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In a search for ω mesic states, the production of ω-mesons in coincidence with forward going protons has been studied in photon induced reactions on 12C for incident photon energies of 1250–3100 MeV. The π0γ pairs from decays of bound or quasi-free ω-mesons have been measured with the CBELSA/TAPS detector system in coincidence with protons registered in the MiniTAPS forward array. Structures in the total energy distribution of the π0γ pairs, which would indicate the population and decay of bound ω 11B states, are not observed. The π0γ cross section of 0.3 nb MeV−1 sr−1 observed in the bound state energy regime between −100 and 0 MeV may be accounted for by yield leaking into the bound state regime because of the large in-medium width of the ω-meson. A comparison of the measured total energy distribution with calculations suggests the real part V0 of the ω 11B potential to be small and only weakly attractive with V0(ρ=ρ0)=−15±35(stat)±20(syst) MeV in contrast to some theoretical predictions of attractive potentials with a depth of 100–150 MeV.

Study Design: Retrospective literature review analysis OBJECTIVE: Analyze the risk factors, characteristics, outcome, and follow up of surgical management of disc herniation in children ≤15 years old (y.o) through a review of the literature., Background: Disc herniation is a rare disease in the pediatric population. While conservative treatment if very often tried, some cases require surgical treatment., Methods: A literature search was conducted using PubMed data base using the terms 'pediatric/children/adolescent disc herniation' and 'surgical management' as key words. Significant manuscripts i.e: case reports, case series, reviews were identified and analyzed. The exclusion criteria were: series and cases with patients >15 y.o, results non-individualizable and medical management., Results: 49 studies were identified, 28 were retained. 69 children <15 y.o were identified with a median age of 13 y.o (SD 1-15). The mean FU was 3.3 years. Trauma and repeated micro traumatism were identified as the main causes of disc herniation in this population. It is mostly a lumbar disease, with very anecdotic cases of thoracic or cervical herniation described. In the absence of neurological deficit, conservative treatment should be tried. Different types of surgery exist (open, endoscopic, tubular), with no difference in outcome or complications. The post-operative outcome was very satisfactory, with no neurological sequalae described with excellent recovery., Conclusions: Pediatric disc herniation is often caused by precipitating factors such as trauma. In the absence of resolution with conservative treatment, surgical options yield favorable short term clinical outcomes with minimal complications and no neurological sequalae., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Purpose: This study aims to examine whether Pridie drilling, a form of bone marrow stimulation, can expedite the healing process and enable a faster return to sports activity in patients with knee Osteochondritis dissecans (OCD). The primary objective is to assess the effectiveness of Pridie drilling in stable OCD lesions that do not respond to non-operative treatment, by evaluating the absence of painful symptoms 6 months after the procedure. Secondary objectives include evaluating radiographic reconstruction 6 months post-surgery and determining the time it takes to resume sports participation., Materials and Methods: This single-center retrospective study included all cases of stable OCD in the knee that underwent anterograde chondral drilling between 2008 and 2020. Diagnosis of OCD was established using knee radiographs, and the surgical technique involved multiple multidirectional subchondral drilling. Treatment efficacy was defined by the absence of painful symptoms for 6 months postoperatively., Results: A total of 41 knees were included in the study, and no complications were observed before or after surgery. At 6 months postoperatively, 32 knees (78%) showed complete resolution of symptoms. Complete radiographic reconstruction was observed in 66% of cases. Asymptomatic patients at 6 months returned to sports activity of similar intensity to that practiced previously in an average time of 7.9 months; while, patients who were symptomatic at 6 months returned in an average time of 16.5 months., Conclusion: This study provides evidence supporting the short-term efficacy of anterograde chondral drilling in stable lesion of OCD in children and adolescents after failed functional treatment., Level of Evidence: Level III (retrospective cohort study)., (© 2023. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Purpose: The objective was to analyze the role of the thoracolumbar sagittal flexibility on the outcome after posterior spinal fusion of Lenke 1 and 2 adolescent idiopathic scoliosis with last touched vertebra as the lowest instrumented vertebra., Methods: We included 105 thoracic AIS patients who had a posterior spinal fusion with a 2 years minimum follow-up. Thoracolumbar junction flexibility was assessed on dynamic sagittal X-rays and compared to the standing position. Adding-on was defined according to radiographic Wang criteria. The junction was considered flexible if the variability from the static position to flexion and/or extension was greater than 10°., Results: Mean age of the patients was 14 ± 2 years. The preoperative mean Cobb angle was 61 ± 12.7° and 27.5 ± 7.7° after surgery. Mean follow-up was 3.1 years. Twenty-nine patients (28%) developed an adding-on. Thoracolumbar junction range of motion was higher (p = 0.017) with higher flexibility in flexion (p < 0.001) in the no adding-on group. In no adding-on group, 53 patients (70%) had a flexible thoracolumbar junction, and 23 patients (30%) had a stiff thoracolumbar junction in flexion and flexible in extension. In adding-on group, 27 patients (93%) had a stiff thoracolumbar junction, and 2 patients (7%) had a flexible junction in flexion and stiff in extension., Conclusion: The flexibility of the thoracolumbar junction is a determining factor in the surgical outcome after posterior spinal fusion for AIS and should be considered in correlation with the frontal and sagittal alignment of the spine., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: The choice of surgical technique for high-grade spondylolisthesis (HGS) associated with lumbosacral kyphosis remains controversial. Are non-instrumented techniques still relevant, what with the multiplicity and modernity of patient-specific instrumentation?, Hypothesis: Our hypothesis was that a non-instrumented circumferential arthrodesis performed after a period of gradual reduction of HGS, associated with lumbosacral kyphosis, provided satisfactory long-term functional and radiographic results in children and adolescents while minimizing the risk of complications., Materials and Methods: Thirty-one L5-S1 HGS associated with a lumbosacral kyphosis operated by non-instrumented circumferential arthrodesis after a period of traction and suspension were included in our study. The first stage of this technique consisted of a gradual reduction using traction followed by immobilization in the corrected position. The second stage involved a posterior, followed by an anterior, surgical procedure and a spica cast immobilization for 4 months. The mean age at surgery was 13.9±2.3 years (6-18) and the mean follow-up was 10.3±4.5 years (2.1-17.8)., Results: The overall complication rate was 26% (n=8/31): 13% neurologic complications, 10% bone fusion defects and 3% skin complications. The reoperation rate was 13% (n=4/31). The mean ODI (/50) was 3±4.6 (0-22) and the SRS-30 126.7±15 (72-143). The Taillard index decreased by 25% (p<.001) and remained stable throughout the follow-up period (p=.65). The lumbosacral angle was corrected by 13.5% (p=.03) and the correction was maintained throughout the follow-up period (p=.71). At the last follow-up, the lumbosacral angle was significantly correlated with a low ODI score and a high SRS-30 score (p<.05)., Conclusion: Even though this technique achieved a smaller reduction of the lumbosacral angle, it reduced by at least a factor of three the incidence of neurologic complications and resulted in satisfactory functional outcomes when compared to instrumented and intraoperative correction series., Level of Evidence: IV., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Background: Spinal osteochondroma (or exostosis) is a rare benign tumour whose clinical manifestations are delayed due to their slow growth and location. Few studies have addressed the characteristics and the diagnostic and therapeutic peculiarities of spinal osteochondroma in children. The objective of this multicentre observational study was to assess the outcomes of a cohort of children after surgery for spinal osteochondroma., Hypothesis: Surgical excision of spinal osteochondroma in children is not followed by complications or recurrences., Material and Methods: We included consecutive children who had surgery between 2010 and 2018 at any of eight participating centres to remove spinal osteochondromas. The cause, clinical manifestations, and location of the lesions were collected. The surgical outcomes were evaluated after at least 2 years' follow-up., Results: We identified 22 patients who had surgery to remove 26 spinal osteochondromas at a mean age of 12.8±2.6 years. Among them, 7 had a solitary osteochondroma (SO group) and 15 had hereditary multiple osteochondromas (HMO group). At diagnosis, 72% of patients had clinical signs (spinal pain, n=4; one or more lumps, n=5; and neurological manifestations, n=3). In the HMO group, the diagnosis was made during routine MRI screening for tumours involving the spinal canal. Most osteochondromas involved the cervical spine (n=13), with no difference between the two groups (p=0.9). The lamina was the most common location but 54% of the tumours were growing within the canal (92% in the HMO group). After a mean follow-up of 5.2±4.4 years, no patients had experienced any recurrences or complications related to the disease or treatment., Discussion: Surgical excision of spinal osteochondromas in children is effective, with no medium-term recurrences. Our results also confirm the low peri-operative morbidity, even when the canal is involved, and the absence of any effect at last follow-up on spinal alignment. All patients with neurological manifestations at diagnosis made a full recovery., Level of Evidence: IV, retrospective observational cohort study., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Introduction: Neurogenic thoracic outlet syndromes (TOS) are dominated by the nonspecific forms. This study focuses only on the rarer true forms related to proximal nerve compression in the thoracic outlet, with the presence of motor (atrophy of the hand's intrinsic muscles) and/or sensory (hypoesthesia in the territory of the medial antebrachial cutaneous nerve) deficits. The objectives of this study were to define the clinical characteristics, anatomical causes and surgical results of this condition., Patients and Methods: Single-center retrospective study of 53 consecutive cases in 50 patients with an objective clinical deficit confirmed by nerve conduction studies. The population consisted of 47 adults and 3 children, 9 males and 41 females, with a mean age of 39 years (9-80 years), diagnosed between July 1994 and December 2019. An objective motor deficit was present in 50 cases, with the remaining 3 having a sensory deficit only. Forty cases underwent surgery, most often via the supraclavicular approach, while 13 cases did not undergo surgery because their deficit was longstanding and non-progressive., Results: One operated patient was lost in follow-up. An analysis of the medical records of 18 cases, including 15 operated cases found complete recovery in 4 cases, significant improvement in 9 cases and small improvement in 2 cases at a mean follow-up of 53 months (1-162 months). Thirty-four cases were reviewed in person, including 24 operated cases and evaluated with a mean follow-up of 135 months (36-284 months): the pain had disappeared in 21 cases, thenar atrophy persisted in 17 cases, which was associated with a claw-hand deformity in 3 cases, while 2 cases had an isolated claw-hand deformity. The patients were very satisfied with the procedure in 15 cases and satisfied in 9 cases. They evaluated the benefit of surgery at 87% and their upper limb function increased from 38% (10-60%) preoperatively to 77% (60-100%) at the review., Conclusion: Few studies in the literature have focused on true neurogenic TOS cases. The treatment is surgical in progressive cases; an anatomical anomaly is always present. Surgical treatment eliminates the pain and helps to stabilize or even partially resolve the deficit. Despite a moderate objective gain, the patients' feeling of functional improvement is important with a high satisfaction rate., Level of Evidence: IV, retrospective., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Background: The Petit-Morel method allows the treatment of developmental hip dysplasia in toddlers by combining gradual traction to achieve reduction followed by immobilisation during which pelvic osteotomy is performed. The objective of this study was to assess the radiographic and clinical outcomes in a retrospective cohort of patients., Hypothesis: The Petit-Morel method is associated with low rates of avascular necrosis and residual acetabular dysplasia at skeletal maturity, as well as with satisfactory medium-term clinical outcomes., Material and Methods: We conducted a single-centre retrospective study of 34 patients (35 hips) treated between 1997 and 2014. The radiological assessment criteria included an evaluation for avascular necrosis classified according to Kalamchi and MacEwan, the vertical centre edge (VCE) angle, femoral head sphericity according to Mose, and acetabular dysplasia at skeletal maturity according to Severin. Hip function was assessed by determining the Postel-Merle d'Aubigné (PMA) score., Results: Mean age at treatment was 19±4 months (range, 14-29). Mean follow-up was 11 years (range, 5-20). There were two failures including one case of recurrent dislocation requiring surgical reduction. Group II avascular necrosis occurred in 1 (3%) patient. Tönnis Grade IV dysplasia was significantly associated with resolving irregularity of the ossification centre, seen in 19 (54%) cases (p=0.002). In the 18 patients followed-up to skeletal maturity, with a mean follow-up of 15 years (range, 12-20 years), 17 hips were Severin Class I. The mean VCE angle was 29° (range, 15°-38°), and the head was spherical for 34 (98%) hips. The PMA score at last follow-up was excellent (17-18). The mean VCE angle was greater in all 5 patients who experienced pain during long walks (35° [range, 32°-37°]) than in the asymptomatic patients (28° [range, 15°-38°]) (p=0.009)., Discussion: The Petit-Morel method is a reliable treatment that provides good clinical and radiological outcomes. Overcorrection of the VCE angle was noted in the patients who experienced walking-related pain in adulthood., Level of Evidence: IV, retrospective observational cohort study., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Residual hip dysplasia may exist despite appropriate treatment of congenital hip dysplasia (CHD). The abnormalities chiefly affect the acetabulum and can lead to premature osteoarthritis. Although the main cause is delayed treatment of CHD, primary lesions are also possible and may be worsened by the initial treatment itself. Residual hip dysplasia must be detected during the follow-up of patients with CHD. The antero-posterior radiograph of the pelvis is the main diagnostic tool. However, the importance of non-ossified anatomical structures requires additional investigations such as arthrography and magnetic resonance imaging. The risk of premature osteoarthritis is difficult to predict based only on the imaging-study findings. Hip dysplasia is best treated before 5 years of age. The work-up at this age should allow determination of the best treatment. Surgery is required but should not be performed unnecessarily. The decision rests on the absence of improvement in the radiographic criteria and on the findings from additional imaging studies. The usual treatment is Salter's osteotomy, during which excessive anterior displacement should be avoided. At adolescence, the information provided by radiography in the coronal plane should be completed by a three-dimensional evaluation of the acetabulum and an assessment of the quality of the labrum. The shelf procedure has been proven to relieve pain and to significantly postpone the need for hip arthroplasty, when performed early, before the development of visible osteoarthritis, and on a congruent hip. Chiari's osteotomy has a role to play in complex dysplasia affecting both the acetabulum and the femur. Periacetabular osteotomy is getting more used thanks to cooperation between paediatric and adult orthopaedic surgeons. This osteotomy provides optimal correction in all three dimensions., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Purpose: To evaluate the AOSpine Thoracolumbar Spine Injury Classification System and if it is reliable and reproducible when applied to the paediatric population globally., Methods: A total of 12 paediatric orthopaedic surgeons were asked to review MRI and CT imaging of 25 paediatric patients with thoracolumbar spine traumatic injuries, in order to determine the classification of the lesions observed. The evaluators classified injuries into primary categories: A, B and C. Interobserver reliability was assessed for the initial reading by Fleiss's kappa coefficient (k F ) along with 95% confidence intervals (CI). For A and B type injuries, sub-classification was conducted including A0-A4 and B1-B2 subtypes. Interobserver reliability across subclasses was assessed using Krippendorff's alpha (α k ) along with bootstrapped 95% CIs. A second round of classification was performed one-month later. Intraobserver reproducibility was assessed for the primary classifications using Fleiss's kappa and sub-classification reproducibility was assessed by Krippendorff's alpha (α k ) along with 95% CIs., Results: In total, 25 cases were read for a total of 300 initial and 300 repeated evaluations. Adjusted interobserver reliability was almost perfect (kF = 0.74; 95% CI 0.71 to 0.78) across all observers. Sub-classification reliability was substantial (α k = 0.67; 95% CI 0.51 to 0.81), Adjusted intraobserver reproducibility was almost perfect (kF = 0.91; 95% CI 0.83 to 0.99) for both primary classifications and for sub-classifications (α k = 0.88; 95% CI 0.83 to 0.93)., Conclusion: The inter- and intraobserver reliability for the AOSpine Thoracolumbar Spine Injury Classification System was high amongst paediatric orthopaedic surgeons. The AOSpine Thoracolumbar Spine Injury Classification System is a promising option as a uniform fracture classification in children., Level of Evidence: III., (Copyright © 2021, The author(s).)

Purpose: To report an original technique for the surgical correction of adolescent idiopathic scoliosis (AIS) based on hybrid construct using polyester bands placed under the thoracic transverse processes., Methods: We reviewed 105 patients operated between 2013 to 2017 for AIS with a minimum of 2 years follow-up. Clinical and radiological data, including 2D measurements of scoliosis parameters preoperatively, and at 3- and 24-months postoperatively were analyzed. Radiological analysis was performed by two independent observers using KEOPS (Smaio, Lyon-France, CE 2014) database., Results: Mean age was 14.6 ± 2 years. Mean follow-up was 23.7 ± 1.2 months. Most curves were classified as Lenke-1 (78%). The average number of vertebra involved in the construct was 10.9 ± 1.3 (range 7-13). A mean of 12.8 implants were used per construct (mean implant density 0.59). The number of subtransverse bands placed averaged 3 (range 1-5 bands). The mean frontal Cobb angle decreased from 57.9 to 21.2 ° (p < 0.05) postoperatively. The average T4-T12 kyphosis increased from 20.2°to 32.4° (p < 0.05) postoperatively and no loss of correction was observed at 2 years follow-up. We encountered one case of transverse process fracture intraoperativley. No neurological complications were observed. In the postoperative period, 4 cases were diagnosed with distal junctional failure, three of which required an extension of the construct, while the fourth case was treated by bracing., Conclusions: The use of subtransverse band is an efficient and safe method for the surgical correction of AIS. This technique incorporates technical ease (minimal risk of neurological injury), provides good frontal correction and restores thoracic kyphosis. Results are maintained over a two-year period., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Epidemiological studies of fractures of the spine in children are all old, mostly single-centre, with series spanning periods of 5 to 20 years., Hypothesis: As lifestyle is constantly changing, notably with an increase in sports activities and improvements in the prevention of road and household accidents, epidemiology has likely changed., Objective: To update the description of spinal trauma in children and adolescents compared to the existing literature., Material and Method: A multicentre cross-sectional study of spinal fracture, dislocation and spinal cord injury without radiological abnormality (SCIWORA) in children was carried out in 15 French university hospital centres, for a period of one year (2016)., Results: One hundred and sixty-five children were identified: 85 girls, 80 boys; mean age 11 years (range, 10 months-17 years); median, 12 years 6 months. One hundred and fifty-two children (92%) had fracture, 8 (5%) dislocation (including 7 C1-C2 rotary dislocations), and 5 (3%) SCIWORA. Fractures were multiple in 80 cases (49%), contiguous in 73 cases (91%) and non-contiguous in 7 (9%). Locations were cervical in 25 cases (15%), thoracic in 85 (52%), lumbar in 75 and sacral in 4 (2%). Fracture types comprised 234 vertebral compactions (78%), 25 burst fractures (8%), 5 chance fractures (2%), 2 odontoid fractures, and 33 other lesions. Causes comprised fall in 77 cases (47%), sports accidents in 56 (34%), road accidents in 29 (18%), and others in 3. In 52 cases (32%), there was ≥1 associated lesion: appendicular in 35 cases (67%), thoracic or abdominal in 31 (60%), and head in 16 (31%). Twenty-one cases had multiple lesions (40%). Eighteen cases showed neurological involvement (11%) including 5 SCIWORAs. Neurological complications were more frequent before 9 years of age., Conclusion: The epidemiology of spine fractures in children has slightly changed. There are now fewer cervical lesions. Causes are less often road accidents and more often sports accidents. Multi-level lesions remain frequent and the rate of neurological complications is around 10%. Compaction fracture is the most common type., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Children are physiologically protected against venous thromboembolism (VTE). Specific triggering events or contributing factors have been identified in the majority of reported cases, which differs from the adult pathology where 50% of the thromboses are considered "idiopathic". This is a rare disease in children with an estimated frequency of less than 1/1000. The risk is highest in neonates, then decreases and increases again around 13 years to reach the same level as adults at 16 years. The risk of VTE is clearly higher in certain situations: significant trauma, prolonged immobilization, central venous catheter, stay in intensive care unit, inherited thrombophilia, cancer, obesity, oral contraceptives, etc. Thromboprophylaxis should not be used systematically, even in adolescents. Proper hydration and early mobilization form the basis of mechanical thromboprophylaxis. A prescription is only given after careful analysis of the child's risk factors and the orthopedic context. Thrombotic risk assessment scores - which are based on expert opinion and large VTE registers but have not been evaluated in clinical studies - are currently the most reliable method to evaluate the thrombotic risk in children and to prescribe thromboprophylaxis. Low-molecular weight heparin are the most commonly used thromboprophylaxis agents in children, with good tolerance and efficacy., (Copyright © 2019. Published by Elsevier Masson SAS.)

Introduction: Prognosis of dumbbell neuroblastoma (NBL) is mainly determined by the sequelae induced by the tumor itself and the neurosurgical approach. However, after primary chemotherapy, surgical management of the residual tumor, especially the spinal canal component, remains controversial., Methods: We conducted a single-center retrospective cohort study over the last 15 years (2002-2017) including patients treated for NBL with spinal canal extension focusing on timing and type of surgery, complications, and functional and oncological follow-up., Results: Thirty-two children (14 M, 18 F) were managed for NBL, with the majority (26) presenting with NBL stroma poor while four had ganglioneuroblastoma intermixed, one nodular, and one ganglioneuroma. All but two patients received neoadjuvant chemotherapy. Upfront laminotomy for spinal cord decompression was performed in two patients; nine patients had extraspinal surgery with a follow-up neurosurgical procedure in seven cases; eight patients had initial neurosurgery followed by an extraspinal procedure, while six patients underwent a combined multidisciplinary approach. With a median follow up of 3.6 years (0.1-14.9), 29 patients (90.6) are alive and two out of three (19, 65.5%) have functional sequelae., Conclusion: Patients with NBL with persistent spinal canal extension of the tumor after neoadjuvant chemotherapy treated at our center had outcomes that compare favorably with the literature. This is likely due to the multidisciplinary approach to optimal surgical strategy and continuous evaluation of the respective risks of tumor progression. Neurological disability results from initial spinal cord compression or the radicular sacrifice required for tumor resection., (© 2019 Wiley Periodicals, Inc.)

A 14-year-old boy was admitted to the hospital after an episode of blunt trauma to the thorax, resulting in a Chance fracture of L1 and a compressive chylothorax 72h after admission. After initial drainage in the operating room, conservative management was successful. This case study documents one of the rare complications of spinal fractures in the context of high-energy blunt trauma. It is the first detailing a noniatrogenic post-traumatic compressive chylothorax in pediatrics responding positively to conservative management. Drainage should be considered the first-line procedure for both therapeutic and diagnostic purposes. Surgery is required if the leakage is still present after parenteral feeding and the implementation of a fat-free diet for 5-7 days., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Introduction: Proximal junctional kyphosis (PJK) is a frequent complication, up to 46%, in adolescent idiopathic scoliosis surgical treatment (AIS). Several risk factors have been evoked but remain controversial. The purpose of this study was to analyze the incidence of PJK in a multicenter cohort of AIS patient and to determine risk factor for PJK., Materials and Methods: Lenke I and II AIS patients operated between 2011 and 2015 (minimum of 2-years follow-up) were included. On fullspine X-rays, coronal and sagittal radiographic parameters were measured preoperatively, postoperatively and at final follow-up. Occurrence of radiological PJK corresponding to a 10° increase in the sagittal Cobb angle, measured between the upper instrumented vertebra (UIV) and UIV + 2, between postoperative and 2-years follow-up X-rays, was reported., Results: Among the 365 patients included, 15.6% (n = 57) developed a PJK and only 10 patients required a revision surgery. Preoperatively, PJK patients had significantly larger pelvic incidence (57° ± 13° vs. 51° ± 12°), larger lumbar lordosis (LL) (63° ± 12° vs. 57° ± 11°) and bigger C7 slope. Postoperatively (3 months), in the non-PJK group, thoracic kyphosis (TK) was increased and LL was not significantly different. However, postoperatively, in the PJK group, no significant change was observed in TK, whereas C7 slope decreased and LL significantly increased. There was also a postoperative change in inflection point which was located at a more proximal level in the PJK group. Between postoperative time and final follow-up, TK and LL significantly increased in the PJK group., Conclusion: PJK is a frequent complication in thoracic AIS, occurring 16%, but remains often asymptomatic (less than 3% of revisions in the entire cohort). An interesting finding is that patients with high pelvic incidence and consequently large LL and TK were more at risk of PJK. As demonstrated in ASD, one of the causes of PJK might be postoperative posterior imbalance that can be due to increased LL, insufficient TK or inflection point shift during surgery. These slides can be retrieved under Electronic Supplementary Material.

Aim: Published studies have suggested that two to five days of intravenous treatment could effectively treat paediatric bone and joint infections (PBJI), allowing a faster discharge. This study analysed the factors associated with PBJI hospital stays lasting longer than five days using the French National Hospital Discharge Database., Methods: We selected children under 15 years hospitalised in 2013 with haematogenous PBJIs using a validated French algorithm based on specific diagnosis and surgical procedure codes. Risk factors for stays of more than five days were analysed using logistic regression., Results: In 2013, 2717 children were hospitalised for PBJI, with 49% staying more than five days. The overall incidence of 22 per 100 000, was highest in males and toddlers. The main causes were septic arthritis (50%) and osteomyelitis (46%) and 50% of the pathogens were Staphylococci. The odd ratios for stays of five days or more were infancy, coded bacteria and sickle cell disease (7.0), having spondylodiscitis rather than septic arthritis (2.2) and being hospitalised in a general hospital rather than a teaching hospital (1.6)., Conclusion: Half of the hospital stays exceeded five days, despite scientific evidence supporting a shorter intravenous antibiotherapy regimen. Greater knowledge and widespread use of short treatment regimens are needed., (©2017 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Purpose: Although lumbar spondylolysis is encountered in general population with an incidence estimated to be 3-10%, limited information is available for children. The aim of the study is to determine the prevalence of spondylolysis according to associated vertebral bony malformation and spinopelvic parameters in children under eight requiring CT evaluation for unrelated lumbar conditions., Methods: Seven hundred and seventeen abdominal and pelvic multi-detector CT scans were obtained in patients under 8 years of age were reviewed. Two board certificated radiologists and two resident radiologists retrospectively evaluated CT scans for lumbar spondylolysis and associated malformations. Pelvic incidence and spondylolisthesis were reported., Results: Our analysis included 717 CT scans in 532 children (259 girls and 273 boys). Twenty-five cases of spondylolysis were diagnosed (16 bilateral and 9 unilateral, 64 and 36%, respectively) in 14 boys (56%) and 11 girls (44%), associating with 12 grade I spondylolisthesis. The mean normal pelvic incidence was 45° (median 44°, SD 7°). The prevalence of spondylolysis was 1% in children under age 3 (n = 3 among 292 patients), 3.7% in children under age 6 (n = 17 among 454 patients) and 4.7% among the 532 patients. Unilateral spondylolysis was significantly associated with a spinal malformation (p = 0.04, Fisher's exact test), with normal pelvic incidence. Half of the patients with bilateral spondylolysis had high pelvic incidence., Conclusions: We observed a prevalence peak of unilateral spondylolysis in the context of a specific malformation in young infants under age 4 with normal pelvic incidence, and, then, a progressive increase in the prevalence of bilateral isolated spondylolysis.

Lipin-1 is a Mg 2+ -dependent phosphatidic acid phosphatase (PAP) that in mice is necessary for normal glycerolipid biosynthesis, controlling adipocyte metabolism, and adipogenic differentiation. Mice carrying inactivating mutations in the Lpin1 gene display the characteristic features of human familial lipodystrophy. Very little is known about the roles of lipin-1 in human adipocyte physiology. Apparently, fat distribution and weight is normal in humans carrying LPIN1 inactivating mutations, but a detailed analysis of adipose tissue appearance and functions in these patients has not been available so far. In this study, we performed a systematic histopathological, biochemical, and gene expression analysis of adipose tissue biopsies from human patients harboring LPIN1 biallelic inactivating mutations and affected by recurrent episodes of severe rhabdomyolysis. We also explored the adipogenic differentiation potential of human mesenchymal cell populations derived from lipin-1 defective patients. White adipose tissue from human LPIN1 mutant patients displayed a dramatic decrease in lipin-1 protein levels and PAP activity, with a concomitant moderate reduction of adipocyte size. Nevertheless, the adipose tissue develops without obvious histological signs of lipodystrophy and with normal qualitative composition of storage lipids. The increased expression of key adipogenic determinants such as SREBP1 , PPARG , and PGC1A shows that specific compensatory phenomena can be activated in vivo in human adipocytes with deficiency of functional lipin-1., (Copyright © 2017 by the American Society for Biochemistry and Molecular Biology, Inc.)

The pig spine is widely used as a large animal model for preclinical research in human medicine to test new spinal implants and surgical procedures. Among them, pedicle screw is one of the most common method of fixation of those implants. However, the pedicle of the porcine vertebra is not as well defined and not as large as the pedicle of the human vertebra. Therefore, the position of the screw should be adapted to the pig and not merely transposed based on the literature on humans. The purpose of this study is to determine the characteristics of the optimum implantation corridors for pedicle screws in the thoracolumbar spine of piglets of different ages using computed tomography (CT) and to determine the size and length of these corridors in pigs of different ages. CT scans from five groups of age: 6, 10, 14, 18, and 26 weeks were reviewed. For each thoracolumbar vertebrae, the pedicle width, pedicle axis length, and the pedicle angle was measured for the left and right pedicle. A total of 326 thoracic vertebrae and 126 lumbar vertebrae were included in the study. Pedicles are statistically larger but not longer for the lumbar vertebrae. An important variation of the pedicle angle is observed along the spine. In all pigs, an abrupt modification of the pedicle angle between T10 and T11 was observed, which corresponds to the level of the anticlinal vertebra which is the vertebra for which the spinous process is nearly perpendicular to the vertebral body. In conclusion, this study provides a quantitative database of pedicle screw implantation corridors in pigs of different ages. When using pedicle screws in experimental studies in pigs, these results should be considered for selecting the most suitable implants for the study but also to ensure a correct and safer screw position. Improving study procedures may limit postoperative complications and pain, thereby limiting the use of live animals.

Purpose: Anterior fusion is a well-established procedure for the treatment of Lenke 5C adolescent idiopathic scoliosis (AIS). This retrospective study aimed to assess preoperative and postoperative radiographic differences between the conventional anterior fusion and anterior short fusions (ASF) in Lenke 5C AIS., Methods: Radiographic data of 42 consecutive cases of Lenke 5C AIS, which were surgically treated through anterior segmental fusion, were analyzed retrospectively. Patients have been divided into two groups: C group (n = 19) treated by the conventional fusion from the proximal end vertebra to the distal end vertebra of the main curve and C-1 group (n = 23) treated by ASF, sparing the lowest end vertebra of the main curve. Cobb angles of main curve, apical vertebra tilt, C7 coronal tilt, lowest instrumented vertebra angle (LIV angle), LIV adjacent level disc angulation (LIVDA), and the first uninstrumented vertebra angle (FUV angle) were measured on anteroposterior radiographs of the entire spine. LIVDA, FUV wedging, and rotation were measured on stretch films. C7 sagittal tilt, lumbar lordosis, thoracic kyphosis, and proximal kyphosis junction were measured on lateral radiographs of the entire spine. Repeated-measures analysis of variance and fisher test were used for the statistical analysis., Results: The preoperative main curve was 39.9 ± 9.1° in the C group vs. (versus) 42.8 ± 11.8° in the C-1 group. At a mean follow-up of 26.2 months (range 10.5-66.3 months), postoperative main curve improved of 75.8 ± 21.2 % (10.4 ± 9.2°, p < 0.001) in the C group and of 52.7 ± 18.7 % (20.1 ± 8.1°, p < 0.001) in the C-1 group. All parameters improved except the LIVDA, which was slightly impaired, especially in the C-1 group with 5.6 ± 4.2° vs. 4.4 ± 2.2° in the C group. On stretch films, FUV rotation was the only parameter to differ; it was graded at 1 ± 0.7 in the C-1 group vs. 0.6 ± 0.5 in the C group (p = 0.04). No disk obliquity just under the future instrumentation and equivalent FUV wedging were found in any of the two groups., Conclusions: The conventional anterior fusion and ASF give equivalent correction in Lenke 5C AIS, but ASF seems to induce adding-on of the disk below the LIV. FUV rotation on stretch films does not seem to be a predictive factor of postoperative radiological outcome.

Background: Cervical kyphosis may occur with neurofibromatosis type I (NF1) and is often associated with vertebral dysplasia. Outcomes of cervical spinal fusion in patients with NF1 are not well described because of the rarity of the condition. We aimed to (1) characterize the clinical presentation of cervical kyphosis and (2) report the outcomes of posterior and anteroposterior cervical fusion for the condition in these children., Methods: The medical records and imaging studies of 22 children with NF1 who had undergone spinal fusion for cervical kyphosis (mean, 67°) at a mean age of 11 years and who had been followed for a minimum of 2 years were reviewed., Results: Thirteen children presented with neck pain; 10, with head tilt; 9, with a previous cervical laminectomy or fusion; and 5, with a neurologic deficit. Two patients had spontaneous dislocation of the mid-cervical spine without a neurologic deficit. Eleven had scoliosis, with the major curve measuring a mean of 61°. Nine patients underwent posterior and 13 underwent anteroposterior surgery. Twenty-one received spinal instrumentation, and 1 was not treated with instrumentation. Preoperative halo traction was used for 9 patients, and it reduced the mean preoperative kyphosis by 34% (p = 0.0059). At the time of final follow-up, all spinal fusion sites had healed and the cervical kyphosis averaged 21° (mean correction, 69%; p < 0.001). The cervical kyphosis correction was significantly better after the anteroposterior procedures (83%) than after the posterior-only procedures (58%) (p = 0.031). Vertebral dysplasia and erosion continued in all 17 patients who had presented with dysplasia preoperatively. Thirteen patients had complications, including 5 new neurologic deficits and 8 cases of junctional kyphosis. Nine patients required revision surgery. Junctional kyphosis was more common in children in whom ≤5 levels had been fused (p = 0.054)., Conclusions: Anteroposterior surgery provided better correction of cervical kyphosis than posterior spinal fusion in children with NF1. Erosion of vertebral bodies continued during the postoperative follow-up period in all patients who had presented with dysplastic changes preoperatively. The cervical spine should be screened in all children with NF1. Fusion should include at least 6 levels to prevent junctional kyphosis., Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence., (Copyright © 2016 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: Few published data are available on long-term outcomes of anterior spinal fusion for adolescent idiopathic scoliosis (AIS). The objective of this single-centre retrospective study was to assess clinical and radiological outcomes of one-stage anterior spinal fusion achieved using precontoured titanium anterior screw-plates., Hypothesis: Our hypothesis was that anterior instrumentation produced both good functional outcomes and good correction in the coronal and sagittal planes., Material and Methods: This procedure was performed in 111 patients between 1975 and 1993. Among them, those who underwent a comprehensive evaluation at least 15 years later were included. The SRS-30 questionnaire and Oswestry Disability Index (ODI) were used to assess functional outcomes. Radiographic outcomes were evaluated on antero-posterior and lateral full-spine radiographs obtained pre-operatively, post-operatively, and at last follow-up., Results: The study included 35 patients, who were re-evaluated after a mean of 21 years (15-31 years). Mean pre-operative Cobb's angle was 44°, mean age at surgery was 14.7 years, mean SRS-30 score was 3.65/5, and mean ODI was 14.9%. At last follow-up, mean Cobb's angle was 14.7° and 25 patients exhibited coronal misalignment with a mean deviation of 12mm. In the sagittal plane, the mean sagittal vertical axis (SVA) measured using the C7 plumb line was -28mm, with 8mm of anterior translation compared to the post-operative value (36mm). The functional outcome assessed using the SRS-30 score correlated significantly with pelvic tilt and anterior SVA translation., Conclusion: Anterior spinal fusion produces good long-term functional outcomes in AIS. Correction is both satisfactory and sustained. Anterior SVA translation over time may be associated with better functional outcomes., Level of Evidence: IV (retrospective study)., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

The porcine model is frequently used during development and validation of new spinal devices, because of its likeness to the human spine. These spinal devices are frequently composed of pedicle screws with a reputation for stable fixation but which can suffer pullouts during preclinical implantation on young animals, leading to high morbidity. With a view to identifying the best choices to optimize pedicle screw fixation in the porcine model, this study evaluates ex vivo the impact of weight (age) of the animal, the level of the vertebrae (lumbar or thoracic) and the type of screw anchorage (mono- or bi-cortical) on pedicle screw pullouts. Among the 80 pig vertebrae (90- and 140-day-old) tested in this study, the average screw pullout forces ranged between 419.9N and 1341.2N. In addition, statistical differences were found between test groups, pointing out the influence of the three parameters stated above. We found that the the more caudally the screws are positioned (lumbar level), the greater their pullout resistance is, moreover, screw stability increases with the age, and finally, the screws implanted with a mono-cortical anchorage sustained lower pullout forces than those implanted with a bi-cortical anchorage. We conclude that the best anchorage can be obtained with older animals, using a lumbar fixation and long screws traversing the vertebra and inducing bi-cortical anchorage. In very young animals, pedicle screw fixations need to be bi-cortical and more numerous to prevent pullout.

Background: Surgical treatment of early-onset scoliosis has greatly developed in recent years. Early-onset scoliosis covers a variety of etiologies (idiopathic, neurologic, dystrophic, malformative, etc.) with onset before the age of 5 years. Progression and severity threaten respiratory development and may result in respiratory failure in adulthood. Many surgical techniques have been developed in recent years, aiming to protect spinal and thoracic development., Material and Methods: Present techniques are based on one of two main principles. The first consists in posterior distraction of the spine in its concavity (single growing rod, or vertical expandable prosthetic titanium rib [VEPTR]), or on either side (dual rod); this requires iterative surgery, for lengthening, unless motorized using energy provided by a magnetic system. The second option is to use spinal growth force to lengthen the assembly; these techniques (Luque Trolley, Shilla), using a sliding assembly, are known as growth guidance., Results: These techniques are effective in controlling early scoliotic deformity, and to some extent restore spinal growth. However, they show a high rate of complications: infection, rod breakage, spinal fixation pull out and, above all, progressive spinal stiffness, reducing long-term efficacy. Respiratory gain is harder to assess, as thoracic expansion does not systematically improve respiratory function, particularly due to impaired compliance of the thoracic cage., (Copyright © 2015. Published by Elsevier Masson SAS.)

Introduction: In haemophiliacs, recurrent hemarthrosis and chronic synovitis lead to chronic arthropathy. Synovectomy is indicated when medical treatment fails. Few studies report the results of open synovectomy of the ankle in haemophiliacs with a small number of procedures and also a limited follow-up., Aim: The aim of this paper is to report the long-term results of open surgical synovectomy of the tibio-talar joint., Methods: Thirty-two open synovectomies were performed in 21 young haemophiliacs in the same haemophilia center using an antero-lateral and postero-medial approaches. The median follow-up was 15.4 years. Clinical (Petrini scores) and radiological evaluations (Pettersson scores) were made preoperatively and at each multidisciplinary follow-up visit. Wilcoxon and Spearman's tests were used for the statistical analysis., Results: Preoperative median Petrini score was 6 (range 3-12), and improved at 2 and 5 years follow-up (P = 0.0003 and P = 0.0001 respectively). At 10 and 15 years follow-ups, median score remained below preoperative score (median 3.5, range 0-11). Ten ankles had a follow-up of more than 20 years. Preoperative median Petterson score presented a slight but continuous worsening in the first 2 and 5 years of follow-ups (P = 0.02, P = 0.003), but not correlation between clinical and radiological results was observed., Conclusion: Our long-term results support that clinical scores are improved even if radiological scores progress. Open synovectomy retards the progression of the arthropathy, but not stops it. Bleeding and pain are controlled and even if recurrence of bleedings is frequent, it is less severe, less painful and requiring less factors replacement., (© 2015 John Wiley & Sons Ltd.)

Introduction: Solid aneurysmal bone cyst (S-ABC) is a variant of aneurysmal bone cyst (ABC), an uncommon benign bone tumor. There are few cases described in the cervical spine in kids up today. We treated a recurrent case with neurological involvement that needed multiple surgical procedures and radiotherapy., Case Presentation: We report a case of C4 located S-ABC concerning a 2-year-old boy treated surgically by anterior and posterior approach. Three months after the initial procedure appearance of a tetraparesis led to diagnose a local recurrence treated by sclerotherapy and a second surgery. The patient had a full neurological recovery. Three months later, a follow-up CT scan showed a second recurrence requiring a new surgical revision by anterior approach and radiotherapy., Outcome: At 6-year follow-up after four surgical procedures, sclerotherapy and radiotherapy, the aneurysmal bone cyst has been healed. Patient had neurological impairment after a local recurrence but had full recovered after final revision surgery.

Background: Upper cervical instability is common in many skeletal dysplasias, and surgical treatment can be difficult because of small, fragile osseous elements. In this study of children with skeletal dysplasia and upper cervical instability, we compared fusion rates and complications between (1) patients treated with no instrumentation or with wiring techniques and (2) those who underwent rigid cervical spine instrumentation. We also sought to characterize the presentation and common parameters of upper cervical instability in this population., Methods: A multicenter study identified twenty-eight children with skeletal dysplasia who underwent surgery from 2000 through 2011 for C1-C2 instability and were followed for a minimum of two years. Fourteen children were treated with no instrumentation or with instrumentation with wires or cables (nonrigid-fixation group) and fourteen were treated with screws (or hooks) and rods (rigid-fixation group). All patients received autograft, and twenty (twelve in the nonrigid group and eight in the rigid group) were treated with a halo-body jacket., Results: Fourteen children had C1-C2 fusion, and fourteen had occipitocervical fusion. Eleven (39%) underwent spinal cord decompression. The nonunion rate was significantly higher in the nonrigid-fixation group (six of fourteen) than in the rigid-fixation group (zero of fourteen; p = 0.0057). Complications, including nonunion, occurred in nine patients in the nonrigid group and one patient in the rigid group. However, two of the complications in the nonrigid-fixation group were vertebral artery bleeding episodes that actually occurred during an attempt at rigid fixation (the fixation was subsequently done with wiring). No new neurologic deficits were observed. Five of the seven children with a preoperative neurologic deficit showed at least partial recovery, with significant improvement in the Japanese Orthopaedic Association upper-extremity score (p = 0.047)., Conclusions: The nonunion rate is relatively high after patients undergo spinal fusion for C1-C2 instability with nonrigid instrumentation, even if a halo-body jacket is applied. Rigid fixation with screws and rods improves fusion rates., (Copyright © 2015 by The Journal of Bone and Joint Surgery, Incorporated.)

Purpose: Evaluation of effectiveness of internal rigid fixation for occipito-cervical fusion with occipital hooks and cervical screws and/or hooks to improve fusion in young or small children with various bone dysplasia or congenital abnormality., Methods: Fourteen children with mean age of 8.4 years and height and weight below 140 cm and 35 kg, respectively, who underwent occipito-cervical fusion for miscellaneous pathologies reading to stenosis and/or instability were reviewed. The posterior instrumentation consisted of a precontoured titanium plate rod with an occipital fixation by hooks and a vertebral fixation by screws and/or hooks. Eight patients had cervical fixation with C2 pedicle screws. The mean follow-up was 48 months (27-81 months)., Results: Thirteen patients (92.8 %) had a complete fusion and no failure of the fixation was observed. We had two deep wound infections not related to implants., Conclusion: Instrumentation was technically feasible even in a very young child. Rigid internal fixation in the little children did not increase surgical complications and significantly increased the union rate of occipito-cervical arthrodesis.

Study Design: A review of clinical publications, current knowledge, and recent developments regarding the etiology of ischiovertebral dysplasia was combined with a clinical review of the condition., Objective: To acquaint orthopedic spine surgeons with identification patterns of ischiovertebral dysplasia in order to provide them with guidelines about spine management and which complications to expect., Summary of Background Data: Ischiovertebral dysplasia is a rare skeletal dysplasia that may appear in a sporadic fashion or be inherited with an autosomal dominant inheritance pattern. It is defined by the association of an ischiopubic ramus hypoplasia and a vertebral dysplasia. It leads to a specific spine deformity whose management and complications should be clarified., Methods: Thirty consecutive patients from 0 to 31 years of age with ischiovertebral dysplasia were included from 5 centers specialized in congenital spinal deformities. Frontal and sagittal Cobb angles before treatment, natural history of the curves, therapeutic options, and their complications were systematically analyzed., Results: All the patients had a vertebral dysplasia and 28 of them developed a spinal deformity. This deformity was an extremely severe thoracic kyphoscoliosis in 25 cases. The other deformities were a thoracolumbar scoliosis in 1 case and a thoracolumbar kyphosis in 2 cases. The management of the thoracic kyphoscoliosis was always challenging and complications included death by respiratory failure (3 cases) and neurological impairment (9 cases)., Conclusion: Recognizing the occurrence of ischioverterbral dysplasia is very important to allow for dedicated treatment. The authors advocate preoperative distraction and circumferential fusion to prevent progression of the curve and to avoid the potentially fatal sequelae associated with this disorder., Level of Evidence: 4.

Study Design: Outcome study to determine the internal consistency, reproducibility, and concurrent validity of the French-Canadian version of the Scoliosis Research Society 22 (SRS-22 fcv) patient questionnaire in France., Objective: To determine whether the SRS-22 fcv can be used in a population from France., Summary of Background Data: The SRS-22 has been translated and validated in multiple countries, notably in the French-Canadian language in Quebec, Canada. Use of SRS-22 fcv seems appropriate for evaluating adolescent idiopathic scoliosis in France. However, French-Canadian French is noticeably different from the French spoken in France, and no study has investigated the use of a French-Canadian version of a health-quality questionnaire in another French population., Methods: The methods used for validating the SRS-22 fcv in Quebec were adopted for use with a group of 200 adolescents with idiopathic scoliosis and 60 healthy adolescents in France. Reliability and reproducibility were measured by the Cronbach α and intraclass correlation coefficient (ICC), construct validity by factorial analysis, concurrent validity by the Short-Form of the survey, and discriminant validity by analysis of variance and multivariate linear regression., Results: In France, the SRS-22 fcv showed good global internal consistency (Cronbach α = 0.87, intraclass correlation coefficient = 0.92), a coherent factorial structure, and high correlation coefficients between the SRS-22 fcv and Short-Form of the survey (P < 0.001). However, reliability and validity were slightly less than that for the instrument's original validation and the validation of the SRS-22 fcv in Quebec. These differences could be explained by language and cultural differences., Conclusion: The SRS-22 fcv is relevant for use in France, but further development and validation of a specific French questionnaire remain necessary to improve the assessment of functional outcomes of adolescents with scoliosis in France., Level of Evidence: N/A.

Background: Mucopolysaccharidosis type I (MPS-I) is a lysosomal storage disorder characterized by progressive multi-organ disease. The standard of care for patients with the severe phenotype (Hurler syndrome, MPS I-H) is early hematopoietic stem cell transplantation (HSCT). However, skeletal disease, including hip dysplasia, is almost invariably present in MPS I-H, and appears to be particularly unresponsive to HSCT. Hip dysplasia may lead to pain and loss of ambulation, at least in a subset of patients, if left untreated. However, there is a lack of evidence to guide the development of clinical guidelines for the follow-up and treatment of hip dysplasia in patients with MPS I-H. Therefore, an international Delphi consensus procedure was initiated to construct consensus-based clinical practice guidelines in the absence of available evidence., Methods: A literature review was conducted, and publications were graded according to their level of evidence. For the development of consensus guidelines, eight metabolic pediatricians and nine orthopedic surgeons with experience in the care of MPS I patients were invited to participate. Eleven case histories were assessed in two written rounds. For each case, the experts were asked if they would perform surgery, and they were asked to provide information on the aspects deemed essential or complicating in the decision-making process. In a subsequent face-to-face meeting, the results were presented and discussed. Draft consensus statements were discussed and adjusted until consensus was reached., Results: Consensus was reached on seven statements. The panel concluded that early corrective surgery for MPS I-H patients with hip dysplasia should be considered. However, there was no full consensus as to whether such a procedure should be offered to all patients with hip dysplasia to prevent complications or whether a more conservative approach with surgical intervention only in those patients who develop clinically relevant symptoms due to the hip dysplasia is warranted., Conclusions: This international consensus procedure led to the construction of clinical practice guidelines for hip dysplasia in transplanted MPS I-H patients. Early corrective surgery should be considered, but further research is needed to establish its efficacy and role in the treatment of hip dysplasia as seen in MPS I.

Purpose: The purpose of this study was to provide the readers with a reliable source of animal models currently being utilized to perform state-of-the-art scoliotic research., Materials and Methods: A comprehensive search was undertaken to review all publications on animal models for the study of scoliosis within the database from 1946 to January 2011., Results: The animal models have been grouped under specific headings reflecting the underlying pathophysiology behind the development of the spinal deformities produced in the animals: genetics, neuroendocrine, neuromuscular, external constraints, internal constraints with or without tissue injury, vertebral growth modulation and iatrogenic congenital malformations, in an attempt to organize and classify these multiple scoliotic animal models. As it stands, there are no animal models that mimic the human spinal anatomy with all its constraints and weaknesses, which puts it at risk of developing scoliosis. What we do have are a multitude of models, which produce spinal deformities that come close to the idiopathic scoliosis deformity., Conclusion: All these different animal models compel us to believe that the clinical phenotype of what we call idiopathic scoliosis may well be caused by a variety of different underlying pathologies.

Obturator muscles haematoma are rarely reported. The most often reported cases are primary pyomyositis or posttraumatic haematomas occurring during pelvic fractures. We firstly report herein two cases of spontaneous obturator internus haematoma (OIH) in two haemophiliacs with inhibitor. Clinical data and imaging of two patients treated in our clinic are reported here according to previously defined criteria of OIH in posttraumatic situation. Both patients were children suffering from severe and moderate haemophilia A, respectively, with an inhibitor at the time of the event. The clinical feature was marked by an iliopelvic pain letting discussing hip haemarthrosis, appendicitis or iliopsoas haematoma. For both patients ultrasonography (US) failed to provide the diagnosis. Careful and repeated clinical examinations eventually lead to suspect obturator haematoma which was confirmed by abdominopelvic computed tomography (CT) and magnetic resonance imaging (MRI). Respectively, high dose of FVIII or rFVIIa regimen allowed a rapid control of the muscular bleeding in the low and high responder inhibitor patients. Spontaneous OIH may be added to the differential diagnosis of iliopelvic pain in severe forms of haemophilia. US still often performed at first in such case remains unhelpful; abdominopelvic CT or MRI should be performed to discriminate among different diagnoses, including OIH which stays probably undiagnosed., (© 2012 Blackwell Publishing Ltd.)

The aim of this article is to provide an up-to-date overview on paediatric haemophilia care in the world, with emphasis on medical treatment, rehabilitation, and orthopaedic surgery. The reason these specific professions caregivers are included is that over 90% of bleeding episodes in people with haemophilia (PWH) occur within the musculoskeletal system; and of these 80% of bleedings occur in joints., (© 2012 Blackwell Publishing Ltd.)

Several models of scoliosis were developed in the past 10 years. In most of them, deformations are induced in old animals and required long time observation period and a chest wall ligation ± resection. The purpose of the study was to create a scoliosis model with a size similar to an early onset scoliosis and an important growth potential without chest wall injuring. An original offset implant was fixed posteriorly and connected with a cable in seven (6 + 1 control) one-month-old Landrace pigs. The mean initial spinal length (T1-S1) was 25 cm and the mean weight was 9 kg. After 2 months observation, spinal deformities were assessed with a three dimension stereographic analysis. In four animals, the cable was sectioned and the deformities followed-up for next 2 months. No post-operative complication was observed. Mean weight growth was 10 kg/month and mean spine lengthening (T1-S1) was 7 cm/month. In 2 months, we obtained structural scoliotic curves with vertebral and disk wedging which were maximal at the apex of the curve. Mean frontal and sagittal Cobb angles was 45°. Chest wall associated deformities were similar to those observed in scoliotic deformities and were correlated to spinal deformities (p = 0.03). The cable section resulted in a partial curve regression influenced by disk elasticity and could probably be influenced by gravity loads (Decrease of the Cobb angle of 30% in the sagittal plane and 45% in the frontal plane). According to the results, the model creates a structural scoliosis and chest wall deformity that is similar to an early onset scoliosis. The spinal deformities were obtained quickly, and were consistent between animals in term of amount and characteristic.

Background: Abnormal mechanical loading occurs in scoliosis as compared to normal spines. Intervertebral disc degeneration has been correlated with alteration of bone density in adjacent vertebral bodies. How vertebral end plate remodels in scoliosis and the consequences on disc homeostasis are not well understood. Permeability is a relevant physical measure to quantify mass transport in porous media. We hypothesized that effective permeability of the vertebral end plate was modified by growth modulation in a scoliosis animal model., Methods: Flexible asymmetric posterior instrumentation was undertaken on six healthy four-week-old pigs. Two sets of left pedicle screws were inserted and connected with a stainless steel cable. After two months, the apical intervertebral unit and three units located cranially and caudally, were harvested. One central and two lateral specimens were investigated using a previously validated method for measuring permeability., Findings: A three-dimensional deformity was obtained in all six animals with an average of 42° right thoracic curve, 44° lordosis and 21° rotation. Permeability was significantly greater in the center of the end plates than in the periphery and it was decreased by -45% towards the apex of the deformity. Fluid flow direction did not play a significant role. No significant difference was found between the convex side and the concave side., Interpretation: The end plate is a crucial zone for diffusive and convective transport and we showed in a scoliosis animal model that a growth modulation may decrease its effective permeability. The proposed methodology and associated results could help to understand degenerative changes in human spine., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

Background/aims: Prader-Willi syndrome (PWS) is a complex genetic disorder whose many manifestations include obesity and short stature. Diabetes, osteoporosis, and scoliosis are common. We evaluated the effects of human growth hormone (hGH)., Methods: A prospective cohort study of 36 children (1-15 years of age) with genetically confirmed PWS who were given hGH (mean dose 0.033 ± 0.006 mg/kg/day) for 36 months. At baseline and once yearly, we evaluated growth, insulin-like growth factor-1 (IGF-1), body composition, bone mineral density (BMD), glucose tolerance, serum lipids, and spinal radiographs., Results: Height gain over the 3-year period was 1.2 SD score. Lean body mass increased significantly during each treatment year. Total body fat decreased by 5.42 and 1.17% in the 1st and 2nd years, respectively. BMD remained unchanged during therapy. IGF-1 and homeostasis model assessment index of insulin resistance increased, and glucose intolerance was found in 22.7% of patients at baseline and 0% at 3 years. None of the patients had diabetes. Their lipid profile improved. Scoliosis was present in 27.8% of the patients at baseline and 47.2% at 3 years., Conclusion: GH treatment in children with PWS has multiple beneficial effects on growth and body composition. Tolerance is good, with an improvement in glucose metabolism, although IGF-1 levels and insulin resistance parameters should be monitored closely. The high rate of scoliosis warrants monitoring by a pediatric orthopedic surgeon., (Copyright © 2010 S. Karger AG, Basel.)

Achondroplasia (ACH), the most common form of human dwarfism is caused by a mutation in the Fibroblast Growth Factor Receptor 3 (FGFR3) gene, resulting in constitutive activation of the receptor. Typical radiological features include shortening of the tubular bones and macrocephaly, due to disruption of endochondral ossification. Consequently, FGFR3 has been described as a negative regulator of bone growth. Studying a large cohort of ACH patients, a delay in bone age was observed shortly after birth (for boys p=2.6×10(-9) and for girls p=1.2×10(-8)). This delay was no longer apparent during adolescence. In order to gain further insight into bone formation, bone development was studied in a murine model of chondrodysplasia (Fgfr3(Y367C/+)) from birth to 6weeks of age. Delayed bone age was also observed in Fgfr3(Y367C/+) mice at 1week of age followed by an accelerated secondary ossification center formation. A low level of chondrocyte proliferation was observed in the normal growth plate at birth, which increased with bone growth. In the pathological condition, a significantly high level of proliferative cells was present at birth, but exhibited a transient decrease only to rise again subsequently. Histological and in situ analyses suggested the altered endochondral ossification process may result from delayed chondrocyte differentiation, disruption of vascularization and osteoblast invasion of the femur. All these data provide evidence that FGFR3 regulates normal chondrocyte proliferation and differentiation during bone growth and suggest that constitutive activation of the receptor disrupts both processes. Therefore, the consequences of FGFR3 activation on the physiological process of bone development appear to be dependent on spatial and temporal occurrence. In conclusion, these observations support the notion that FGFR3 has a dual effect, as both a negative and a positive regulator of the endochondral ossification process during post-natal bone development., (Copyright © 2010 Elsevier Inc. All rights reserved.)

An achondroplastic patient with a thoracolumbar kyphosis was first seen at the age of 16 at our institution. His only concern at that time was the aesthetic implication of his deformity. His physical examination was normal except for loss of the neurologic reflexes in the lower limbs. The radiographs showed a fixed 180° thoracolumbar kyphosis with correct frontal and sagittal balances. No spinal cord anomaly was found on MRI. Two years later, he developed a progressive neurogenic claudication of the lower limbs. He was still neurologically intact at rest. The MRI showed an abnormal central spinal cord signal in front of the apex of the kyphosis associated with the narrow congenital spinal canal. In regards to this progressive neurological worsening, a surgical treatment was decided. We decided to perform a front and back arthrodesis combined with a spinal cord decompression without reduction of the deformity. A five-level hemilaminotomy was performed with a posterior approach at the kyphosis deformity. The spinal cord was individualised onto 10 cm and the left nerve roots were isolated. A decancellation osteotomy of the three apex vertebrae and a disc excision were performed. The posterior aspect of the vertebral body was then translated forward 2 cm and in association with the spinal cord. Two nerve roots were severed laterally to approach the anterior part of the kyphosis and a peroneal strut graft was inlayed anterolaterally. A complementary anterior and a right posterolateral fusion was made with cancellous bone. The patient was immobilised in a cast for 3 months relayed by a thoracolumbosacral orthosis for 6 months. At 3 years follow-up, the neurogenic claudication had disappeared. No worsening of the kyphosis was observed. His only complaint is violent electric shock in the lower limbs with any external sudden pressure on the spinal cord in the area uncovered by bone.

Background: In haemophiliacs, synovectomy is indicated for recurrent joint bleedings, despite medical treatment., Method: We report a series of 23 surgical synovectomies of the knee with a median follow-up of 8.8 years. The median age of patients at surgery was 13.5 years. Clinical and radiological evaluations were made according to the Petrini and the Pettersson scores, at 1 and 5 years after surgery, and at the last follow-up. Wilcoxon and Spearman's tests were used for the statistical analysis., Result: The Petrini score improved at 1 and 5 years (P < 0.001). Nine patients have 20 years of follow-up and a stable result. In more than half of the knees, no episode of recurrent bleeding occurred. The effect of surgery on the range of motion (ROM) was moderate and mobilisation under anaesthesia did not improve it significantly. There was a progressive worsening of the radiological score, but no correlation between clinical and radiological score was noticed (ρ = 0.08, P = 0.77)., Conclusion: Complete synovectomy gives good long-term results in term of bleeding recurrence and overall function.

We conducted a micro-CT analysis of subchondral bone of the vertebral end-plates after application of compressive stress. Thoracic and lumbar vertebral units were instrumented by carrying out left asymmetric tether in eleven 4-week-old pigs. After 3 months of growth, instrumented units and control units were harvested. Micro-CT study of subchondral bone was performed on one central and two lateral specimens (fixated side and non-fixated side). In control units, bone volume fraction (BV/TV), number of trabeculae (Tb.N), trabecular thickness (Tb.Th), and degree of anisotropy (DA) were significantly higher, whereas intertrabecular space (Tb.Sp) was significantly lower in center than in periphery. No significant difference between the fixated and non-fixated sides was found. In instrumented units, BV/TV, Tb.N, Tb.Th, and DA were significantly higher in center than in periphery. BV/TV, Tb.N, and Conn.D were significantly higher in fixated than in non-fixated side, while Tb.Sp was significantly lower. We noted BV/TV, Tb.N, and Tb.Th significantly lower, and Tb.Sp significantly higher, in the instrumented levels. This study showed, in instrumented units, two opposing processes generating a reorganization of the trabecular network. First, an osteolytic process (decrease in BV/TV, Tb.N, Tb.Th) by stress-shielding, greater in center and on non-fixated side. Second, an osteogenic process (higher BV/TV, Tb.N, Conn.D, and lower Tb.Sp) due to the compressive loading induced by growth on the fixated side. This study demonstrates the densification of the trabecular bone tissue of the vertebral end-plates after compressive loading, and illustrates the potential risks of excessively rigid spinal instrumentation which may induce premature osteopenia., ((c) 2009 Orthopaedic Research Society.)

We implemented an experimental model of asymmetrical compression loading of the vertebral end plate (VEP) in vivo. The macroscopic permeability of the VEP was measured. We hypothesized that static asymmetrical loading on vertebrae altered the macroscopic permeability of the VEP. In scoliosis, solute transport to and from the disc is dramatically decreased especially at the apical intervertebral disc. The decrease in permeability could be induced by mechanical stress. Nine skeletally immature pigs were instrumented with left pedicle screws and compression rod at the T5/T6 and L1/L2 levels. After 3 months, three cylindrical specimens of the VEP were obtained from each of the tethered levels. A previously validated method for measuring permeability, based on the relaxation pressure due to a transient-flow rate was used. A pistoning device generated a fluid flow that fully saturated the cylindrical specimen. The decrease in upstream pressure was measured using a pressure transducer, which allowed the macroscopic permeability to be derived. A microscopic study completed the approach. Overall macroscopic permeability was lower for the tethered VEPs than for the VEPs of the control group, respectively -47% for flow-in (p = 0.0001) and -46% for flow-out (p = 0.0001). In the tethered group, macroscopic permeability of the specimens from the tethered side was lower than macroscopic permeability of those from the non-tethered side, -39% for flow-out (p = 0.024) and -47% for flow-in (p = 0.13). In the control group, the macroscopic permeability was greater in the center of the VEP than in its lateral parts for flow-out (p = 0.004). Macroscopic permeability of the center of the VEPs was greater for flow-out than for flow-in (p = 0.02). There was no significant difference between thoracic and lumbar. This study demonstrated that compression loading applied to a growing spine results in decreased permeability of the VEP. This result could be explained by local remodeling, such as calcification of the cartilage end plate or sclerosis of the underlying bone.

Background: Surgical correction of coxa vara in children with osteopenic bone diseases could be very challenging. In this study, we describe a modified surgical technique for the correction of coxa vara in children with bone fragility. We also report the results and complications of this technique in 16 children (21 coxa vara) with osteopenic bone diseases., Methods: Charts, clinic notes, and radiology images of 16 patients (21 hips) who had a proximal femoral osteotomy for the treatment of coxa vara in osteogenesis imperfecta (18 hips) and fibrous dysplasia (3 hips) in our institution between 1996 and 2005 were reviewed. The modified surgery involved an intertrochanteric osteotomy and the use of Kirshner wires and intramedullary rods. Neck-shaft angle, Hilgenreiner-epiphyseal angle, and head-shaft angle were assessed at preoperative, postoperative, and final follow-up., Results: The average age at surgery was 8.3 years (range, 3.3-15.8 years). The average correction of the neck-shaft angle was from 84.6 to 114.4 degrees, and for the Hilgenreiner-epiphyseal angle, it was from 67.7 to 42 degrees at final follow-up. All osteotomies were healed at 3 months postoperatively. The mean follow-up was 4.29 years (range, 1.70-8.12 years). The average improvement in abduction and external rotation of the hips was 14 and 15 degrees, respectively. There were no cases of infection and 2 cases (12%) of implant-related complications. One patient with fibrous dysplasia needed replacement of the intramedullary rod and additional distal femoral osteotomy 1 week postsurgery. In another patient, the intramedullary rod had migrated proximally, which was corrected 5 months postsurgery by advancing the rod distally., Conclusion: Results suggest that this surgical technique provides satisfactory correction of coxa vara in children with osteopenic bone diseases., Clinical Relevance: The described surgical technique used to correct coxa vara is reproducible and safe and has few complications. Furthermore, the size of the implants used allows surgery to be performed in very young children.

Objective: Our goals were to determine the prevalence and estimate the evolution of spinal deformities in patients suffering from Prader-Willi syndrome; find out which kind of spine deformity predominates regarding genotype and clinical patterns; and evaluate the affect of growth-hormone treatment on the onset and progression of spinal deformities., Patients and Methods: This was a retrospective longitudinal, clinical, and radiologic study. One hundred forty-five children followed between 1980 and 2006 were studied in 2 referral centers for Prader-Willi syndrome. Genetic testing confirmed the diagnosis in 133 patients. Ninety-three patients (64%) received growth-hormone therapy. For statistical analysis, age-adjusted comparison between groups was performed by using multivariate logistic regression., Results: Mean age of the patients was 10.2 +/- 6.2 years. Sixty-three (43.4%) patients were afflicted with scoliosis. Scoliosis frequency steadily rose with age, and a large majority of patients were affected at skeletal maturity (66.7%). Scoliosis prevalence was not affected by the genotype or by growth-hormone treatment. Patients with higher BMI values had an increased risk of developing a kyphotic deformity in association with scoliosis. We found a statistical association between kyphotic deformity and the need for surgical treatment., Conclusions: Scoliosis is a major concern for patients with Prader-Willi syndrome, and a regular (annual) systematic back examination is mandated. The role of growth-hormone treatment on the natural history of scoliosis could not be determined, and careful monitoring during treatment is recommended.