Steen Bach Christensen

Outcome at 10 years after treatment for adolescent idiopathic scoliosis

Study Design. A total of 215 consecutive patients treated either by bracing or operation received a questionnaire after 9.7 years. Objective. The aim of the present study was to elucidate the long-term outcome in a group of brace (BT) or surgical treated (ST) patients suffering AIS. Summary of Background Data. In adolescent idiopathic scoliosis (AIS), the options are BT or ST and, together with the disease itself, they may both be physically and psychologically demanding. Methods. The main topics of a questionnaire were demographics, back pain, activities of daily living, and SF-36. Results. A total of 181 replied. The mean age at follow-up was 26.0 years. The level of back or leg pain was relatively low, and the BT patients had more pain than their ST peers. We saw a generally high level of ADL and found no significant difference between BT and ST patients in the present study for any of the SF-36 variables. Compared with age-matched controls, the SF-36 scores were lower in the AIS patients. Brace related questions revealed a significant impact of the disease and the treatment on the patients’ lives. Conclusions. The patients had moderately reduced perceived health status and activities of daily living, and increased pain with the ST patients generally at a better level than the BT.

Early weaning might reduce the psychological strain of Boston bracing: a study of 136 patients with adolescent idiopathic scoliosis at 3.5 years after termination of brace treatment.

From 1983 to 1990 a total of 136 patients with adolescent idiopathic scoliosis were treated with the Boston brace. With the aim of examining the social and psychological impact of the brace treatment all patients received a questionnaire at an average of 3.5 years after termination of the treatment. Ninety-three percent responded to the questionnaire. The overall picture was that the patients daily activities and social contacts, not least with the opposite sex, were affected both during the bracing period and at follow-up. It is important that the indications for the treatment are assured, and weaning should be initiated at the latest 3 years after menarche.

Selection of Fusion Levels in Idiopathic Adolescent Scoliosis Treated by Harrington-DDT Instrumentation: A Short-Term Radiologic Study

Clinical records and radiographs of 106 patients treated by Harrington-dorsal transverse traction (DDT) instrumentation for idiopathic adolescent thoracolumbar scoliosis were reviewed. Our strategy was to fuse from one vertebra above the measured curve to two vertebrae below the curve, but to avoid fusions below the third lumbar vertebra. With this strategy, the lower level of fusion rarely coincided with the stable vertebra. In King type 2 and type 3 scolioses, the best results were obtained when the lower fusion level coincided with the stable vertebra. In King type 4 and in most King type 5 scolioses, the lower level of fusion was two or three vertebrae short of the stable vertebra; nevertheless, we obtained good corrections. We conclude that in King type 4 and type 5 scolioses extensive lumbar fusion can be avoided.

Radiological Outcomes in Adolescent Idiopathic Scoliosis Patients More Than 22 Years After Treatment

STUDY DESIGN Longitudinal cohort. OBJECTIVES To evaluate the long-term radiologic outcomes in adolescent idiopathic scoliosis (AIS) patients more than 22 years after treatment. SUMMARY OF BACKGROUND DATA Although treatment for AIS is prophylactic and is aimed at preventing curve progression, very few studies report long-term outcomes of treatment. METHODS AIS patients treated with Boston brace or posterior spinal fusion (PSF) with Harrington-dorso-transverse traction (DTT) instrumentation from 1983 to 1990 were requested to return to clinic. Subsequently, 36-inch standing radiographs were obtained after patient consent. Cobb angles were compared with pretreatment and immediate posttreatment radiographs. Any evidence of adjacent-level disease or local kyphosis was also noted. RESULTS One hundred fifty-nine (78%) of 219 patients were available for follow-up, 66 braced and 93 surgical. There were 85 females with an average age at surgery of 14.3 years and an average age at follow-up of 37.6 years. The mean length of follow-up was 24.5 years (range, 22-30 years). There was a statistically significant curve progression of 2.9° in the PSF group. There was a greater degree of curve progression in the braced group (5.5°), but this was not statistically significant. Proximal segment degeneration was seen in 8 (5%), 2 in the brace cohort and 6 in the PSF cohort. Distal segment degeneration was seen in 26 (16%) patients, 4 treated with brace and 22 treated with PSF. No patient developed proximal junction kyphosis. Three patients in the PSF cohort required additional surgery for distal adding-on. Four patients had a noncontiguous L5-S1 fusion, three from the PSF cohort and one from the braced cohort. CONCLUSION In this cohort with an average follow-up of 24.5 years, with 78% available for follow-up, both the braced and surgically treated patients had a very small degree of curve progression, with a small incidence of distal segment degeneration and reoperation. LEVEL OF EVIDENCE III.

CELSR2 is a candidate susceptibility gene in idiopathic scoliosis

A Swedish pedigree with an autosomal dominant inheritance of idiopathic scoliosis was initially studied by genetic linkage analysis, prioritising genomic regions for further analysis. This revealed a locus on chromosome 1 with a putative risk haplotype shared by all affected individuals. Two affected individuals were subsequently exome-sequenced, identifying a rare, non-synonymous variant in the CELSR2 gene. This variant is rs141489111, a c.G6859A change in exon 21 (NM_001408), leading to a predicted p.V2287I (NP_001399.1) change. This variant was found in all affected members of the pedigree, but showed reduced penetrance. Analysis of tagging variants in CELSR1-3 in a set of 1739 Swedish-Danish scoliosis cases and 1812 controls revealed significant association (p = 0.0001) to rs2281894, a common synonymous variant in CELSR2. This association was not replicated in case-control cohorts from Japan and the US. No association was found to variants in CELSR1 or CELSR3. Our findings suggest a rare variant in CELSR2 as causative for idiopathic scoliosis in a family with dominant segregation and further highlight common variation in CELSR2 in general susceptibility to idiopathic scoliosis in the Swedish-Danish population. Both variants are located in the highly conserved GAIN protein domain, which is necessary for the auto-proteolysis of CELSR2, suggesting its functional importance.

Radiological Outcome in Adolescent Idiopathic Scoliosis Patients 20 Years after Treatment

Introduction The purpose of this study was to evaluate the long-term radiological outcome, curve progression, and adjacent level degeneration 20 years after idiopathic scoliosis treatment with Boston brace or Harrington rods. Materials and Methods A total of 219 patients treated with Boston brace or posterior spinal fusion a.m. Harrington were invited to participate in a long-term evaluation with clinical examination and X-ray evaluation. The old medical charts and X-ray descriptions were available. Standing X-ray was examined, the Cobb angle measured and compared with the patients previous X-ray and the adjacent levels were evaluated for any signs of adjacent level disease or local kyphosis. Results A total of 159 patients (78%) participated. Of them, 66 patients were treated with Boston brace and 92 patients were treated with posterior spinal fusion a.m. Harrington. In the brace group, the Cobb angle before treatment was 37.5 degrees (35.1–40.0 degrees) and after treatment was 34.7 degrees (31.9–37.5 degrees). Cobb angle after 20 years was 40.2 degrees (36.7–43.6 degrees). In the surgical group, the Cobb angle before treatment was 54.5 degrees (50.4–58.8 degrees), 1-year postoperatively was 29.5 degrees (25.7–33.9 degrees). Cobb angle after 20 years was 32.35 degrees (27.9–39.5 degrees). A total of 26 patients had distal segment degeneration in X-ray (16.5%), 4 patients were treated with brace and 22 patients with posterior spinal fusion. A total of 8 patients had proximal segment degeneration (5%), 2 patients were treated with brace and 6 patients with posterior spinal fusion. A total of 4 patients were treated with posterior fusion of the distal adjacent segment (2.6%), 1 was treated with brace and 3 were treated with posterior spinal fusion. Conclusion The average follow-up was 24.5 years (range, 15–35 years). The brace group had a small reduction in the spinal deformity during the treatment period, and X-rays show a small progression of the deformity, with the Cobb angle increasing by 5.5 degrees within 20 years. The surgically treated patients had a large correction during surgery and there is no statistical significant progression or loss of correction over a 20-year period. Only 4 patients in the brace group have distal segment degeneration and only 1 patient was treated with a one-level spinal fusion. The surgically treated groups had a significant deformity correction during surgery and have maintained the correction after 20 years. A total of 22 patients have distal degeneration and 3 patients were treated with distal adding on surgery.