Instrumentation failure following pediatric spine deformity growth-sparing surgery using traditional growing rods or vertical expandable prosthetic titanium ribs.

BACKGROUND: Instrumentation failure (IF) is a major complication associated with growth-sparing surgery for pediatric spinal deformities; however, studies focusing on IF following each surgical procedure are lacking. We aimed to evaluate the incidence, timing, and rates of unplanned return to the operating room (UPROR) associated with IF following each surgical procedure in growth-sparing surgeries using traditional growing rods (TGRs) and vertical expandable prosthetic titanium ribs (VEPTRs). METHODS: We reviewed 1,139 surgical procedures documented in a Japanese multicenter database from 2015 to 2017. Of these, 544 TGR and 455 VEPTR procedures were included for evaluation on a per-surgery basis. IF was defined as the occurrence of an implant-related complication requiring revision surgery. RESULTS: The surgery-based incidences of IF requiring revision surgery in the TGR and VEPTR groups were 4.3% and 4.0%, respectively, with no significant intergroup difference. Remarkably, there was a negative correlation between IF incidence per surgical procedure and the number of lengthening surgeries in both groups. In addition, rod breakage in the TGR group and anchor-related complications in the VEPTR group tended to occur relatively early in the treatment course. The surgery-based rates of UPROR due to IF in the TGR and VEPTR groups were 2.0% and 1.5%, respectively, showing no statistically significant difference. CONCLUSIONS: We found that IF, such as anchor related-complications and rod breakage, occurs more frequently earlier in the course of lengthening surgeries. This finding may help in patient counseling and highlights the importance of close postoperative follow-up to detect IF and improve outcomes.

Does Flexion Varus Osteotomy Improve Radiographic Findings Compared With Patients Treated in a Brace for Late-onset Legg-Calvé-Perthes Disease?

BACKGROUND: Legg-Calvé-Perthes disease (LCPD) is a childhood hip disease characterized by osteonecrosis of the femoral head. Because severe deformity of the femoral head can cause secondary osteoarthritis in adulthood, progressive collapse should be prevented in children with a necrotic epiphysis. The prognosis of patients with LCPD generally worsens as the age at disease onset increases, and the appropriate treatment for late-onset LCPD remains unclear. Based on the limited effect of nonoperative treatment using a nonweightbearing brace, flexion varus osteotomy (FVO) was introduced in 2010 as an initial treatment for late-onset LCPD in place of brace treatment, which we used in our institution before that time. QUESTIONS/PURPOSES: We asked, (1) Which treatment, FVO or a nonweightbearing brace, is associated with a lower likelihood of progressive femoral head collapse in children whose diagnosis of LCPD was made at the age of ≥ 8 years and who were followed for a minimum of 3 years after their intervention? (2) What proportion of patients in the brace group had surgery despite the treatment, and what percentage of children in the FVO group had a second operation to remove hardware and/or additional operations? METHODS: The initial treatment was applied in 181 patients with LCPD between 1995 and 2018 in our institution. Patients whose disease onset was at ≥ 8 years old (late-onset LCPD) with complete clinical and radiologic data were considered potentially eligible. In 2010, treatment for these patients changed from brace treatment to FVO for all patients. A total of 35% (42 of 121) of patients who were treated with a nonweightbearing brace between 1995 and 2009 and 40% (24 of 60) of patients who were treated with FVO between 2010 and 2018 were eligible. Among patients treated with a brace, 21% (nine of 42 patients) were excluded because of hospital transfer (three patients), short-term follow-up (three), the period from onset to the first visit was ≥ 7 months (two), and inability to use the brace because of mental incapacity (one patient). In patients treated with FVO, 12% (three of 24 patients) were excluded (two patients with a period from onset to the first visit ≥ 7 months and one with a comorbidity and multiple-epiphyseal dysplasia). Among the remaining patients, 79% (33 of 42 patients) were classified into the brace group and 88% (21 of 24 patients) were classified into the FVO group for analyses. There were no overlapping patients at the timepoint when the treatment strategy for late-onset LCPD changed. In the FVO group, subtrochanteric osteotomy with 35° to 40° of flexion and 15° to 20° of varus was performed using a locking compression plate for pediatric use. Patient demographics, radiographic parameters, and the assessment of femoral head deformity using the Stulberg classification were compared between the two groups. There was a greater proportion of boys than girls in both groups (brace: 88% and FVO: 86%), and there were no differences in the distribution of genders between the groups (p = 0.82). The right side was more frequently treated in the brace group, but there was no difference in laterality between the groups (brace: 58% right and FVO: 62% left; p = 0.16). There was no difference between groups in the median age at disease onset (9.0 years [range 8.0 to 12.5 years] in the brace group and 9.6 years [range 8.0 to 12.4 years] in the FVO group; p = 0.26). There was no difference between the groups in the period of treatment from onset (1.7 ± 1.9 months in the brace group and 1.5 ± 1.5 months in the FVO group; p = 0.73) or the follow-up period (6.7 ± 2.1 years in the brace group and 6.2 ± 2.1 years in the FVO group; p = 0.41). The LCPD stage at the first visit was assessed using the modified Waldenström classification. The intraobserver and interobserver values of the modified Waldenström classification, evaluated using kappa statistics, were excellent (kappa value 0.89 [95% CI 0.75 to 0.97]; p < 0.01) and good (kappa value 0.65 [95% CI 0.43 to 0.87]; p < 0.01). The radiographic degree of collapse at the maximum fragmentation stage was assessed using the lateral pillar classification. The intraobserver and interobserver reliabilities of the lateral pillar classification were excellent (kappa value 0.84 [95% CI 0.73 to 0.94]; p < 0.01) and excellent (kappa value 0.83 [95% CI 0.71 to 0.94]; p < 0.01). The degree of femoral head deformity at the most recent follow-up examination was compared between the groups in terms of the Stulberg classification, in which Classes I and II were classified as good and Classes III through V were classified as poor. The intraobserver and interobserver reliabilities of the Stulberg classification were good (kappa value 0.74 [95% CI 0.55 to 0.92]; p < 0.01) and good (kappa value 0.69 [95% CI 0.50 to 0.89]; p < 0.01). The evaluators were involved in the patients' clinical care as part of the treating team. RESULTS: Good radiographic results (Stulberg Class I or II) were obtained more frequently in the FVO group (76% [16 of 21 patients]) than in the brace group (36% [12 of 33 patients]), with an odds ratio of 5.6 (95% CI 1.7 to 18.5; p < 0.01). In the brace group, a subsequent femoral varus osteotomy was performed in 18% (six of 33) of patients with progressive collapse and hinge abduction, and implant removal surgery was performed approximately 1 year after the first procedure. This traditional varus osteotomy was occasionally performed in patients who were considered for conversion from nonoperative treatment before 2009 because FVO had not yet been introduced. In the FVO group, all patients (n = 21) had a second procedure to remove the implant at a mean of 10.5 ± 1.2 months postoperatively. Additional procedures were performed in 24% (five of 21) of patients, including a second FVO for progressive collapse (one patient), guided growth for a limb length discrepancy (one patient), and flexion valgus osteotomy for coxa vara in patients with a limb length discrepancy (three patients). CONCLUSION: Our historical control study found that FVO may increase the possibility of obtaining good radiographic results (Stulberg Class I or II) compared with brace treatment for patients with late-onset LCPD, although surgical interventions after the first and second implant removal procedures may be indicated. Surgeons can consider FVO if they encounter patients with late-onset LCPD, which is a challenging condition. A larger study with long-term follow-up is needed to confirm the efficacy of FVO. LEVEL OF EVIDENCE: Level III, therapeutic study.

A Novel Method for Assessing the 3-dimensional Morphology of Cartilaginous Acetabulum Via Childhood Magnetic Resonance Imaging.

BACKGROUND: Periarticular cartilage is abundant in children, making evaluations of 3-dimensional (D) cartilaginous acetabular morphology using x-ray or computed tomography (CT) difficult. The study aimed to visualize the 3D cartilaginous acetabular morphology in normal children and patients with pediatric developmental dysplasia of the hip (DDH). METHODS: Magnetic resonance imaging (MRI) of 17 female children without acetabular dysplasia at 7.5 years and CT of 33 normal female adolescents with mature bones at 14.6 years were used as controls. Subjects were 26 female patients with unilateral DDH who underwent angulated Salter innominate osteotomy (A-SIO) at 5.5 years. Preoperative and postoperative MRIs were performed at 5.2 and 7.0 years, respectively. The MRI sequence was 3D-MEDIC. The medial intersection (point A) of the line connecting the centers of the bilateral femoral head and the femoral head were defined as point zero. The 3D coordinates (X, Y, Z) of the cartilaginous acetabular edge (point C) from anterior to posterior were calculated. Subsequently, a 3D scatter plot was created using 3D graph software. The subjects were divided into 6 groups, including control MRI, control CT, unaffected DDH before and after A-SIO, and affected DDH before and after A-SIO. The femoral head coverage ratio (FHCR: AC/AB) was used to quantify coverage and was compared in each group. RESULTS: In the control MRI group, the acetabular coverage was small anteriorly, largest anterolaterally, and gradually decreased posteriorly, similar to the bony acetabulum in adolescents. In the affected DDH before A-SIO group, the coverage was significantly lower than that of the control MRI and unaffected DDH groups. After A-SIO, the morphology improved beyond the unaffected DDH and the control MRI group. CONCLUSIONS: The global defect of the cartilaginous acetabulum in the affected DDH group was significantly improved to normal morphology after A-SIO. Evaluating the cartilaginous acetabulum using MRI was useful for assessing hip morphology in childhood. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Hip Prognosis in Patients With Congenital Diastasis of the Pubic Symphysis.

BACKGROUND: Congenital diastasis of the pubic symphysis (CDPS) is a rare musculoskeletal malformation in the exstrophy-epispadias complex that is potentially associated with the development of hip dysplasia. The purpose of this study was to investigate the incidence and prognostic factors of hip dysplasia in patients with CDPS. METHODS: Fifty-four hips in 27 patients with CDPS initially evaluated between 1983 and 2016 were retrospectively reviewed. The mean age at the first visit was 2.3 (0 to 8) years. The mean follow-up duration was 10.5 (1 to 36) years. Patient characteristics at the first visit, the clinical course at the most recent follow-up, and radiologic parameters on pubic malformation and hip dysplasia during at least 2 time points (first visit or age 1, and either the most recent visit or before hip surgery) were evaluated. Prognostic factors associated with the development of hip dysplasia were analyzed using univariate/multivariate analysis. The Kaplan-Meier survival curves were generated and compared based on these factors. RESULTS: Nine of 27 patients (33%) and 13 of 54 hip joints (24%) with CDPS developed hip dysplasia. Paraplegia (odds ratio, 10.0; 95% confidence interval, 1.7-76.6) and center-edge angle of <5 degrees at the first visit or age 1 (P<0.001) were independent predictors of the development of hip dysplasia. Patients with CDPS and either paraplegia or center-edge angle <5 degrees at the first visit or age 1 were significantly more likely to develop hip dysplasia than other patients (hazard ratio, 29.3; 95% confidence interval, 3.4-250). CONCLUSIONS: Approximately one third of patients with CDPS develop hip dysplasia. Paraplegia and center-edge angle of <5 degrees at the first visit are independent risk factors. LEVEL OF EVIDENCE: Level III.

Associations between curve severity and revised Scoliosis Research Society-22 and scoliosis Japanese Questionnaire-27 scores in female patients with adolescent idiopathic scoliosis: a multicenter, cross-sectional study.

BACKGROUND: Patient-reported outcome measures are widely utilized to assess health-related quality of life (HRQOL) in patients with adolescent idiopathic scoliosis (AIS). However, the association between HRQOL and curve severity is mostly unknown. The aim of this study is to clarify the association between HRQOL and curve severity, and to determine the optimal cutoff values of patient-reported outcomes for major curve severity in female patients with AIS. METHODS: Female patients with AIS treated conservatively were recruited. The patients' HRQOL outcomes were examined using the revised Scoliosis Research Society-22 (SRS-22r) and the Scoliosis Japanese Questionnaire-27 (SJ-27). The correlations of the SRS-22r and SJ-27 scores with the major Cobb angle were assessed using Spearman's correlation coefficient analysis. The association between HRQOL issues in the SJ-27 and the major Cobb angle was evaluated by calculating Akaike's Information Criterion (AIC). Furthermore, the optimal cutoff values of the SRS-22r and SJ-27 scores for the major Cobb angle were determined by AIC analysis. RESULTS: The study cohort comprised 306 female patients with AIS. The SRS-22r and SJ-27 scores were significantly correlated with the major Cobb angle. Questions in the SJ-27 regarding discomfort when wearing clothes showed a lower AIC value in patients with severe scoliosis. The optimal cutoff values were a SRS-22r score of 3.2 for the discrimination of severe scoliosis (Cobb angle ≥48°), and a SJ-27 score of 32 for the discrimination of moderate scoliosis (Cobb angle ≥33°). CONCLUSION: Discomfort when wearing clothes was the most important HRQOL problem caused by severe scoliosis. The SRS-22r and SJ-27 scores are useful for the discrimination of clinical status in female patients with severe scoliosis or moderate scoliosis.

A longitudinal study for the prediction of the mature acetabular morphology using childhood magnetic resonance imaging.

BACKGROUND: Although acetabular dysplasia is a common etiology of osteoarthritis of the hip regardless of the history of developmental dysplasia of the hip (DDH), whether or not corrective surgeries are beneficial for the childhood asymptomatic acetabular dysplasia remains controversial due to a lack of evidence. We conducted a longitudinal study to compare the cartilaginous morphology on childhood magnetic resonance imaging (MRI) and the mature hip morphology of the same patient and to assess the predictive indicators for future acetabular dysplasia. METHODS: A total of 92 unaffected hips (47 unilateral DDH and 45 unilateral Legg-Calvé-Perthes disease) were reviewed for X-ray and MRI findings on childhood (mean age: 6.0 years) and X-ray findings from a skeletally mature age with a mean follow-up period of 15.1 years. The following parameters were measured and compared: the immature-acetabular index (AI) and center edge angle (CE) on immature X-ray; the cartilage- and bone- AI, CE, Sharp and acetabular head index (AHI) on childhood MRI; and the mature-acetabular roof obliquity (ARO), CE, Sharp and AHI on skeletally mature X-ray. The prognostic factors on childhood MRI for acetabular dysplasia, defined by a CE of <20° on skeletally mature X-ray were also assessed. RESULTS: Positive correlations were shown between the cartilage-AI and mature-ARO (7.6°/6.3°; r = 0.44), the cartilage-CE and mature-CE (27.8°/28.0°; r = 0.62), the cartilage-Sharp and mature-Sharp (44.4°/41.8°; r = 0.52) and the cartilage-AHI and mature-AHI (78.7%/80.3%; r = 0.46). A multivariate analysis indicated cartilage-CE to be an independent predictor for acetabular dysplasia with a cut-off value of 22°. Children with a cartilage-CE <22° developed more frequently acetabular dysplasia compared to the others (52.4% vs. 1.4%). CONCLUSIONS: Childhood MRI findings are useful for the prediction of acetabular dysplasia without a DDH history. Children with a cartilage-CE ≥23° are likely to achieve a non-dysplastic hip without the need for surgical intervention.

Terminal syringomyelia associated with lumbar limited dorsal myeloschisis.

PURPOSE: Limited dorsal myeloschisis (LDM) is characterized by a fibroneural tethering stalk linking the skin lesion to the underlying spinal cord. Terminal syringomyelia, which is located at the lower third of the cord, is often associated with a tethered cord caused by various spinal dysraphisms; however, terminal syringomyelia has not been documented in LDM. The purpose of this study was to clarify the pathophysiological mechanisms of syringomyelia in LDM. METHODS: In our 16 patients with lumbar LDM, three patients had terminal syringomyelia. We retrospectively analyzed the clinical, neuroradiological, intraoperative, and histopathological findings for these patients, with particular attention to the clinical course of the syrinx. RESULTS: Patient 1 had a saccular skin lesion and patients 2 and 3 had flat lesions. In all patients, the syringomyelic cavity was located in the lower thoracolumbar cord, immediately rostral to the stalk-cord attachment at the lumbar level. The caudal pole of the syrinx extended to the thickened stalk at the attachment instead of at the caudal cord. Patient 3 had another syrinx in the stalk itself. The longitudinal axis of the syrinx and central canal coincided with the traveling angle of the LDM stalk at the stalk-cord attachment. In patient 1, histology revealed an ependyma-lined central canal in both the LDM stalk and meningocele sac. Patients 1 and 2 underwent syringostomy, but long-term effects were not obtained. Preoperative spontaneous resolution occurred in patient 3. CONCLUSIONS: The histological findings in patient 1 supported the idea that segmental myelocystocele is involved in the development of saccular LDM. The hydromyelic central canal herniates and distends the stalk, resulting in the formation of the myelocystocele. It is possible that the hydromyelic central canal also distends the stalk of flat LDM lesions. The syrinx in patient 3 differed from that in patients 1 and 2, in that the syrinx resolved spontaneously. Further studies are needed to clarify the outcomes of syrinxes associated with LDM stalks.

Comparison of Early Definitive Fusion and Traditional Growing Rods in Early-onset Dystrophic Scoliosis in Neurofibromatosis Type 1: A Preliminary Report.

BACKGROUND: Early definitive spinal fusion (EF) has been widely recommended to avoid spinal deformity progression for early-onset scoliosis (EOS) with neurofibromatosis type 1 (NF-1). In contrast, growing rod (GR) procedure has recently been recommended for EOS associated with the dystrophic type NF-1. However, no studies have compared the surgical outcomes between EF and GR procedure for EOS with NF-1. The purpose of this study was to compare the surgical outcomes of EF versus GR for early onset and dystrophic scoliosis with NF-1. METHODS: This was a retrospective multicenter study; 26 EOS patients with dystrophic type of NF-1 who underwent EF (16 patients) or GR (10 patients) were investigated. We compared age at first surgery, fused levels, total number of surgeries, coronal major curve, complication rates, spinal height (T1-T12 and T1-S1 length), and forced vital capacity between the 2 groups. RESULTS: The mean age at first surgery was 7.3 years in the EF and 5.8 years in the GR (P<0.05). The mean follow-up period was 12.8 and 10.5 years, respectively. The 2 groups did not show significant differences in preoperative major Cobb angle (75.1 vs. 83.1 degrees), complication rate (53% vs. 60%), and increase rate of T1-T12 (20.0% vs. 30.4%). However, significant differences (P<0.05) were recognized in fusion segments (13.9 vs. 15.8), total numbers of surgery (2.6 vs. 10.1), correction rate of major curve (59.0% vs. 40.6%), increase rate of T1-S1 (19.0% vs. 33.9%), body height at final follow-up (151 vs. 142 cm), and forced vital capacity at final follow-up (2.23 vs. 1.46 L). CONCLUSIONS: Early fusion is still a viable option for progressive EOS with dystrophic type of NF-1 even in the growth-friendly era. Surgeons should consider the best procedure for each patient taking into account the severity of dystrophic change when treating EOS with NF-1. LEVEL OF EVIDENCE: Level IV.

A functional variant in MIR4300HG, the host gene of microRNA MIR4300 is associated with progression of adolescent idiopathic scoliosis.

Adolescent idiopathic scoliosis (AIS) is a common spinal deformity affecting millions of children. Since treatment and prognosis of AIS depend on curve progression, identifying factors related to AIS curve progression is important in its management. Although several genetic loci for AIS occurrence are reported, no locus for curve progression has been identified. To identify genes associated with AIS progression, we conducted a genome-wide association study followed by a replication study using a total of 2,543 AIS subjects who were evaluated for the curve progression. We identified a significantly associated locus on chromosome 11q14.1 (P = 1.98 × 10-9, odds ratio = 1.56). In silico and in vitro analyses identified a functional variant, rs35333564 in MIR4300HG, the host gene of a microRNA, MIR4300. The genomic region containing rs35333564 had enhancer activity, which was decreased in its risk allele. Our data suggest that decrease of MIR4300 is related to AIS progression.

A Functional SNP in BNC2 Is Associated with Adolescent Idiopathic Scoliosis.

Adolescent idiopathic scoliosis (AIS) is the most common spinal deformity. We previously conducted a genome-wide association study (GWAS) and detected two loci associated with AIS. To identify additional loci, we extended our GWAS by increasing the number of cohorts (2,109 affected subjects and 11,140 control subjects in total) and conducting a whole-genome imputation. Through the extended GWAS and replication studies using independent Japanese and Chinese populations, we identified a susceptibility locus on chromosome 9p22.2 (p = 2.46 × 10(-13); odds ratio = 1.21). The most significantly associated SNPs were in intron 3 of BNC2, which encodes a zinc finger transcription factor, basonuclin-2. Expression quantitative trait loci data suggested that the associated SNPs have the potential to regulate the BNC2 transcriptional activity and that the susceptibility alleles increase BNC2 expression. We identified a functional SNP, rs10738445 in BNC2, whose susceptibility allele showed both higher binding to a transcription factor, YY1 (yin and yang 1), and higher BNC2 enhancer activity than the non-susceptibility allele. BNC2 overexpression produced body curvature in developing zebrafish in a gene-dosage-dependent manner. Our results suggest that increased BNC2 expression is implicated in the etiology of AIS.

Reliability and validity of a novel quality of life questionnaire for female patients with adolescent idiopathic scoliosis: Scoliosis Japanese Questionnaire-27: a multicenter, cross-sectional study.

BACKGROUND: A progressive deformity associated with adolescent idiopathic scoliosis (AIS) negatively affects a patient's health-related quality of life (HRQOL). Although the Scoliosis Research Society-22 (SRS-22) is the standard measurement tool for assessing HRQOL in patients with AIS, it is partially suboptimal for evaluating HRQOL in Japanese patients with AIS because of cultural differences. The purpose of this study was to develop a novel patient-reported outcome measure for Japanese female patients with AIS and to evaluate the reliability and validity of this questionnaire in comparison with the SRS-22 tool. METHODS: We developed 27 questions based on the psychosocial problems in the daily life of young female patients with AIS in Japan, the Scoliosis Japanese Questionnaire-27 (SJ-27). To evaluate its reliability, the internal consistency was assessed using Cronbach's alpha coefficient. Concurrent validity was evaluated using Spearman's correlation coefficient between the SJ-27 and the SRS-22. To investigate the construct validity of the SJ-27, the correlation between the SJ-27 questions was assessed using Akaike's information criterion (AIC). RESULTS: We analyzed 384 female patients with AIS. Cronbach's alpha coefficients were 0.914 and 0.829 for the SJ-27 and the SRS-22, respectively. Spearman's correlation coefficient between the SJ-27 and the SRS-22 was 0.692 (p < 0.001). The AIC analysis indicated that the SJ-27 items are divided into five domains, indicating that the SJ-27 covered a wide range of health-related problems among female patients with AIS. CONCLUSIONS: The results suggest that the SJ-27 is a reliable and valid patient-reported outcome measure for evaluating HRQOL in female patients with AIS in Japan.

Postoperative shoulder imbalance in Lenke Type 1A adolescent idiopathic scoliosis and related factors.

BACKGROUNDS: The purpose of this study was to investigate the occurrence and factors associated with postoperative shoulder imbalance (PSI) in Lenke type 1A curve. METHODS: This study included 106 patients with Lenke Type 1A curve who were followed up more than two years after posterior correction surgery. Pedicle screw (PS) constructs were used in 84 patients, and hybrid constructs in 22. The upper instrumented vertebra was rostral to the upper-end vertebra (UEV) in 70 patients, at UEV in 26, and below UEV in 10. The clavicle angle and T1 tilt angle were measured as PSI indicators, and correlations between radiographic parameters of shoulder balance and other radiographic parameters and associations between PSI and clinical parameters were investigated. For statistical analyses, paired and unpaired t-tests were used. RESULTS: The mean Cobb angles of the main and proximal thoracic curves were 54.6 ± 9.5 and 26.7 ± 7.9 degrees before surgery, 14.5 ± 7.5, and 14.9 ± 7.1 at follow-up. Clavicle angle and T1 tilt angle were -2.9 ± 2.8 and -2.6 ± 6.3 before surgery, 2.4 ± 2.8 and 4.4 ± 4.3 immediately after surgery, and 1.8 ± 2.1 and 3.4 ± 5.5 at follow-up. Twenty patients developed distal adding-on. Clavicle angle at follow-up correlated weakly but significantly with preoperative clavicle angle (r = 0.34, p = 0.001) and with the correction rates of the main thoracic curve (r = 0.34, p = 0.001); it correlated negatively with the proximal curve spontaneous correction rate (r=-0.21, p = 0.034). The clavicle angle at follow-up was significantly larger in patients with PS-only constructs (PS 2.1 degrees vs. hybrid 0.9, p = 0.02), and tended to be smaller in patients with distal adding-on (adding-on 1.1 vs. non adding-on 2.0, p = 0.09). CONCLUSIONS: PSI was more common with better correction of the main curve (using PS constructs), in patients with a larger preoperative clavicle angle, and with a larger and more rigid proximal curve. Distal adding-on may compensate for PSI.

Sprengel deformity: morphometric assessment and surgical treatment by the modified green procedure.

BACKGROUND: We evaluated the pathologic anatomy of the Sprengel deformity using radiographs, 3-dimensional computed tomography (3D-CT), and magnetic resonance imaging (MRI), and reviewed our results of the modified Green procedure. METHODS: Between 2002 and 2009, 23 scapulae in 22 patients were treated. The average age at the time of surgery was 3.4 years (range, 1.9 to 7.1 y). The outcome of surgery was assessed on the basis of changes in shoulder abduction and the radiographic findings using Cavendish classification, Rigault classification, scapular elevation, and medialization. Preoperative appearance was classified as grade III in 13 cases and grade IV in 10 cases according to the Cavendish classification, and as grade II in 4 cases and grade III in 19 cases according to the Rigault classification. Using 3D-CT, we assessed the height to width ratio on the scapular posterior view, the superior displacement ratio and rotational difference on the trunk posterior view, and the anterior curvature of the supraspinous portion on the scapular medial view. RESULTS: The average follow-up postoperative period was 4.4 years (range, 2.2 to 8.7 y). Postoperatively, the shoulder abduction improved well, with a mean improvement of 63 degrees. Improvement of at least 1 Cavendish grade and 1 Rigault grade was attained in all cases. On preoperative 3D-CT, the mean height to width ratio of the affected scapula was significantly smaller than that of the contralateral scapula. There was an inverse relationship between the superior displacement ratio and the rotational difference. Anterior curvature of the supraspinous portion was seen in all cases. Omovertebral bone was found in 20 cases. MRI identified unossified omovertebral cartilage that was not evident by radiographs or CT. Transient brachial plexus palsy occurred in 2 patients, both of whom recovered within 4 months. Postoperative scapula winging and unsightly scarring were seen in 3 and 8 cases, respectively. CONCLUSIONS: 3D-CT and MRI were helpful in evaluating the pathologic anatomy of the deformity and in planning the surgery. The modified Green procedure provided successful functional and cosmetic results. LEVEL OF EVIDENCE: Level IV.

Mid- to long-term radiological and respiratory outcomes following surgery for older early-onset scoliosis patients with Marfan syndrome.

This study aimed to elucidate mid- to long-term radiological and respiratory outcomes in patients aged 7-11 years at index surgery with Marfan syndrome and early-onset scoliosis (EOS) in a retrospective multicenter study. Primary outcomes were final thoracic height and final percentage of predicted vital capacity (%VC) at or after 16 years of age. We identified 21 (6 male and 15 female) patients with a mean age of 9.9 years and mean follow-up period of 149.3 months. Fifteen patients underwent primary fusion, whereas six underwent growth-friendly surgery (GFS). The mean preoperative and final T1-T12 heights were 204.0 mm and 248.0 mm, respectively. Final pulmonary function tests were available for 16 patients, and the mean final %VC was 54.0% with 10 patients exhibiting a final %VC < 60%. A significant moderate association was observed between the final T1-T12 height and final %VC. The predicted final T1-T12 height required for a final %VC of 60% was approximately 260 mm. Although most older patients with Marfan syndrome and EOS acquired a considerably large final T1-T12 height, a larger thoracic height was required for satisfactory respiratory function in many cases; hence, GFS may be indicated even in this population.

Outcomes of Linear Circumferential Skin Closure for Congenital Constriction Ring Syndrome.

Background: Congenital constriction ring syndrome (CCRS) is a rare condition diagnosed at birth characterised by deformation due to a constriction ring. The usual treatment for CCRS involves excision of the constriction ring and suture of the skin incorporating a Z-plasty to prevent scar contracture. A Z-plasty often results in an unsightly scar. In order to avoid this, we performed linear circumferential skin closure (LCSC). The aim of this paper is to report the outcomes of LCSC for CCRS. Methods: We retrospectively investigated all patients with CCRS who underwent LCSC between 2002 and 2020. Two linear incisions were placed proximal and distal to the constriction ring in parallel, and the constriction ring was excised carefully so as not to damage nerves or vessels. The deep subcutaneous and dermis layers were sutured. The skin was closed using adhesive tape. Two-stage surgery was performed in two patients with severe CCRS of the lower leg to avoid problems with distal circulation. Patients were followed up for at least 1 year and assessed for complications and quality of scar. Results: We performed LCSC for 31 sites in 19 patients, including one forearm, 14 fingers, 10 lower legs and six toes. The median age at the operation was 16 months (range: 4-175). The median follow-up period after surgery was 5.8 years (range: 1.9-16.0). The linear surgical scar had healed well in all patients and there were no complications. There was no recurrence of the constriction ring and no scar hypertrophy, though we did not perform fat mobilisation in all cases. None of the patients required additional surgery and the aesthetic outcome of the linear circumferential surgical scar was maintained at the final observation. Conclusions: Treatment for CCRS using LCSC resulted in no complications, no recurrence of constriction and an excellent aesthetic outcome. Level of Evidence: Level IV (Therapeutic).

Incidence and causes of instrument-related complications after primary definitive fusion for pediatric spine deformity.

OBJECTIVE: Various complications have been reported in the treatment of pediatric spinal deformities. Among these, instrument-related complications could be critical concerns and risks of reoperation. This study aimed to identify the incidence and causes of complications after primary definitive fusion for pediatric spine deformities. METHODS: The authors retrospectively collected data from 14 institutions about patients who underwent primary definitive fusion between 2015 and 2017. There were 1490 eligible patients (1184 female and 306 male), with a mean age of 13.9 years. The incidence, causes, and reoperation rates were analyzed according to 4 etiologies of pediatric spine deformity (congenital, neuromuscular, syndromic, idiopathic). The complications were also categorized as screw-, hook-, or rod-related complications, implant loosening or backout, and junctional problems. RESULTS: The incidence of overall instrument-related complications was 5.6% (84 cases). Regarding etiology, the incidence rates were 4.3% (idiopathic), 6.8% (syndromic), 7.9% (congenital), and 10.4% (neuromuscular) (p < 0.05). The most common causes were pedicle screw malposition (60.7%), followed by implant backout or loosening (15.4%), junctional problems (13.1%), rod breakage (4.8%), and other complications (6.0%). Univariate analysis showed that etiology, type of deformity (kyphosis), surgical procedure, operation time, and estimated blood loss were significant factors. Multivariate analysis revealed that etiology (neuromuscular), surgical procedure (combined approach), and operation time (> 5 hours) remained as significant risk factors. Among all patients with instrument-related complications, 45% (38/84) required revision surgery. Of these cases, > 50% were related to pedicle screw malposition. Medial breach was the most common complication regardless of location, from upper thoracic to lumbar spine. CONCLUSIONS: Pedicle screw malposition was the primary cause of overall complications and subsequent reoperation. In addition to more precise screw insertion techniques, meticulous confirmation of pedicle screw placement, especially of medial breach, may reduce the overall instrument-related complications and revision rates.

Evidence of causality of low body mass index on risk of adolescent idiopathic scoliosis: a Mendelian randomization study.

Introduction: Adolescent idiopathic scoliosis (AIS) is a disorder with a three-dimensional spinal deformity and is a common disease affecting 1-5% of adolescents. AIS is also known as a complex disease involved in environmental and genetic factors. A relation between AIS and body mass index (BMI) has been epidemiologically and genetically suggested. However, the causal relationship between AIS and BMI remains to be elucidated. Material and methods: Mendelian randomization (MR) analysis was performed using summary statistics from genome-wide association studies (GWASs) of AIS (Japanese cohort, 5,327 cases, 73,884 controls; US cohort: 1,468 cases, 20,158 controls) and BMI (Biobank Japan: 173430 individual; meta-analysis of genetic investigation of anthropometric traits and UK Biobank: 806334 individuals; European Children cohort: 39620 individuals; Population Architecture using Genomics and Epidemiology: 49335 individuals). In MR analyses evaluating the effect of BMI on AIS, the association between BMI and AIS summary statistics was evaluated using the inverse-variance weighted (IVW) method, weighted median method, and Egger regression (MR-Egger) methods in Japanese. Results: Significant causality of genetically decreased BMI on risk of AIS was estimated: IVW method (Estimate (beta) [SE] = -0.56 [0.16], p = 1.8 × 10-3), weighted median method (beta = -0.56 [0.18], p = 8.5 × 10-3) and MR-Egger method (beta = -1.50 [0.43], p = 4.7 × 10-3), respectively. Consistent results were also observed when using the US AIS summary statistic in three MR methods; however, no significant causality was observed when evaluating the effect of AIS on BMI. Conclusions: Our Mendelian randomization analysis using large studies of AIS and GWAS for BMI summary statistics revealed that genetic variants contributing to low BMI have a causal effect on the onset of AIS. This result was consistent with those of epidemiological studies and would contribute to the early detection of AIS.

Identification of a Functional Susceptibility Variant for Adolescent Idiopathic Scoliosis that Upregulates Early Growth Response 1 (EGR1)-Mediated UNCX Expression.

Adolescent idiopathic scoliosis (AIS) is a serious health problem affecting 3% of live births all over the world. Many loci associated with AIS have been identified by previous genome wide association studies, but their biological implication remains mostly unclear. In this study, we evaluated the AIS-associated variants in the 7p22.3 locus by combining in silico, in vitro, and in vivo analyses. rs78148157 was located in an enhancer of UNCX, a homeobox gene and its risk allele upregulated the UNCX expression. A transcription factor, early growth response 1 (EGR1), transactivated the rs78148157-located enhancer and showed a higher binding affinity for the risk allele of rs78148157. Furthermore, zebrafish larvae with UNCX messenger RNA (mRNA) injection developed body curvature and defective neurogenesis in a dose-dependent manner. rs78148157 confers the genetic susceptibility to AIS by enhancing the EGR1-regulated UNCX expression. © 2022 American Society for Bone and Mineral Research (ASBMR).

The recent bacterial etiology of childhood osteoarticular infections focusing on the vaccine initiation for Streptococcus pneumoniae and Haemophilus influenzae: A single-center retrospective analysis in Japan.

Background: Pediatric acute osteomyelitis and septic arthritis can destroy growth plate cartilage and joint cartilage, causing permanent deformities and growth disorders. Preventing the contraction of osteoarticular infections is important. Various types of bacteria cause osteoarticular infections in children. Since 2013, when routine vaccination against Streptococcus pneumoniae and Haemophilus influenzae was initiated in Japan, diseases caused by these bacteria (other than osteoarticular infection) are reported to decrease. In this study, we aimed to re-confirm the actual situation including the presence of pathogenic bacteria of pediatric bone and joint infections. Methods: The subjects were patients of 15 years old or younger who had been diagnosed with acute osteomyelitis or septic arthritis and received initial treatment in our hospital from April 1995 to March 2019. We obtained information from the medical records and analyzed them statistically. Results: There were 65 patients with 65 bones with acute osteomyelitis, and 120 patients with 124 joints with septic arthritis. The pathogenic bacteria were identified in 26 (40.0%) osteomyelitis patients and 59 (49.2%) septic arthritis patients. Staphylococcus aureus was the most common pathogenic bacterium, and S. pneumoniae and H. influenzae were identified in four and seven patients respectively, frequently in younger patients. After routine vaccination against S. pneumoniae and H. influenzae, these bacteria were no longer detected in patients under five years old. Conclusions: The efficacy of the S. pneumoniae and H. influenzae vaccine against orthopedic infectious diseases in Japan was indicated.

Severe scoliosis with an impaired pulmonary allograft function after pediatric unilateral lung transplantation.

Left-unilateral single-lobe lung transplantation from a living donor was performed in a 4-year-old boy who suffered from severe respiratory failure caused by bronchiolitis obliterans (BO) as a result of graft versus host disease (GVHD) after peripheral blood stem cell transplantation (PBSCT). The patient grew well during his early childhood years, with an excellent lung allograft function. However, severe thoracic scoliosis occurred 7 years after lung transplantation, which ultimately resulted in compression of the lung allograft followed by severe respiratory dysfunction, and the patient became dependent on mechanical ventilation support. Posterior spinal fusion of Th2-L3 with instrumentation and bone grafting was performed to correct scoliosis in the hope of recovering his thoracic capacity. The left thoracic volume was dramatically improved immediately after spinal fusion surgery, and the patient was ultimately weaned off of mechanical ventilation after a year of pulmonary rehabilitation.