to 330.6 mm (SD 30.4) immediately after surgery. The mean T1 to S1 and T1 to T12 growth after surgery were 64.1 mm (SD 19.9) and 47.4 mm (SD 18.8), respectively, thus accounting for a mean T1 to S1 and T1 to T12 spinal growth after surgery of 10.5 mm/year (SD 3.7) and 7.8 mm/year (SD 3.3), respectively. A total of six patients (five idiopathic EOS, one cerebral palsy EOS) had broken rods during their growth spurt but were uneventfully revised with a fusion procedure. No other complications were noted. Conclusion Our data show that SGR is a safe and effective technique for the treatment of EOS in nonambulatory hypotonic patients with a neuromuscular condition. Significant spinal growth can be expected after surgery and is comparable to other published techniques for EOS. While satisfactory correction of the deformity can be achieved and maintained with this technique, a high rate of rod breakage was seen in patients with an idiopathic or cerebral palsy EOS. Cite this article: Bone Joint J 2020;102-B(11):1560–1566.