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Purpose: To analyze how pedicle subtraction osteotomy (PSO) treatment of severe Scheuermann thoracolumbar kyphosis (STLK) using pedicle screw instrumentation affects sagittal spinopelvic parameters., Background: The medical literature on the post-surgical effects of treatments such as Ponte osteotomy is limited, but suggests few effects on spinopelvic profiles. Currently, there is no research regarding changes in sagittal spinopelvic alignment upon PSO treatment in STLK patients., Methods: We performed a retrospective study on 11 patients with severe STLK. These patients underwent posterior-only correction surgeries with PSO and pedicle screw instrumentation between 2012 to 2017 in a single institute. Patients were measured for the following spinopelvic parameters: global kyphosis (GK), thoracic kyphosis (TK), thoracolumbar kyphosis (TL), lumbar lordosis (LL), sagittal vertical axis (SVA), pelvic incidence (PI), pelvic tile (PT), sacral slope (SS), and administered a Scoliosis Research Society-22 questionnaire (SRS-22) pre-operation, post-operation and at final follow-up., Results: GK improved from a median of 74.1° to 40.0° after surgery, achieving a correction rate of 48.8% with a median correction loss of 0.8°. TK, TL and LL all showed significant difference (P < 0.05) and SVA improved 22.7 (11.6, 30.9) mm post operation. No significant difference was found in pelvic parameters (PI, PT, SS, all P < 0.05). The absolute value of LL- PI significantly improved from a median of 26.5° pre-operation to 6.1° at the final follow-up. 72.7% in this series showed an evident trend of thoracic and lumbar apices migrating closer to ideal physiological segments after surgery. Self-reported scores of pain, self-image, and mental health from SRS-22 revealed significant improvement at final follow-up (all P < 0.05)., Conclusions: PSO treatment of severe STLK with pedicle screw instrumentation can improve spine alignment and help obtain a proper alignment of the spine and the pelvis.

Background: Early-onset scoliosis (EOS), defined by an onset age of scoliosis less than 10 years, conveys significant health risk to affected children. Identification of the molecular aetiology underlying patients with EOS could provide valuable information for both clinical management and prenatal screening., Methods: In this study, we consecutively recruited a cohort of 447 Chinese patients with operative EOS. We performed exome sequencing (ES) screening on these individuals and their available family members (totaling 670 subjects). Another cohort of 13 patients with idiopathic early-onset scoliosis (IEOS) from the USA who underwent ES was also recruited., Results: After ES data processing and variant interpretation, we detected molecular diagnostic variants in 92 out of 447 (20.6%) Chinese patients with EOS, including 8 patients with molecular confirmation of their clinical diagnosis and 84 patients with molecular diagnoses of previously unrecognised diseases underlying scoliosis. One out of 13 patients with IEOS from the US cohort was molecularly diagnosed. The age at presentation, the number of organ systems involved and the Cobb angle were the three top features predictive of a molecular diagnosis., Conclusion: ES enabled the molecular diagnosis/classification of patients with EOS. Specific clinical features/feature pairs are able to indicate the likelihood of gaining a molecular diagnosis through ES., Competing Interests: Competing interests: JRL has stock ownership in 23andMe, is a paid consultant for Regeneron Pharmaceuticals and Novartis and is a coinventor on multiple US and European patents related to molecular diagnostics for inherited neuropathies, eye diseases and bacterial genomic fingerprinting. The Department of Molecular and Human Genetics at Baylor College of Medicine derives revenue from the chromosomal microarray analysis (by comparative genomic hybridisation microarray and/or single-nucleotide polymorphism arrays), clinical exome sequencing and whole-genome sequencing offered in the Baylor Genetics Laboratory (http://bmgl.com)., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

BACKGROUND Adolescent idiopathic scoliosis (AIS) is the most common spinal deformity, but its etiology is unclear. Multiple genetic mutations have been reported to be associated with AIS. MATERIAL AND METHODS We enrolled a cohort of 113 surgically treated AIS patients with available parental subjects from the Peking Union Medical College Hospital. We performed whole-exome sequencing in 10 trio families and whole-genome sequencing in 103 singleton patients. Luciferase assay was used to detect the functional alterations of candidate ESR1 and ESR2 variants. RESULTS Using a de novo strategy, a missense variant in ESR1 (c.868A>G) was selected as a candidate gene for AIS. The main Cobb angle of this patient was 41° (T6-T10). Another potential pathogenic variant in ESR2 (c.236T>C) was identified. The main curve of the patient was 45° at T10-L3. The transactivation capacities of the mutated ESR1 and ESR2 protein were both significantly decreased (p=0.026 and 0.014, respectively). CONCLUSIONS Potential pathogenic variants in ESR1 and ESR2 were identified in 113 AIS patients, suggesting that genetic mutations in ESR1/2 were associated with the risk of AIS.

Congenital scoliosis (CS) is a form of scoliosis caused by congenital vertebral malformations. Genetic predisposition has been demonstrated in CS. We previously reported that TBX6 loss-of-function causes CS in a compound heterozygous model; however, this model can explain only 10% of CS. Many monogenic and polygenic CS genes remain to be elucidated. In this study, we analyzed exome sequencing (ES) data of 615 Chinese CS from the Deciphering Disorders Involving Scoliosis and COmorbidities (DISCO) project. Cosegregation studies for 103 familial CS identified a novel heterozygous nonsense variant, c.2649G>A (p.Trp883Ter) in FBN1. The association between FBN1 and CS was then analyzed by extracting FBN1 variants from ES data of 574 sporadic CS and 828 controls; 30 novel variants were identified and prioritized for further analyses. A mutational burden test showed that the deleterious FBN1 variants were significantly enriched in CS subjects (OR = 3.9, P = 0.03 by Fisher's exact test). One missense variant, c.2613A>C (p.Leu871Phe) was recurrent in two unrelated CS subjects, and in vitro functional experiments for the variant suggest that FBN1 may contribute to CS by upregulating the transforming growth factor beta (TGF-β) signaling. Our study expanded the phenotypic spectrum of FBN1, and provided nove insights into the genetic etiology of CS.

Background: The mid-long term outcomes of posterior spinal fusion in pediatric neurofibromatosis type 1 (NF-1) patients are rarely reported, so does the effectiveness of itsorthopeidc maintenance function. This study aims to evaluate the mid-long term surgical outcomes of posterior only instrumented spinal fusion for early-onset scoliosis (EOS) in NF-1 patients., Methods: A retrospective review was performed on a cohort of 10 NF-1 patients having EOS from 2008 to 2014 in our hospital, the age averaged at 7.8 years old when they underwent posterior only instrumented spinal fusion for their EOS. Both general clinical data and surgical specific data of the patients were collected and reviewed, and the dystrophic progression of EOS was evaluated during the follow-up., Results: The average duration of follow-up was 54 months (24 to 88 months). All patients underwent posterior only instrumented spinal fusion at 1 stage. The primary curves of EOS were thoracic in 9 cases and 1 patient had lumbar scoliosis. Preoperative major curve was significantly corrected (from 66.1 to 31.1 degrees). However, the major curve deteriorated significantly to 40.1 degrees on average at the end of the follow-up. The T1-S1 distance increased 2.8 cm on average and kept increasing at a rate of 0.6 cm/year during the follow-up., Conclusions: Posterior only fusion surgery was not a good option to treat the EOS in NF-1 patients despite the relatively short segments involvement in the disease. The maintenance of orthopedic effect after treatment was not satisfactory.

Background: The original Peking Union Medical College (PUMC) classification of adolescent idiopathic scoliosis (AIS) is one system to combine each type with corresponding surgical fusion guidance, presenting comparable interobserver reliability, and reproducibility with Lenke classification. However, during its application in previous over 10 years, we found 2 main problems of this classification, which required modification., Purpose: (1) To evaluate the interobserver and intraobserver reliability, (2) to assess the effects of the added fusion criteria of proximal thoracic (PT) curve on improving postoperative shoulder balance of the modified PUMC classification of AIS., Study Design/setting: Retrospective analysis of our AIS cohort and prospective validation of its effectiveness., Patient Sample: Fifty sets of preoperative radiographs of AIS patients were randomly chosen from our AIS database. Furthermore, 46 consecutive AIS cases with PT curve were enrolled who underwent surgeries in our center from July 2007 to July 2013, with at least 2-year follow-up., Outcome Measures: The classification results of 50 sets of preoperative radiographs by 5 surgeons. The shoulder balance was evaluated using radiographic shoulder height., Methods: Five surgeons independently evaluated and classified presurgical radiographs of 50 AIS patients based on the modified PUMC classification. Inter- and intraobserver reliabilities were calculated. Furthermore, the post-op shoulder balance was investigated in 46 consecutive cases of AIS with PT curve who were treated strictly based on the modified PUMC classification., Results: The Kappa coefficients of inter- and intraobserver reliability of the overall modified PUMC classification are 0.889 and 0.865, respectively. The Kappa coefficients of inter- and intraobserver reliability for the type II are 0.791 and 0.746, respectively. In addition, the shoulder balance rate of the 46 AIS patients with PT curve at the final follow-up was 95.7%., Conclusions: Modified PUMC classification presents incremental improvement compared to our original published version, with high interobserver and intraobserver reliability and better performance of postoperative shoulder balance. Furthermore, the modified PUMC classification provides corresponding surgical fusion guidance for each subtype. Multicenter prospective studies with larger samples are still needed to further improve this system., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To characterize clinically measurable endophenotypes, implicating the TBX6 compound inheritance model., Methods: Patients with congenital scoliosis (CS) from China(N = 345, cohort 1), Japan (N = 142, cohort 2), and the United States (N = 10, cohort 3) were studied. Clinically measurable endophenotypes were compared according to the TBX6 genotypes. A mouse model for Tbx6 compound inheritance (N = 52) was investigated by micro computed tomography (micro-CT). A clinical diagnostic algorithm (TACScore) was developed to assist in clinical recognition of TBX6-associated CS (TACS)., Results: In cohort 1, TACS patients (N = 33) were significantly younger at onset than the remaining CS patients (P = 0.02), presented with one or more hemivertebrae/butterfly vertebrae (P = 4.9 × 10 ‒8 ), and exhibited vertebral malformations involving the lower part of the spine (T8-S5, P = 4.4 × 10 ‒3 ); observations were confirmed in two replication cohorts. Simple rib anomalies were prevalent in TACS patients (P = 3.1 × 10 ‒7 ), while intraspinal anomalies were uncommon (P = 7.0 × 10 ‒7 ). A clinically usable TACScore was developed with an area under the curve (AUC) of 0.9 (P = 1.6 × 10 ‒15 ). A Tbx6 -/mh (mild-hypomorphic) mouse model supported that a gene dosage effect underlies the TACS phenotype., Conclusion: TACS is a clinically distinguishable entity with consistent clinically measurable endophenotypes. The type and distribution of vertebral column abnormalities in TBX6/Tbx6 compound inheritance implicate subtle perturbations in gene dosage as a cause of spine developmental birth defects responsible for about 10% of CS.

Study Design: A case-control association study was performed to investigate the relationship between ladybird homeobox (LBX1) and adolescent idiopathic scoliosis (AIS) in northern Chinese Han population., Objective: To explore the prevalence and functional importance of LBX1 polymorphisms in patients with AIS within the northern Chinese Han population., Summary of Background Data: AIS is the most common subtype of idiopathic scoliosis. Genetic factors such as LBX1 polymorphisms have been recently proved to be associated with AIS in some populations. In this study we explored the prevalence and functional importance of the polymorphisms around LBX1 in patients with AIS within the northern Chinese Han population., Methods: Five tag single nucleotide polymorphisms (SNPs) around or in LBX1 were genotyped in 180 patients with AIS and 182 controls. And the luciferase assay was performed to explore the functional importance of the most significant SNPs., Results: We replicated that rs11190870, previously reported as the most significantly associated SNP, was enriched in our AIS cohort. In addition, we found that the T allele of rs1322331 was associated with a novel risk allele (odds ratio = 3.349, 95% confidence interval 1.742-6.436). In the following luciferase assay, the TT-type promoter showed significantly reduced transcription activity in vitro., Conclusion: Two SNPs around LBX1, rs11190870 and rs1322331 are associated with AIS in northern Chinese Han population. The T allele of rs1322331 is a novel risk allele. We hypothesize that rs1322331 might increase patients' susceptibility to AIS by reducing LBX1-AS1 transcription and thus upregulating the function of LBX1., Level of Evidence: 3.

Introduction: Posterior selective thoracolumbar or lumbar (TL/L) fusion with pedicle screw constructs for adolescent idiopathic scoliosis (AIS) has been studied in a few researches. However, few studies have discussed the indication for selective TL/L fusion and the behaviors of its adjacent disc angle. The present study aims to discuss the indication for posterior selective TL/L fusion and the behavior of the adjacent disc angle., Methods: 45 consecutive cases of AIS undergoing posterior selective TL/L fusion were retrospectively evaluated, with an average follow-up of 36 months. Radiographs were reviewed to determine the coronal curve magnitude and the sagittal alignment preoperatively, postoperatively and at final follow-up. Thoracic curves in groups A had a correction loss of more than 5°, while thoracic curves in group B had a correction loss of not more than 5°., Results: The coronal curve magnitude of the TL/L curve averaged 44° preoperatively and it was corrected to 6° immediately with a correction rate of 84.8 %. At final follow-up it was 9° with a correction loss of 3°. The minor thoracic curve was 26° preoperatively, and the convex side bending curve magnitude averaged 8° with a flexibility of 72.7 %. It was corrected to 13° immediately with a spontaneous correction of 48.5 %. At final follow-up it was 14° with a correction loss of 1°. UIVA decreased from 4° to 2° after surgery, and it was 2° at final follow-up. LIVA decreased from 7° to 4° after surgery, and it was 5° at final follow-up. Maximal correction of TL/L curves in group A is significantly less than that in group B. 1 patient received revision surgery to fuse the progressive thoracic curve., Conclusion: Posterior selective TL/L fusion with pedicle screw constructs allows for spontaneous thoracic correction and maintains coronal and sagittal balance during the follow-up. Maximal correction instead of undercorrection was recommended for moderate Lenke 5C curves. Disc wedging could be improved after surgery and well maintained during the follow-up., Competing Interests: The authors declare that they have no conflict of interest. Ethical approval All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study formal consent is not required.

Study Design: Retrospective study., Objective: To study the behavior of the unfused thoracic curve in Lenke type 5C during the follow-up and to identify risk factors for its correction loss., Summary of Background Data: Few studies have focused on the spontaneous behaviors of the unfused thoracic curve after selective thoracolumbar or lumbar fusion during the follow-up and the risk factors for spontaneous correction loss., Methods: We retrospectively reviewed 45 patients (41 females and 4 males) with AIS who underwent selective TL/L fusion from 2006 to 2012 in a single institution. The follow-up averaged 36 months (range, 24-105 months). Patients were divided into two groups. Thoracic curves in group A improved or maintained their curve magnitude after spontaneous correction, with a negative or no correction loss during the follow-up. Thoracic curves in group B deteriorated after spontaneous correction with a positive correction loss. Univariate analysis and multivariate analysis were built to identify the risk factors for correction loss of the unfused thoracic curves., Results: The minor thoracic curve was 26° preoperatively. It was corrected to 13° immediately with a spontaneous correction of 48.5%. At final follow-up it was 14° with a correction loss of 1°. Thoracic curves did not deteriorate after spontaneous correction in 23 cases in group A, while 22 cases were identified with thoracic curve progressing in group B. In multivariate analysis, two risk factors were independently associated with thoracic correction loss: higher flexibility and better immediate spontaneous correction rate of thoracic curve., Conclusion: Posterior selective TL/L fusion with pedicle screw constructs is an effective treatment for Lenke 5C AIS patients. Nonstructural thoracic curves with higher flexibility or better immediate correction are more likely to progress during the follow-up and close attentions must be paid to these patients in case of decompensation., Level of Evidence: 4.

Purpose: To explain the intra-operative transcranial motor evoked potential (MEP) monitoring can work well in patients with neural axis abnormality (NAA)., Methods: One hundred eighteen consecutive NAA and 334 adolescent idiopathic scoliosis (AIS) patients who underwent spinal deformity surgery between June 2010 and April 2013 in our spine center were included. The MEP data including the success rate of obtaining a baseline, amplitude, sensitivity and specificity were analyzed., Results: High-efficiency MEPs baseline could be obtained in 117/118 NAA (74 congenital scoliosis, 32 neuromuscular scoliosis, 8 adult scoliosis, 3 congenital kyphoscoliosis and 1 neurofibromatosis scoliosis) and 334 AIS cases. They had an approximate level in success rate of MEPs baseline (99.2 vs. 99.7 %) and MEPs amplitude (317 μV, n = 118; vs. 312 μV, n = 334). The sensitivity and specificity for MEP were 100 and 98.2 % in patients with NAA. And the MEPs amplitude value fitted positive-skewed distribution in both of NAA and AIS., Conclusions: Intraoperative MEP monitoring can be used accurately and satisfactorily in NAA patients and show no difference compared with AIS.

Study Design: Retrospective study., Objectives: The aim of this study was to analyze whether there were differences in mental health scores of Scoliosis Research Society-22 (SRS-22) questionnaire and Short Form-36 (SF-36) questionnaire between adolescent idiopathic scoliosis (AIS) patients with/without preoperative brace treatment., Summary of Background Data: Mental health is an important domain of SRS-22 and SF-36 questionnaire. There are few studies focused on the effect of preoperative brace treatment on mental health in surgically treated AIS patients., Materials and Methods: The SRS-22 and SF-36 questionnaires of 211 AIS patients who underwent posterior correction surgery were retrospectively reviewed. The average age of the patients was 14.4 years (range, 11-18 y), and the patients were classified into 2 groups: with preoperative brace treatment (group BS-32 cases, 5 male and 27 female) and without preoperative brace treatment (group S-179 cases, 23 male and 156 female). The radiographic data and mental health scores of SRS-22 and SF-36 questionnaires were compared between the 2 groups., Results: There was no significant difference in terms of height, rib hump, the mean preoperative coronal Cobb angle or apical vertebral translation of the major curve, sagittal thoracic kyphosis, or coronal trunk balance between group BS and group S (all P>0.05). The average and total mental health scores of SRS-22 between group BS and group S were 3.6±0.7 and 3.7±0.5, and 18.1±3.5 and 18.3±2.6, respectively. The total mental health scores of SF-36 between group BS and group S were 71.1±8.7 and 68.7±11.5, respectively. There was no significant difference with regard to the mental health scores of SRS-22 and SF-36 questionnaires between group BS and group S (all P>0.05)., Conclusions: Preoperative brace treatment did not have obvious influence on the mental health scores of SRS-22 and SF-36 questionnaires in surgically treated AIS patients.

Background: The treatment of congenital scoliosis associated with split cord malformation (SCM) raises the issue of how to best manage such patients to avoid neurological deficit while achieving a satisfactory correction., Methods: This prospective clinical study was performed at our center from March 2000 through June 2013. We enrolled a total of 214 patients (61 male and 153 female) with congenital scoliosis associated with SCM who were undergoing spinal correction surgery. The mean age at surgery was 14.1 years. The inclusion criteria were congenital scoliosis with confirmed SCM; status as neurologically intact or stable over the preceding 2 years; and no neurological deterioration as evidenced on traction, side-bending, or fulcrum-bending radiographs. Patients with unstable neurological status or for whom vertebral column resection surgery was planned were excluded. All patients underwent scoliosis surgery without prophylactic detethering., Results: On the basis of the Pang classification, 73 patients were in the type-I SCM group, and 141 were in the type-II SCM group. The groups did not differ significantly with respect to preoperative characteristics, operative time, blood loss, or number of levels fused. The mean follow-up was 37 months (range, 24 to 108 months). The rate of scoliosis correction was lower in the type-I group than in the type-II group (p < 0.05). In the type-I group, the correction rate was 48.9% at 1 week postoperatively and 42.2% at the last follow-up. In the type-II group, the correction rate was 54.7% at 1 week postoperatively and 47.9% at the last follow-up. Eleven (5.1%) of the patients experienced transient neurological complications, with no significant difference between the groups (p = 0.415). No patient experienced permanent neurological deficit during surgery or follow-up., Conclusions: Patients with congenital scoliosis associated with SCM, regardless of type, can safely and effectively undergo spinal deformity correction and achieve spinal balance without neurological intervention. For such patients with intact or stable neurological status, prophylactic detethering prior to scoliosis surgery may not be necessary., Level of Evidence: Therapeutic Level II. See Instructions for Authors for a complete description of levels of evidence., (Copyright © 2016 by The Journal of Bone and Joint Surgery, Incorporated.)

Purpose: To determine: (1) the relationship of thoracic cage parameters and preoperative pulmonary function tests (PFTs) in congenital scoliosis (CS) patients. (2) if patients with rib deformity have greater impairment of PFTs than those without rib deformity., Methods: A total of 218 patients with CS and pulmonary dysfunction (FVC < 80 %) were conducted in one spine center between Jan 2009 and Mar 2013. The demographic distribution, medical records, PFTs and radiographs of all patients were collected. The association of PFTs and thoracic cage deformities was analyzed., Results: In total, 143 patients (65.6 %) had a clinically relevant impairment of pulmonary function. They had smaller BMI, larger thoracic transverse and anteroposterior diameter, more thorax height, scoliotic angle and number of involved vertebra than no clinically impairment. PFTs were negative correlation significantly with scoliotic angle, number of involved vertebra and thoracic sagittal diameter, while thorax height is significant positive. The T1-12 height was significant positive correlation with age, stature, weight, thoracic transverse and sagittal longitudinal diameter, while negative correlation with scoliotic curve, number of involved vertebra, as well as kyphotic angle. PFTs do not correlate with T1-12 height, but significantly associated with the rib anomalies. The FVC and FEV1 were significantly lower in patients with rib anomalies than without rib anomalies. There was no association between the pulmonary function and the location of rib deformities., Conclusions: PFTs correlate significantly with scoliotic angle, number of involved vertebra, thoracic sagittal diameter, and thorax height. PFTs were significantly lower in patients with rib anomalies, particular to the patients with fused rib.

Background: The X-ray films of the patients with Marfan syndrome scoliosis (MSS) look like those with adolescent idiopathic scoliosis (AIS). In literature, there are many reports on the correction results of AIS, while there are a few studies focused on the difference of the correction results between MSS and AIS. This study aims to analyze whether there are differences of posterior correction surgery in MSS and AIS., Methods: All the patients included underwent posterior correction surgery. The radiographic data, operation duration, estimated blood loss, transfusion, fusion levels, and correction rate were retrospectively reviewed and analyzed between the two groups., Results: Group MSS included 42 patients, 11 male and 31 female, with an average age of 15.2 years old. Group AIS included 168 patients (ratio, 1:4), 34 male and 134 female, with an average age of 14.5 years old. Twenty-three patients in group MSS and 94 patients in group AIS were followed up regularly, with an average time of 18.4 and 18.5 months, respectively. The mean coronal Cobb angle of the major curve before operation and at final follow-up, the correction rate, fusion level, operation duration, estimated blood loss during operation, and transfusion between the two groups were 60.4 and 57.5°, 14.6 and 15.2°, 76.4 and 74.1%, 11.5 and 11.0 vertebrae, 4.6 and 4.0 h, 845 and 698 ml, and 1151 and 894 ml, respectively. The age, gender ratio, curve type, and coronal Cobb angle of the major curve were all matched (all P > 0.05). Group MSS had a longer operation duration and more estimated blood loss compared with those of group AIS (both P < 0.05), while there was no significant difference in terms of fusion level, transfusion, coronal Cobb angle of the major curve at final follow-up, and the correction rate (all P > 0.05)., Conclusions: When performing posterior correction for scoliosis, the surgeons should be aware that the patients with Marfan syndrome scoliosis had more estimated blood loss and longer operation duration than AIS patients, while the correction rate was similar.

Purpose: To analyze risk factors for an increase in proximal junctional angle (PJA) after posterior selective thoracolumbar/lumbar (TL/L) curve fusion in patients with adolescent idiopathic scoliosis (AIS)., Methods: AIS patients that underwent selective posterior TL/L curve fusion with a minimum of 2-year follow-up were identified. Demographic and radiographic data were collected before surgery, at first erect after surgery and at final follow-up. Multiple linear regression analysis was performed to determine the relation of PJA changes during follow-up and eight potential risk factors, including locations of upper instrumented vertebra (UIV), locations of lower instrumented vertebra (LIV), length of fusion segments, types of pedicle screw alignment, lumbar lordosis (LL) at first erect after surgery, LL changes before and after surgery, sagittal vertical axis (SVA) at first erect after surgery and SVA changes before and after surgery., Results: A total of 41 patients were included in this study. There were 37 female and 4 male with a mean age of 14.7 years at surgery. PJA was increased from 5.5° immediately after surgery to 10.8° at the last follow-up (P < 0.0001). Regression analysis showed that locations of LIV, LL changes before and after surgery and SVA changes before and after surgery were risk factors for increased PJA. Pearson correlation test showed that postoperative LIV inclination was significantly correlated with PJA changes., Conclusions: Location of LIV above or equal to L3, higher postoperative LL and deteriorative negative SVA with surgery were potential risk factors for increased PJA during follow-up. Postoperative LIV inclination more than 5ºmight be also an indicator for an increase in PJA.

Study Design: A retrospective radiographical analysis., Objective: To assess the effect of unfused segments in the reconstitution of coronal balance after posterior selective thoracolumbar/lumbar (TL/L) curve fusion for adolescent idiopathic scoliosis., Summary of Background Data: Current literature suggests that compensatory changes occur after selective TL/L curve fusion surgery. Hitherto, the roles of the unfused levels in coronal balance reconstitution have not been fully understood., Methods: Radiographical data of patients with idiopathic scoliosis who underwent selective posterior TL/L curve fusion Peking Union Medical College Hospital before October 2011 with a minimum of 2 years of follow-up period were retrospectively analyzed. Changes of coronal trunk shift during the follow-up period were studied and multiple linear regression analysis was performed to determine its correlation with changes of upper and lower curve of unfused thoracic segments, instrumented segments angle, distal unfused segments angle, and coronal sacral inclination., Results: A total of 43 patients were included in this study. Preoperative and first erect radiographs demonstrated trunk shifts of 21.1 mm and 18.7 mm respectively, showing no significant differences (P = 0.205). At the last follow-up, it compensated to 9 mm, which showed significant differences (P < 0.01). Regression analysis of all patients showed that coronal trunk shift changes only correlated with distal unfused segment angle changes. Subgroup analysis based on the magnitude of preoperative thoracic curve found that only distal unfused segments had an impact on coronal balance reconstitution. However, subgroup analysis based on the flexibility of preoperative thoracic curve showed that both proximal unfused thoracic segments and distal unfused lumbar segments contributed to coronal balance compensation in patients with a thoracic curve flexibility rate of more than 70%., Conclusion: The reconstitution of coronal balance was mainly compensated by distal unfused segments after selective posterior fusion of TL/L idiopathic scoliosis. The effect of unfused thoracic segments in coronal balance reconstitution mainly depended on its flexibility.

Study Design: Retrospective review., Objective: To investigate the clinical manifestations and radiological characteristics of Klippel-Feil syndrome (KFS) in congenital scoliosis (CS). To identify the incidence of spinal or extraspinal abnormities in KFS., Summary of Background Data: KFS is an uncommon condition, characterized as improper segmentation of one or more cervical spine segments. "Scoliosis" is potentially the most common manifestation associated with KFS. However, the clinical manifestations and radiological characteristics of KFS in patients with CS are less reported., Methods: A total of 516 patients with CS from January 2009 to March 2013 were identified from a single institution. The demographic distribution and clinical and radiographical data were collected. Cervical regions were also designated as high (O-C2), mid (C2-C4), and low (C4-T1). The incidence of intra- and extraspinal abnormalities associated with KFS was investigated., Results: In total, 28 patients (5.42%) had been identified with KFS, which included 8 males and 20 females. The mean coronal cervical alignment was 20.6° and sagittal alignment was 29.9°. KFS type I was found in 14 patients (50.0%), type II in 6 (21.4%), and type III in 8 (28.6%). Congenitally fused cervical segment is more common in the mid and lower cervical spine region (85.7%, 24/28). In the 28 patients with KFS, 11 have intraspinal anomalies (32.1%) and 6 have extraskeletal anomalies (21.4%). Thirteen patients (46.4%) exhibited rib anomalies. The incidence of rib anomalies showed no significant difference in CS patients with KFS and without KFS (P>0.05). A half of the patients with KFS have hemivertebrae; however, the incidence of hemivertebrae showed no significant difference in CS patients with KFS and without KFS (P>0.05)., Conclusion: The incidence of KFS was 5.42% in patients with CS. Congenitally fused cervical patterns are more common in the mid and lower cervical spine region. The incidence of rib anomalies, intraspinal abnormities, and hemivertebra was not increased in CS patients with KFS., Level of Evidence: 4.

Study Design: A genetic association study of single nucleotide polymorphisms (SNPs) for the LMX1A gene with congenital scoliosis (CS) in the Chinese Han population., Objective: To determine whether LMX1A genetic polymorphisms are associated with susceptibility to CS., Summary of Background Data: CS is a lateral curvature of the spine due to congenital vertebral defects, whose exact genetic cause has not been well established. The LMX1A gene was suggested as a potential human candidate gene for CS. However, no genetic study of LMX1A in CS has ever been reported., Methods: We genotyped 13 SNPs of the LMX1A gene in 154 patients with CS and 144 controls with matched sex and age. After conducting the Hardy-Weinberg equilibrium test, the data of 13 SNPs were analyzed by the allelic and genotypic association with logistic regression analysis. Furthermore, the genotype-phenotype association and haplotype association analysis were also performed., Results: The 13 SNPs of the LMX1A gene met Hardy-Weinberg equilibrium in the controls, which was not in the cases. None of the allelic and genotypic frequencies of these SNPs showed significant difference between case and control groups (P > 0.05). However, the genotypic frequencies of rs1354510 and rs16841013 in the LMX1A gene were associated with CS predisposition in the unconditional logistic regression analysis (P = 0.02 and 0.018, respectively). Genotypic frequencies of 3 SNPs at rs6671290, rs1354510, and rs16841013 were found to exhibit significant differences between patients with CS with failure of formation and the healthy controls (P = 0.019, 0.007, and 0.006, respectively). Besides, in the model analysis by using unconditional logistic regression analysis, the optimized model for the 3 genotypic positive SNPs with failure of formation were rs6671290 (codominant; P = 0.025, Akaike information value = 316.6, Bayesian information criterion = 333.9), rs1354510 (overdominant; P = 0.0017, Akaike information value = 312.1, Bayesian information criterion = 325.9), and rsl6841013 (overdominant; P = 0.0016, Akaike information value = 311.1, Bayesian information criterion = 325), respectively. However, the haplotype distributions in the case group were not significantly different from those of the control group in the 3 haplotype blocks., Conclusion: To our knowledge, this is the first study to identify that the SNPs of the LMX1A gene might be associated with the susceptibility to CS and different clinical phenotypes of CS in the Chinese Han population., Level of Evidence: 4.

Purpose: To determine fusion necessity to one level below lower-end vertebra (LEV+1) in selective posterior fusion of moderate thoracolumbar/lumbar (TL/L) idiopathic scoliosis., Methods: A total of 37 patients with moderate TL/L idiopathic scoliosis (Cobb angle of TL/L curve between 30° and 60°) were identified and three patients with TL/L curve Cobb angle more than 60° were excluded. And the follow-up period was at least 2 years. Lowest instrumented vertebra (LIV) was one level proximal to LEV in three patients, LEV in 22 patients and LEV+1 in 12 patients. The three patients with TL/L Cobb angle more than 60° were all fused to LEV+1. Clinical and various radiographic measurements were collected before surgery, post-surgery and during last follow-up, and analytical comparisons were made between LIV = LEV patients and LIV = LEV+1 patients., Results: No significant difference was observed regarding clinical and radiographic parameters between LEV group and LEV+1 group preoperatively except LIV disc angle and LIV translation. The correction rate of unfused thoracic curve and TL/L curve was 52.7 and 79.9 % in LEV group and 52.5 and 83.7 % in LEV+1 group at the last follow-up, indicating no significant difference (P = 0.976 and P = 0.415, respectively). Coronal balance and sagittal alignments were also comparable between the two groups. LIV translation was slightly less in LEV+1 group (P = 0.028) at the last follow-up on the basis that LEV+1 was less translated than LEV preoperatively., Conclusions: Our analysis almost showed no benefit for fusing to LEV+1 in moderate TL/L idiopathic scoliosis patients undergoing posterior selective fusion with pedicle screws. For patients with TL/L Cobb angle more than 60°, the distal fusion level probably needs to be LEV+1.

Study Design: Retrospective study., Objective: To evaluate the clinical outcomes of the hybrid technique of 1-stage posterior osteotomy with short segmental fusion and dual growing rod (GR) technique for severe rigid congenital scoliosis., Summary of Background Data: As some types of congenital scoliosis consist of long curve including sharp deformities producing enormous asymmetric growth at apex, they cannot be well corrected with osteotomy and short segmental fusion. GR technique may be the best option. However, enormous asymmetric growth potential due to the sharp deformities at the apex will increase risk of complications, especially implant failures., Methods: Seven patients (2 males, 5 females) undergoing this hybrid technique for severe rigid congenital scoliosis were retrospectively reviewed. The patients' charts were reviewed. The analysis included age at initial surgery and the latest follow-up, number and frequency of lengthening, and complications. Radiographical evaluation included measured changes in scoliosis Cobb angle, thoracic kyphosis, lumbar lordosis, trunk shift, length of T1-S1, and instrumentation., Results: The mean follow-up was 53.3 (30-77) months. The mean age at the initial surgery is 5.9 (2-10) years. The averaged lengthenings were of 5.3 per patient. The mean scoliosis improved from 81.4° to 40.1° after initial surgery and was 41.0° at the latest follow-up. The average T1-S1 length was of 1.23 cm per year. The space available for lung ratio increased from 0.86 to 0.96., Conclusion: Osteotomy with short fusion could help to improve the correction of the GR and eliminate the large asymmetric growth potential around the apex, with little influence to the length of the spine. Dual GR technique could maintain correction achieved at initial surgery while allowing spinal growth to continue. This hybrid technique may be an option for young patients who present sharp deformities with large asymmetric growth potential in a long congenital spinal deformity., Level of Evidence: 4.

Study Design: Retrospective study., Objectives: To analyze the influence of preoperative brace treatment on the pulmonary function tests (PFTs) in females with adolescent idiopathic scoliosis (AIS)., Summary of Background: Brace is a selection of conservative treatment for some patients with AIS. Some authors reported that wearing a brace could impair the PFTs, although there are no reports that analyze the related factors., Methods: Preoperative PFTs were evaluated in 270 female patients with AIS. The patients were classified into 2 groups: group A, with preoperative brace treatment, 70 cases; and group B, without preoperative brace treatment, 200 cases. The differences of the PFTs between the 2 groups were compared., Results: The predicted values of forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) in group A and B were 3.26 L and 3.20 L, 2.78 L and 2.73 L, respectively, (both P>0.05). The actual values of FVC and FEV1 in group A and B were 2.61 L and 2.72 L, 2.37 L and 2.48 L, respectively, (both P>0.05). The percentage of actual value and predicted value of FVC(FVC%) and FEV1(FEV1%) in group A and B were 80.6% and 85.2%, 85.7% and 91.1%, respectively, and group A had significantly lower values when compared with those of group B (both P<0.05). This difference was significant in patients with a primary thoracic curve (166 cases) (P<0.05), whereas not in patients with a primary thoracolumbar/lumbar curve (104 cases) (P>0.05). In group A, there were significant correlations between the sagittal Cobb angle of the thoracic curve and the actual values of FVC and FEV1 and FVC% and FEV1% (both P<0.05)., Conclusions: Preoperative brace treatment can reduce the FVC% and FEV1% in thoracic AIS. The sagittal Cobb angle of the thoracic curve may be the influential factors.

Study Design: Retrospective study., Objective: To evaluate clinical outcomes of dual growing rod (GR) technique in treating children with congenital scoliosis (CS)., Summary of Background Data: Published reports on the dual GR technique results of early-onset scoliosis demonstrate it to be safe and effective. However, the use of GR in congenital spinal deformities is controversial, and there have been no reports on the results and complications of dual GR technique for CS with large series of patients., Methods: During 2004 to 2009, a total of 30 patients with CS underwent dual GR procedures. Of the 159 total procedures conducted within the treatment period, 125 were lengthenings with an average of 4.2 lengthenings per patient. Five patients with severe rigid deformity or kyphosis had an osteotomy at apex vertebra with short segmental fusion. The analysis included age at initial surgery and final fusion (if applicable), number and frequency of lengthenings, and complications. Radiographical evaluation was conducted., Results: The mean scoliosis improved from 72.3° to 34.9° after initial surgery and was 35.2° at the last follow-up or after final fusion. T1-S1 length increased from 25.42 to 29.03 cm after initial surgery and to 33.32 cm at last follow-up or after final fusion with an average T1-S1 length increase of 1.49 cm per year. The space available for lung ratio in patients with thoracic curves improved from 0.84 to 0.96 at the latest follow-up. Three patients reached final fusion. Complications occurred in 7 of the 30 patients, and they had a total of 13 complications., Conclusion: The dual GR technique is safe and effective in the treatment of selected cases of long, complex CS. It maintains correction achieved at initial surgery while allowing spinal growth to continue. And it has an acceptable rate of complications. The osteotomy at the apex vertebra with short segmental fusion of the severe rigid scoliosis or the patients with kyphosis could help to improve the correction and decrease the implant failures, with little influence on the length of the spine.

Study Design: An analysis of computed tomography (CT) images of patients with adolescent thoracic idiopathic scoliosis for posterior pedicle screw placement., Objective: To evaluate the relative position of the aorta to the spine by the axial CT scans in patients with right thoracic idiopathic scoliosis, and to discuss the safe trajectory and screw length for posterior pedicle screw placement., Summary of Background Data: Posterior pedicle screw instrumentation and fusion are widely used in the correction of scoliosis. Pedicle screw placement in the thoracic spine implies risk of injuring the aorta. Literatures on the relative position of the aorta to the spine are mostly focused on the anterior surgery. However, few are reported about the posterior surgery., Methods: A total of 47 patients with adolescent right thoracic idiopathic scoliosis who underwent CT scan of total spine before surgery were included in this study. Transverse plane including bilateral pedicles, lamina, and transverse process was selected for measurement from T4 to T12. We defined a new coordinate system, and 5 parameters were measured in each vertebral body from T4 to T12 of the patients. The point where the left pedicle axial line and the base of the left transverse process intersected was defined as the origin of the coordinate system. A line connecting bilateral intersection was defined as x-axis. y-axis perpendicular to the x-axis is drawn ventrally from the origin. The left pedicle-aorta angle (α), the left aorta angle (β), the left pedicle-aorta distance (PAD), the aorta-x-axis distance (AXD), and the vertebral rotation angle (γ) were measured., Results: The values of angles α and β had a tendency of first increasing and then decreasing, and increasing again from T4 to T12. The lowest value of angle α occurred at T10 (7.45±6.10 degrees), followed by T4 (8.89±6.49 degrees), T11 (9.13±7.59 degrees), and T9 (9.74±6.11 degrees). PAD and AXD values had a tendency of decreasing first and then increasing from T4 to T12. The lowest values of PAD and AXD occurred at T6; 25.94±5.33 and 23.64±6.53 mm, respectively. From the cephalad to the caudal spine, angle γ increased first and then decreased. The highest value occurred at T7 (2.32±6.83 degrees), which rotated to the right side. The second largest value occurred at T8 (2.23±7.76 degrees). Statistical analysis indicated that the apical vertebral translation and vertebral rotation angle were significantly positively correlated to angles α and β (P<0.05)., Conclusions: The highest risk of injuring the aorta when placing pedicle screw during posterior scoliosis surgery was at T10, followed by T4, T11, and T9 in right thoracic idiopathic scoliosis. Taking a spine CT scan and evaluating the relative position of the aorta to the thoracic spine before surgery are significant in precise and safe pedicle screw placement.

Study Design: A multicenter cross-cultural study to re-evaluate the reliability and validity of the simplified Chinese version of Scoliosis Research Society-22 (SRS-22) Patient Questionnaire., Objective: To assess the concurrent validity and reliability of this instrument in mainland China., Summary of Background Data: The SRS-22 Questionnaire was developed to assess the health-related quality of life for patients with scoliosis in the United States. More recently, the reliability and validity analyses of traditional and simplified Chinese versions of this questionnaire were performed, respectively. However, with respect to the diversity of population and large proportion of rural patients in China, a multicenter study might be necessary to re-evaluate its adaptation., Methods: The original English version of the SRS-22 Questionnaire was translated into simplified Chinese. Considering the different health care systems in mainland China, the question 15 was modified; the respondent might be the parent if the patient was younger than 18 years. This multicenter study was performed by 13 scoliosis surgeons involving seven spine centers. The SRS-22 Questionnaire and a previously validated 36-Item Short-Form Health Survey Questionnaire were given to 391 patients with idiopathic scoliosis or congenital scoliosis without neurologic deficits. Of those, 136 patients were randomly selected for a second identical SRS-22 Questionnaire filled 1 week later and mailed back with a stamped return envelope., Results: A total of 333 patients fully completed both SRS-22 and 36-Item Short-Form Health Survey Questionnaires. Good consistency was observed for all the five domains of the SRS-22. Poorer internal consistency was found in questions 15 and 18. If question 15 was excluded, the α coefficient would increase to 0.68. The pain domain of SRS-22 had a high ceiling effect being 67.5%. The test-retest reproducibility was observed "good" in the pain domain and "excellent" for the remaining domains of SRS-22 Questionnaire. Good (10 domains) and moderate (23 domains) correlations were demonstrated while seven domains had poor correlation., Conclusion: The simplified Chinese version of the SRS-22 Questionnaire was re-evaluated by a multicenter study to respect the diversity of population and cultural differences in mainland China, which showed good internal consistency and satisfactory test-retest reproducibility. It might be useful for clinical evaluation of Chinese adolescents and young adults with scoliosis treated with bracing or surgery, although it also encountered previously reported difficulties with some of the items related to different cultures and health care systems.

Study Design: Retrospective clinical study., Objective: To evaluate the predictive factors of postoperative pulmonary complications in unselected scoliotic patients with moderate or severe pulmonary dysfunction., Background: Many scoliotic patients with moderate or severe pulmonary dysfunction develop postoperative pulmonary complications during hospitalization. However, little is reported about the postoperative pulmonary complication events in this population at the first visit to the hospital., Methods: A total of 88 scoliotic patients with moderate or severe pulmonary dysfunction were identified. The demographic distribution, medical history, and clinical data were collected to investigate the predictive factors of immediate postoperative pulmonary complications by logistic regression., Results: A total of 16 (18.2%) patients were documented with postoperative pulmonary complications including increased requirement of postoperative ventilatory support (6.3%), atelectasis (31.3%), pneumothorax (6.3%), pneumonia (12.5%), and hypoxemia (43.8%). There were no statistical differences in age, sex, body mass index, abnormal echocardiogram, and prior symptoms between the 2 groups (P>0.05). Of the patients with postoperative pulmonary complications, a slightly higher proportion of patients underwent anterior approach, 2 (12.5%) versus 2 (2.8%) (P=0.091). The Cobb angle before surgery was 102.1+/-25.5 degree with postoperative pulmonary complications versus 87.7+/-23.8 degree without postoperative pulmonary complications (P=0.034). Forced vital capacity was 47.0+/-10.5% versus 52.4+/-9.0% (P=0.037). Number of levels fused was 13.7+/-2.3 versus 13.1+/-2.2 (P=0.319). Operation time was 376.8+/-125.6 minutes versus 350.4+/-97.8 minutes (P=0.357). Anesthesia time was 415.3+/-126.8 minutes versus 388.0+/-97.5 minutes (P=0.341). Estimated blood loss was 1050.0+/-769.4 mL versus 777.8+/-460.9 mL (P=0.065), respectively. Logistic regression analysis showed that thoracoplasty correlated with postoperative pulmonary complications (odds ratio 3.81, 95% confidence interval 1.11-13.16)., Conclusions: Thoracoplasty is the only independent predictor of postoperative pulmonary complications. However, the abnormal echocardiogram, prior symptoms, forced vital capacity, and other clinical characteristics play a less relevant role in the prediction of postoperative pulmonary complications.

Study Design: A case-control association study was conducted to investigate the genetic etiology for congenital scoliosis (CS) in a Chinese Han population., Objective: To identify whether TBX6 polymorphisms are associated with susceptibility to CS in a Chinese Han population., Summary of Background Data: CS is a 3-dimensional deformity of the spine, resulting from defection of normal vertebral development. Although there are many types of defects observed in CS, all result from abnormal formation and segmentation of the vertebral precursors, called somites. Developmental studies in animal models have identified many genes regulating somite formation and segmentation. T-box factor, TBX6, is a prerequisite for somite segmentation in vertebrates. In mouse TBX6 knockouts, the phenotypes are similar with that of some human birth defects, such as CS, raises the possibility that TBX6 gene may be a potential susceptibility gene for CS, so we investigated the relations between TBX6 polymorphisms and CS., Methods: Two known single-nucleotide polymorphisms (SNPs) of TBX6 gene were genotyped among 254 Chinese Han subjects (127 CS patients and 127 controls with matched sex and age) by GenomeLab SNPstream genotyping system. The 2 markers (the only tagging SNP and a functional SNP) with minor allele frequency above 5% were analyzed by the allelic and genotypic association analysis, the genotype-phenotype (CS patients were divided into type I 31 cases [failure of formation], type II 46 cases [a failure of segmentation], and type III 50 cases [mixed defects]) association analysis, and the haplotype analysis., Results: The single SNP analysis showed allele frequency of rs2289292 (exon 8, the only tagging SNP) and rs3809624 (5' untranslated region) demonstrated significant difference between CS cases and controls (P = 0.017 and P = 0.033). No SNP was found to be correlated with clinical phenotype. Moreover, the 2 makers (rs2289292 and rs3809624) in TBX6 gene were found to be in strong linkage disequilibrium (D' = 1.0; gamma = 0.984; 95% confidence interval, 0.96-1.0; LOD = 57.48) in the controls. Both global haplotype analysis and individual haplotype analysis showed that the haplotype of SNP1/SNP2 showed significant association with the disease (P = 0.017), G-A haplotype was more frequently observed in controls than in cases (odds ratio, 0.71; 95% confidence interval, 0.51-0.99)., Conclusion: This is the first report on SNPs of TBX6 gene in CS that suggests genetic variants of TBX6 gene is associated with CS and may play an important role in mediating susceptibility to developing CS in the Chinese Han population.

The etiology of adolescent idiopathic scoliosis is undetermined despite years of research. A number of hypotheses have been postulated to explain its development, including growth abnormalities. The irregular expression of growth hormone and insulin-like growth factor-1 (IGF-1) may disturb hormone metabolism, result in a gross asymmetry, and promote the progress of adolescent idiopathic scoliosis. Initial association studies in complex diseases have demonstrated the power of candidate gene association. Prior to our study, 1 study in this field had a negative result. A replicable study is vital for reliability. To determine the relationship of growth hormone receptor and IGF-1 genes with adolescent idiopathic scoliosis, a population-based association study was performed. Single nucleotide polymorphisms with potential function were selected from candidate genes and a distribution analysis was performed. A conclusion was made confirming the insufficiency of an association between adolescent idiopathic scoliosis and the single-nucleotide polymorphism of the growth hormone receptor and IGF-1 genes in Han Chinese.

Study Design: Comparison of 2 radiographic scoliosis classification systems by multiple surgeons., Objective: Compare the reliability of Peking Union Medical College (PUMC) and Lenke scoliosis classification systems and analyze their differences., Summary of Background Data: The PUMC classification is a newly reported system based on radiographic measurements with recent popularity, while the Lenke classification is widely accepted worldwide in surgical design. Both these classification systems have their own individual characteristics, hence it is necessary to compare their reliability., Methods: Five scoliosis surgeons independently evaluated and classified presurgical radiographs of 62 adolescent idiopathic scoliosis patients based on the PUMC and Lenke classification systems on 2 separate occasions. Radiographs were cleaned before each evaluation. Inter- and intraobserver reliabilities were quantified using Kappa statistics. Data were compared using chi2 analysis., Results: The PUMC classification's inter- and intraobserver percentage of agreement averaged to 91.0% (Kappa coefficient 0.896) and 90.2% (Kappa coefficient 0.892), respectively. While those of the Lenke curve type classification were 86.5% (Kappa coefficient 0.808) and 87.4% (Kappa coefficient 0.826). The PUMC classification from 10 individual measurements had 17 cases (27.4%) of disagreements, while in the Lenke curve type classification, 24 cases (38.7%) had disagreements. PUMC classification normally has discrepancies between type IIb, IIc, and IId, while Lenke classification has discrepancies in curve types 1 and 2. Out of 17 inconsistent PUMC curve type cases, 7 did not affect surgical fusion levels, while in the Lenke's only 2 out of 24 cases with discrepancies did not affect fusion range selection, with an obvious statistical difference., Conclusion: The reliability of both PUMC classification and Lenke curve type classification were categorized as good-to-excellent. PUMC classification is relatively simple, with less confusion among inter- and intraobservers, with corresponding surgical fusion guidance and planning. The mismatch of curve classification had less influence on PUMC's fusion range selection than Lenke's.

Study Design: A prospective study., Objective: Comparison study of radiologic and clinical outcomes, efficiency, and cost between anterior spinal fusion (ASF) and posterior spine fusion (PSF) in surgical treatment of moderate lumbar/thoracolumbar adolescent idiopathic scoliosis (AIS)., Summary of Background Data: ASF and PSF indicated for lumbar and thoracolumbar adolescent idiopathic scoliosis surgical treatment have respective advantages and disadvantages. However, up until today, a related prospective AIS comparative study has rarely been reported., Methods: Thirty-two cases in this prospective study with patients enrolled in either method A or B alternately in a sequence were divided into 2 groups. Group A underwent ASF with single solid rod and single screw constructs, and group B underwent PSF with segmental total pedicle screw system. Inclusion criteria were: (1) AIS diagnosis; (2) diagnosis classification as Lenke5CN type; (3) Cobb angles 35 degrees-60 degrees on anteroposterior view radiographs. Exclusion criteria were: (1) a history of spinal surgery; (2) age younger than 10 years; (3) Risser sign 0 degree; (4) lumbar/thoracolumbar kyphosis. All patients were observed with 2-year minimum follow-up (24-46 months). Clinical and radiologic outcomes of both groups A and B were analyzed., Results: Statistical t test or Mann-Whitney U test demonstrated no significant difference in preoperative age (P = 0.380), Risser sign (P = 0.733), magnitude (P = 0.936), flexibility (P = 0.815), apical vertebra rotation (AVR, P = 0.756), and apical vertebra translation (AVT, P = 0.355) of the lumbar/thoracolumbar curves, trunk shift (TS, P = 0.448), sagittal kyphosis from T5-T12 (P = 0.792) and sagittal lordosis from L1-L5 (P = 0.299). Average coronal correction of thoracolumbar/lumbar curves was 83% after surgery and 77% at follow-up in group A and 87% after surgery and 82% at follow-up in group B (P = 0.236 and P = 0.138). No significant differences were observed regarding correction of sagittal alignment, TS, AVT, AVR and hospitalization days on last follow-up between both groups (P > 0.05). No pseudarthrosis, reoperation, neurologic complications, infection, and no other problems were observed. Excellent clinical fusion results were present in all patients on their last follow-up. However, significant differences were evident in group A in regards to reduced operative time (P = 0.046), reduced estimated blood loss (P = 0.003), decreased blood transfusion (P = 0.006), reduced implants cost and hospitalization expenses (P = 0.000). Additionally, group A had shorter fusion levels than group B (p50 = 4 vs. p50 = 5, P = 0.003)., Conclusion: ASF versus PSF comparison in treating moderate lumbar/thoracolumbar AIS did not show significant differences in regards to safety or efficacy but demonstrated shorter fusion levels, reduced surgical trauma and costs in ASF.

Study Design: A genetic association study of tryptophan hydroxylase 1 gene (TPH1) and arylalkylamine N-acetyltransferase gene(AANAT) with adolescent idiopathic scoliosis (AIS) in Han Chinese., Objective: To access whether TPH1 and AANAT polymorphisms are associated with the predisposition, gender, and/or severity of AIS., Summary of Background Data: Studies have shown that AIS is a multifactorial inheritance disease, but the etiology is still unknown. In addition, several lines of evidence show that melatonin deficiency is closely associated with AIS, although there are still doubts and debates. Some polymorphisms in TPH1 and AANAT, the genes of 2 critical enzymes involved in melatonin biosynthesis, may contribute to variability of melatonin production in pineal glands., Methods: We genotyped 16 reported single nuclear polymorphisms (SNPs) present in TPH1 and AANAT in 103 AIS patients and 108 controls with matched sex and age. The data of 6 SNPs with minor allele frequence (MAF) above 5% were analyzed by the allelic and genotypic association analysis, the genotype-phenotype (gender and Cobb angle) association analysis, and the haplotype analysis., Results: The single SNP analysis showed that rs10488682, located in the promoter region of TPH1, was related with the occurrence of AIS (P < 0.05). No SNP was found to be correlated with gender or Cobb angle. Two makers (rs8176799 and rs2108977) in TPH1 were found to be in strong LD [ D' = 1.0 (95% CI, 0.9-1.0), gamma = 0.501, LOD = 18.93] in the controls. Both global haplotype analysis and individual haplotype analysis showed that there was no haplotype significantly associated with AIS in this LD block., Conclusion: TPH1 polymorphisms were associated with AIS but not with gender and Cobb angle in AIS patients. AANAT polymorphisms were not associated with AIS. These results suggested that TPH1 was an AIS predisposition gene, and there was a close relationship between the dyssynthesis of melatonin and AIS.

Study Design: A randomized prospective study., Objective: To compare safety, efficacy, and cost between 1-stage and 2-stage anterior and posterior spinal fusion in treating rigid thoracic adolescent idiopathic scoliosis (AIS)., Summary of Background Data: Retrospective study issued different outcomes comparing 1-stage with 2-stage surgical procedures in treating neuromuscular and congenital scoliosis. A randomized prospective study in AIS has rarely been reported. METHODS.: Twenty-four patients were randomly divided into 2 groups. Group A underwent a 2-stage procedure, group B a 1-stage procedure. Inclusion criteria were: (1) AIS; (2) rigid single thoracic curve, > or =90 degrees on anteroposterior and > or =60 degrees on side bending view; and (3) pulmonary function tests, vital capacity > or =500 mL or FEV1 > or =50%. Exclusion criteria were: (1) curves either <90 degrees on anteroposterior or <60 degrees on bending view; and (2) pulmonary function tests, both vital capacity <500 mL and FEV1 <50%. The mean follow up was 3.3 years (range 2-5.1). Clinical outcomes between the 2 groups were analyzed., Results: A statistical t test demonstrated no significant difference in age (P = 0.299), curves on anteroposterior (P = 0.908), on bending (P = 0.905), after surgery (P = 0.699), and at last follow-up (P = 0.946). No significant difference in blood loss (P = 0.089), transfusion (P = 0.334), operation time (P = 0.172), fusion segments anterior (P = 0.161), posterior (P = 0.546), wound drainage (P = 0.557), thoraco drainage (P = 0.948), and loss of correction at follow-up (P = 0.596). However, significant differences were found in length of stay (P < 0.001), patient cost of hospitalization (P < 0.001) between 2 groups, and curve correction (P < 0.001) in each group. Perioperative complications included 1 patient each with pneumonia, pneumothorax (group A), and ileus (group B). Screw nut loosing was found in 1 patient in group B, but no curve deteriorated. No neurologic deficit occurred in either group., Conclusion: A comparison of 1-stage versus 2-stage fusion in treating rigid thoracic curves in AIS did not show significant differences in safety or efficacy but did demonstrate significant difference in length of stay and patient cost.

Study Design: To compare the expression of cytokines and core protein of proteoglycan in the scoliotic concave and convex cartilaginous endplate using immunohistochemical staining., Objectives: To define the possible role of transforming growth factor beta 1 (TGFbeta1), basic fibroblast growth factor (bFGF), and core protein of proteoglycan in the development of adolescent idiopathic scoliosis., Summary of Background Data: Changes in the endplate composition have been implicated as possible etiologic factors in the pathogenesis of adolescent idiopathic scoliosis. Cytokines have exclusive effects on cartilage. Thus comparing the expression of the cytokines and matrix on the convex and concave sides of scoliotic endplate tissues may help to understand the role of endplate tissues in the induction and/or progression of idiopathic scoliosis., Methods: The convex and concave half of cartilage endplate was collected at the apex and end vertebrae from 12 patients. The expression of TGFbeta1, bFGF, and core protein on both sides was examined with the immunohistochemistry method, and results were analyzed with the image analysis system., Results: TGFbeta1, bFGF, and core protein of proteoglycan were all expressed in the cytoplasm of chondrocytes in the cartilaginous endplate. The area density and quantity density of TGFbeta1 and bFGF on the concave side are expressed in an even significantly higher level than that on the convex side (P > or = 0.05). The expression of the core protein of proteoglycan on the convex side is higher than that on the concave side, the difference is not significant (P > 0.05)., Conclusion: There was a significantly higher expression of TGFbeta1 and bFGF, although a lower expression of the core protein on the concave side, which suggests a possible etiological factor or a secondary change in the development of adolescent idiopathic scoliosis.

Study Design: A retrospective radiographic study on the type of surgically treated idiopathic scoliosis, with a prospective study on the reliability of the type-related fusion guide., Objectives: To identify and classify surgically treated idiopathic scoliosis, and define its related fusion levels by a new classification system., Summary of Background Data: Some classification methods for idiopathic scoliosis have been suggested. However, poor intraobserver reproducibility and interobserver reliability were experienced in these studies, and were not appropriate for guiding surgical planning., Methods: A total of 427 surgically treated idiopathic scoliosis cases were reviewed. Preoperative and postoperative standing anteroposterior, lateral, and preoperative supine side-bending radiograph were analyzed using the Scoliosis Research Society definition of scoliosis and curve apex. The resulting classification was tested for intraobserver reliability and interobserver reliability, and by 6 surgeons. Apical frequencies were determined for each type, and prospective surgical testing of the new type and its related fusion guide was performed., Results: Three major types and 13 subtypes were identified, of which the Peking Union Medical College type I accounted for 56.62%, type II 42.16%, and type III 1.22%. The interobserver reliability testing was 85% (kappa coefficient 0.832), while intraobserver reproducibility was 91% (kappa coefficient 0.898). Each type had its corresponding fusion levels. A prospective study of 152 cases was performed according to the classification. All of these cases were followed over 18 months, and no postoperative decompensation was noted., Conclusion: The Peking Union Medical College classification of idiopathic scoliosis is one system to combine each type with its corresponding fusion level, and it had much higher interobserver reliability and intraobserver reproducibility than the King system. Further prospectivestudies would help to clarify and expand this system.

Objective: To introduce a new spinal internal fixation system, Texas Scottish Rite Hospital (TSRH), and to investigate its early clinical outcomes., Methods: The preliminary clinical outcomes of 15 patients with thoracolumbar or lumbar scoliosis treated by anterior spinal fusion with TSRH instrumentation were studied retrospectively. Fourteen patients were diagnosed as idiopathic scoliosis and 1 as neuromuscular scoliosis., Results: Preoperatively, the Cobb's angle on the coronal plane was 55.8 degrees (range 35 degrees to approximately 78 degrees), and 14 degrees postoperatively, with an average correction of 74.8%. The average unfused thoracical curve was 35.9 degrees preoperatively (range 26 degrees to approximately 51 degrees) and 21.8 degrees (10 degrees to approximately 42 degrees) postoperatively, with 40% correction. The sagittal curve of lumbar was kept physiologically, preoperative 27.9 degrees and postoperative 25.7 degrees respectively. The trunk shift was 13.4 mm (5 to approximately 28 mm) preoperatively and 3.5 mm (0 to approximately 7 mm) postoperatively. The averaged apic vertebra derivation was 47.8 mm (21 to approximately 69 mm) before operation and 10.8 mm (3 to approximately 20 mm) after operation. The distance of C7 to center sacrum vertical line (CSVL) was 19.5 mm (16 to approximately 42) preoperatively and 11.3 mm (0 to approximately 32 mm) postoperatively. The apical vertebra rotation was 3 degree in 15 patients preoperatively, and were improved to normal in 10 patients, 1 degree in 4 patients, and 2 degree in 1 patients postoperatively. None had neurological injury and infection. Only 1 patient complained a cool feeling in the low extremity of concave side, and the symptom vanished at 3-month followed up., Conclusion: If used appropriately, TSRH anterior spinal system is a good teatment for low thoracic or thoracic lumbar scoliosis.

Objective: To evaluate the results of the TRAFIX instrumentation in correcting scoliosis., Methods: Since October 1997, 47 patients with scoliosis received spinal fixation with the TRAFIX instrumentation at the Peking Union Medical College Hospital. The average age was 14. 3 years (range 10 to 38 years). There were 27 idiopathic cases, 16 congenital cases, 2 cases with Marfan syndrome and 2 with neurofibromatosis. Twelve of the 47 patients underwent anterior release, while 4 patients received the revision approach. The average follow-up time was 26 months (13 -38 months)., Results: The measurements of primary coronal deformity before and after surgery were 74 degrees (50 degrees approximately 115 degrees) and 38.7 degrees (11 approximately 95 degrees), respectively. The average curve correction was 54%. The average number of fused segments was 12.5 (7 approximately 17) vertebrae. The distance between the center of apex and the C7 plumb line was 56.8 mm before surgery (25 approximately 107 mm) and 31 mm after surgery (10 approximately 87 mm)., Conclusion: The TRAFIX instrumentation provides three-dimensional correction with refinement, convenience and reliable fixation.

Background: The treatment of congenital scoliosis associated with split cord malformation (SCM) raises the issue of how to best manage such patients to avoid neurological deficit while achieving a satisfactory correction.Methods: This prospective clinical study was performed at our center from March 2000 through June 2013. We enrolled a total of 214 patients (61 male and 153 female) with congenital scoliosis associated with SCM who were undergoing spinal correction surgery. The mean age at surgery was 14.1 years. The inclusion criteria were congenital scoliosis with confirmed SCM; status as neurologically intact or stable over the preceding 2 years; and no neurological deterioration as evidenced on traction, side-bending, or fulcrum-bending radiographs. Patients with unstable neurological status or for whom vertebral column resection surgery was planned were excluded. All patients underwent scoliosis surgery without prophylactic detethering.Results: On the basis of the Pang classification, 73 patients were in the type-I SCM group, and 141 were in the type-II SCM group. The groups did not differ significantly with respect to preoperative characteristics, operative time, blood loss, or number of levels fused. The mean follow-up was 37 months (range, 24 to 108 months). The rate of scoliosis correction was lower in the type-I group than in the type-II group (p < 0.05). In the type-I group, the correction rate was 48.9% at 1 week postoperatively and 42.2% at the last follow-up. In the type-II group, the correction rate was 54.7% at 1 week postoperatively and 47.9% at the last follow-up. Eleven (5.1%) of the patients experienced transient neurological complications, with no significant difference between the groups (p = 0.415). No patient experienced permanent neurological deficit during surgery or follow-up.Conclusions: Patients with congenital scoliosis associated with SCM, regardless of type, can safely and effectively undergo spinal deformity correction and achieve spinal balance without neurological intervention. For such patients with intact or stable neurological status, prophylactic detethering prior to scoliosis surgery may not be necessary.Level Of Evidence: Therapeutic Level II. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Evaluates the results of Texas Scottish Rite Hospital instrumentation in the correction of coronal, sagittal and rotational deformity of scoliosis in China. Description of adolescent iodiopathic scoliosis; Classification of idiopathic scoliosis; Difference between CD and TRSH instrumentation.