Posterior Corrective Surgery for Type II Congenital Kyphosis: SRS-Schwab Grade 4 Osteotomy or Vertebral Column Resection?

OBJECTIVE: Surgical decision-making for congenital kyphosis (CK) with failure of anterior segmentation (type II) has been contradictory regarding the trade-off between the pursuit of correction rate and the inherent risk of the osteotomy procedure. This study was designed to compare the clinical and radiographic measurement in type II CK underwent SRS-Schwab Grade 4 osteotomy and vertebral column resection (VCR), the most-adapted osteotomy techniques for CK, and to propose the strategy to select between the two procedures. METHODS: This retrospective observational comparative study evaluated surgical outcomes in type II CK patients underwent VCR or SRS-Schwab Grade 4 osteotomy at our institution between January 2015 and January 2020. Patients operated with VCR and SRS-Schwab Grade 4 osteotomy were allocated to Group 1 and Group 2 respectively. Radiographic parameters and SRS-22 quality of life metrics were assessed at pre-operation, post-operation, and during follow-up visits for both groups, allowing for a comprehensive comparison of surgical outcomes. RESULTS: Thirty-one patients (19 patients in Group 1 and 12 patients in Group 2) aged 16.3 ± 10.4 years were recruited. Correction of segmental kyphosis was similar between groups (51.1 ± 17.6° in Group 1 and 48.4 ± 19.8° in Group 2, p = 0.694). Group 1 had significantly longer operation time (365.9 ± 81.2 vs 221.4 ± 78.9, p < 0.001) and more estimated blood loss (975.2 ± 275.8 ml vs 725.9 ± 204.3 mL, p = 0.011). Alert event of intraoperative sensory and motor evoked potential (SEP and MEP) monitoring was observed in 1 patient of Group 2. Both groups had 1 transient post operative neurological deficit respectively. CONCLUSION: SRS-Schwab Grade 4 osteotomy was suitable for kyphotic mass when its apex is the upper unsegmented vertebrae or the neighboring disc, or when the apical vertebrae with an anterior/posterior (A/P) height ratio of vertebral body higher than 1/3. VCR is suitable when the apex is located within the unsegmented mass with its A/P height ratio lower than 1/3. Proper selection of VCR and SRS-Schwab Grade 4 osteotomy according to our strategy, could provide satisfying radiographic and clinical outcomes in type II CK patients during a minimum of 2 years follow-up. Patients undergoing VCR procedure might have longer operation time, more blood loss and higher incidence of peri- and post-operative complications.

Differential Gene Expression Profiles and Pathways Highlight the Role of Osteoimmunology in Neurofibromatosis Type 1-Related Dystrophic Scoliosis With Osteopenia.

STUDY DESIGN: Microarray approach and integrated gene network analysis. OBJECTIVE: To explore the differential genetic expression profile, Gene Ontology terms, and Kyoto Encyclopedia of Genes and Genomes pathways in human trabecular bone (HTB)-derived cells of dystrophic scoliosis secondary to neurofibromatosis type 1 (DS-NF1) and compare these to normal controls. SUMMARY OF BACKGROUND DATA: The pathogenesis of DS-NF1 and the accompanying generalized osteopenia remain unclear. We hypothesized that HTBs may play a significant role in the etiology and pathogenesis of DS-NF1. MATERIALS AND METHODS: Microarray analysis was used to identify differentially expressed genes of HTBs from patients with DS-NF1 compared with those from healthy individuals. Functional and pathway enrichment analysis were implemented through Gene Ontology and Kyoto Encyclopedia of Genes and Genomes pathway database. Then, the "search tool for the retrieval of interacting genes/proteins" database, Cytoscape, and "Molecular Complex Detection" were applied to construct the protein-protein interaction (PPI) network and screen hub genes. Pathway enrichment analysis was further performed for hub genes and gene clusters identified through module analysis. Six potential crucial genes were selected for validation by reverse transcription polymerase chain reaction. RESULTS: Bioinformatic analysis revealed that there are 401 previously unrecognized differentially expressed genes (238 up and 163 downregulated genes) in HTBs from patients with DS-NF1, and they were mainly enriched in terms of immune response, type-I interferon (IFN) signaling, TNF signaling pathway and etinoic acid inducible gene I-like receptor signaling pathway. Five hub genes, including signal transducer and activator of transcription 1, 2'-5'-oligoadenylate synthetase-like, IFN induced with helicase C domain 1, IFN regulatory factor 7, and MX dynamin-like GTPase 1 were identified through PPI network, which were mainly enriched in terms of Jak-STAT and etinoic acid inducible gene I-like receptor signaling pathway. An independently dysregulated protein cluster containing CCL2, CXCL1, CXCL3, CX3CL1, TLR1 , and CXCL12 was also identified through the PPI network. This indicated that the upper abnormally expressed genes may play essential roles in DS-NF1 pathogenesis and accompanied osteopenia. CONCLUSION: Six key genes were identified in the progression of DS-NF1-related osteopenia. Immune response might play a key role in the progression of osteopenia, whereas a CXCL12 -mediated osteogenic effect might play a protective role.

How does congenital cervicothoracic scoliosis bring about early trunk tilt and coronal imbalance during curve progression: a radiographic analysis to dissect the mechanism of proximal takeoff phenomenon.

PURPOSE: To dissect the mechanism of how congenital cervicothoracic scoliosis (CTS) drive the occurrence of early trunk tilt, namely proximal takeoff phenomenon (PTO) during curve progression. METHODS: CTS patients were stratified into case and control groups according to the presence of PTO. The radiographic deformity parameters of head-neck-shoulder complex were measured and compared between the two groups. The main risk factors for PTO were identified through multiple linear regression analysis. RESULTS: 16 CTS patients with PTO were recruited, and the non-PTO group consisted of 19 CTS patients without PTO. The average Cobb angle was 64.9 ± 19.8° in PTO group and 57.7 ± 21.9° in control group (p > 0.05). Significant difference could be observed for head shift, neck tilt, trunk inclination, apex-C7 deformity angular ratio (DAR), apex translation ratio, C6 tilt, clavicle angle (CA), radiographic shoulder height (RSH), head-neck translation and coronal balance distance (CBD) (All p < 0.05) but not head tilt (p > 0.05). Multiple linear regression analysis revealed that head shift, but not neck tilt correlated significantly with the severity of trunk inclination (ß = 0.106, p = 0.003), while apex-C7 DAR and apex translation ratio were the two factors contributing significantly to the severity of head shift (ß = 0.620, p = 0.020; ß = - 0.371, p = 0.004). CONCLUSIONS: Development and progression of head shift rather than neck tilt is a significant causative factor initiating the occurrence of trunk tilt and proximal takeoff in CTS. A higher apex-C7 DAR representing a short angular upper hemi curve and a lower apex translation ratio representing poor proximal coronal compensation are key risk factors predisposing to head shift.

Posterior-only correction surgery for idiopathic scoliosis Lenke type 5c: differences of strategies and outcomes between adult patients and adolescent patients.

PURPOSE: To compare radiographic parameters, and functional and surgical outcomes between lumbar adolescent idiopathic scoliosis (AIS) and lumbar adult idiopathic scoliosis (AdIS). METHODS: A retrospective study was performed to identify Lenke 5c type AIS and AdIS patients from our scoliosis database who had undergone posterior surgical treatment for scoliosis. Preoperative and postoperative radiographic and clinical outcomes were compared between the two groups. RESULTS: A total of 22 patients were included in AdIS group, and 44 matched patients in AIS group. AdIS group had significantly larger L3 and L4 tilt and translation than AIS group (P < 0.05). AdIS group had larger T10-L2 angle and smaller T5-T12 angle (P < 0.05). AdIS group had higher VAS scores (P < 0.05) and pain domain of SRS-22 scores (P < 0.05) as compared to AIS group. Correlation analysis demonstrated positive relationship between VAS scores and T10-L2 angle (r = 0.492, P < 0.05). AdIS group was fused longer than AIS group (P < 0.05). Cobb angle of TL/L curve was larger and correction ratio was smaller at AdIS group (P < 0.05). AdIS group still had significantly larger L3 and L4 tilt and translation than AIS group (P < 0.05). CT measurements demonstrated larger postoperative vertebral body rotation at apical vertebrae and LIV at AdIS group (P < 0.05). Vertebral correction ratio was smaller at AdIS group (P < 0.05). CONCLUSION: Lenke 5c AdIS patients had greater preoperative and postoperative L3 and L4 tilt and translation, as well as less correction of major curve and vertebral body derotation than AIS patients. However, the incidence of adding-on was similar between the two groups.

The Utilization of Dual Second Sacral Alar-Iliac Screws for Spinopelvic Fixation in Patients with Severe Kyphoscoliosis.

OBJECTIVES: As a new pelvic fixation technique, the dual S2AI screws fixation technique could provide highly stable distal strength, and have wide clinical prospect in the correction of severe kyphoscoliosis. However, the ideal trajectory parameters, indications and clinical outcomes of this technique have not been reported so far. This study aimed to determine the anatomical parameters of dual S2AI screws in the normal Chinese adult population, investigating the indications of this technique and evaluating the feasibility and clinical outcomes. METHODS: Fifteen males and 15 females with normal pelvis underwent a pelvic CT scan to determine ideal dual S2AI screws trajectories. Sagittal angle (SA), transverse angle (TA), maximal length (ML), sacral length, and skin distance were measured. Subsequently, we retrospectively reviewed the data of 16 patients (seven males and nine females) who underwent dual S2AI screw fixation and 23 patients who underwent single S2AI screw fixation between January 2014 and December 2019. Preoperative, postoperative, and latest follow-up measurements of Cobb angle, coronal balance (CB), spinal pelvic obliquity (SPO), and regional kyphosis (RK) were obtained. The mean follow-up time was 16.7 ± 7.1 months (range: 12-30 months). Independent t-test was used to determine the difference in the analysis of the trajectories. The paired sample non-parametric Wilcoxon test was performed to assess the changes in radiographic parameters between different time points and different groups. RESULTS: For both male and females, the proximal S2AI screws had significantly higher TA and ML, but a lower SA than distal screws. Females showed significantly more caudal (SA: 25.03° ± 2.32° vs. 29.82° ± 2.47°, t = 7.742, P < 0.001) trajectories of distal screw. Additionally, ML in the females were significantly shorter than that in males (106.81 mm ± 6.79 mm vs. 101.63 mm ± 6.55 mm, t = 3.007, P = 0.003, 124.41 mm ± 7.57 mm vs. 116.23 mm ± 7.03 mm, t = 4.337, P < 0.001). Eight had unilateral and eight had bilateral dual S2AI screw placement. Respectively, both the single S2AI and dual S2AI groups showed significant postoperative improvement of Cobb angle, RK angle and SPO angle. In patients with dual S2AI screws fixation, two patients found that screws loosening occurred in one of dual screws at 1-year follow-up, and in patients with single S2AI screws fixation, six patients found screw loosing as well as two patients found screw breakage at 1-year follow-up. None of all patients had any prominent loss of correction. CONCLUSION: The ideal trajectory of dual S2AI screw could be well established. The dual S2AI screw fixation technique is feasible in patients with severe kyphoscoliosis, and provides satisfactory correction of deformity with few postoperative complications.

Scoliosis: an unusual clinical presentation of paraspinal ganglioneuroma.

PURPOSE: To comprehensively present the clinical characteristics and treatment strategies in patients with scoliosis secondary to ganglioneuroma (S-GN). METHODS: Six patients with S-GN treated surgically at a median age of 12 years were retrospectively reviewed and the median follow-up period was 6 years (4-14 years). The radiological features of GN and the associated scoliosis were evaluated. The surgical strategies and the corresponding outcomes were investigated. RESULTS: All patients had a delayed diagnosis age of GN than scoliosis (12 vs. 9 years). GN was located at the posterior mediastinum in four patients (66.7%) and at retroperitoneum in two, respectively. Tumor occupancies were frequently detected on the X-ray films for four patients (66.7%), being uniformly on the convexity of the main curve. All patients complained of rapid progressive deformities during the growth period. Five patients (83.3%) received total tumor resections, one accepted partial resection. Deformity correction was implemented for all patients with an average rate of 66.4% on the main curve. No recurrence of the GN was detected for all totally tumor-resected patients at the latest follow-up. CONCLUSION: S-GN is often misdiagnosed clinically. Paravertebral mass neighboring the apex of scoliosis can be meticulously detected from the X-ray films. Total tumor resection should be aggressively performed if possible. The deformity correction could be satisfactorily obtained and the risk of recurrence of the GN was relatively low.

How to rectify the convex coronal imbalance in patients with unstable dystrophic scoliosis secondary to type I neurofibromatosis: experience from a case series.

BACKGROUND: There was a paucity of valid information on how to rectify the convex coronal imbalance effectively in dystrophic scoliosis secondary to Type I neurofibromatosis (DS-NF1), while postoperative inadvertent aggravation of CCI occurred regularly resulting in poor patient satisfaction. We aimed to identify the risk factors for persistent postoperative CCI in DS-NF1, and to optimize the coronal rebalancing strategies based on the lessons learned from this rare case series. METHODS: NF1-related scoliosis database was reviewed and those with significant CCI (> 3 cm) were identified, sorted and the outcomes of surgical coronal rebalance were analyzed to identify the factors being responsible for failure of CCI correction. RESULTS: CCI with dystrophic thoracolumbar/lumbar apex was prone to remain uncorrected (7 failure cases in 11) when compared to those with thoracic apex (0 failure cases in 4) (63.6% vs. 0.0%, p = 0.077). Further comparison between those with and without post-op CCI showed a higher correction of main curve Cobb angle (65.9 ± 9.1% vs. 51.5 ± 37.3%, p = 0.040), more tilted instrumentation (10.3 ± 3.6° vs. 3.2 ± 3.1°, p = 0.001) and reverse tilt and translation of upper instrumented vertebra (UIV) to convex side (8.0 ± 2.3° vs. -3.4 ± 5.9°, p < 0.001; 35.4 ± 6.9 mm vs. 12.3 ± 13.1 mm, p = 0.001) in the uncorrected imbalanced group. Multiple linear regression analysis revealed that △UIV translation (pre- to post-operation) (ß = 0.832; p = 0.030) was significantly correlated with the correction of CBD. CONCLUSION: Thoracolumbar/lumbar CCI in dystrophic scoliosis was prone to suffer high risk of persistent post-op CCI. Satisfying coronal rebalance should avoid UIV tilt and translation to the convex side, tilted morphology of instrumentation and over correction maneuvers for main curve, the upper hemi-curve region in particular.

Could screw/hook insertion at the apical vertebrae with rib head dislocation effectively retract the corresponding rib head from spinal canal in dystrophic scoliosis secondary to type 1 neurofibromatosis?

BACKGROUND: Rib head dislocation (RHD) in dystrophic scoliosis of type 1 neurofibromatosis (DS-NF1) is a unique disorder caused by skeletal dystrophy and scoliotic instability. No particular surgical manipulation is mentioned in the literature to instruct the spine surgeons to effectively obtain more migration of the dislocated rib head without resection. The present study aimed to investigate the effectiveness of screw/hook insertion at vertebrae with RHDs on the retraction of penetrated rib head from spinal canal. METHODS: 37 neurologically intact patients with DS-NF1 and concomitant 53 RHDs undergoing scoliosis surgery without rib head excision were retrospectively reviewed. We used pre and postoperative whole-spine radiographs to determine the Cobb angle and the vertebral translation (VT), and the CT scans to evaluate the intraspinal rib length (IRL) and rib-vertebral angle (RVA). The dislocated ribs were assigned into two groups according to the presence of screw/hook insertion at vertebrae with RHD: screw/hook group and non-screw/hook group. RESULTS: 37 dislocated ribs with screws/hooks insertion at corresponding vertebrae were assigned into the screw/hook group and the remaining 16 dislocated ribs consisted of the non-screw/hook group. In the screw/hook group, the correction rates of Cobb angle and VT were significantly higher than the non-screw/hook group after surgery (58.7 ± 16.0% vs. 30.9 ± 12.4%, p = 0.003; 61.8 ± 18.8% vs. 35.1 ± 16.6%, p = 0.001; respectively). Similarly, more correction rates of IRL and RVA were found in the screw/hook group than the non-screw/hook group (63.1 ± 31.3% vs. 30.1 ± 20.7%, p = 0.008; 17.6 ± 9.7% vs. 7.2 ± 3.6%, p = 0.006; respectively). Multiple linear regression analysis revealed that the correction rates of Cobb angle, VT and RVA contributed significantly to correction of IRL (ß = 0.389, 0.939 and 1.869, respectively; p = 0.019, 0.001 and 0.002, respectively). CONCLUSION: Screw/hook insertion at dystrophic vertebrae with RHDs contributed significantly to the degree of retraction of penetrated rib head from spinal canal. This effectiveness is mediated by more corrections of VT and RVA.

Posterior 3-Column Osteotomy for Treatment of Congenital Kyphosis with Multiple Thoracolumbar/Lumbar Anterior Unsegmented Vertebrae: A Comparison Between Patients with Increasing Number of Anterior Unsegmented Vertebrae.

BACKGROUND: A 3-column osteotomy is sometimes challenging in congenital kyphosis (CK) with many anterior unsegmented vertebrae (AUVs). This study compared surgical outcomes of single-level 3-column osteotomy and associated complications in CK with increasing number of AUVs. METHODS: We retrospectively reviewed 25 consecutive patients with AUVs in CK who underwent surgery at a mean age of 16.2 ± 10.3 years. Patients were stratified into 2 groups according to the number of AUVs: 3 AUVs and ≥4 AUVs. Osteotomy types, surgical outcomes, and related complications were analyzed and compared between groups. RESULTS: The 3 AUVs group comprised 13 patients, and the ≥4 AUVs group comprised 12 patients. Pedicle subtraction osteotomy, grade 4 osteotomy, vertebral column resection, and vertebral column decancellation accounted for 15.4%, 38.5%, 46.1%, and 0% of procedures in the 3 AUVs group and 8.3%, 0%, 83.3%, and 8.3% of procedures in the ≥4 AUVs group. Preoperative focal kyphosis, which was significantly higher in the ≥4 AUVs group (82.9° ± 28° vs. 59.7° ± 9.4°, P = 0.010), was corrected in both groups postoperatively. The ≥4 AUVs group had significantly higher remaining kyphosis (33.6° ± 13.4° vs. 15.1° ± 9.1°, P < 0.001) with a significantly lower correction rate (61.2% ± 13.6% vs. 75.0% ± 15.6%, P = 0.001). The complication rate, mainly involving vertebral subluxation and proximal junctional kyphosis, was significantly higher in the ≥4 AUVs group than the 3 AUVs group (8/12 vs. 1/13, P = 0.004). CONCLUSIONS: Posterior single-level 3-column osteotomy can achieve satisfactory kyphosis correction in CK with 3 AUVs. Decreasing kyphosis correction and increasing surgery-related complications are prone to develop when treating CK with ≥4 AUVs.

Curve evolution during bracing in children with scoliosis secondary to early-onset neurofibromatosis type 1: indicators of rapid curve progression.

BACKGROUND: Scoliosis secondary to neurofibromatosis type 1 (NF1) in children aged <10 years is an important etiology of early-onset scoliosis (EOS). This study was performed to investigate the curve evolution of patients with EOS secondary to NF1 undergoing bracing treatment and to analyze high-risk indicators of rapid curve progression. METHODS: Children with EOS due to NF1 who underwent bracing treatment from 2010 to 2017 were retrospectively reviewed. The angle velocity (AV) at each visit was calculated, and patients with rapid curve progression (AV of >10°/year) were identified. The age at modulation and the AV before and after modulation were obtained. Patients with (n = 18) and without rapid curve progression (n = 10) were statistically compared. RESULTS: Twenty-eight patients with a mean age of 6.5 ±â€Š1.9 years at the initial visit were reviewed. The mean Cobb angle of the main curve was 41.7°â€Š±â€Š2.4° at the initial visit and increased to 67.1°â€Š±â€Š8.6° during a mean follow-up of 44.1 ±â€Š8.5 months. The overall AV was 6.6°â€Š±â€Š2.4°/year for all patients. At the last follow-up, all patients presented curve progression of >5°, and 20 (71%) patients had progressed by >20°. Rapid curve progression was observed in 18 (64%) patients and was associated with younger age at the initial visit and a higher incidence of modulation change during follow-up (t = 2.868, P = 0.008 and <0.001, respectively). The mean AV was 4.4°â€Š±â€Š1.2°/year before modulation and 11.8°â€Š±â€Š2.7°/year after modulation (t = 11.477, P < 0.010). CONCLUSIONS: Curve progression of >10°/year is associated with younger age at the initial visit, and modulation change indicated the occurrence of the rapid curve progression phase.

Both structural damage and inflammation of the lumbar spine contribute to the sagittal imbalance in ankylosing spondylitis patients with thoracolumbar kyphosis.

BACKGROUND: The relationship between structural damage and inflammation of the spine and the sagittal imbalance in ankylosing spondylitis (AS) is not well understood. The present study aimed to investigate the correlation between structural damage and inflammation of the lumbar spine and the sagittal imbalance in AS patients with thoracolumbar kyphosis. METHODS: Forty-five AS patients with thoracolumbar kyphosis were retrospectively reviewed. Six sagittal spinal parameters, including the C7 tilt (C7T), spino-sacral angle (SSA), global kyphosis (GK), the sagittal vertical axis (SVA), thoracic kyphosis (TK), and lumbar lordosis (LL), were measured. Structural damage of the lumbar spine was assessed by the modified Stoke AS Spine Score (mSASSS) on radiographs. Lumbar spinal inflammation was evaluated by the AS spinal magnetic resonance imaging (MRI) activity (ASspiMRI-a) on MRI. Correlation analysis was performed using the paired sample t-test. Multivariable linear regression models were constructed to analyze the contributions of mSASSS and ASspiMRI-a to the sagittal parameters. RESULTS: The average values of the sagittal parameters C7T, SSA, GK, SVA, TK, and LL were 68.1°, 80.1°, 77.3°, 168.7 mm, 47.7°, and -0.7°, respectively. The average mSASSS and ASspiMRI-a scores were 9.8 and 10.8, respectively. Correlation analysis showed that the mSASSS and ASspiMRI-a were correlated with C7T, SSA, SVA, and LL (the Spearman correlation coefficients were -0.439, -0.390, 0.424, and 0.530 for mSASSS; -0.406, -0.402, 0.378, and 0.486 for ASspiMRI-a; P<0.05). The C7T, SSA, and SVA were significantly correlated with LL (r=-0.696, -0.779, and 0.633, respectively; P<0.05). There was a weak correlation between the mSASSS and ASspiMRI-a (ß=0.299, P=0.046). The multivariable regression models indicated that the sagittal imbalance was determined to a greater extent by the mSASSS than ASspiMRI-a (the ß values were -1.550 vs. -0.649 for C7T, -1.865 vs. -1.231 for SSA, 9.161 vs. 3.823 for SVA, and 3.128 vs. 1.717 for LL). CONCLUSIONS: Both structural damage and inflammation of the lumbar spine contributed to the sagittal imbalance in AS patients with thoracolumbar kyphosis. In the late stages of AS, the sagittal imbalance was more attributable to the structural damage than the inflammation of the lumbar spine.

Assessing the unique characteristics associated with surgical treatment of dystrophic lumbar scoliosis secondary to neurofibromatosis type 1: a single-center experience of more than 10 years.

OBJECTIVE: Dystrophic lumbar scoliosis secondary to neurofibromatosis type 1 (DLS-NF1) may present an atypical, unique curve pattern associated with a high incidence of coronal imbalance and regional kyphosis. Early surgical intervention is complicated and risky but necessary. The present study aimed to assess the unique characteristics associated with the surgical treatment of DLS-NF1. METHODS: Thirty-nine consecutive patients with DLS-NF1 treated surgically at a mean age of 14.4 ± 3.9 years were retrospectively reviewed. Patients were stratified into three types according to the coronal balance classification: type A (C7 translation < 30 mm), 22 patients; type B (concave C7 translation ≥ 30 mm), 0 patients; and type C (convex C7 translation ≥ 30 mm), 17 patients. Types B and C were considered to be coronal imbalance. The diversity of surgical strategies, the outcomes, and the related complications were analyzed. RESULTS: The posterior-only approach accounted for 79.5% in total; the remaining 20.5% of patients received either additional anterior supplemental bone grafting (12.8%) to strengthen the fixation or convex growth arrest (7.7%) to reduce growth asymmetry. The lower instrumented vertebra (LIV) being L5 accounted for the largest share (41%), followed by L4 and above (35.9%), the sacrum (15.4%), and the pelvis (7.7%). Type C coronal imbalance was found in 23 patients (59%) postoperatively, and the incidence was significantly higher in the preoperative type C group (14/17 type C vs 9/22 type A, p = 0.020). All the patients with postoperative coronal imbalance showed ameliorative transition to type A at the last visit. The rate of screw malposition was 30.5%, including 9.9% breached medially and 20.6% breached laterally, although no serious neurological impairment occurred. The incidence of rod breakage was 16.1% (5/31) and 0% in patients with the posterior-only and combined approaches, respectively. Four revisions with satellite rods and 1 revision with removal of iliac screw for penetration into the hip joint were performed. CONCLUSIONS: Surgical strategies for DLS-NF1 were diverse across a range of arthrodesis and surgical approaches, being crucially determined by the location and the severity of dystrophic changes. The LIV being L5 or lower involving the lumbosacral region and pelvis was not rare. Additional posterior satellite rods or supplementary anterior fusion is necessary in cases with insufficient apical screw density. Despite a high incidence of postoperative coronal imbalance, improvement of coronal balance was frequently confirmed during follow-up. Neurological impairment was scarce despite the higher rate of screw malposition.

Utilization of distal radius and ulna classification scheme in predicting growth peak and curve progression in idiopathic scoliosis girls undergoing bracing treatment.

PURPOSE: Distal radius and ulna (DRU) classification scheme has been proposed for predicting skeletal maturity in patients with idiopathic scoliosis (IS). However, the utilization of DRU classification scheme in the assessment of growth peak and curve progression in IS was still inconclusive. This study aimed to correlate the distal radius and ulna stages with several indicators for growth potential and to evaluate the predictive value of DRU system for curve progression in braced female IS patients. METHODS: This was a consecutive longitudinal study including physically immature IS girls receiving standardized bracing treatment and regularly followed up every 3-6 months until brace weaning. The following data of each visit were collected: chronologic age, standing height, Cobb angle, spinal length, Risser sign, digital skeletal age (DSA) scores and DRU scores. The height velocity (HV), spinal growth velocity (SGV) and angle velocity (AV) of each visit were calculated. The correlation among radius stage, ulna stage, Risser sign, height, spinal length, HV, SGV and AV was studied. RESULTS: Forty braced IS girls with 349 longitudinal whole spine X-rays were reviewed. The average DRU scores at initial visit were R6.5 ± 1.1 and U4.5 ± 1.2 for radius and ulna, respectively. Both the radius stages between R5 and R8 and ulna stages between U3 and U6 indicated high SGV and high HV. The DSA scores were 402.1 ± 48.8 and 430.8 ± 44.4 at R7 and R8, respectively. The AV values were - 5.9 ± 12.4°/y and - 0.4 ± 1.5°/y at R5 and R6, which increased to 5.9 ± 17.3°/y, 3.1 ± 15.7°/y and 4.2 ± 12.2°/y at R7, R8 and R9, respectively. The DSA scores were 387.3 ± 65.7 for U5 and 432.9 ± 48.5 for U6, respectively. The AV values were - 3.1 ± 0.3°/y at U3, - 1.7 ± 9.3°/y at U4, 2.3 ± 16.1°/y at U5, 5.4 ± 15.5°/y at U6 and 4.4 ± 12.9°/y at U7. CONCLUSIONS: Both distal radius and ulna scores correlate with the longitudinal growth potential, and thus, the DRU scoring scheme is an alternative predictor for growth potential and curve progression in girls with IS. These slides can be retrieved under Electronic Supplementary Material.

Quantitation Analysis of PCDH10 Methylation in Adolescent Idiopathic Scoliosis Using Pyrosequencing Study.

STUDY DESIGN: A retrospective and comparative study. OBJECTIVE: To evaluate the difference of DNA methylation in protocadherin10 (PCDH10) genes between adolescent idiopathic scoliosis (AIS) and normal controls, and to assess the association between DNA methylation and the etiology of AIS. SUMMARY OF BACKGROUND DATA: The PCDH10 gene showed abnormal expression in AIS. However, the mechanism was still unclear. DNA methylation was an important epigenetic mechanism at the interface between genetics and environmental phenotype, seeming to be a suitable epigenetic mark for the abnormal expression of PCDH10 in AIS. METHODS: There were 50 AIS patients and 50 healthy controls included in the study. The peripheral blood sample of each participant was taken. The pyrosequencing assay was used to assess the methylation status of PCDH10 promoter and real time PCR (RT-PCR) was used to detect the PCDH10 gene expression. The comparison analysis was performed using independent t test and 2-tailed Pearson coefficients was calculated for the correlation analysis. RESULTS: The average methylation level was 4.32 ±â€Š0.73 in AIS patients and 3.14 ±â€Š0.97 in healthy controls (P < 0.001). The PCDH10 gene expression was 0.23 ±â€Š0.04 in AIS patients and 0.36 ±â€Š0.08 in normal controls (P < 0.0001). Statistically significant linear correlation was found between PCDH10 gene methylation level and Cobb angle of major curve (P < 0.001). Besides, a significant negative correlation between PCDH10 methylation and PCDH10 gene expression was found (P < 0.001). CONCLUSION: AIS patients were associated with high DNA methylation level and low gene expression of PCDH10 gene rather than normal controls. The high methylation level indicated high Cobb angle of major curves in AIS. The abnormal DNA methylation may widely exist and serve as a potential mechanism for AIS. LEVEL OF EVIDENCE: 3.

Sequential correction technique to avoid postoperative global coronal decompensation in rigid adult spinal deformity: a technical note and preliminary results.

PURPOSE: This study aims to evaluate this new sequential correction technique for preventing postoperative coronal imbalance. METHODS: Adult Spinal deformity (ASD) patients were stratifies into two types: primary thoracolumbar/lumbar (TL/L) curve with compensatory lumbosacral (LS) curve (Type I) and primary LS curve with compensatory TL/L curve (Type II): for Type I patients: correction of major TL/L curve and one- or two-level segmental rod installed at the convexity of the TL/L curve, L4-S1 TLIF to correct fractional curve and a short rod installed on the contralateral side and installation of long rods; for Type II patients: horizontalize L4 and L5, short rod installation at the convexity of the LS curve, distraction of curve with regional rod and installation of long rods. ASD patients were enrolled with inclusion criteria: with pre-op TL/L Cobb angle more than 30°, with pelvic fixation and with UIV over T10. Radiographic parameters were analyzed. RESULTS: Twenty-one patients were recruited (14 patients Type I and 7 Type II patients). Both Cobb angle and coronal offset were significantly improved after surgery. In Type I patients, Cobb angle was improved from 50.48° to 26.91° and coronal offset from 2.94 to 0.95 cm; in Type II patients, Cobb angle was improved from 61.42° to 28.48° and coronal offset from 2.82 to 1.38 cm. In the 10 patients with baseline coronal imbalance, 9 were corrected to coronal balance after surgery. CONCLUSION: The sequential correction technique allows decomposing the complex correction surgery into several steps, and each step focuses only on one task. It can also reduce the difficulty of rod installation due to the separated maneuvers and multi-rod system. LEVEL OF EVIDENCE: IV These slides can be retrieved under Electronic Supplementary Material.

Does relocation of the apex after osteotomy affect surgical and clinical outcomes in patients with ankylosing spondylitis and thoracolumbar kyphosis?

OBJECTIVE: Relocation of the apex is often found in patients with ankylosing spondylitis (AS)-associated thoracolumbar/lumbar kyphosis after corrective surgery. This study evaluates the influence of different postoperative apex locations on surgical and clinical outcomes of osteotomy for patients with AS and thoracolumbar kyphosis. METHODS: Sixty-two patients with a mean age of 34.6 ± 9.7 years (range 17-59 years) and a minimum of 2 years of follow-up, who underwent 1-level lumbar pedicle subtraction osteotomy for AS-related thoracolumbar kyphosis, were enrolled in the study, as well as 62 age-matched healthy individuals. Patients were divided into 2 groups according to the postoperative location of the apex (group 1, T8 or above; group 2, T9 or below). Demographic data, radiographic measurements (including 3 postoperative apex-related parameters), and clinical outcomes were compared between the 2 groups preoperatively, postoperatively, and at the last follow-up. Furthermore, a subgroup analysis was performed among patients with a postoperative apex located at T6-11 and postoperatively the entire AS cohort was compared with normal controls regarding the apex location of the thoracic spine. RESULTS: In the majority of the enrolled patients, the apex location changed from T12-L2 preoperatively to T6-9 postoperatively. The sagittal vertical axis (SVA) differed significantly both postoperatively (25.7 vs 59.0 mm, p = 0.001) and at the last follow-up (34.6 vs 59.9 mm, p = 0.003) between the 2 groups, and the patients in group 1 had significantly smaller horizontal distance between the C7-vertical line and the apex (DCA) than the patients in group 2 (67.5 vs 103.7 mm, p = 0.001). Subgroup analysis demonstrated similar results, showing that the patients with a postoperative apex located at T8 or above had an average SVA < 47 mm. Notably, a significant correlation was found between postoperative SVA and DCA (r = 0.642, p = 0.001). Patients who underwent an osteotomy at L3 had limited apex relocation but larger SVA correction than those at L1 or L2. However, no significant difference was found in health-related quality of life between the 2 groups. CONCLUSIONS: AS patients with an apex located at T8 or above after surgery tended to have better SVA correction (within 47 mm) than those who had a more caudally located apical vertebra. For ideal postoperative apex relocation, a higher (closer to or at the preoperative apex) level of osteotomy is more likely to obtain the surgical goal.

Radiologic and Pathological Investigation of Pseudarthrosis in Ankylosing Spondylitis: Distinguishing Between Inflammatory and Traumatic Etiology.

OBJECTIVE: To investigate the pathogenesis of pseudarthrosis in ankylosing spondylitis (AS) based on the pathological analysis of specimens harvested during surgery. METHODS: Radiographic and clinical data for 17 consecutive AS patients with pseudarthrosis were retrospectively analyzed. Meanwhile, the pathological analysis of specimens obtained during surgery was also performed. RESULTS: In total, 18 extensive Andersson lesions were included. Pseudarthrosis located at the apical region were noted in 12 patients. Complete ossified anterior longitudinal ligaments above or below pseudarthrosis and fracture through posterior elements or facet joints were observed in 7 and 6 lesions, respectively. The most definitive pathological characteristic in all cases was proliferating hypovascular edematous fibrous tissue involving disc, bone-disc border, and vertebral body. Fibrinoid necrosis, necrotic bone fragments, hemosiderin deposits, and active subchondral osteogenesis were found, indicating trauma process. Mild perivascular collections of inflammatory cells were detected in only 2 cases. CONCLUSION: AS-related pseudarthrosis is more likely to originate from mechanical trauma than inflammation. The above-mentioned radiological and histological findings showed that multiple mechanisms lead to the formation of pseudarthrosis. These mechanisms include excessive stress, insufficiency fracture, and an acute fracture involving a 3-column structure.

Complications of spinal osteotomy for thoracolumbar kyphosis secondary to ankylosing spondylitis in 342 patients: incidence and risk factors.

OBJECTIVETo describe the incidence of complications in spinal osteotomy for thoracolumbar kyphosis caused by ankylosing spondylitis (AS) and to investigate the risk factors for these complications.METHODSFrom April 2000 to July 2017, 342 consecutive AS patients with a mean age (± SD) of 35.4 ± 9.8 years (range 17-71 years) undergoing spinal osteotomy were enrolled. Patients with complications within the 1st postoperative year were identified. Demographic, radiological, and surgical data were compared between patients with and without complications. The complications were classified into intraoperative and postoperative complications.RESULTSA total of 310 consecutive pedicle subtraction osteotomy (PSO) and 37 multiple Smith-Petersen osteotomy (SPO) procedures were performed in 342 patients. Overall, 47 complications were identified in 47 patients (13.7%), including 31 intraoperative complications and 16 postoperative complications. Patients with complications were older than those without (p = 0.006). A significant difference was observed in preoperative global kyphosis (GK), lumbar lordosis (LL), sagittal vertical axis (SVA), and the correction of these radiographic parameters between patients with and without complications (p < 0.05). Two-level PSO (p = 0.022) and an increased number of instrumented vertebrae (p = 0.019) were significantly associated with an increased risk of complications.CONCLUSIONSThe overall incidence of complications was 13.7%. Age; preoperative GK, LL, and SVA; the correction of GK, LL, and SVA; 2-level PSO; and number of instrumented vertebrae were risk factors. Therefore, the potential risk of extensive surgeries with large correction and long fusion in older AS patients with severe GK should be seriously considered in surgical decision-making.

Accuracy of Freehand Pedicle Screw Placement in Surgical Correction of Thoracolumbar Kyphosis Secondary to Ankylosing Spondylitis: A Computed Tomography Investigation of 2314 Consecutive Screws.

OBJECTIVE: To evaluate the accuracy and safety of freehand pedicle screw placement in surgical correction for thoracolumbar kyphosis caused by ankylosing spondylitis (AS). METHODS: We retrospectively reviewed 266 consecutive patients with AS who underwent osteotomy for kyphosis correction with freehand screw insertion from January 1998 to April 2015 at our institution. A total of 2314 pedicle screws in 158 patients with AS with postoperative computed tomography scans were included in the study. Postoperative computed tomography was performed to classify accuracy of screws, using the established Gertbein classification (grade 0: no perforation, grade 1: perforation <2 mm, grade 2: perforation between 2 and 4 mm, and grade 3: perforation >4 mm). Patients were divided into 2 groups according to coronal Cobb angle: group A (n = 21, Cobb angle ≥10°), group B (n = 137, Cobb angle <10°). RESULTS: Among the 2314 pedicle screws, 2168 pedicle screw placements were categorized as grade 0, 71 were grade 1, 51 were grade 2, and 24 were grade 3. Breaches occurred more frequently in L1-S1 than the thoracic spine (7.1% and 5.4%, respectively). T5 (25.0%) and S1 (17.7%) experienced the greatest breach rate, whereas T8, L1, and L3 had the lowest breach rate. The breach rate of group A was greater than that of group B (7.9% vs. 6.1%). None of the breaches resulted in either neurologic deficits or vascular complications. CONCLUSIONS: Freehand pedicle screw placement can be performed safely with acceptable breach rate in patients with AS and thoracolumbar kyphosis.

Abnormal PITX1 gene methylation in adolescent idiopathic scoliosis: a pilot study.

BACKGROUND: The gene of pituitary homeobox 1 (PITX1) has been reported to be down-regulated in adolescent idiopathic scoliosis (AIS), of which the cause has not been well addressed. The abnormal DNA methylation was recently assumed to be an important mechanism for the down-regulated genes expression. However, the association between PITX1 promoter methylation and the etiology of AIS was not clear. METHODS: The peripheral blood samples of 50 AIS patients and 50 healthy controls were collected and the genomic DNA was extracted. The pyrosequencing assay was used to assess the methylation status of PITX1 promoter and real-time quantitative polymerase chain reaction (PCR) was used to detect the PITX1 gene expression. Comparison analysis was performed using independent t test and Chi-square tests, while correlation analysis were performed with 2-tailed Pearson coefficients. RESULTS: The mean methylation level was (3.52 ± 0.96)% in AIS and (1.40 ± 0.81)% in healthy controls (P < 0.0001). The PITX1 gene expression was 0.15 ± 0.08 in AIS and 0.80 ± 0.55 in healthy controls (P < 0.0001). The comparative analysis showed significant difference in age (P = 0.021) and Cobb angle of the main curve (P = 0.0001) between AIS groups with positive and negative methylation. The methylation level of 6 CpG sites in PITX1 promoters was significantly associated with Cobb angle of the main curve (P < 0.001) in AIS. No statistical relationship between PITX1 promoter methylation and gene expression was found in AIS (P = 0.842). CONCLUSION: Significantly higher methylation level and lower PITX1 gene expression are found in AIS patients. PITX1 methylation is associated with Cobb angles of the main curves in AIS. DNA methylation thus plays an important role in the etiology and curve progression in AIS.