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Abstract Background Anesthesia for spinal muscular atrophy (SMA) patients undergoing spinal deformity surgery is challenging. We report an unusual case of an SMA girl who developed severe intraoperative hypoxemia and hypotension during posterior spinal fusion related with surgical positioning. Case presentation A 13-yr-old girl diagnosed with SMA type 2, severe kyphoscoliosis and thoracic deformity was scheduled for elective posterior spinal fusion. She developed severe hypoxemia and profound hypotension intraoperatively in the prone position with surgical table tilted 45° to the right. Though transesophageal echocardiography (TEE) could not be performed due to limited mouth opening, her preoperative computed tomography revealed a severely distorted thoracic cavity with much reduced volume of the right side. A reasonable explanation was when the surgeons performed surgical procedure with the tilted surgical table, the pressure was directly put on the shortest diameter of the significantly deformed thoracic cavity, causing severe compression of the pulmonary artery, resulting in both hypoxemia and hypotension. The patient stabilized when the surgical table was tilted back and successfully went through the surgery in the leveled prone position. Conclusions Spinal fusion surgery is beneficial for SMA patients in preventing scoliosis progression and improving ventilation. However, severe scoliosis and thoracic deformities put them at risk of both hemodynamic and respiratory instability during surgical positioning. When advanced monitoring like TEE is not practical intraoperatively, preoperative imaging may help with differential diagnosis, and guide the surgical positioning to minimize mechanical compression of the thoracic cavity, thereby helping the patient complete the surgery safely.

Abstract Purpose The natural history of congenital scoliosis (CS) caused by hemivertebra varies greatly. This study aimed to explore the association between the morphology of hemivertebra and the severity of CS, since the diagnosis of the hemivertebra. Methods Patients with isolated (single fully segmented) hemivertebra were enrolled. The degree and progression of deformity were compared by three morphological parameters of hemivertebra, comprising whether the width of hemivertebra extends across the central vertical line of lower adjacent vertebra (midline); the lateral height ratio (LHR, lateral height of hemivertebra× 2/(lateral height of HV-1 plus HV + 1) with the cut-point being 0.9; and the sagittal position of hemivertebra that was divided into the lateral and posterolateral group. Results In total, 156 patients (mean age 9.7 ± 6.2 years, 81 males) were enrolled. The number of thoracic, thoracolumbar (T12/13-L1), and lumbar hemivertebrae were 63, 41, and 52, respectively. Hemivertebrae across the midline had larger scoliosis and kyphosis (58.3 ± 20.6° vs. 42.8 ± 15.0°, P

Abstract Background As the first gene therapy for spinal muscular atrophy (SMA), nusinersen is supposed to be administrated via intrathecal injection regularly for a lifetime. However, for SMA patients with severe spinal deformities, bony fusion following posterior spinal instrumentation sets great obstacles for the application of nusinersen. Therefore, efforts have been devoted to the exploration of appropriate approach for nusinersen administration. This study aims to evaluate the safety and reliability of unilateral interlaminar fenestration on the convex side during spinal fusion surgery for intrathecal nusinersen injection in SMA. Results SMA patients receiving posterior spinal fusion and interlaminar fenestration in Peking Union Medical College Hospital from January 2020 to October 2021 were retrospectively analyzed. 13 patients were included. Of the 13 patients, 10 were classified into SMA type II and 3 into SMA type III. Distal fusion to pelvis was undertaken in 11 patients; while L5 was selected as the lowest instrumented vertebra in the other 2 patients. All patients received interlaminar fenestration on the convex side only with an area of about 15 mm × 20 mm. Fenestration at L2–L3 level was performed in 6 patients; while L3–L4 level was selected for windowing in the remaining 7 patients. 9 of the 13 patients received lumbar puncture and intrathecal nusinersen administration during the 1-year follow-up, with an accumulative total of 50 times. All injections were performed successfully under ultrasound guidance, with no one transferred to radiographic assistance. No severe complications occurred after injection. Conclusions In SMA with severe scoliosis planning to receive posterior spinal fusion, unilateral lumbar interlaminar fenestration on the convex side provides a feasible and reliable access for intrathecal nusinersen administration after surgery.

Abstract Background Previous studies have analyzed paraspinal muscle imbalance in idiopathic scoliosis (IS) with methods including imaging, histology and electromyography. However, whether paraspinal muscle imbalance is the cause or the consequence of spinal deformities in IS remains unclear. Comparison of paraspinal muscle imbalance between IS and congenital scoliosis (CS) may shed some light on the causality of paraspinal muscle imbalance and IS. This study aimed to elucidate the generality and individuality of paraspinal muscle imbalance between IS and CS from gene expression. Methods Five pairs of surgical‐treated IS and CS patients were matched. Bilateral paraspinal muscles at the apex were collected for transcriptome sequencing. Differentially expressed genes (DEGs) between the convexity and concavity in both IS and CS were identified. Comparison of DEGs between IS and CS was conducted to discriminate IS‐specific DEGs from DEGs shared by both IS and CS. Bioinformatics analysis was performed. The top 10 hub genes in the protein–protein interaction (PPI) network of IS‐specific DEGs were validated by quantitative PCR (qPCR) in 10 pairs of IS and CS patients. Results A total of 370 DEGs were identified in IS, whereas 380 DEGs were identified in CS. Comparison of DEGs between IS and CS identified 59 DEGs shared by IS and CS, along with 311 DEGs specific for IS. These IS‐specific DEGs were enriched in response to external stimulus and signaling receptor binding in GO terms and calcium signaling pathway in KEGG pathways. The top 10 hub genes in the PPI network of IS‐specific DEGs include BDKRB1, PRH1‐TAS2R14, CNR2, NPY4R, HTR1E, CXCL3, ICAM1, ALB, ADIPOQ, and GCGR. Among these hub genes, the asymmetrical expression of PRH1‐TAS2R14 and ADIPOQ in IS but not CS were validated by qPCR. Conclusions Transcriptomic differences in bilateral paraspinal muscles between the convexity and concavity in IS share few similarities with those in CS.

Abstract Background Oculopharyngodistal myopathy (OPDM) is an autosomal dominant adult-onset degenerative muscle disorder characterized by ptosis, ophthalmoplegia and weakness of the facial, pharyngeal and limb muscles. Trinucleotide repeat expansions in non-coding regions of LRP12, G1PC1, NOTCH2NLC and RILPL1 were reported to be the etiologies for OPDM. Results In this study, we performed long-read whole-genome sequencing in a large five-generation family of 156 individuals, including 21 patients diagnosed with typical OPDM. We identified CGG repeat expansions in 5’UTR of RILPL1 gene in all patients we tested while no CGG expansion in unaffected family members. Repeat-primed PCR and fluorescence amplicon length analysis PCR were further confirmed the segregation of CGG expansions in other family members and 1000 normal Chinese controls. Methylation analysis indicated that methylation levels of the RILPL1 gene were unaltered in OPDM patients, which was consistent with previous studies. Our findings provide evidence that RILPL1 is associated OPDM in this large pedigree. Conclusions Our results identified RILPL1 is the associated the disease in this large pedigree.

Objectives: To investigate the challenges of patients with spinal muscular atrophy (SMA) during the Omicron variant COVID-19 pandemic. Design: A cross-sectional survey was conducted in China from January 02, 2023, to January 12, 2023, using a questionnaire that covered three aspects: (1) Demographic information; (2) SMA-related information; and (3) COVID-19-related information. We recruited patients with SMA from 33 provinces. The prevalence, course, and clinical manifestations of COVID-19 were calculated. The relationships between independent and outcome variables were investigated. Results: In total, 677 patients (male: 349; female: 328) were included in this study (average age = 11.40 years); 534 (78.88%) suffered from COVID-19. The most common symptoms were fever (95.51%), cough (57.87%), and sputum (49.44%). Of the infected patients, 91.57% recovered with at-home care, and 8.43% were hospitalized; 1.31% were admitted to the intensive care unit (ICU). A positive correlation was observed between the SMA severity and hospitalization rate. The ICU stay rate in patients with SMA type I was significantly higher than that in other SMA types. Conclusion: This is the first large sample survey to timely reveal the living situation of patients with SMA during the COVID-19 pandemic's Omicron variant. Patients with SMA type I should be paid more attention in terms of hospitalization and ICU stay.

Objective This study aims to compare the proteomic profiles of paraspinal muscle imbalance between idiopathic scoliosis (IS) and congenital scoliosis (CS). Methods Bilateral paraspinal muscles of 5 pairs of matched IS and CS patients were collected. Proteome patterns of paraspinal muscles were established. Differentially expressed proteins (DEPs) in paraspinal muscles between the convexity and the concavity were screened out. DEPs shared by both IS and CS and IS-specific DEPs were identified. Bioinformatic analyses of DEPs were performed. Results Among 105 DEPs identified in IS, 30 displayed predominant expression on the convexity, whereas other 75 exhibited predominant expression on the concavity. DEPs in IS were mainly enriched in calcium ion binding and DNA binding in gene ontology (GO) term and glycolysis/gluconeogenesis and purine metabolism in Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway. Among 48 DEPs identified in CS, 25 were predominantly expressed on the convexity and 23 on the concavity. DEPs in CS were mainly enriched in receptor activity and immune response in GO term and glycolysis/gluconeogenesis and cellular senescence in KEGG pathway. Comparison of DEPs between IS and CS identified only 8 proteins shared by both types of scoliosis. Among the 97 IS-specific DEPs, 28 were predominantly expressed on the convexity and 69 on the concavity. IS-specific genes were enriched in calcium ion binding and protein glycosylation in GO term and glycolysis/gluconeogenesis and hypertrophic cardiomyopathy in KEGG pathway. Conclusion IS and CS exhibit proteomic imbalance in bilateral paraspinal muscles but share few similarities. Paraspinal muscle imbalance in IS might not be the consequence of spinal deformities.

Objective Menstruation is considered a contraindication for elective surgery for higher operative blood loss. Progesterone is often used to postpone menstruation to avoid surgery during menstruation. This study aimed to explore whether using progesterone to postpone menstruation affects perioperative blood loss and complications in female patients with adolescent idiopathic scoliosis (AIS) who underwent posterior spinal fusion (PSF) surgery. Methods A retrospective study was performed for female patients diagnosed with AIS who underwent PSF surgery between March 2013 and January 2021. Patients scheduled to undergo PSF surgery from 2 days before menstruation to 3 days after menstruation were treated with progesterone preoperatively. The patients were divided into two groups according to progesterone use (progesterone injection group; control group). Demographic and surgical data including intraoperative blood loss (IBL), normalized blood loss (NBL), total blood loss (TBL), transfusion rate, perioperative complications, postoperative drainage time, postoperative hospital stay, and preoperative coagulation function data were collected. Results A total of 206 patients were included in the study. Among them, the progesterone injection group included 41 patients, with an average age of 14.8 years. While the control group included 165 patients, with an average age of 14.9 years. The two groups were matched for age, height, weight, operation time, Risser sign, correction rate, mean curve Cobb angle, bending Cobb angle, number of internal fixations, and number of fused levels (all P > 0.05). Regarding coagulation function, no significant differences were found in thrombin time, activated partial thromboplastin time, fibrinogen, prothrombin time, and platelet count between the two groups (all P > 0.05). IBL, NBL, and TBL were higher in progesterone injection group; however, the difference was nonsignificant (all P > 0.05). Transfusion rate, perioperative complications, postoperative drainage time, and postoperative hospital stay were not statistically different between groups (all P > 0.05). Conclusion Intramuscular injection of progesterone to avoid menstruation during PSF surgery did not affect perioperative blood loss and complications in AIS patients. It may be a safe method for AIS patients to avoid menstrual problems affecting the operation time and receive PSF surgery as scheduled.

Introduction Postoperative anaemia is prevalent in adult spinal deformity (ASD) surgery in association with unfavourable outcomes. Ferric derisomaltose, a novel iron supplement, offers a promising solution in rapidly treating postoperative anaemia. However, the clinical evidence of its effect on patients receiving spinal surgery remains inadequate. This randomised controlled trial aims to evaluate the safety and efficacy of ferric derisomaltose on postoperative anaemia in ASD patients.Methods and analysis This single-centre, phase 4, randomised controlled trial will be conducted at Department of Orthopaedics at Peking Union Medical College Hospital and aims to recruit adult patients who received ASD surgery with postoperative anaemia. Eligible participants will be randomly assigned to receive ferric derisomaltose infusion or oral ferrous succinate. The primary outcome is the change in haemoglobin concentrations from postoperative days 1–14. Secondary outcomes include changes in iron parameters, reticulocyte parameters, postoperative complications, allogeneic red blood cell infusion rates, length of hospital stay, functional assessment and quality-of-life evaluation.Ethics and dissemination This study has been approved by the Research Ethics Committee of Peking Union Medical College Hospital and registered at ClinicalTrials.gov. Informed consent will be obtained from all participants prior to enrolment and the study will be conducted in accordance with the principles of the Declaration of Helsinki. The results of this study are expected to be disseminated through peer-reviewed journals and academic conferences.Trial registration number NCT05714007.

Abstract Study design A combined retrospective and prospective analysis on the therapeutic effect of physiotherapeutic scoliosis-specific exercise (PSSE) in mild juvenile idiopathic scoliosis (JIS) patients. Background At present, patients with mild JIS are generally treated by observation without any interventional treatment. This study analyzed the effects of PSSE on mild JIS, which provided a new approach for the treatment of JIS. Method A total of 52 patients with mild JIS (Cobb angle 10–19°), aged 4–9 years, self-selected into an observation group and a PSSE group. Patients performed the corrective posture exercises daily based on the Scientific Exercise Approach to Scoliosis (SEAS) to the best of their ability, and performed the over-corrective training based on Schroth methods for 30 min each day. Before and one year after the treatment, the Cobb angle and the angle of trunk rotation (ATR) were evaluated, and the results were compared between the two groups. Results After one year of treatment, the Cobb angle in the PSSE group decreased from 15.0(11.0–17.0)° to 5.0(2.0–12.0)°(p ≤ 0.001), while the Cobb angle in the observation group increased from 13.5(11.0–17.3)° to 16.0(10.8–20.0)° (p = 0.010). The ATR in the PSSE group decreased from 5.0(2.0–7.0)° to 3.0(2.0–4.0)° (p = 0.009), while the change of ATR in the observation group was not significant. Compared with the observation group, 69.57% of patients in PSSE group had a decreased Cobb angle of more than 5 degrees, which was statistically significant(p ≤ 0.001). Conclusion For mild JIS, PSSE decreased the Cobb angle and ATR.

Abstract Background The treatment of severe and rigid scoliosis is challenging. We developed a surgical strategy for severe and rigid scoliosis since 2014. This study aimed to retrospectively analyze the safety and efficacy of apical region correction and global balance with 3 rods as a surgical strategy for the treatment of severe and rigid scoliosis. Methods A retrospective study was performed for patients with severe and rigid scoliosis who underwent one-stage posterior corrective operation using the apical region correction and global balance with 3 rods surgical strategy between February 2014 and April 2020. The inclusion criteria were as follows: [1] Cobb angle > 90°; [2] flexibility 20 mm) restored coronal balance at the final follow-up. 87% of the patients (14/16) with preoperative sagittal imbalance (SVA > 40 mm) restored sagittal balance at the final follow-up. The mean operation time and blood loss were 286 min and 941 mL, respectively. No patients had neurological complications or implant failure. Conclusion The surgical strategy of apical region correction and global balance with 3 rods is a safe and effective alternative for the surgical treatment of severe and rigid scoliosis.

Adiposity and bone mineral density (BMD) are closely associated. The aim of this research was to investigate the association between BMD and adiposity measures in adults, including gynoid percent fat (GPF), android percent fat (APF), total percent fat (TPF), visceral adipose tissue percent (VAT%), and total lean mass percent (TLM%). Participants (n = 11,615) aged 18 years and older were analyzed using data from the National Health and Nutrition Examination Survey (NHANES) spanning from 1999 to 2018. Associations between BMD and adiposity measures were investigated, and potential differences based on gender and age were explored. Significant negative associations were observed among TPF, APF, GPF, VAT%, and BMD in the fully adjusted models, while TLM% and BMD were positively associated. Stratifying by age and sex, TPF, GPF, and VAT% consistently demonstrated a negative correlation with BMD. In the young adult group, a TPF of 38.2% eliminated the negative correlation between BMD and TPF. Male BMD exhibited an inverted U-shaped relationship with APF, peaking at 35.6%, while a similar pattern was observed for the middle-aged group BMD and APF, with a peak at 31.7%. This large-sample research found a significant negative association between adiposity measures and BMD, providing valuable revelations regarding the intricate connection between adiposity and bone health.

Abstract Background Postoperative pulmonary complications are common and associated with morbidity and mortality. Congenital scoliosis is a failure of vertebral formation and/or segmentation arising from abnormal vertebral development. Posterior fusion and osteotomy are necessary for these patients to prevent deterioration of spine deformity. The incidence of postoperative pulmonary complications in this specific group of patients, especially young children were unknown. Methods A retrospective study was conducted and electronic medical records of early-onset scoliosis patients who had primary posterior fusion and hemivertebra resection at our institution from January 2014 to September 2019 were reviewed. The demographic characteristics, the intraoperative and postoperative parameters were collected to identify the predictors of postoperative pulmonary complications. Results A total of 174 patients (57.5% boys) with a median age of 3 years old were included for analysis. Eighteen patients (10.3%) developed perioperative pulmonary complications and pneumonia (n=13) was the most common. History of recent upper respiratory infection was not related to postoperative pulmonary complications. Multifactorial regression analysis showed thoracoplasty was the only predictive risk factor of postoperative pulmonary complications. Conclusions For congenital scoliosis patients younger than 10 years old, thoracoplasty determine the occurrence of postoperative pulmonary complications. Both surgeons and anesthesiologists should pay attention to patients undergoing thoracoplasty and preventive measures are necessary.

Abstract Background Contradictory opinions about whether early correction and fusion surgeries should be performed for congenital scoliosis (CS) patients at a young age exist. The objectives of this study were to analyze the association between patient characteristics and fusion-surgery outcomes in CS patients treated with spinal correction and fusion surgeries and to report risk factors for extended length of stay (LOS), more estimated blood loss (EBL), longer fused segments and higher medical costs. Methods We analyzed data of 1,207 CS inpatients treated with fusion surgeries in our institute from January 2010 - December 2019. All patients underwent spinal X-ray, CT, MRI, echocardiogram and urogenital ultrasound. We analyzed demographic and clinical information and outcome measures, including LOS, EBL, fused segments and medical costs. Results Age at fusion (OR = 1.053; p

Abstract Background To indicate whether combined topical and intravenous (IV) administration of tranexamic acid (TXA) could further reduce the blood loss after surgery for adolescent idiopathic scoliosis (AIS) compared with IV-TXA alone. Methods Ninety AIS patients who underwent posterior spinal fusion were prospectively randomized to combined group (IV + topical- TXA group) and IV-TXA alone group. TXA was infused at a loading dose of 1 g from the beginning of the surgery with a maintenance dose of 10 mg/kg/h until the wound was closed. In the combined group, 2 g TXA was injected retrogradely through a drain, while an equivalent amount of normal saline was injected in the IV-TXA alone group. The drain tube was clamped for 2 h in both groups. The amount of wound drainage and transfusion rates were analyzed. Results The drainage volume and duration of drain were significantly lower in the combined group compared with that in the IV-TXA alone group (372.0 ± 129.7 mL vs. 545.2 ± 207.7 mL, P

Abstract Introduction Adult non-degenerative scoliosis accounts for 90% of spinal deformities in young adults. However, perioperative complications and related risk factors of long posterior instrumentation and fusion for the treatment of adult non-degenerative scoliosis have not been adequately studied. Methods We evaluated clinical and radiographical results from 146 patients with adult non-degenerative scoliosis who underwent long posterior instrumentation and fusion. Preoperative clinical data, intraoperative variables, and perioperative radiographic parameters were collected to analyze the risk factors for perioperative complications. Potential and independent risk factors for perioperative complications were evaluated by univariate analysis and logistic regression analysis. Results One hundred forty-six adult non-degenerative scoliosis patients were included in our study. There were 23 perioperative complications for 21 (14.4%) patients, eight of which were cardiopulmonary complications, two of which were infection, six of which were neurological complications, three of which were gastrointestinal complications, and four of which were incision-related complication. The independent risk factors for development of total perioperative complications included change in Cobb angle (odds ratio [OR] = 1.085, 95% CI = 1.035 ~ 1.137, P = 0.001) and spinal osteotomy (OR = 3.565, 95% CI = 1.039 ~ 12.236, P = 0.043). The independent risk factor for minor perioperative complications is change in Cobb angle (OR = 1.092, 95% CI = 1.023 ~ 1.165, P = 0.008). The independent risk factors for major perioperative complications are spinal osteotomy (OR = 4.475, 95% CI = 1.960 ~ 20.861, P = 0.036) and change in Cobb angle (OR = 1.106, 95% CI = 1.035 ~ 1.182, P = 0.003). Conclusions Our study indicate that change in Cobb angle and spinal osteotomy are independent risk factors for total perioperative complications after long-segment posterior instrumentation and fusion in adult non-degenerative scoliosis patients. Change in Cobb angle is an independent risk factor for minor perioperative complications. Change in Cobb angle and spinal osteotomy are independent risk factors for major perioperative complications.

This study aims to analyze the potential association between the preoperative coagulation status and perioperative blood loss in spinal deformity correction surgery. The preoperative coagulation status and estimated blood loss (EBL) during operation, postoperative wound drainage, and allogeneic transfusion during and after operation were recorded and analyzed. Among the 164 patients, 26 had a longer prothrombin time (PT), 13 had a lower fibrinogen level, 55 had a longer activated partial thromboplastin time (APTT), and 2 had a longer thrombin time (TT), and the platelet count (PLT) was all normal or higher than the normal level. The mean EBL per surgical level was 77.8 ml (range, 22–267 ml), and the mean drainage per surgical level was 52.7 ml (range, 7–168 ml). Fifty-five patients and 12 patients underwent allogeneic transfusion during and after the operation, respectively. The differences in EBL per surgical level, mean drainage per surgical level, the occurrences of allogeneic transfusion during and after operation between the patients with a longer PT, lower fibrinogen level, longer APTT or longer TT, and the normal controls were not significant (all P’s > 0.05). The Spearman correlation analysis showed that there was no correlation between PT, fibrinogen, APTT, TT or PLT with EBL per surgical level, mean drainage per surgical level, or allogeneic transfusion during and after the operation (all P’s > 0.05). The abnormal preoperative coagulation status but not hemophilia does not lead to more perioperative blood loss or a higher rate of perioperative allogeneic transfusion in spinal deformity correction surgery.

Abstract Background Early onset scoliosis (EOS) may cause malnutrition in affected patients. Growing-rod treatment has been an effective protocol for treating EOS. The objective of this study is to demonstrate whether growing-rod treatment improves nutritional status of EOS patients. Methods Fifty-two EOS patients who had dual growing-rod surgery was enrolled. The minimum follow-up was 3-years. Their body weights were normalized based on the data of two National Population Census of China. Z-scores were used to indicate the standard deviation from the median body weight-for-age. Results The median follow-up time was 6 years. Preoperatively, the prevalence of malnutrition (Z

Abstract Background Spinal deformities constitute one of the most common types of manifestations of neurofibromatosis type-1 (NF-1), which can lead to either dystrophic or non-dystrophic early-onset scoliosis (EOS). Surgical treatment for EOS with NF-1 is challenging, and the outcomes have rarely been reported. The anterior-posterior procedure is widely used, but posterior-only fusion is theoretically easier and safer to perform. Is it possible that a new surgery that accommodates growth is a better choice? A direct comparison between posterior fusion and growth-friendly surgery in terms of surgical outcomes has not yet been conducted in dystrophic EOS with NF-1 patients. Methods Baseline information was extracted from the NF-1 database at our institute with approval from the local ethics committee. All enrolled patients were diagnosed with NF-1. Clinical and radiographic data were recorded preoperatively, after the initial surgery, and at the final follow-up. Implant-related, alignment, neurological complication and unplanned revision surgery data were recorded. We compared the outcomes of these two groups in terms of curve correction, growth parameters, complications and unplanned revision surgeries. Results There were eight patients in the PF group and eight patients in the GR group, with a mean follow-up of 51.0 ± 17.5 months. The main curve size was similar (PF 67.38° ± 17.43° versus GR 75.1° ± 26.43°, P = 0.501), and there were no significant differences in the initial surgery correction rate or the rate of correction. However, the patients in the GR group exhibited more T1-S1 growth during the follow-up overall and per year than did those in the PF group. The operative time was significantly longer for the PF group than for the GR group (PF, 4.39 ± 1.38 vs. GR, 3.00 ± 0.42 h; p = 0.008). Significantly fewer segments were involved in the PF group (8.25 ± 3.20) than in the GR group (13.00 ± 1.60). Conclusion For the initial treatment of dystrophic EOS in patients with NF-1, the GR technique is possibly a more appropriate treatment than is the PF technique in terms of trunk growth. However, the repeated procedures required for GR may be a considerable disadvantage. More studies with direct measurement of pulmonary function must be conducted to determine the effect of GR on pulmonary development. More studies with larger sample sizes and longer follow-up periods are needed to fully assess the treatment strategies.

Abstract 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.

Methylprednisolone (MP) is the drug of choice for treating spinal cord injury (SCI), but the aggressive dosage regimen used often results in adverse side effects. Therefore, MP should be combined with other drugs to lower the required dose. Melatonin is effective in alleviating nerve damage and inhibiting axonal degeneration. The combination of melatonin and half-dose methylprednisolone (HMP) for spinal cord injury treatment has never been reported. In this study, we established a rat model of T9 spinal cord injury by the Allen’s method and assessed the synergistic therapeutic effects of melatonin and HMP by factorial design. Our results demonstrated that melatonin could synergize with HMP to ameliorate acute SCI through PI3K-AKT1 pathway. Combining melatonin with HMP significantly reduced the standard-dose of methylprednisolone and limited its adverse reactions, representing a promising option for treating acute SCI.

As the principal reason for low back pain, intervertebral disc degeneration (IDD) affects the health of people around the world regardless of race or region. Degenerative discs display a series of characteristic pathological changes, including cell apoptosis, senescence, remodeling of extracellular matrix, oxidative stress and inflammatory local microenvironment. As a serine/threonine-protein kinase in eukaryocytes, AMP-activated protein kinase (AMPK) is involved in various cellular processes through the modulation of cell metabolism and energy balance. Recent studies have shown the abnormal activity of AMPK in degenerative disc cells. Besides, AMPK regulates multiple crucial biological behaviors in IDD. In this review, we summarize the pathophysiologic changes of IDD and activation process of AMPK. We also attempt to generalize the role of AMPK in the pathogenesis of IDD. Moreover, therapies targeting AMPK in alleviating IDD are analyzed, for better insight into the potential of AMPK as a therapeutic target.

The debt default risk of local government financing vehicles (LGFVs) has become a potential trigger for systemic financial risks. How to effectively prevent hidden debt risk has always been a hot issue in public-private partnership (PPP) financing management research. In recent years, machine learning has become more and more popular in the study of enterprise credit evaluation. However, most scholars only focus on the output of the model, and do not explain in detail the extent to which variables affect the model and the decision-making process of the model. In this paper, we aim to apply a better credit rating method to the key factors and analysis of LGFV’s default risk, and analyze the decision-making process of the model in a visual form. Firstly, this paper analyzes the financial data of LGFVs. Secondly, the XGBoost-logistic combination algorithm is introduced to integrate the typical characteristics of PPP projects and construct the credit evaluation model of LGFVs. Finally, we verify the feasibility of the model by K-fold cross validation and performance evaluation. The results show that: (1) net worth, total assets, operating income, and return on equity are the most critical factors affecting the credit risk of LGFVs, asset-liability ratio and tax revenue are also potentially important factors; (2) the XGBoost-logistic model can identify the key factors affecting the credit risk of LGFVs, and has better classification performance and predictive ability. (3) The influence of each characteristic variable on model decision can be quantified by the SHAP value, and the classification decision visualization of the model improves the interpretability of the model.

Abstract Background Blood loss during hemivertebra resection may be substantial. Few studies have examined the risk factors of blood loss undergoing hemivertebra resection, especially those in patients under 10 years old. Methods Patients under 10 years old diagnosed with congenital scoliosis and hemivertebra were retrospectively included from January 2014 to October 2017. They all had primary posterior hemivertebra resection at Peking Union Medical College Hospital. Perioperative information was collected and multivariable linear logistic regression was performed to determine the independent risk factors of blood loss. Results One hundred three patients were included. The mean total blood loss was 346 + 178 ml. The percentage of total blood loss to the EBV was 27.0 + 13.3%. Multivariable linear logistic regression indicated that preoperative total Cobb angle (P = 0.046) and the number of fused levels (P

Abstract Backgrounds Adolescent idiopathic scoliosis (AIS) is an adolescent onset spinal deformity, which can negatively affect the mental health of these patients. But no studies about their parental mental health have been reported so far. In this study, the parental mental health of AIS patients and the associated risk factors were evaluated by a cross-sectional survey. Methods 64 AIS patients who underwent conservative or surgical treatments in our hospital from April 2017 to March 2018, and their parents were enrolled in the AIS group. 85 parents of healthy children were enrolled in the control group. Depression and anxiety were separately assessed using the Patient Health Questionnaire (PHQ-9) and the Generalized Anxiety Disorder 7-item scale (GAD-7). Spearman correlation coefficients were first estimated to investigate the relationships among the parental PHQ-9/GAD-7 scores and the patient’s PHQ-9/GAD-7 scores in the AIS group. Then, the morbidities of the parental probable major depressive disorder (pMDD, PHQ score ≥ 10) and probable general anxiety disorder (pGAD, GAD-7 score ≥ 10) were compared between the AIS and control groups. Third, the potential risk factors for parental pMDD or pGAD in the AIS group were compared using the chi-squared test or Student’s t-test, respectively. Finally, the uneven distributive variates were analyzed using the binary logistic regression model. Results Both parental depression and anxiety were moderately associated with those of the patients (r = 0.448~0.515, p

Abstract Background Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome characterized by the triad of venous varicosities, capillary malformations and limb hypertrophy. However, KTS may rarely occur in combination with kyphoscoliosis. Case presentation We presented an 18-year-old female with KTS and kyphoscoliosis. Hypertrophy of bone and soft tissue affected her left face, trunk and lower limb. Moreover, the patient is associated with subacute thyroiditis, vitamin D deficiency and iron deficiency anemia, high level of D-dimer, swollen tonsil, kyphoscoliosis and Chiari-I-malformation without syringomyelia. A posterior correction and spinal fusion from T10 to L5 levels were performed for this patient. The lumbar curve was corrected from 105° to 60° and the kyphosis improved from 58° to 26°. The distance of trunk shift decreased from 10 cm to 1.4 cm. There were no thrombotic events occurred. At the 8th month follow-up, there was no significantly change of the curve in the coronal and sagittal radiographs. During the 31-month follow-up, the patient did not experience any discomfort. And her general appearance did not have any change until the last follow-up. However, she refused to take radiograph for worrying about radiation. Conclusions KTS is a rare disease with classic clinical triad. However, it can also have other different features, including kyphoscoliosis, elevated D-Dimer, vitamin D deficiency and iron-deficiency anemia. These issues should be taken into consideration when planning treatment for kyphoscoliosis in KTS patients.

Background: Adolescent idiopathic scoliosis (AIS) is a complex disease affecting a large number of teenagers, especially in female. This study reveals novel epigenetic perturbation to the pathogenesis of AIS.Methods: A female monozygotic (MZ) twin pair discordant for AIS were examined for whole-exome sequencing and epigenome difference. Sets of differentially methylated regions (DMRs) were validated using MethylTarget™ method in 20 AIS female patients and 20 healthy female controls.Results: Few exome difference but several potential DMRs were found between the MZ twins. We identified 313 hypermethylated DMRs and 397 hypomethylated DMRs, respectively. Most of them were enriched in the MAPK and PI3K-Akt signaling pathway, which may contribute to the discordance of AIS. Several DMRs related to scoliosis genes were tested, and the NDN: TSS-DMR (chr15:23932133-23932304, hg19) was confirmed in additional samples. The methylation level of this DMR was significantly higher in the AIS group than in the control group (p = 0.04).Conclusions: We described the epigenome difference in an AIS female discordant MZ twin pair using Whole Genome Bisulfite Sequencing (WGBS). The NDN: TSS-DMR had higher methylation level in female AIS, which can help elucidate the potential etiology of AIS.

Background/Aims: Congenital scoliosis (CS) is a result of anomalous development of vertebrae and is frequently associated with somitogenesis malformation. Although noncoding RNAs (ncRNAs) have been recently determined to be involved in the pathogenesis of CS, the competing endogenous RNA (ceRNA) regulatory networks in CS remain largely unknown. Methods: Sequencing was conducted to explore the ncRNA expression profiles in rat embryos (gestation day 9) following vitamin A deficiency (VAD) (n = 9 for the vitamin A deficiency-induced congenital scoliosis (VAD-CS) group and n = 4 for the control group). Real-time reverse transcriptase polymerase chain reaction (RT-PCR) was conducted to verify the expression levels of selected mRNAs, long noncoding RNAs (lncRNAs), circular RNAs (circRNAs), and microRNAs (miRNAs). Bioinformatics analysis was used to discover the possible relationships and functions of the ceRNAs. Results: A total of 749 mRNAs, 56 miRNAs, 685 lncRNAs, and 70 circRNAs were identified to have significantly different expression levels in the two groups. Wnt, PI3K-ATK, FoxO, EGFR, and mTOR were found to be the most significant pathways involved in VAD-CS pathogenesis. The circRNA/miRNA/mRNA and lncRNA/miRNA/mRNA networks of CS were built, and the gene expression mechanisms regulated by ncRNAs were unveiled via the ceRNA regulatory networks. Conclusion: We comprehensively identified ceRNA regulatory networks of embryonic somite development in VAD-CS as well as revealed the contribution of different ncRNA expression profiles. Our data demonstrate the association between mRNAs and ncRNAs in the pathogenic mechanism of CS.

Abstract Backgrounds Rare study has been conducted to detect risk factors of perioperative complications, which are closely related to preoperative status of the patients and surgical stress. The aim of this study is to detect these relationships in degenerative scoliosis (DS) patients. Methods Perioperative complications of 226 cases with DS (56 males and 170 females; 65.5 ± 8.1 years old), who accepted posterior fusion in our hospital from January, 2013 to July, 2017, were retrospectively reviewed. Potential risk factors were first compared between patients with or without perioperative complications using student t test or Chi-squared test. Then, the unevenly distributed variables between the two groups were analyzed with binary logistic regression model. Results All patients separately underwent decompression with short limited instrumentation (116, 51.3%) or with long instrumentation for correction (110, 48.7%). The mean operation duration (OD) was 216.9 ± 64.2 min and the average amount of bleeding was 587.4 ± 357.2 ml. 44 cases (19.5%)suffered from the complications during the perioperative phase, including incision complications (5.3%), urinary infection (3.5%), dura tears/cerebrospinal fluid (CSF) leakages (3.5%) and new neurological deficits (2.7%). Hospital stay was significantly extended for the complications (p 2 (p = 0.011) and RBC transfusion≥4 U(p = 0.028) were associated with these complications. Multivariate logistic regressions revealed that only ASA grade > 2(p = 0.011, Odds Ratio[OR] = 4.104, 95% Confidence Interval[CI] = 1.413~ 11.917) and OD (p = 0.013, OR = 2.697, 95% CI = 1.233~ 5.899) were the independent risk factors. Conclusions The high morbidity of perioperative complications for posterior spinal fusion would significantly extend hospital stay of DS patients. It was independently related to higher ASA grade and longer OD.

Abstract Background Vertebrae, ribs, and spinal cord are anatomically adjacent structures, and their close relationships are clinically important for planning better corrective surgical approach. The objective is to identify the radiographic characteristics in surgical patients with congenital scoliosis (CS) and coexisting split cord malformation (SCM). Methods A total of 266 patients with CS and SCM underwent surgical treatment at our hospital between May 2000 and December 2015 was retrospectively identified. The demographic distribution and radiographic data were collected to investigate the characteristics of spine curve, vertebral, rib, and intraspinal anomalies. According to Pang’s classification, all patients were divided into two groups: type I group is defined as two hemicords, each within a separate dural tube separated by a bony or cartilaginous medial spur, while type II group is defined as two hemicords within a single dural tube separated by a nonrigid fibrous septum. Results There were 104 patients (39.1%) in Type I group and 162 patients (60.9%) in Type II group. SCM was most commonly found in the lower thoracic and lumbar regions. The mean length of the septum in Type I SCM was significantly shorter than Type II SCM (2.7 vs. 5.2 segments). Patients in Type I group had a higher proportion of kyphotic deformity (22.1%). The vertebral deformities were simple in only 16.5% and multiple in 83.5% of 266 cases. Patients in Type I group presented higher prevalence of multiple (90.4%) and extensive (5.1 segments) malformation of vertebrae. In addition, hypertrophic lamina and bulbous spinous processes were more frequent in Type I group (29.7%), even developing into the “volcano-shape” deformities. Rib anomalies occurred in 62.8% of all patients and 46.1% of them were complex anomalies. The overall prevalence of other intraspinal anomalies was 42.9%. The most common coexisting intraspinal anomalies was syringomyelia (30.5%). Conclusion The current study, with the largest cohort to date, demonstrated that patients with CS and coexisting SCM presented high prevalence of multiple vertebral deformities, rib and intraspinal anomalies. The length of the split segment in Type I SCM was shorter than that in Type II SCM. Compared with Type II SCM, patients with Type I SCM presented with higher incidence of kyphotic deformity, more extensive and complicated vertebral anomalies, and more complex rib anomalies.

Abstract Introduction In this study, the effects of omega‐3 fatty acids were examined in a rat model of spinal cord injury. Methods The rats were classified into sham, control, spinal cord injury plus 50 mg/kg Omega‐3 fatty acids and spinal cord injury plus 100 mg/kg Omega‐3 fatty acids. The levels of oxidative, apoptotic, and inflammatory markers were examined in each of these groups. Results Altered lipid peroxidation, reduced glutathione (GSH), superoxide dismutase (SOD), glutathione peroxidase (Gpx), and catalase were normalized. Omega‐3 fatty acid supplementation decreased tumor necrosis factor‐alpha (TNF‐α) and interleukin‐6 (IL‐6) levels by >50%. TNF‐α and IL‐6 mRNA expression were reduced. Caspase‐3, p53, bax, and pro‐NGF mRNA expression levels were increased by 1.3‐, 1.4‐, 1.2‐, and 0.9‐fold, respectively, whereas bcl‐2 mRNA expression was decreased by 0.77‐fold in control rats. Omega‐3 fatty acid supplementation decreased p53, caspase‐3, bax, and pro‐NGF mRNA expression by >40%, while the level of bcl‐2 mRNA expression was increased by 286.9%. Omega‐3 fatty acid supplementation decreased caspase‐3 and p53 protein expression by >30%. Conclusion Taken together, our results suggested that omega‐3 fatty acid supplementation reduced oxidative stress, apoptosis, and the levels of inflammatory markers in ischemia‐reperfusion‐induced rats.

Study design. Retrospective characterization of nonspecific low back pain (NSLBP) in young adult female patients with and without lumbar scoliosis. Background. There is no consensus as to whether NSLBP in scoliosis patients is related to scoliosis per se or is just a normal symptom that could happen in anyone. Objectives. The aim of this study was to compare the differences in NSLBP between young adult female patients with and without lumbar scoliosis and to provide a theoretical basis for differential treatment of NSLBP in patients with and without lumbar scoliosis. Methods. Ninety female young adults with NSLBP were divided into scoliosis and nonscoliosis groups. Characteristics of pain, lumbar mobility, muscle strength, Cobb angle, axial trunk rotation (ATR) angle, and surface electromyography (SEMG) signal were compared between the two groups. Results. The pain location in scoliotic patients was more concentrated on the left side of the lumbar spine (P≤0.001). The area affected by pain (P=0.028) and the numerical pain rating scale (NPRS) scores (P=0.014) of scoliotic patients were less than those of nonscoliotic patients. The difference between side-bending in scoliotic patients was greater than that in nonscoliotic patients (P=0.001). Scoliotic patients exhibited a significantly better ability for flexion (P=0.001) and extension (P=0.017) than nonscoliotic patients. The posterior muscles in scoliotic patients were stronger than those in nonscoliotic patients (P=0.014). The ratio of root-mean-square (RMS) on paraspinal muscles in scoliotic patients was greater than that in nonscoliotic patients (P≤0.001). Scoliotic patients exhibited greater relaxation time during the flexion-relaxation phenomenon (FRP) than nonscoliotic patients (P=0.024). Conclusions. The characteristics of NSLBP experienced by patients with lumbar scoliosis were distinct from those of NSLBP experienced by nonscoliotic patients. The treatment of NSLBP in scoliotic patients should be different from that in nonscoliotic patients.

Abstract Background Hyperlordosis is common in facioscapulohumeral muscular dystrophy (FSHD), which cannot be controlled by bracing. While the surgical treatment is neither reported nor recommended in previous studies, we report the first corrective surgery for hyperlordosis in one wheelchair-dependent FSHD patient. Case presentation A 15-year-old, wheelchair-dependent girl complaining of hyperlordosis and lower extremity weakness was diagnosed as FSHD. Preoperative examination showed hyperlordosis of 116° with scoliosis of 44°. Posterior correction and bone graft fusion was performed. Postoperatively, the hyperlordosis was corrected to 72° and better sitting posture was gotten. 12 months’ follow-up showed favorable correction with improvement in SF-36 and ODI score. Conclusions The correction for hyperlordosis in FSHD is controversial. We report the first successful case of operative treatment by corrective spine surgery in these rare and demanding patient collective. Corrective surgery for spinal deformity, such as hyperlordosis and scoliosis, could do some help in posture and improve the quality of life especially in wheelchair-dependent patients.

OBJECTIVES:To analyze the occurrence, risk factors, treatment and prognosis of postoperative pleural effusion after spinal deformity correction surgery. METHODS:The clinical and imaging data of 3325 patients undergoing spinal deformity correction were collected from the database of our hospital. We analyzed the therapeutic process of the 28 patients who had postoperative pleural effusion, and we identified the potential risk factors using logistic regression. RESULTS:Among the 28 patients with postoperative pleural effusion, 24 (85.7%) suffered from hemothorax, 2 (7.1%) from chylothorax, and 2 (7.1%) from subarachnoid-pleural fistula. The pleural effusion occurred on the convex side in 19 patients (67.9%), on the concave side in 4 patients (14.3%), and on both sides in 4 patients (14.3%). One patient with left hemothorax was diagnosed with kyphosis. The treatment included conservative clinical observation for 5 patients and chest tube drainage for 23 patients. One patient also underwent thoracic duct ligation and pleurodesis. All of these treatments were successful. Logistic regression analysis showed that adult patients(≥18 years old), congenital scoliosis, osteotomy and thoracoplasty were risk factors for postoperative pleural effusion in spinal deformity correction surgery. CONCLUSIONS:The incidence of postoperative pleural effusion in spinal deformity correction surgery was approximately 0.84% (28/3325), and hemothorax was the most common type. Chest tube drainage treatment was usually successful, and the prognosis was good. Adult patients(≥18 years old), congenital scoliosis, and had undergone osteotomy or surgery with thoracoplasty were more likely to suffer from postoperative pleural effusion.

Background: Congenital early-onset scoliosis (CEOS) is characterized by a spectrum of vertebral anomalies, including formation failures and segmentation failures at the apex segment, which makes CEOS different from other etiologies of earlyonset scoliosis. To date, studies on patients who have graduated from CEOS treatment using traditional dual growing rods (TDGR) have been scarce, and the preliminary results of TDGR with or without the apical control technique (ACT) have varied. We therefore compared the final outcomes of patients with CEOS who graduated from TDGR with or without the ACT. Methods: A retrospective study of patients with CEOS who had graduated from TDGR treatment performed from 2007 to 2020 was conducted. Graduation included final fusion or observation after reaching skeletal maturity. Patients were divided into the ACT-TDGR group (apical vertebrectomy and/or hemivertebrectomy with short fusion and TDGR) and the TDGR-only group. Demographic characteristics, radiographic data, patient-reported clinical outcomes, pulmonary function, and complications were analyzed. Results: A total of 41 patients with CEOS were enrolled: 13 in the ACT-TDGR group and 28 in the TDGR-only group. The lengthening intervals were longer in the ACT-TDGR group (mean [and standard deviation], 1.26 ± 0.66 years) than in the TDGR-only group (0.80 ± 0.27 years). The preoperative main curve was larger in the ACT-TDGR group (80.53° ± 19.50°) than in the TDGR-only group (64.11° ± 17.50°). The residual curve was comparable between groups (26.31° ± 12.82° in the ACT-TDGR group compared with 27.76° ± 15.0° in the TDGR group) at the latest follow-up. The changes in apical vertebral rotation and thoracic rotation were significantly larger in the ACT-TDGR group. Patients had comparable T1-12 and T1-S1 heights, pulmonary function, and 22-item Scoliosis Research Society (SRS-22) scores at the latest follow-up. The mean number of mechanical-related complications per patient was lower in the ACT-TDGR group (0.77 ± 0.73) than in the TDGR-only group (1.54 ± 1.43). Seventeen patients underwent final fusion. Conclusions: In this small-scale study, we observed that both ACT-TDGR and TDGR-only could correct the deformity while allowing for spinal growth in patients with CEOS. ACT-TDGR yielded better correction in severe cases and did not have a deleterious effect on spinal height. A large number of cases will be needed to validate the clinical value of the ACT. Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]