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Purpose: There is great controversy about the etiologic origin of adolescent idiopathic scoliosis. Multiple theories have been suggested, including metabolic aspects, endocrine dysfunction, neurological central abnormalities, genetic predisposition and epigenetic factors involved in the development of scoliosis. However, there has always been speculations based on human biomechanical behavior., Methods: In this article, we performed a literature review on the biomechanical traits of human posture, and the proposed theories that explain the special characteristics present in idiopathic scoliosis., Results: The current theory on the etiopathogeneis of AIS suggests that dorsally directed shear loads acting on a preexisting axial plane rotation, in a posteriorly inclined sagittal plane of a growing patient, together with disc maturation, collagen quality at this phase of development and immaturity of proprioception, is the perfect scenario to spark rotational instability and create the three-dimensional deformity that defines idiopathic scoliosis., Conclusion: The unique spinal alignment of human bipedalism, gravity and muscle forces acting straight above the pelvis to preserve an upright balance, and the instability of the soft tissue in a period of growth development, is an appealing cocktail to try to explain the genesis of this condition in humans., (© 2023. The Author(s), under exclusive licence to Scoliosis Research Society.)

Study Design: This is a case-control study of prospectively collected data., Objective: To quantify paraspinal muscle size asymmetry in adolescent idiopathic scoliosis (AIS) and determine if this asymmetry is (i) greater than observed in adolescent controls with symmetrical spines; and (ii) positively associated with skeletal maturity using Risser grade, scoliosis severity using the Cobb angle, and chronological age in years., Summary of Background Data: AIS is a three-dimensional deformity of the spine which occurs in 2.5% to 3.7% of the Australian population. There is some evidence of asymmetry in paraspinal muscle activation and morphology in AIS. Asymmetric paraspinal muscle forces may facilitate asymmetric vertebral growth during adolescence., Methods: An asymmetry index [Ln(concave/convex volume)] of deep and superficial paraspinal muscle volumes, at the level of the major curve apex (Thoracic 8-9 th vertebral level) and lower-end vertebrae ( LEV , Thoracic 10-12 th vertebral level), was determined from three-dimensional Magnetic Resonance Imaging of 25 adolescents with AIS (all right thoracic curves), and 22 healthy controls (convex=left); all female, 10 to 16 years., Results: Asymmetry index of deep paraspinal muscle volumes was greater in AIS (0.16±0.20) than healthy spine controls (-0.06±0.13) at the level of the apex ( P <0.01, linear mixed-effects analysis) but not LEV ( P >0.05). Asymmetry index was positively correlated with Risser grade ( r =0.50, P <0.05) and scoliosis Cobb angle ( r =0.45, P <0.05), but not age ( r =0.34, P >0.05). There was no difference in the asymmetry index of superficial paraspinal muscle volumes between AIS and controls ( P >0.05)., Conclusions: The asymmetry of deep apical paraspinal muscle volume in AIS at the scoliosis apex is greater than that observed at equivalent vertebral levels in controls and may play a role in the pathogenesis of AIS., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Study Design: A prospective cohort study., Objective: Detail typical three-dimensional segmental deformities and their rates of change that occur within developing adolescent idiopathic scoliosis (AIS) spines over multiple timepoints., Summary of Background Data: AIS is a potentially progressive deforming condition that occurs in three dimensions of the scoliotic spine during periods of growth. However, there remains a gap for multiple timepoint segmental deformity analysis in AIS cohorts during development., Materials and Methods: Thirty-six female patients with Lenke 1 AIS curves underwent two to six sequential magnetic resonance images. Scans were reformatted to produce images in orthogonal dimensions. Wedging angles and rotatory values were measured for segmental elements within the major curve. Two-tailed, paired t tests compared morphologic differences between sequential scans. Rates of change were calculated for variables given the actual time between successive scans. Pearson correlation coefficients were determined for multidimensional deformity measurements., Results: Vertebral bodies were typically coronally convexly wedged, locally lordotic, convexly axially rotated, and demonstrated evidence of local mechanical torsion. Between the first and final scans, apical measures of coronal wedging and axial rotation were all greater in both vertebral and intervertebral disk morphology than nonapical regions (all reaching differences where P <0.05). No measures of sagittal deformity demonstrated a statistically significant change between scans. Cross-planar correlations were predominantly apparent between coronal and axial planes, with sagittal plane parameters rarely correlating across dimensions. Rates of segmental deformity changes between earlier scans were characterized by coronal plane convex wedging and convexly directed axial rotation. The major locally lordotic deformity changes that did occur in the sagittal plane were static between scans., Conclusions: This novel investigation documented a three-dimensional characterization of segmental elements of the growing AIS spine and reported these changes across multiple timepoints. Segmental elements are typically deformed from initial presentation, and subsequent changes occur in separate orthogonal planes at unique times., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: To provide normative data and to determine accuracy and reliability of preoperative measurements of spondylolisthesis and kyphosis on supine static magnetic resonance imaging (MRI) of patients with degenerative cervical myelopathy., Methods: T2-weighted midsagittal images of the cervical spine were in 100 cases reviewed twice by one junior observer, with an interval of 3 months, and once by a senior observer. The spondylolisthesis slip (SSlip, mm) and the modified K-line interval (mK-line INT, mm) were assessed for accuracy with the standard error of measurement (SEm) and the minimum detectable change (MDC). Intraobserver and interobserver reliability levels were determined using the intraclass correlation coefficient (ICC)., Results: The SEm was 0.5 mm (95% CI 0.4-0.6) for spondylolisthesis and 0.6 mm (95% CI 0.5-0.7) for kyphosis. The MDC, i.e., the smallest difference between two examinations that can be detected with statistical certainty, was 1.5 mm (95% CI 1.2-1.8) for spondylolisthesis and 1.6 mm (95% CI 1.3-1.8) for kyphosis. The highest reliability levels were seen between the second observation of the junior examiner and the senior observer (ICC = 0.80 [95% CI 0.70-0.87] and ICC = 0.96 [95% CI 0.94-0.98] for SSlip and mK-line INT, respectively)., Conclusions: This study provides normative values of alignment measurements of spondylolisthesis and kyphosis in DCM patients. It further shows the importance of taking measurement errors into account when defining cut-off values for cervical deformity parameters and their potential clinical application in surgical decision-making., (© 2023. The Author(s).)

Purpose: Kyphosis involves spines curving excessively backward beyond their physiological curvature. Although the normal structure of the spinal vertebrae is extremely important for maintaining posture and the normal function of the thoracic and abdominal organs, our knowledge concerning the pathogenesis of the disease is insufficient. We herein report that the downregulation of the calcium signaling pathway is involved in the pathogenesis of congenital kyphosis., Methods: The third to fifth lumbar spine segments, the kyphotic region of Ishibashi (IS) rats, which are used as a model of congenital kyphoscoliosis, were collected. A DNA microarray, quantitative PCR, Western blotting, and immunohistochemistry were used to measure the expression of genes and proteins related to intracellular calcium signaling., Results: We found that the expression of calcium-sensing receptor (CaSR) and transient receptor potential vanilloid 1 (Trpv1)-two receptors involved in the calcium signaling-was decreased in the lumbar spine of IS rats. We also observed that the number of CaSR-immunoreactive and Trpv1-immunoreactive cells in the lumbar spine of IS rats was lower than in wild-type rats. Furthermore, the expression of intracellular molecules downstream of these receptors, such as phosphorylated protein kinase C, c-Jun N-terminal kinase, and neural EGFL-like 1, was also reduced. In fact, the calcium content in the lumbar spine of IS rats was significantly lower than that in wild-type rats., Conclusion: These results indicate that adequate calcium signaling is extremely important for the regulation of normal bone formation and may also be a key factor for understanding the pathogenesis of congenital kyphosis., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Spinal lesions in pediatric chronic recurrent multifocal osteomyelitis/chronic non-bacterial osteomyelitis (CRMO/CNO) can cause permanent sequelae; thus, early recognition of these is vital for management., Objective: To characterize the MR imaging features and patterns of pediatric spinal CRMO/CNO., Materials and Methods: This cross-section study received IRB approval. The first available MRI with documented spine involvement in children with CRMO/CNO was reviewed by a pediatric radiologist. Descriptive statistics were used to describe the characteristics of vertebral lesions, disc involvement, and soft tissue abnormality., Results: Forty-two patients were included (F:M, 30:12); median age was 10 years (range 4-17). At diagnosis, 34/42 (81%) had spine involvement. Kyphosis in 9/42 (21%) and scoliosis in 4/42 (9.5%) patients were present at the time of spinal disease recognition. Vertebral involvement was multifocal in 25/42 (59.5%). Disc involvement was found in 11/42 (26%) patients, commonly in the thoracic spine and often with adjacent vertebrae height loss. Posterior element abnormalities were present in 18/42 patients (43%) and soft tissue involvement in 7/42 (17%). One hundred nineteen vertebrae were affected, commonly the thoracic vertebrae (69/119; 58%). Vertebral body edema was focal in 77/119 (65%) and frequently superior (42/77; 54%). Sclerosis and endplate abnormality were present in 15/119 (13%) and 31/119 (26%) vertebrae, respectively. Height loss was present in 41/119 (34%)., Conclusion: Chronic non-bacterial osteomyelitis of spine is usually thoracic. Vertebral body edema is often focal at the superior vertebral body. Kyphosis and scoliosis occur in a quarter and vertebral height loss in a third of children at spinal disease recognition., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To quantify the cervical sagittal alignment in patients with Hirayama disease (HD) and to investigate the effect of loss of cervical sagittal alignment upon the cervical spinal lesions in HD., Methods: Cervical sagittal alignments were measured in 253 HD patients and 63 healthy subjects by C2-C7 Cobb and a modified method of Toyama et al. Motor unit number estimation (MUNE) was performed in bilateral abductor pollicis brevis (APB) in all HD patients, and 31 patients further underwent cervical diffusion tensor imaging (DTI)., Results: Compared with healthy subjects, HD patients showed lower C2-C7 Cobb (P < 0.05), and 83.4% patients showed loss of cervical lordosis (cervical straight or kyphosis), which was greater than healthy subjects (55.6%, P < 0.05). Compared with lordotic/straight group, patients with cervical kyphosis showed lower MUNE values and greater single motor unit potential (SMUP) in bilateral APB, and higher apparent dispersion coefficient (ADC) and lower fractional anisotropy were observed at C4/C5 level in the latter than the former (P < 0.05). C2-C7 Cobb was associated with both C4/C5 ADC and bilateral SMUP (P < 0.05)., Conclusions: Most HD patients showed loss of cervical sagittal alignments, and both MUNE and DTI detections demonstrated a positive correlation between loss of cervical sagittal alignments and cervical spinal lesions in HD. These findings supported that loss of cervical sagittal alignments may worsen motor impairments in HD. Therefore, it is necessary for clinicians to be aware of restoring cervical sagittal alignments during HD treatment., (© 2023. Fondazione Società Italiana di Neurologia.)

Degenerative spinal disorders, including kyphotic deformity, are associated with a range of degenerative characteristics of the paraspinal musculature. It has therefore been hypothesized that paraspinal muscular dysfunction is a causative factor for degenerative spinal deformity; however, experimental studies demonstrating causative relationships are lacking. Male and female mice received either glycerol or saline injections bilaterally along the length of the paraspinal muscles at four timepoints, each separated by 2 weeks. Immediately after sacrifice, micro-CT was performed to measure spinal deformity; paraspinal muscle biopsies were taken to measure active, passive and structural properties; and lumbar spines were fixed for analysis of intervertebral disc (IVD) degeneration. Glycerol-injected mice demonstrated clear signs of paraspinal muscle degeneration and dysfunction: significantly (p < 0.01) greater collagen content, lower density, lower absolute active force, greater passive stiffness compared to saline-injected mice. Further, glycerol-injected mice exhibited spinal deformity: significantly (p < 0.01) greater kyphotic angle than saline-injected mice. Glycerol-injected mice also demonstrated a significantly (p < 0.01) greater IVD degenerative score (although mild) at the upper-most lumbar level compared to saline-injected mice. These findings provide direct evidence that combined morphological (fibrosis) and functional (actively weaker and passively stiffer) alterations to the paraspinal muscles can lead to negative changes and deformity within the thoracolumbar spine., (© 2023. The Author(s).)

Objective: To characterize the paraspinal muscles of adolescent idiopathic scoliosis (AIS) patients, and to further explore its etiology., Methods: Clinical records and paraspinal muscle biopsies at the apex vertebra region during posterior scoliosis correction surgery of 18 AIS were collected from November 2018 to August 2019. Following standardized processing of fresh muscle tissue biopsy, serial sections with conventional hematoxylin-eosin (HE) and histochemical and immunohistochemical (IHC) with antibody Dystrophin-1 (R-domain), Dystrophin-2 (C-terminal), Dystrophin-3 (N-terminal), Dystrophin-total, Myosin (fast), major histocompatibility complex 1 (MHC-1), CD4, CD8, CD20, and CD68 staining were obtained. Biopsy samples were grouped according to the subjects' median Cobb angle (Cobb angle ≥ 55° as severe AIS group and Cobb angle < 55° as mild AIS group) and Nash-Moe's classification respectively, and the corresponding pathological changes were compared between the groups statistically., Results: Among the 18 AIS patients, 8 were in the severe AIS group (Cobb angle ≥55°) and 10 in the mild AIS group (Cobb angle < 55°). Both severe and mild AIS groups presented various of atrophy and degeneration of paraspinal muscles, varying degrees and staining patterns of immune-expression of Dystrophin-3 loss, especially Dystrophin-2 loss in severe AIS group with significant differences, as well as among the Nash-Moe classification subgroups. Besides, infiltration of CD4 + and CD8 + cells in the paraspinal muscles and tendons was observed in all the patients while CD20 + cells were null. The expression of MHC-1 on myolemma was present in some muscle fibers., Conclusion: The histologic of paraspinal muscle biopsy in AIS had similar characteristic changes, the expression of Dystrophin protein was significantly reduced and correlated with the severity of scoliosis, suggesting that Dystrophin protein dysfunctions might contribute to the development of scoliosis. Meanwhile, the inflammatory changes of AIS were mainly manifested by T cell infiltration, and there seemed to be a certain correlation between inflammatory cell infiltration, MHC-1 expression and abnormal expression of Dystrophin. Further research along the lines of this result may open up new ideas for the diagnosis of scoliosis and the treatment of paraspinal myopathy.

Purpose: The pathology of dropped head syndrome (DHS) is diverse, and reports of surgery for DHS are scarce. We aimed to describe surgery for DHS and to investigate the surgical outcomes thereof., Methods: We enrolled 40 consecutive patients (six males and 34 females; average age at surgery, 72.0 years) with DHS who underwent correction surgeries at a single institute. Short fusion (SF), with the extent of fixation mainly at the cervical region, was performed for 27 patients; long fusion (LF), involving the cervical and thoracic spine, for 13. Clinical and radiological outcomes were investigated, and factors analyzed using the Japanese Orthopedic Association Cervical Myelopathy Evaluation Questionnaire (JOACMEQ)., Results: All patients were able to gaze horizontally at the follow-up. Instances of five transient C5 palsy results, and five distal junctional kyphosis results were found, but no revisions were reported due to recurrence. Patients whose T1 slope-20° was smaller than the C2-7 angle postoperatively exhibited better clinical outcomes in the three domains of the JOACMEQ, regardless of the extent of fixation., Conclusion: For cases where the T1 slope is relatively small, and approximately 10° of cervical lordosis is predicted to be obtained postoperatively, SF is appropriate. Alternatively, for cases with higher T1 slope, obtaining a cervical lordosis over 20° has a risk of postoperative complications. For such cases, it is an option to perform an LF involving the cervical and thoracic spine., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Study Design: A retrospective cross-sectional study., Objective: To assess the association between spinal muscle morphology and spinopelvic parameters in lumbar fusion patients, with a special emphasis on lumbar lordosis (LL)., Summary of Background Data: Maintenance of sagittal alignment relies on muscle forces, but the basic association between spinal muscles and spinopelvic parameters is poorly understood., Materials and Methods: Patients operated between 2014 and 2017 who had both lumbar magnetic resonance imaging scan and standing whole-spine radiographs within six months before surgery were included. Muscle measurements were conducted on axial T2-weighted magnetic resonance images at the superior endplate L3-L5 for the psoas and L3-S1 for combined multifidus and erector spinae (paraspinal) muscles. A pixel intensity threshold method was used to calculate the total cross-sectional area (TCSA) and the functional cross-sectional area (FCSA). Spinopelvic parameters were measured on lateral standing whole-spine radiographs and included LL, pelvic incidence (PI), PI-LL mismatch, pelvic tilt, sacral slope, thoracic kyphosis, and sagittal vertical axis. Analyses were stratified by biological sex. Multivariable linear regression analyses with adjustments for age and body mass index (BMI) were performed., Results: A total of 104 patients (62.5% female) were included in the analysis. The patient population was 90.4% White with a median age at surgery of 69 years and a median BMI of 27.8 kg/m 2 . All muscle measurements were significantly smaller in women. PI, pelvic tilt, and thoracic kyphosis were significantly greater in women. PI-LL mismatch was 6.1° (10.6°) in men and 10.2° (13.5°) in women ( P =0.106), and sagittal vertical axis was 45.3 (40.8) mm in men and 35.7 (40.8) mm in women ( P =0.251). After adjusting for age and BMI, paraspinal TCSA at L3-L5, and paraspinal FCSA at L4 showed significant positive associations with LL in women. In men, psoas TCSA at L5 and psoas FCSA at L5 showed significant negative associations with LL, but none of the paraspinal muscle measurements., Conclusion: Our findings indicate that psoas and lumbar spine extensor muscles interact differently on LL among men and women, creating a unique mechanical environment., Level of Evidence: Level 4., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Study Design: Retrospective cross-sectional study., Objective: The aim was to describe spinopelvic alignment types by pelvic incidence (PI) and age to compare the Roussouly classification between pediatric and adult populations., Summary of Background Data: The Roussouly classification was validated for adults. Alignment types may vary during growth., Materials and Methods: Radiographs of 1706 non pathologic individuals (5-49 yr) were analyzed. Individuals ≤19 years were stratified by chronological age and skeletal maturity (triradiate cartilage, Risser), and compared with adults. Global and spinopelvic alignment parameters were assessed. Roussouly Types 1, 2, 3, 3A (anteverted pelvis), and 4 were determined. The distribution of parameters was analyzed by Bayesian inference. The relationship between PI and age by Roussouly type was modeled by linear regression., Results: The Sagittal Vertical Axis C7 decreased during growth and was significantly smaller in adults (20-34 yr) (Pr>0.99). Thoracic kyphosis and lumbar lordosis increased during growth and were larger in adults (Pr<0.025). Lordosis increased mainly in the cranial arch (Pr<0.025). PI and pelvic tilt increased during growth and were larger in adults (Pr<0.025). In children and adolescents, PI<45° represented the largest proportion, significantly larger compared with adults (Pr>0.99). Proportions of Roussouly Types 1 and 2 were similar throughout ages. Types 3 and 4 were rarer during the prepubertal period (Pr<0.025). The proportion of Type 3A was significantly higher in children and adolescents (Pr>0.99). Linear regression showed that Type 4 had the largest PI increase with age, with significantly higher curve slope compared with other types (Pr>0.9999). Types 3, 3A and 2 had similar slopes and lowest PI increase with age., Conclusion: Global and spinopelvic alignment changed during childhood and adolescence, leading to different kyphosis and lordosis distribution compared with adults. Growth-related PI increase influenced Roussouly types with typical predominance of Type 3A in the pediatric population and larger PI increase in Type 4., Level of Evidence: Level III., Competing Interests: Y.P.C. is consultant for Stryker, Clariance, SpineVision, Philips and Ceraver; he received royalties and grants unrelated to this study from Stryker and Clariance. B.I. is consultant for Zimmer Biomet, Medtronic and Implanet. B.B. is associate editor for OTSR Elsevier-Masson and consultant for Medicrea, Medtronic, Implanet, Vexim Stryker, and 3M. F.L. is consultant for Spineart and SMAIO. Guillaume Riouallon is consultant for Medtronic, Stryker and NewClip; he received royalties from Euros. V.C. is shareholder of Follow Health SA and consultant for Clariance. I.O. is consultant for Medtronic and Depuy Synthes; he received grants from DePuy Synthes unrelated to this study and royalties from Clariance, Alphatec and Spineart. L.B.is consultant for Neo and Euros; he received grants from DePuy Synthes unrelated to this study. F.S. received funding to attend meetings from Medicrea, Medtronic and Euros. J.-C.L.H. is consultant for Medtronic and BD Bard; he received royalties and grants unrelated to this study from Medtronic. V.F. is consultant for Clariance; he received royalties Medicrea and Clariance. A.F. is consultant for OSD. The remaining authors report no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Our previous studies found disproportionate anteroposterior vertebral size is associated with severity of the scoliotic curves in adolescent idiopathic scoliosis (AIS) patients. Subsequent studies showed wedging of vertebral bodies (VB) had less contribution than intervertebral discs (IVD) to the anterior-posterior vertebral column length discrepancy in severe-AIS. However, the exact morphological changes of IVD were not clearly defined. This study aimed to evaluate the morphological and pathological changes of IVD and VB in AIS girls and healthy female controls., Methods: This study included 33 age-matched female controls and 76 AIS girls with a right-sided thoracic curvature. Wedge angle, height ratio and distance ratio of VB and IVD were measured on the best midline coronal and sagittal planes from reformatted MRI spine. Volumes of VB, IVD and nucleus pulposus (NP) were also evaluated on volumetric images. One-way ANOVA with Bonferroni correction and Pearson correlation tests were used., Results: There was significant difference in wedge angle and height ratio of VB and IVD between AIS and controls. In severe-AIS, the position of NP was significantly shifted to the convexity when compared with non-severe AIS and controls. Whereas, the volume of IVD and NP in severe-AIS was found to be significantly smaller. In addition, Cobb angle was significantly correlated with wedge angle and height ratio, and inversely correlated with the volume of NP., Conclusions: In addition to wedging of VB and IVD, there was significantly reduced volume of IVD and NP in AIS patients with severe curve, insinuating the mechanical effect of scoliosis leads to a compression on both IVD and NP before significant disc desiccation occurs. We postulate that the compression of IVD and NP can contribute to curve progression in severe-AIS, these patients are more prone to disc degeneration in adulthood if no operative treatment is offered. Further longitudinal study on these parameters is still warranted., (© 2022. The Author(s).)

Spinal kyphosis involves the vertebrae curving excessively backward, beyond their physiological curvature. Although the normal structure of the spinal vertebrae is extremely important for maintaining posture, the normal function of the thoracic and abdominal organs, and cosmetics, our knowledge concerning the pathogenesis of this disease is lacking. Furthermore, the responsible gene has not yet been identified. In this short review, we summarize the current state of kyphosis research and introduce the molecular and cellular mechanisms associated with the pathogenesis of this disease, based on findings obtained using rats that develop kyphosis., (© 2021 Wiley Periodicals LLC.)

The aim of this study was to analyze the impact of surgical correction of the thoracic deformity on the cardiorespiratory function of patients with moderate-severe Scheuermann's hyperkyphosis (SK). A series of 23 adolescents with SK who underwent surgery through an only posterior approach using all pedicle screw constructs were included in the study. Cardiorespiratory parameters were measured during a maximal exercise tolerance test before and 2 years after surgery. Heart rate, oxygen saturation (SatO2), maximum oxygen uptake (VO2 max), ventilatory capacity at maximal exercise (VEmax), and energy costs were recorded. There were statistically significant differences in the forced vital capacity (FVC) (P < 0.05), total VO 2 max (ml/min) (P < 0.01), maximum expired volume (VEmax) per minute (P < 0.01) and cardiovascular efficiency (HR/VO2 ratio) (P < 0.05). None of these changes were clinically relevant. There were no changes in the VO 2 max per kg of body mass. The magnitude of the kyphosis correction did not correlate with the change in normalized VO2max or VEmax. In conclusion, patients with moderate-severe SK improve their baseline respiratory limitations and the tolerance to maximum exercise 2 years after surgery. However, the slight cardiorespiratory functional improvements should not necessarily be attributed to the surgery, and could also be caused solely by the residual growth of the lungs and thorax. Furthermore, respiratory functional changes are under thresholds considered as clinically relevant., (© 2021. The Author(s).)

Objective: To identify the apical vertebra is relate to the occurrence of vertebral rotatory subluxation (VRS) in degenerative lumbar scoliosis., Methods: In total, 86 patients with degenerative lumbar scoliosis included in this retrospective study were divided into 2 groups: with VRS and without VRS. Coronal and sagittal parameters were measured on whole-spine anteroposterior and lateral radiographs. The impact of VRS on the spine and pelvis parameters was statistically analyzed, and the correlation between the occurrence of VRS and the apical vertebra was analyzed by using logistic regression., Results: VRS was present in 49 patients with degenerative lumbar scoliosis and absent in 37 patients. Compared with the patients without VRS, the patients with VRS had a smaller lumbar lordosis and thoracic kyphosis (P < 0.05) and a larger apical vertebra translation, apical vertebra tilt angle, Cobb angle, and pelvic incidence-lumbar lordosis (P < 0.05). Logistic regression analysis showed that apical vertebra translation and apical vertebra tilt angle were risk factors for the occurrence of VRS. The receiver operating characteristic curve analysis showed that apical vertebra translation greater than 23.2 mm and apical vertebra tilt angle greater than 11.8° were related to a greater probability of VRS in patients with degenerative lumbar scoliosis., Conclusions: VRS is an important characteristic to consider in degenerative lumbar scoliosis that can affect the coronal and sagittal alignment. The apical vertebra is correlated with the occurrence of VRS; an apical vertebra translation greater than 23.2 mm and apical vertebra tilt angle greater than 11.8° are highly correlate with the occurrence of VRS., (Copyright © 2021 Elsevier Inc. All rights reserved.)

The kyphoscoliotic Ehlers-Danlos syndrome (kEDS) is a rare autosomal recessive connective tissue disorder characterized by hyperextensible skin and joints, kyphoscoliosis, and severe muscle hypotonia at birth. Causal variants have been identified in PLOD1 resulting in lysyl hydroxylase deficiency responsible for kEDS. However, the detailed phenotype of kEDS during the perinatal period is still poorly recognized. Here, we describe a case of a female newborn presenting with prenatal hydrocephalus and severe hypotonia after birth with two novel compound heterozygous variants, c.2T > C (p.?) and c.1462del (p. Arg488Glyfs*9) in the PLOD1 gene. Our case suggests that in addition to the reported phenotype during the neonatal period, prenatal hydrocephalus should also be differentially diagnosed to exclude the potential of kEDS., (Copyright © 2021. Published by Elsevier Masson SAS.)

The aim of this study was to determine age-related changes and sex-specific differences in sagittal alignment, range of motion (ROM), and intervertebral disc height of the thoracolumbar spine in healthy subjects. Lateral neutral and flexion-extension radiographs of the thoracolumbar spine of 627 asymptomatic subjects (307 males and 320 females; average age, 49.6 ± 16.5 years) were evaluated. We included at least 50 males and 50 females in each decade of life between the 20s and the 70s. Intervertebral disc height from T10/T11 to L5/S1, local lordotic alignment, and ROM from T10-T11 to L5-S1 were measured. T10-L2 kyphosis and T12-S1 lordosis as well as flexion, extension, and total ROM were measured. T10-L2 kyphosis did not markedly change with age in subjects of either sex but a sudden increase was noted in the 70s females. T12-S1 lordosis increased with age in both sexes, except the 70s. Flexion, extension, and total ROM at T10-L2 and T12-S1 decreased with age in most subjects. The levels from L3-L4 to L5-S1 were conspicuous as mobile segments. Intervertebral disc height gradually increased from T10/T11 to L4/L5; the shortest was at T10/T11 and the longest at L3/L4 or L4/L5 in all subjects. Age-related decreases in intervertebral disc height were most prominent at L4/L5 in middle-aged and elderly individuals of both sexes. Normative values of sagittal alignment, ROM, and intervertebral disc height at each segmental level were established in both sexes and all age groups in healthy subjects., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Objective: In this study, we evaluated factors affecting changes in cervical lordosis after deformity correction and during follow-up period in adult spinal deformity (ASD) patients with severe sagittal imbalance., Methods: Seventy-nine patients, with an average age of 71.6 years, who underwent long-segment fixation from T10 to S1 with sacropelvic fixation were included. We performed a comparative analysis of the radiographic parameters after surgery (Post) and at the last follow-up (Last). We calculated the Pearson's correlation coefficient and performed multilinear regression analysis to predict independent parameters for Post and Last cervical lordosis (CL), T1 slope (T1S), and thoracic kyphosis (TK)., Results: Hyperlordotic changes of -23.3° in CL before surgery was reduced to -7° after surgery, and Last CL had increased to -15.3°. T1S was reduced from 27° before surgery to 14.4° after surgery and had increased to 18.8° at the last follow-up. Through multilinear regression analysis, we found that Post CL and T1S were more significantly affected by the amount of LL correction (p = .045 and .049). The effect of Last T1S was significantly associated with the Last CL; the effect of Last TK, with the Last T1S; and the effect of Post PI-LL, with the Last TK (p < .05)., Conclusion: The postoperative kyphotic change in CL in ASD patients with preoperative cervical hyperlordosis is not permanent and is affected by drastic LL correction and SVA restoration. To achieve spinopelvic harmony proportional to the difference in LL relative to PI, TK becomes modified over time to increase T1S and CL, in an effort to achieve optimal spine curvature., Competing Interests: The authors have declared that no competing interests exist.

Back pain may be related to an improper sitting position. The aim of the study was to assess the sagittal curvatures of the spine in a sitting position in children with generalized joint hypermobility (GJH). The study included 302 children aged 8-14 years. The sagittal curvatures of the spine (sacral slope, lumbar lordosis, thoracic kyphosis with its lower and upper part) were assessed using the Saunders digital inclinometer. In order to assess GJH a 9-point Beighton scale was used. The study revealed no significant differences (p > 0.05) in sagittal curvatures of the spine in a relaxed sitting position between children with and without GJH. Regardless of the occurrence of GJH, kyphotic alignment of the spine was noted in a relaxed sitting. GJH does not affect the position of the trunk in a sagittal plane in a relaxed sitting position in children aged 8-14 years. A relaxed sitting position in children with and without GJH is characterized by a kyphotic position of the spine caused by an improper position of pelvis and lumbar segment of the spine.

Background: Degenerative lumber spondylolisthesis (DLS) is a common orthopedic condition, described as a condition that compared with the lower vertebra, the superior vertebra slides forward or backward in the sagittal plane without accompanying isthmic spondylolisthesis. Information pertaining to different types of double-level DLS is scarce. This study aims to analyze parameters of patients with different types of double-level DLS to provide a reference for guiding surgical treatment and restoring sagittal balance of patients with DLS., Methods: From January 2014 to January 2020, records of patients with double-level DLS were retrospectively reviewed. Patients with double-level DLS were divided into 3 types: anterior, posterior, and combined; the anterior and combined types were studied. The sagittal spinopelvic parameters included C7 tilt, maximal thoracic kyphosis, maximal lumbar lordosis (LLmax), pelvic incidence (PI), pelvic tilt (PT), and sacral slope (SS). After descriptive analysis, demographic and radiographic data were compared., Results: Forty and 18 patients were included in the anterior and combined type groups, respectively. Both groups had different levels of chronic low back pain, but the incidence of radiating leg pain and neurogenic claudication was significantly higher in the anterior type. Oswestry Disability Index and visual analog scale low back scores were also higher in the anterior type. In the anterior type, C7 tilt (7.14 ± 2.15 vs. 5.41 ± 2.28, P = 0.007), LLmax (50.02 ± 14.76 vs. 36.96 ± 14.56, P = 0.003), PI (68.28 ± 9.16 vs. 55.53 ± 14.19, P < 0.001), PT (28.68 ± 7.31 vs. 19.38 ± 4.70, P < 0.001), and PT/PI (42.45 ± 11.22 vs. 36.04 ± 9.87, P = 0.041) were significantly higher. In the anterior type, PI correlated positively with LLmax (r = 0.59) and SS (r = 0.71). LLmax and SS (r = 0.65) had a positive correlation. PT/PI and SS (r = -0.77) had a negative correlation. In the combined type, PI correlated positively with LLmax (r = 0.61) and SS (r = 0.88), and PT/PI correlated negatively with SS (r = -0.81)., Conclusions: In patients with double-level DLS, the sagittal spinopelvic parameters differed between the anterior and combined types. Overall, spinal surgeons should focus on correcting sagittal deformities, relieving postoperative clinical symptoms, and improving quality of life during fusion surgery., (Copyright © 2021 Elsevier Inc. All rights reserved.)

There is a lack of studies comparing the effects of different exercise types in patients with thoracic hyperkyphosis. Twenty-four subjects were divided into three groups: corrective exercise, resistance exercise, and physical therapy. The groups performed their respective interventions, two times per week for three months. Clinical outcomes, including the value of Cobb's angle, cervical muscle strength and endurance, and the cross-sectional area of the cervical deep muscles were measured pre- and post-intervention. There was a significant difference in the changes in the thoracic Cobb's angle between the groups (P < 0.001). The corrective exercise group revealed a significantly superior increase in muscle strength and endurance between pre- and post-intervention (P < 0.012). There was a significant difference in the cross-sectional area of the cervical deep muscles included longus capitis and multifidus between the groups (P < 0.036 and 0.007, respectively). The corrective exercise group showed the most significant increase in cross-sectional area between pre- and post-intervention (P < 0.012). A corrective exercise program is a more effective intervention than traditional resistance exercise and physical therapy for improving the thoracic Cobb's angle, cervical muscle strength and endurance, and the cross-sectional area of the deep muscles in patients with thoracic hyperkyphosis.Trial registration: KCT0005292.

Study Design: Retrospective cohort study., Objective: The aim of this study was to compare the incidence of proximal junctional kyphosis (PJK), proximal junctional failure (PJF), and clinical outcomes of patients who did and did not receive posterior ligament complex (PLC) augmentation using a semitendinosus allograft when undergoing long-segment posterior spinal fusion for adult spinal deformity., Summary of Background Data: Clinical research on the augmentation of the PLC to prevent PJK and PJF has been limited to small case series without a comparable control group., Methods: From 2014 to 2019, a consecutive series of patients with adult spinal deformity who underwent posterior long-segment spinal fusion with semitendinosus allograft to augment the PLC (allograft) or without PLC augmentation (control) were identified. Preoperative and postoperative spinopelvic parameters were measured. PJK, PJF, and Oswestry Disability Index (ODI) scores were recorded and compared between the two groups. Univariate and multivariate analysis was performed. P ≤ 0.05 was considered significant., Results: Forty-nine patients in the allograft group and 34 patients in the control group were identified. There were no significant differences in demographic variables or operative characteristics between the allograft and control group. Preoperative and postoperative spinopelvic parameters were also similar between the two groups. PJK was present in 33% of patients in the allograft group and 32% of patients in the control group (P = 0.31). PJF did not occur in the allograft group, whereas six patients (18%) in the control group developed PJF (P = 0.01). Postoperative absolute ODI was significantly better in the allograft group (P = 0.007)., Conclusion: The utilization of semitendinosus allograft tendon to augment the PLC at the upper instrumented vertebrae in patients undergoing long-segment posterior spinal fusion for adult deformity resulted in a significant decrease in PJF incidence and improved functional outcomes when compared to a cohort with similar risk of developing PJK and PJFLevel of Evidence: 3., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background: This study was undertaken to establish a rat bipedal walking model of cervical kyphosis (CK) associated with chronic forward flexed neck and assess the effects of chronic forward flexed neck on endplate chondrocytes., Methods: Forty-eight 1-month-old Sprague-Dawley rats were randomly divided into 3 groups: forward flexed neck group (n = 16), bipedal group (n = 16), and normal group (n = 16). Cervical curves were analyzed on a lateral cervical spine X-ray using Harrison's posterior tangent method before the experiment and at 2-week intervals for a 6-week period. Histologic changes in cartilaginous endplate chondrocytes were observed using hematoxylin and eosin (H&E) staining, transmission electron microscopy (TEM), and terminal deoxyribonucleotidyl transferase (TdT)-mediated dUTP nick-end labeling., Results: Radiographic findings suggested a significantly decreased cervical physiological curvature in the forward flexed neck group over the 6-week follow-up; normal cervical curves were maintained in other groups. The average cervical curvature (C2-C7) was - 7.6 ± 0.9° in the forward flexed neck group before the experiment, - 3.9 ± 0.8° at 2 weeks post-experiment, 10.7 ± 1.0° at 4 weeks post-experiment, and 20.5 ± 2.1° at the last follow-up post-experiment. Histologically, results of H&E staining unveiled that cartilaginous endplate chondrocytes were arranged in an irregular fashion, with the decreased number at the observation period; the incidence of apoptotic cells in the forward flexed neck group was noticeably higher at the 6-week follow-up than that in other groups., Conclusions: CK developed as the result of chronic forward flexed neck. Histologic changes suggested that chondrocyte apoptosis may play a critical role in the development of cervical kyphotic deformity associated with chronic forward flexed neck.

Magnetically controlled growing rods (MCGR) are commonly implanted for the treatment of early-onset scoliosis. While most authors report favorable short-term results, little is known about long-term deformity correction. This prospective cohort study assesses spinal deformity control in a homogeneous spinal muscular atrophy (SMA) patient group treated with MCGR implants, a standardized lengthening protocol and a minimum follow-up of four years. 17 SMA patients with progressive scoliosis were treated with MCGR implanted parallel to the spine with rib-to-pelvis fixation. Radiologic measurements were performed before and after MCGR implantation and during external lengthening procedures. These included measurements of the scoliotic curve, kyphosis, lordosis, pelvic obliquity and the spinal length. Additional clinical data of the complications were also analyzed. 17 children (mean age 7.4 years) were surgically treated and underwent a total of 376 lengthenings. Complication rates were 3.5% in respect to all interventions or 41% of the patients had complications during 3.5% of the lengthening sessions. The initial implantation significantly reduced the main scoliotic curve by 59%, with the correction remaining constant throughout the follow-up. Pelvic obliquity was also significantly and permanently corrected by 72%, whereas kyphosis and lordosis were not influenced. The spinal length could be significantly increased mostly during the first year of treatment. Bilateral implantation of MCGRs for correction of spinal deformity in children with SMA showed no decrease of the lengthening potential during a four-year follow-up. Therefore, the previously described 'law of diminishing returns' could not be applied to this patient population.Level of Evidence/Clinical relevance: Therapeutic Level IV.

Study Design: A cross-sectional radiological study., Objectives: We aimed to examine the degenerative changes of sagittal alignment in patients with Roussouly type 1. Roussouly type 1 is unique in shape, characterized by short lumbar lordosis (LL) with the apex at L5 and thoracolumbar kyphosis (TLK). Because of the unique shape of sagittal alignment and the small pelvic incidence (PI) in Roussouly type 1, the degenerative changes of sagittal alignment may differ., Methods: A total of 145 patients with Roussouly type 1 were recruited and distributed into three age groups; Group I (N = 40) were young patients (20-40 years of age), Group II (N = 47) were middle-aged patients (45-60 years of age), and Group III (N = 48) were elderly patients (>65 years of age). Sagittal parameters including sagittal vertical axis (SVA), PI, pelvic tilt (PT), L1S1 LL, L4S1 LL, thoracic kyphosis (TK), and TLK were measured using Surgimap ® software. The occurrence of lumbar retrolisthesis was also examined., Results: The SVA, PI, PT, L1S1 LL, L4S1 LL, TK, and TLK in group I were - 25.9° ± 23.4 mm, 37.1° ± 5.3°, 10.3° ± 5.5°, 42.7° ± 8.8°, 35.5° ± 6.9°, 29.5° ± 23.5°, and 9.7° ± 5.9°, respectively. Among the Groups I, II, and III, there was a stepwise increase in the SVA, PT, TLK, and lumbar retrolisthesis (all P < 0.001). The PI, L4S1 LL, and TK were identical among the three groups., Conclusions: Degenerative changes of Roussouly type 1 include increase in the SVA, PT, TLK, and lumbar retrolisthesis, while the PI, L4S1 LL, and TK remain unchanged., Level of Evidence: Level IV.

Study Design: Retrospective study., Objective: To analyze the relationships between three-dimensional (3D) measurements of spinal deformity and Scoliosis Research Society-22 (SRS-22) scores in preoperative patients with major thoracic adolescent idiopathic scoliosis (AIS). Previous studies reported 2D measurements were not or only weakly correlated with preoperative SRS-22 scores. However, 2D measures do not always accurately represent the 3D deformity., Methods: A multicenter prospective registry of surgically treated AIS patients was reviewed for patients with right major thoracic AIS (Lenke type 1-4) who underwent biplanar radiography and completed the SRS-22 questionnaire preoperatively. For the 3D measurements, two reference frames were utilized: global (gravity/patient-based) and local (vertebra/disc-based). To obtain regional measurements, the individual segments in the appropriate reference plane were summed between the levels of interest. Patients were divided into two groups for each SRS-22 domain according to their scores: low (< 4) and high (≥ 4) score groups. Group differences and correlations with SRS-22 scores were analyzed with p < 0.01 as the threshold for significance., Results: There were 405 eligible patients (mean age, 14.4 years). The mean 3D thoracic curve was 59° (45°-115°). The only significant group difference of 3D measurements occurred in the local lumbar lordosis (LL) with a small mean difference (- 3.4°, p = 0.008) in the mental health domain. In the correlation analyses, global and local thoracic kyphosis (TK) and TK/LL ratio demonstrated significant, but weak, correlations with function and total scores (|r|< 0.2, p < 0.01)., Conclusion: 3D measurements of scoliosis severity have only weak associations with preoperative SRS-22 scores, which might indicate a limit to the discriminative capacity of the SRS-22 within surgical range major thoracic AIS curves. Interestingly, the sagittal plane was the principle 3D plane in which significant correlations existed., Level of Evidence: II, prognostic.

Congenital scoliosis is defined by the presence of structural anatomical malformations that arise from failures of vertebral formation or segmentation before and after birth. The understanding of genetic background and key genes for congenital scoliosis is still poor. We herein report that the excess expression of plasminogen activator inhibitor-1 (Pai-1) induced by the upregulation of miR-224-5p is involved in the pathogenesis of congenital kyphoscoliosis through impaired osteoblast differentiation. We first investigated the variety and progression of abnormalities of the lumbar spines in Ishibashi (IS) rats, a rat model of congenital kyphoscoliosis. The rats had already shown fusion and division of the primary ossification center at postnatal day 4. Over time, the rats showed various abnormalities of the lumbar spine, including the fusion of the annular epiphyseal nucleus. At postnatal day 42, spinal curvature was clearly observed due to the fusion of the vertebral bodies. Using a microRNA array, we found that the expression of miR-224-5p was increased in the lumbar spine of the rats at postnatal day 4. The expression of Pai-1, which is involved in osteoblast differentiation regulated by miR-224-5p, was also increased, while the levels of type I collagen, a marker of osteoblast differentiation, were decreased in the lumbar spine. These results indicate that the aberrant expression of miRNA-224-5p and its target genes is involved in the impaired osteoblast differentiation and may provide a partial molecular explanation for the pathogenesis of congenital scoliosis.

Study Design: This was a retrospective analysis of a prospectively collected consecutive case series of patients with adult spinal deformity (ASD)., Objective: This study aimed to investigate the impact of the geometrical sagittal shape of the corrected spine on the development of proximal junctional kyphosis (PJK). Several studies have documented risk factors for PJK in ASD surgery. Geometrical assessment is vital for evaluating sagittal spinal deformity. It is essential to assess the postoperative geometrical shape of the spine and the location of the correction in the spine to decrease postoperative junctional stress and PJK., Methods: Consecutive patients with ASD who underwent corrective fusion with long constructs to the pelvis were included. Patients with neuromuscular disease, congenital and adolescent scoliosis, infection, and spinal tumor were excluded. We investigated the spinopelvic and geometrical parameters of the whole spine. The locations of the thoracic and lumbar apical vertebrae and the inflection vertebrae (IV), where the curvature of the associated adjacent vertebral bodies changes from kyphosis to lordosis, were investigated. The subjects were divided into PJK included patients who underwent revision surgery for junctional failure or with a change in proximal junctional angle ≥ 20°, and non-PJK groups., Results: A total of 139 patients (mean age, 69.6 years; range 18-82 years) were included. There were 47 and 92 patients in the PJK and non-PJK groups, respectively. The IV were located significantly cranial and posterior, the lumbar apex were located significantly posterior in the PJK group at the immediate postoperative time points. The significant risk factors for PJK on binary logistic regression were cranial IV and posterior lumbar apical vertebrae. The incidence of PJK in patients with IV at T12 or cranial tends PJK significantly higher (69%) than at L1 or caudal (26%)., Conclusions: Geometrical spinal shape should be taken into account to reduce the rate of postoperative mechanical complications., Level of Evidence: Level of evidence III.

Background: Kyphoscoliotic Ehlers-Danlos syndrome (kEDS) is a rare autosomal recessive connective tissue disorder characterized by progressive kyphoscoliosis, congenital muscular hypotonia, marked joint hypermobility, and severe skin hyperextensibility and fragility. Deficiency of lysyl hydroxylase 1 (LH1) due to mutations of PLOD1 (procollagen-lysine, 2-oxoglutarate 5-dioxygenase 1) gene has been identified as the pathogenic cause of kEDS (kEDS-PLOD1). Up to now, kEDS-PLOD1 has not been reported among Chinese population., Case Presentation: A 17-year-old Chinese male patient presenting with hypotonia, joint hypermobility and scoliosis was referred to our hospital. After birth, he was found to have severe hypotonia leading to delayed motor development. Subsequently, joint hypermobility, kyphoscoliosis and amblyopia were found. Inguinal hernia was found at age 5 years and closed by surgery. At the same time, he presented with hyperextensible and bruisable velvety skin with widened atrophic scarring after minor trauma. Dislocation of elbow joint was noted at age of 6 years. Orthopedic surgery for correction of kyphoscoliosis was performed at age 10 years. His family history was unremarkable. Physical examination revealed elevated blood pressure. Slight facial dysmorphologies including high palate, epicanthal folds, and down-slanting palpebral fissures were found. He also had blue sclerae with normal hearing. X-rays revealed severe degree of scoliosis and osteopenia. The Echocardiography findings were normal. Laboratory examination revealed a slightly elevated bone turnover. Based on the clinical manifestations presented by our patient, kEDS was suspected. Genetic analysis revealed a novel homozygous missense mutation of PLOD1 (c.1697 G > A, p.C566Y), confirming the diagnosis of kEDS-PLOD1. The patient was treated with alfacalcidol and nifedipine. Improved physical strength and normal blood pressure were reported after 12-month follow-up., Conclusions: This is the first case of kEDS-PLOD1 of Chinese origin. We identified one novel mutation of PLOD1, extending the mutation spectrum of PLOD1. Diagnosis of kEDS-PLOD1 should be considered in patients with congenital hypotonia, progressive kyphoscoliosis, joint hypermobility, and skin hyperextensibility and confirmed by mutation analysis of PLOD1.

Purpose: Ankylosing spondylitis and hereditary hypophosphatemia with long-term high dose supplementation of phosphorous and calcitriol can both lead to severe structural abnormalities of the vertebrae. Impairment of spinal mobility and spinal deformity may ultimately necessitate surgical treatment. A severe fixed hyperkyphosis in a patient with ankylosing spondylitis is a surgically demanding condition, therefore, the indication for surgical treatment should be thoroughly considered and chosen individually., Methods: This is an uncommon case with a combination of a severe fixed hyperkyphosis with a Cobb-angle of 105 degrees between Th2 and L4 in an adult male patient suffering from ankylosing spondylitis and X-linked hypophosphatemia with surprisingly massive osteopetrosis. In this paper, the coexisting conditions of late-stage ankylosing spondylitis and long-term treated hereditary hypophosphatemia are highlighted. The surgical treatment with different techniques, complications, and results are well explained., Results: A normal gait and stand were achieved by a long posterior fusion with 3 pedicle subtraction osteotomies on L1, L3, and L5. The surgical correction was performed in 3 stages. Postoperative the patient was administered to a rehabilitation center for 3 months. The hyperkyphosis, the C7 plumbline, and the pelvic retroversion were corrected., Conclusions: Surgical treatment of a severe fixed hyperkyphosis due to ankylosing spondylitis is technically demanding but can be successfully achieved if all surgical challenges and comorbidities are adequately addressed including intraoperative surprising findings like osteopetrotic bone in a patient with hereditary hypophosphatemia as in our case.

Purpose: As the most poorly tolerated and debilitating form of spinal malalignment, sagittal imbalance is becoming an increasingly recognized cause of pain and disability in adults. However, there is evidence showing that sagittal imbalance has a weak or no correlation with health-related quality-of-life (HRQoL) outcomes. The objective of this study was to describe the direct factor associated with HRQoL in terms of Oswestry Disability Index (ODI) assessment., Patients and Methods: This study retrospectively evaluated the clinical and radiographic information of 179 elderly patients with degenerative lumbar disorders and suboptimal sagittal standing posture (sagittal vertical axis>50 mm). Patient-reported outcomes were assessed using ODI. Patients with ODI≥40% were assigned to Group D (disability), while those with ODI<40% were assigned to Group ND (non-disability)., Results: Compared with Group ND (n=104), patients in Group D (n=75) had greater thoracolumbar kyphosis, pelvic incidence-lumbar lordosis (PI-LL), sagittal vertical axis (SVA), T1 pelvic angle, and fat infiltration, and smaller LL and muscle mass ratio. Pearson analysis revealed a high correlation between the percentage of fat infiltrated and ODI (r=768, P <0.01) and moderate correlation between SVA and ODI (r=0.408, P <0.001). Linear regression results indicated that fat infiltration was an independent factor associated with ODI. ODI significantly correlated with SVA in patients with major fat infiltration (r=0.328, P =0.001), while having no correlation with SVA in those with moderate or minor fat infiltration (r=0.083, P =0.464)., Conclusion: Lumbar muscle fat infiltration is an independent factor associated with the living quality in terms of ODI assessment in the elderly population with degenerative lumbar disorders, which has more correlations with ODI scores than the sagittal imbalance. The relationship between HRQoL outcomes and sagittal imbalance depends on the quality of lumbar muscle., Competing Interests: The authors have no conflicts of interest to declare for this work., (© 2020 Zhu et al.)

Background: The importance of sagittal alignment in healthy individuals and in reconstructive spinal surgery has been studied over the last 15 years. The aim of the present study was to assess the long-term effects of abnormal sagittal alignment on hardware after posterior thoracolumbar spinal fusion., Methods: Patients who had undergone revision surgery (revision cohort, n = 34) due to breakage of their implants were compared retrospectively with patients who had intact implants at the final follow-up investigation after a long posterior thoracolumbar and/or lumbar spinal fusion (control cohort, n = 22). Clinical data and radiological parameters including the sagittal vertical axis (SVA), pelvic incidence (PI), lordosis gap (LG), pelvic tilt (PT), sacral slope (SS), lumbar lordosis (LL), thoracic kyphosis (TK), and the femoral obliquity angle (FOA) were assessed on full-spine lateral radiographs obtained in regular standing position. Data were analysed using descriptive statistics, parametric and non-parametric inferential statistics., Results: Patients in the breakage group (female n = 21, male n = 9, mean age 60.9 ± 15.6 years) had a higher anterior shift of the C7 plumb line (SVA) (p = 0.02), retroversion of the pelvis (PT) (p < 0.001), PI-LL mismatch (LG) (p = 0.001), and PI (p = 0.002) than the intact group (female n = 10, male n = 12, mean age 65.7 ± 12.4 years). No significant difference was registered between groups in regard of SS, LL, TK, FOA, and the mean number of comorbidities., Conclusion: Failure of restoration of the SVA and the LG to the acceptable ranges, especially in patients with a high PI, may be regarded as a risk factor for the long-term failure of implants after posterior thoracolumbar spinal fusion.

Background: Severe kyphotic deformities carry high risk for neurological injuries as osteotomies are often required for correction. Surgeons often utilize a staged approach for dealing with these conditions starting with a period of halo traction to stretch tight soft tissues and partially correct the deformity, followed by surgery. Halo traction is a relatively safe procedure and complications are uncommon. We report a unique case of iatrogenic fracture of the cervical spine during gradual halo traction for deformity correction of a severe cervical kyphosis., Case Presentation: An 80-year-old female with previous cervical spine tuberculosis infection and C5-C6 anterior spinal fusion developed severe cervical kyphosis of 64° from C2-C6 and neck pain requiring deformity correction surgery. Gradual increase in traction weight was applied, aiming for a maximum traction weight of 45 pounds or half body weight. During the 1st stage halo-gravity traction, sudden neck pain and a loud cracking sound was witnessed during increase of the traction weight to 14 pounds. Imaging revealed a fracture through the C4 and reduction in kyphosis deformity to 11° from C2-C6. There was no neurological deficit. No further traction was applied and the patient underwent an in-situ occipital to T3 fusion without osteotomies. At 3-year follow-up, the patient was symptom-free and radiographs showed solid fusion and maintenance of alignment., Conclusions: Iatrogenic fracture may occur with halo traction. Elderly patients with osteoporotic and diseased bone should be closely monitored during the treatment. A fracture without complications was a fortunate complication as the patient was able to avoid any high-risk osteotomies for deformity correction., Level of Evidence: IV.

Backgrounds: Cervical posterior decompression surgery is used to relieve ventral compression indirectly by incorporating a backward shift of the spinal cord, and this indirect decompression is bound to be limited. This study aimed to determine the decompression limit of posterior surgery and the effect of the decompression range., Methods: We retrospectively reviewed the data of 129 patients who underwent cervical open-door laminoplasty through 2008 to 2012 and were grouped as follows: C4-C7 (n = 11), C3-C6 (n = 61), C3-C7 (n = 32), and C2-C7 (n = 25). According to the relative location of spinal levels within a decompression range, the type of decompression at a given level was categorized as external decompression (ED; achieved at the levels located immediately external to the decompression range margin), internal decompression (ID; achieved at the levels located immediately internal to the decompression range margin), and central decompression (CD; achieved at the levels located in the center, far from the decompression range margin). The vertebral-cord distance (VCD) was used to evaluate the decompression limit. The C2-C7 angle and VCD on post-operative magnetic resonance images were analyzed and compared between groups. The relationship between VCD and decompression type was analyzed. Moreover, the relationship between the magnitude of the ventral compressive factor and the probability of post-operative residual compression at each level for different decompression ranges was studied., Results: There was no significant kyphosis in cervical curvature (> -5°), and there was no significant difference among the groups (F = 2.091, P = 0.105). The VCD of a specific level depended on the decompression type of the level and followed this pattern: ED < ID < CD (P < 0.05). The decompression type of a level was sometimes affected by the decompression range. For a given magnitude of the ventral compressive factor, the probability of residual compression was lower for the group with the larger VCD at this level., Conclusions: Our study suggests that the decompression range affected the decompression limit by changing the decompression type of a particular level. For a given cervical spinal level, the decompression limit significantly varied with decompression type as follows: ED < ID < CD. CD provided maximal decompression limit for a given level. A reasonable range of decompression could be determined based on the relationship between the magnitude of the ventral compressive factor and the decompression limits achieved by different decompression ranges.

Objective: To assess the role of the difference between the proximal junctional angle (PJA) and rod contouring angle (RCA) (PJA-RCA) in the development of postoperative proximal junctional angle (PJK) in Lenke I and II adolescent idiopathic scoliosis (AIS) patients., Methods: We performed a retrospective analysis of 84 Lenke I and II AIS patients who underwent posterior segmental spinal instrumentation and fusion between 2012 and 2018 (minimum follow-up of 1.5 years and an average follow-up of 2 years) at a single institution. The full-spine x-ray films taken at the preoperative, postoperative, and final follow-ups were measured for each patient. The radiographic parameters were compared between the PJK and non-PJK groups, and binary logistic regression with forward elimination (conditional) was also performed to identify the risk factors for the occurrence of PJK., Results: Among the 84 patients (mean age: 14.63 ± 1.33 years), the overall incidence of PJK was 23.81%. The PJK group showed a larger preoperative pelvic incidence (55.66° ± 8.66° vs. 50.29°±8.27°, P = 0.045), thoracic kyphosis (TK) (32.44° ± 5.60° vs. 27.19° ± 5.14°, P = 0.007) and sagittal vertical axis (SVA) (40.99 ± 21.82 mm vs. 18.13 ± 28.64 mm, P = 0.013) than the non-PJK group. Postoperatively, the PJK group showed a larger decrease in the TK (-10.62° ± 3.19° vs. -5.56° ± 1.17°, P < 0.001) and SVA (-24.28 ± 18.22 mm vs. -10.83 ± 15.02 mm, P = 0.007). In addition, the PJK group had significantly larger postoperative PJA (9.83° ± 2.64° vs. 5.77° ± 3.06°, P < 0.001) and postoperative PJA-RCA (6.56° ± 3.69° vs. 1.55° ±3.32°, P < 0.001). The proportion of patients with a PJA-RCA value greater than 5° was significantly larger in the PJK group (65.00% vs. 18.75%, P < 0.001). At the last follow-up, the PJK patients had significantly larger TK (35.11° ± 5.51° vs. 26.53° ± 4.71°, P < 0.001) and SVA (22.83 ±23.12 mm vs. 3.46 ±26.24 mm, P = 0.038). Binary logistic regression analysis showed that decreases in TK and postoperative PJA-RCA were the primary contributors to PJK in patients with AIS., Conclusions: Large postoperative PJA-RCA and decreased TK are risk factors for PJK in Lenke I and II AIS patients, especially those with PJA-RCA greater than 5°, and the occurrence of PJK should be highly considered., (Copyright © 2020 Elsevier Inc. All rights reserved.)

BACKGROUND This retrospective clinical study aimed to compare the efficacy of preoperative halo-gravity traction with postoperative halo-femoral traction after posterior spinal release in corrective surgery for patients with severe kyphoscoliosis. MATERIAL AND METHODS A retrospective clinical study included patients who underwent elective corrective surgery for severe kyphoscoliosis (N=60) between 2013 and 2015. Two patient groups were compared, the postoperative halo-femoral traction after posterior spinal release (R-HF) group (N=30) and the preoperative halo-gravity traction (HGT) group (N=30). Demographic and clinicopathological data included age, gender, Cobb angle, degree of spinal curvature, history of osteotomy, and etiological factors. Patients in the two study groups were matched. Postoperative surgical outcome was evaluated by the radiographic coronal Cobb angle, global kyphosis, coronal balance, and the sagittal vertical axis (SVA). Clinical outcome was assessed using the Scoliosis Research Society Outcomes Questionnaire (SRS-22). RESULTS The preoperative Cobb angle was similar between the R+HF group and the HGT group (123.5±12.7° vs. 123.1±14.1°; P=0.909). Following postoperative traction, a significantly higher correction rate was found in the R+HF group than the HGT group (31.8±7.8% vs. 19.3±12.9%; P=0.001). The postoperative correction rate in the R+HF group was significantly higher than the HGT group (44.7±7.8% vs. 39.0±12.8%; P=0.042). In both study groups, the postoperative SRS-22 scores were significantly improved with no statistical difference between the two groups, and no neurological complications occurred. CONCLUSIONS Patients with severe kyphoscoliosis who underwent postoperative halo-femoral traction after posterior spinal release achieved satisfactory radiographic correction.

Background Context: The head's center of gravity (COG) plumb line (PL) and C7 PL could be simultaneously positioned over the pelvis in adult spinal deformity with normal cervical mobility. However, the position of the head in relation to the global spinal alignment has yet to be investigated in ankylosing spondylitis (AS) patients with thoracolumbar kyphosis., Purpose: The objective of this study was to analyze the position of head in relation to the global spinal alignment in AS-related thoracolumbar kyphosis., Study Design/setting: Retrospective single-center study., Patient Sample: AS patients who underwent lumbar pedicle subtraction osteotomy for thoracolumbar kyphosis from January 2010 to August 2016 were reviewed. Only patients with a visible ear canal on the preoperative, immediate postoperative, and final follow-up radiographs were included., Outcome Measures: The chin-brow angle, cervical range of motion (ROM), lumbar lordosis (LL), thoracolumbar angle, thoracic kyphosis (TK), L1 pelvic angle, pelvic incidence (PI), pelvic tilt (PT), sacral slope (SS), TK+LL+PI, PI-LL, maximal kyphosis (MK), deformity angular rate, T1 pelvic angle, T1 tilt, spinosacral angle, sagittal vertical axis (SVA) COG-C7, SVA COG-femoral head (FH), and SVA C7-S were evaluated. Data regarding the health-related quality of life, including the Oswestry disability index (ODI) and visual analogue scale (VAS)-back score, were also collected preoperatively, 2 years postoperatively, and at the latest follow-up., Methods: The cohort was divided into patients with occiput-trunk (OT) concordance or with OT discordance according to the SVA COG-C7 ≤30 mm or >30 mm, respectively. There was no funding in this study and there are no conflict of interest-associated biases., Results: A total of 43 patients (36 males and 7 females) with a mean age of 34.2 years (range, 18-59 years) were identified. There were 17 patients accompanied with OT concordance and 26 patients with OT discordance preoperatively. The cervical ROM was significantly lower (24.0° vs 56.1°, p<.001) and SVA COG-C7 was significantly larger (71.7 mm vs 7.4 mm, p<.001) in patients with OT discordance. Furthermore, the PT was larger (41.0° vs 33.5°, p=.010) in patients with OT discordance. After surgery, the whole cohort showed an improvement in LL (-8.6° vs -52.8°, p<.001). Moreover, the CBVA (25.4° vs 1.3°, p<.001) and SVA COG-C7 (46.2 mm vs 21.6 mm, p<.001) were significantly decreased following lumbar PSO. There were 13 patients accompanied with OT discordance postoperatively, and the cervical ROM was still lower (22.5° vs 42.8°, p=.024) in these patients. Postoperative PT was larger (26.5° vs 20.1°, p=.033) in the patients with OT discordance. At the latest follow-up, there were 17 patients accompanied with OT discordance. In these 17 patients, the cervical ROM was significantly lower (21.0° vs 47.0°, p=.001) and PT was significantly higher (26.2° vs 19.2°, p=.012). The ODI and VAS-back scores demonstrated no significant differences between the two groups preoperatively, 2 years postoperatively, or at the latest follow-up., Conclusions: OT discordance in AS-related thoracolumbar kyphosis could be caused by the reduced cervical ROM. To maintain global spinal balance, the pelvis rotated further backward in response to the larger SVA COG-C7. Moreover, the larger SVA COG-C7 could be decreased after the lumbar PSO. Although there were radiographic differences between the patients with OT concordance and with OT discordance, there was no difference in clinical outcomes, and that a larger sample size and longer follow-up are needed., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Selective fusion of double curves in patients with scoliosis is considered to spare fusion levels. In 2011, we studied the lumbosacral takeoff angle, defined as the angle between the center-sacral vertical line and a line through the centra of S1, L5, and L4. The lumbosacral takeoff angle was shown to moderately correlate with the lumbar Cobb angle, and a predictive equation was developed to predict the lumbar Cobb angle after selective fusions. The purposes of the present study were to validate that equation in a separate cohort and to assess differences in outcomes following selective and nonselective fusion., Methods: Patients with Lenke 1B, 1C, 3B, or 3C curve patterns undergoing fusion (both selective and nonselective) with pedicle screw constructs and a minimum of 2 years of follow-up were included. Selective fusion was defined as a lowest level of fixation cephalad to or at the apex of the lumbar curve. To validate the previously derived equation, we used this data set and analysis of variance to check for differences between the actual and calculated postoperative lumbar Cobb angles. Pearson correlation, multiple linear regression, and t tests were used to explore relationships and differences between the selective and nonselective fusion groups., Results: The mean calculated postoperative lumbar Cobb angle (and standard deviation) (22.35° ± 3.82°) was not significantly different from the actual postoperative lumbar Cobb angle (21.08° ± 7.75°), with an average model error of -1.268° (95% confidence interval, -2.649° to 0.112°). The preoperative lumbar Cobb angle was larger in patients with deformities that were chosen for nonselective fusion (50.2° versus 38.9°; p < 0.001). Performing selective fusion resulted in a 3.5° correction of the lumbosacral takeoff angle (p < 0.001), whereas nonselective fusion resulted in a 9.3° correction (p < 0.001)., Conclusions: The lumbosacral takeoff angle can be used to predict the residual lumbar Cobb angle and may be used by surgeons to aid in the decision between selective and nonselective fusion. The change in the lumbosacral takeoff angle following selective fusion is small. Improvement in the lumbosacral takeoff angle and coronal balance is greater in association with nonselective fusion., Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Calcium signaling plays a central role in bone development and homeostasis. Store operated calcium entry (SOCE) is an important calcium influx pathway mediated by calcium release activated calcium (CRAC) channels in the plasma membrane. Stromal interaction molecule 1 (STIM1) is an endoplasmic reticulum calcium sensing protein important for SOCE. We generated a mouse model expressing the STIM1 R304W mutation, causing Stormorken syndrome in humans. Stim1 R304W/R304W mice showed perinatal lethality, and the only three animals that survived into adulthood presented with reduced growth, low body weight, and thoracic kyphosis. Radiographs revealed a reduced number of ribs in the Stim1 R304W/R304W mice. Microcomputed tomography data revealed decreased cortical bone thickness and increased trabecular bone volume fraction in Stim1 R304W/R304W mice, which had thinner and more compact bone compared to wild type mice. The Stim1 R304W/+ mice showed an intermediate phenotype. Histological analyses showed that the Stim1 R304W/R304W mice had abnormal bone architecture, with markedly increased number of trabeculae and reduced bone marrow cavity. Homozygous mice showed STIM1 positive osteocytes and osteoblasts. These findings highlight the critical role of the gain-of-function (GoF) STIM1 R304W protein in skeletal development and homeostasis in mice. Furthermore, the novel feature of bilateral subgingival hair growth on the lower incisors in the Stim1 R304W/R304W mice and 25 % of the heterozygous mice indicate that the GoF STIM1 R304W protein also induces an abnormal epithelial cell fate., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Objective: This study was carried out to analyze the surgical effect of cervical spine sagittal alignment for patients with Hirayama disease (HD)., Methods: Forty-four subjects were retrospectively analyzed for the parameters of cervical spine sagittal alignment. The case group consisted of 23 patients with HD, whereas the control group consisted of 21 healthy adolescent subjects. Pre- and postoperative cervical spine sagittal parameters of the patients with HD were collected; the cervical sagittal parameters of the healthy adolescent subjects were also collected. Sagittal alignment parameters were compared between the patients with HD and the healthy adolescent subjects, and between the pre- and postoperative parameters for the patients with HD., Results: Forty-four subjects completed the follow-up, with the average follow-up period being 18.0 months. No significant differences were detected between the HD and control groups for clinical parameters (P > 0.05). The preoperative HD group had smaller values compared with the control group in the sagittal parameters of C2-7 cervical lordosis (CL) angle, T1 slope, thoracic inlet angle (TIA), and cervical tilt angle (P < 0.05). For the patients with HD, the preoperative values were smaller compared with the postoperative HD values for the parameters of C2-7 CL angle, T1 slope, and cervical tilt angle (P < 0.05). We found no significant differences between the postoperative patients with HD and the healthy subjects, including C2-7 CL angle, C2-7 sagittal vertical axis, T1 slope, TIA, neck tilt angle, cervical tilt angle, and cranial tilt angle (P > 0.05)., Conclusions: Patients with HD have sagittal imbalance of the cervical spine compared with age-matched healthy adolescent subjects, and surgical treatment could correct the sagittal imbalance., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Study Design: Retrospective review of a multicenter, prospective database., Objective: Our aim was to compare complication rates and maintenance of radiographic correction at 2 years after posterior spinal fusion (PSF) with or without cross-links in patients with cerebral palsy (CP)-related scoliosis., Summary of Background Data: Cross-links are frequently used in PSF to correct scoliosis in patients with CP because they are thought to increase the stiffness and torsional rigidity of the construct., Methods: We reviewed the records of patients with CP who underwent primary PSF with or without cross-links between August 2008 and April 2015. Inclusion criteria were minimum follow-up of 2 years, availability of complications data (implant failure, surgical site infection, revision), and pre- and postoperative measurements of the major curve (measured using the Cobb method). The 256 patients included in this analysis had a mean age of 14.1 ± 2.7 years. Ninety-four patients had cross-links (57% using one cross-link; 43% using two cross-links) and 162 patients did not have cross-links. P < 0.05 was considered statistically significant., Results: The two groups did not differ significantly with regard to sex, age at surgery, preoperative menarche status, Gross Motor Function Classification System level, major curve magnitude, pelvic obliquity, kyphosis, and lordosis angles. There were no significant differences between groups in the correction achieved or the maintenance of correction at 2 years for the major curve, pelvic obliquity, kyphosis, or lordosis (all P > 0.05). Complication rates were similar between the cross-link group (16%, N = 15) and the non-cross-link group (14%, N = 22)., Conclusion: At 2 years after PSF to treat CP-related scoliosis, patients had no significant differences in the degree of correction achieved, the maintenance of correction, or the rate of complications between those whose fusion constructs used cross-links and those whose constructs did not., Level of Evidence: 3.

Purpose: This study aims to describe the spinopelvic sagittal alignment in transfemoral amputees (TFAs) from a radiologic study of the spine with a postural approach to better understand the high prevalence of low back pain (LBP) in this population., Methods: TFAs underwent X-rays with 3-D reconstructions of the full spine and pelvis. Sagittal parameters were analyzed and compared to the literature. Differences between TFAs with and without LBP were also observed., Results: Twelve subjects have been prospectively included (TFA-LBP group (n = 5) and TFA-NoP group (n = 7)). Four of the five subjects of the TFA-LBP group and two of the seven in TFAs-NoP group had an imbalanced sagittal posture, especially regarding the T9-tilt, significantly higher in the TFA-LBP group than in the TFA-NoP (p = 0.046). Eight subjects (6 TFA-NoP and 2 TFA-LBP) had abnormal low value of thoracic kyphosis (TK). Moreover, the mean angle of TK in the TFA-NoP group was lower than in the TFA-LBP group (p = 0.0511)., Conclusion: In the considered sample, TFAs often present a sagittal imbalance. A low TK angle seems to be associated with the absence of LBP. It can be hypothesized that this compensatory mechanism of the sagittal imbalance is the most accessible in this population. This study emphasizes the importance of considering the sagittal balance of the pelvis and the spine in patients with a TFA to better understand the high prevalence of LBP in this population. It should be completed by the analysis of the spinopelvic balance and the lower limbs in 3D. These slides can be retrieved under Electronic Supplementary Material.

Purpose: The thoracolumbar junction (TLJ) has not been explored in regard to its contribution to global sagittal alignment. This study aims to define novel sagittal parameters of the TLJ and to assess their roles within global sagittal alignment., Methods: Included for cross-sectional, retrospective analysis were asymptomatic volunteers and symptomatic patients who had undergone operation for adult spinal deformity. Unique sagittal parameters of the TLJ were measured using the midline of the T12-L1 disk space: The TLJ orientation [TLJO; thoracolumbar tilt (TLT) and slope (TLS)]. Thoracic kyphosis (TK; T5-12), C7-S1 sagittal vertical axis (SVA), lumbar lordosis (LL; L1-S1), sacral slope (SS), pelvic tilt (PT), and pelvic incidence (PI) were measured. Continuous variables were compared using the independent t test. Pearson correlations examined relationships between the parameters in each group. The asymptomatic TK was calculated using the measurement of the asymptomatic volunteer's TLJO by linear regression., Results: One hundred fifteen asymptomatic volunteers and 127 symptomatic patients were included. Only LL among the lumbopelvic parameters correlated with TK (asymptomatic volunteers: r = - 0.42; symptomatic patients: r = - 0.40). All the pelvic parameters have no direct correlation with TK in both groups. TLJO had stronger correlation with TK [asymptomatic volunteers: r = - 0.68 (TLS), r = 0.41 (TLT); symptomatic patients: r = - 0.56 (TLS), r = 0.44 (TLT)] than the lumbopelvic parameters. TLS correlated with LL (asymptomatic volunteers: r = 0.78; symptomatic patients: r = 0.73). Most pelvic parameters correlated with TLJO except for PI. The asymptomatic TK was estimated by the derived formula: 20.847 + TLS × (- 1.198)., Conclusion: The TLJO integrates the status of the lumbopelvic sagittal parameters and simultaneously correlates with thoracic and global sagittal alignment. These slides can be retrieved under Electronic Supplementary Material.

There are still controversies on characteristics and risk factors for proximal junctional kyphosis (PJK) in adult spinal deformity (ASD) patients. The objective of this study is to explore the characteristics and risk factors for PJK in ASD. A systematic online search in databases including PubMed, EMBASE, Web of Science, and the Cochrane Library was performed to identify eligible studies. OR and weight mean difference with 95% CI were used to evaluate characteristics and risk factors. A total of 31 studies were finally included. ASD patients with PJK had larger proximal junctional angle (PJA), thoracic kyphosis (TK), pelvic incidence minus lumbar lordosis (PI-LL), and sagittal alignment. Age, female gender, and low BMD/osteoporosis were demographic risk factors for PJK. Using hooks at upper instrumented vertebra (UIV) and the selection of UIV above T8 could reduce the occurrence of PJK, while pelvic fixation was significantly associated with increased occurrence of PJK. Preoperative LL, preoperative pelvic tilt (PT), preoperative LL-TK, preoperative PI-LL, preoperative sagittal vertical axis (SVA), preoperative global spine alignment (GSA), postoperative PJA, change in PJA, postoperative TK, change in LL, change in SVA, and postoperative GSA were identified as risk factors for PJK. In conclusion, PJK patients had larger PJA, larger TK, smaller PI-LL, and larger sagittal alignment. Older female ASD patients with low BMD/osteoporosis are more likely to suffer from PJK. We recommend the following: (1) using hooks at UIV; (2) UIV should be chosen above T8, and pelvic fixation should be avoided if possible; (3) ideal correction of sagittal alignment should be performed to prevent the occurrence of PJK.

Although congenital scoliosis is defined as a genetic disease characterized by a congenital and abnormal curvature of the spinal vertebrae, our knowledge of the genetic underpinnings of the disease is insufficient. We herein show that the downregulation of the retinol-retinoic acid metabolism pathway is involved in the pathogenesis of congenital scoliosis. By analyzing DNA microarray data, we found that the expression levels of genes associated with the retinol metabolism pathway were decreased in the lumbar spine of Ishibashi rats (IS), a rat model of congenital kyphoscoliosis. The expression of Adh1 and Aldh1a2 (alcohol dehydrogenase), two enzymes that convert retinol to retinoic acid in this pathway, were decreased at both the gene and protein levels. Rarα, a receptor of retinoic acid and bone morphogenetic protein 2, which play a central role in bone formation and are located downstream of this pathway, were also downregulated. Interestingly, the serum retinol levels of IS rats were higher than those of wild-type control rats. These results indicate that the adequate conversion from retinol to retinoic acid is extremely important in the regulation of normal bone formation and it may also be a key factor for understanding the pathogenesis of congenital scoliosis., (© 2019 Wiley Periodicals, Inc.)