Search

Background: X-linked hypophosphatemic rickets (XLHR) is due to mutations in PHEX leading to unregulated production of FGF23 and hypophosphatemia. XLHR is characterized by leg bowing of variable severity. Phosphate supplements and oral vitamin analogs, partially or, in some cases, fully restore the limb straightness. Surgery is the alternative for severe or residual limb deformities. Objective: To retrospectively assess the results of surgical limb correction in XLHR (osteotomies and bone alignment except for 3 transient hemiepiphysiodesis). Methods: We analyzed the incidence of recurrence and post-surgical complications in 49 XLHR patients (29F, 20M) (mean age at diagnosis 6.0 years (± 7.1)). Results: At first surgery, the mean age was 13.4 years (± 5.0). Recurrence was observed in 14/49 (29%) patients. The number of additional operations significantly decreased with age (2.0 (± 0.9), 1.7 (± 1.0) and 1.2 (± 0.4) in children 15 years; P < 0.001). Incidence of recurrence seemed to be lower in patients with good metabolic control of the rickets (25% vs 33%). Complications were observed in 57% of patients. Conclusion: We report a large series of surgical procedures in XLHR. Our results confirm that phosphate supplements and vitamin D analog therapy is the first line of treatment to correct leg bowing. Surgery before puberty is associated with a high risk of recurrence of the limb deformity. Such procedures should only be recommended, following multidisciplinary discussions, in patients with severe distortion leading to mechanical joint and ligament complications, or for residual deformities once growth plates have fused.

Background: Adolescent idiopathic scoliosis (AIS) is a common spinal disease affecting 2% of adolescents, and women in 90% of the cases. When a surgical treatment is opted for, many questions are frequently asked by families and patients about the course of pregnancy and childbirth after the spinal fusion. This subject remains little studied in the literature, especially with modern instrumentation techniques., Hypothesis: The goal was to describe pregnancy and childbirth after AIS surgery in terms of access to epidural analgesia, need for cesarean section (c-section), and low back pain during and after pregnancy. We thus hypothesized that women undergoing spinal surgery for AIS have subsequently uncomplicated pregnancies and childbirths, and have access to epidural analgesia as women without AIS do., Patients and Methods: In this retrospective multicenter study, 198 women who underwent surgery between 1984 and 2014 were reviewed from two university hospitals. Among them, 50 women became pregnant, for a total of 80 pregnancies. Surgical data were collected [approach, uppermost and lowermost instrumented vertebra (UIV, LIV)]. Pregnancy characteristics were evaluated: time between surgery and pregnancy, number of births, mode of analgesia, type of delivery, weight gain. Occurrence of low back pain during pregnancy and at follow-up was recorded using ODI., Results: Of the 50 women, 34 had posterior surgery and 16 had anterior surgery. Deliveries took place from 1988 to 2018. Of the 80 pregnancies, 81% were delivered by vaginal route (n=65/80), and an effective epidural anesthesia was performed for 49% of them (n=39/80). Epidural analgesia failed in 9% of pregnancies (n=7/80), and was denied in 35% of cases (n=28/80), half of the time by anesthesiologists (n=15/80). Patients refused epidural in 13 pregnancies (16%, n=13/80). A general anesthesia was used in six pregnancies (8%, n=6/80), for c-sections only. Back pain was reported in 48% of the pregnancies (n=38/80). The level of fusion was correlated with c-section, and conversely with epidural anesthesia., Discussion: A normal pregnancy with vaginal delivery seems to be the rule for women undergoing spinal fusion for AIS. The c-section rate in AIS women was similar to the general population (19%). Yet, access to epidural anesthesia still seems problematic with only 49% of births in this series, compared with 81% in the French population., Level of Evidence: IV, retrospective cohort., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Introduction: Early-onset scoliosis is a common deformity in neuromuscular disease. When conservative treatment becomes ineffective, several surgical options can be proposed. The most common technique is posterior spinal fusion (PSF) consisting of performing a multiple segmental instrumentation with pedicular screws on the full spine associated with decortication and bone graft. Minimally invasive fusionless surgery (MIFS) is an alternative to correct and fix definitively the spine without graft. The objective of this study was to compare early surgical inpatient period between PSF and MIFS in neuromuscular scoliosis., Material and Methods: 140 NMS operated by PSF or MIFS between 2012 and 2017 was retrospectively reviewed. The following data were compared between groups: general characteristics (age, sex, etiology), preoperative preparation (halo traction, noninvasive ventilation or tracheostomy), Cobb angle and pelvic obliquity correction, use of drugs (vasopressor and/or inotropes, expansion fluids, transfusion and volumes), postoperative complications, and need of noninvasive ventilation., Results: 75 patients were managed by PSF with a mean age of 14.3 ± 2.3y and 65 by MIFS with a mean age of 11.8 ± 3y. Average pelvic obliquity and major curve correction were similar postoperatively. Intraoperative blood transfusion was significantly more common in PSF group (OR, 14; 95% CI [6.3-33.0]). Vasopressors were used non-significantly more often in the PSF group and expansion fluids similar in the two groups. PSF group had more overall complications (OR, 4.6; 95% CI [2.3-9.8]), more infections (OR, 3.6; 95% CI [1.5-9.3]) and more hemodynamic complications (OR, 4.1; 95% CI [1.4-15.1]). Average intubation duration was 5 days in the PSF and 4 days in MIFS (p = 0.05)., Conclusion: In this series of neuromuscular patients, the complication rate was reduced in MIFS comparatively to PSF, with lower blood transfusion and less infections., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Juvenile idiopathic arthritis is the most common chronic rheumatic disease encountered in children under the age of sixteen and causes significant impairments in daily life. Over the last two decades, the introduction of new drug treatments (including disease-modifying antirheumatic drugs and biologics) has changed the course of this disease, thus reducing the indication for surgery. However, some patients fail to respond to drug therapy and thus require personalized surgical management, e.g., the local reduction of joint effusion or a synovial pannus (via intra-articular corticosteroid injections, synovectomy, or soft tissue release), and management of the sequelae of arthritis (such as growth disorders and joint degeneration). Here, we provide an overview of the surgical indications and outcomes of the following interventions: intra-articular corticosteroid injections, synovectomy, soft tissue release, surgery for growth disorders, and arthroplasty.

Introduction: Percutaneous Achilles tenotomy (PAT) was recently added to functional treatment of congenital talipes equinovarus (aka clubfoot). The aim of this study was to determine the relevance of a carefully chosen radiological criterion for the PAT indication and to evaluate its results., Hypothesis: When the tibiocalcaneal angle (aTiCa) is larger than 75° at 4 months, doing PAT will improve the results of the functional method in the medium term and will reduce the surgery rate., Patients and Methods: This prospective study involved 101 patients (151 feet) born between 2011 and 2014 with clubfoot who were treated with the French functional method and had at least 4 years' follow-up. The initial severity of the deformity was evaluated using the Diméglio scoring system. In our sample, 30 feet had a Diméglio rating of II (20%), 61 had a Diméglio rating of III (40%) and 60 feet had a Diméglio rating of IV (40%). The indication for PAT was made at 4 months of age when the aTiCa on a lateral radiograph of the foot in maximum correction was greater than 75°. The mean follow-up was 5 years. The final assessment was done using the modified Ghanem and Seringe classification., Results: In the entire cohort, PAT was done in 113 feet (75%). None of the feet required a repeat PAT. Surgical release of the soft tissues was done in 20 feet (13%). None of the feet developed a rocker bottom deformity. Two feet were operated in the absence PAT (out of 38 in this subgroup) and 18 feet after PAT (out of 113 in this subgroup). The aTiCa angle did not vary in the PAT group based on whether surgical release was indicated afterwards or not. At the final assessment, 140 feet (93%) were classified as very good and 11 feet (7%) as good., Discussion: The tibiocalcaneal angle is a relevant radiological criterion for the PAT indication in children with clubfoot. PAT has a positive impact on the outcomes., Level of Evidence: II; prospective study., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Introduction: Pelvic fixation in patients with neuromuscular scoliosis is difficult, due to their fragile general condition and poor bone quality. Many techniques have been described, associated with high rates of mechanical complications. The objective of this work was to evaluate the mechanical complications and long-term radiological results of ilio-sacral screw pelvic fixation., Materials and Methods: 167 consecutive patients with neuromuscular scoliosis who underwent minimally invasive bipolar fixation with ilio-sacral screw pelvic fixation were retrospectively reviewed. The instrumentation consisted in a bilateral sliding rods construct extended from T1 to the sacrum, anchored proximally by double-hook claws and distally by ilio-sacral screws through a minimally invasive approach. Mechanical complications and radiographic measurements (angle of the major coronal curve, pelvic obliquity, lumbar lordosis) were evaluated preoperatively, postoperatively, and at the last follow-up., Results: Mean operative age was 12 ± 3 years, and follow-up 6.4 years (3.0-10.4 years). Pelvic obliquity decreased from 20° preoperatively to 5° (77% correction) at last follow-up, Angle of the major coronal curve from 75° to 36° (52% correction), and lumbar lordosis from 28° to 38°. 16 mechanical complications in nine patients occurred: screw prominence (n = 1), connector failure (n = 4), screw malposition (n = 11). Unplanned surgery was required in seven cases, two were managed during rod lengthening, seven did not require treatment., Conclusion: In this series of neuromuscular patients operated by ilio-sacral screws as pelvic fixation, the results were stable with a mean follow-up of more than 6 years and the complication rate was reduced comparatively to the literature., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: One of the worst complications of surgery for spinal deformity is postoperative neurological deficit. Multimodal intraoperative neuromonitoring (IONM) can be used to detect impending neurological injuries. This study aimed to analyze IONM in non-idiopathic scoliosis using a minimally invasive fusionless surgical technique., Methods: This retrospective, single-center study was performed from 2014 to 2018. Patients with non-idiopathic scoliosis who underwent a minimally invasive fusionless procedure and had at least 2 years of follow-up were included. IONM was performed using a neurophysiological monitoring work station with somatosensory evoked potentials (SSEP) and neurogenic mixed evoked potentials (NMEP)., Results: A total of 290 patients were enrolled. The mean age at surgery was 12.9±3 years. The main etiology was central nervous system (CNS) disorders (n=139, 48%). Overall, 35 alerts (11%) in the SSEP and 10 (7%) in the NMEP occurred. There were two neurological deficits with total recovery after 6 months. There were no false negatives in either SSEP or NMEP, although there was one false positive in SSEP and two false positives for NMEP in the group without signal recovery. There was no significant relationship between the incidence of SSEP or NMEP loss and age, body mass index (BMI), number of rods used, upper instrumented vertebrae (p=0.36), lower instrumented vertebrae, or type of surgery. A preoperative greater Cobb angle was associated with a significantly higher risk of NMEP loss (p=0.02). In CNS patients, a higher BMI was associated with a statistically significant risk of NMEP loss (p=0.004). The use of a traction table was associated with a higher risk of signal loss (p=0.0005)., Conclusion: A preoperative higher Cobb angle and degree of correction were associated with a significant risk of NMEP loss. In CNS scoliosis, a higher BMI was associated with a significant risk of NMEP loss. The use of a traction table was associated with a higher risk of signal loss., Competing Interests: Declaration of Competing Interest All Authors report no conflict of interest., (Copyright © 2022 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

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.)

Study Design: Retrospective longitudinal study., Objective: The main goal of this study was to measure the disability after AIS correction, according to the LIV., Summary of Background Data: Adolescent idiopathic scoliosis (AIS) is a three-dimensional deformity of the spine that may require surgical correction. If the upper and lower instrumented levels (UIV and LIV) of these fusions are defined by the characteristics of the curve, the long-term consequences of the LIV choices are still partially unknown., Methods: This retrospective longitudinal study collected demographic, radiologic (Lenke classification, Cobb angle), and surgical data (approach, LIV, UIV) on 116 patients operated for AIS fusion on a specialized pediatric spine center were collected. All participants answered SRS30, SF12, lumbar and leg pain Visual Analogue Scales (VAS) at last follow-up. Statistical analysis between LIV (T12L1, L2, L3 or L4L5) and clinical data at last follow-up was realized., Results: The mean follow-up was 87months. The mean increase of back pain VAS per UIV level was 9 mm. No statistically significant difference between the different LIV was found, for SRS30 or SF12 MCS (mental component scale). There was a statistically significant difference between L3 UIV and L4L5 UIV for SF-12 PCS (physical component scale); ( P = 0.03)., Conclusion: The long-term consequences of LIV choice mostly affect levels distal to L3. If the LIV is mostly defined by the characteristics of the curve, one level caudally corresponds to +9 mm of back pain VAS at 7 years of follow-up. Surgeons may be aware of the long-term consequence of LIV choice and patients be informed., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Expandable endoprostheses are used to restore limb function and compensate for the sacrifice physis involved in carcinologic resection. Long-term outcomes of the last generation of knee "non-invasive" expandable endoprostheses are required. Objectives were to report on oncologic results of bone sarcoma resection around the knee with expandable endoprosthesis reconstruction and to compare the surgical outcomes of the "non-invasive" expandable endoprostheses used in our department., Materials and Methods: Retrospective study that included all children with bone sarcoma around the knee that underwent tumor resection reconstructed with non-invasive expandable prosthesis. Phenix-Repiphysis was used from 1994 to 2008 followed by Stanmore JTS non-invasive from 2008 to 2016. Survival and complications were recorded. Functional outcomes included Musculoskeletal Tumor Society (MSTS) score, knee range of motion, lower limb discrepancy (LLD)., Results: Forty children (Sex Ratio = 1) aged a mean 8.8 years (range, 5.6-13.8) at surgery were included in the study. There were 36 osteosarcoma and 4 Ewing sarcoma that involved 33 distal femur and 7 proximal tibia. Cohort (n = 40) consisted of 28 Phenix-Repiphysis and 12 Stanmore with a mean follow-up of 9.8 ± 5.8 years and 6.1 ± 3.1 years, respectively. Postoperative infection rate was 7.5% in the cohort (3 Repiphysis). Functional results were significantly better in the Stanmore group with a mean MSTS of 87.6 ± 5.4% and knee flexion of 112 ± 38°. At last follow-up, implant survival was 100% in Stanmore group, whereas all living Phenix-Repiphysis were explanted. Mechanical failure was the primary cause for revision of Phenix-Repiphysis. Limb length equality was noted in 79% patients with Phenix-Repiphysis and 84% with Stanmore at last follow-up., Conclusion: Chemotherapy and limb-salvage surgery yield good oncologic outcomes. Expandable endoprostheses are effective in maintaining satisfactory function and lower limb equality. With improvements made in the last generation of "non-invasive" prostheses, implants' survival has been substantially lengthened., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature.)

Study Design: A prospective study., Objective: The aim of this study was to report the results of an alternative technique to growing rods (GR) for neuromuscular scoliosis using a minimally invasive fusionless surgery with a minimum of 5 years' follow-up., Summary of Background Data: Conservative treatment is not effective in progressive neuromuscular scoliosis. Early surgery using GR is increasingly advocated to control the deformity while preserving spinal and thoracic growth before arthrodesis. These techniques still provide a high rate of complications., Methods: The technique relies on a bilateral double rod sliding instrumentation anchored proximally by four hooks claws and distally to the pelvis by iliosacral screws through a minimally invasive approach. The clinical and radiological outcomes of 100 consecutive patients with neuromuscular scoliosis who underwent this fusionless surgery with a minimum follow-up of 5 years were reviewed., Results: 6.5 ± 0.7 years after initial surgery, six patients were lost of follow-up and 11 died of unrelated raison. Of the 83 remaining patients at latest follow-up, mean Cobb angle was stable to 35.0° which correspond to 61% correction of the initial deformation. Mean pelvic obliquity was 29.6° (0.3°-80.0°) preoperatively and 7.2 (0.2°-23.5°) at latest follow-up. Correction of the hyper kyphosis remained stable. Skeletal maturitywas reached in 42 of 83 patients (50.6%). None of these patients has required spinal fusion. The global complication rate was 31.3%., Conclusion: The outcomes of this minimally invasive fusionless technique at 5 years follow-up showed a stable correction of spinal deformities and pelvic obliquity over time, with a reduced rate of complication. The arthrodesis was not required for all patients at skeletal maturity. This technique could be a good alternative to arthrodesis for neuromuscular scoliosis.Level of Evidence: 3., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: This study aimed to characterize pharmacokinetics of intravenous and oral ciprofloxacin in children to optimize dosing scheme., Methods: Children treated with ciprofloxacin were included. Pharmacokinetics were described using non-linear mixed-effect modelling and validated with an external dataset. Monte Carlo simulations investigated dosing regimens to achieve a target AUC 0-24 h /MIC ratio ≥ 125., Results: A total of 189 children (492 concentrations) were included. A two-compartment model with first-order absorption and elimination best described the data. An allometric model was used to describe bodyweight (BW) influence, and effects of estimated glomerular filtration rate (eGFR) and age were significant on ciprofloxacin clearance., Conclusion: The recommended IV dose of 10 mg/kg q8h, not exceeding 400 mg q8h, would achieve AUC 0-24 h to successfully treat bacteria with MICs ≤ 0.25 (e.g. Salmonella, Escherichia coli, Proteus, Haemophilus, Enterobacter, and Klebsiella). A dose increase to 600 mg q8h in children > 40 kg and to 15 mg/kg q8h (max 400 mg q8h, max 600 mg q8h if augmented renal clearance, i.e., eGFR > 200 mL/min/1.73 m 2 ) in children < 40 kg would be needed for the strains with highest MIC (16% of Pseudomonas aeruginosa and 47% of Staphylococcus aureus). The oral recommended dose of 20 mg/kg q12h (not exceeding 750 mg) would cover bacteria with MICs ≤ 0.125 but may be insufficient for bacteria with higher MIC and a dose increase according bodyweight and eGFR would be needed. These doses should be prospectively confirmed, and a therapeutic drug monitoring could be used to refine them individually., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Treatment of spinal muscular atrophy (SMA) scoliosis has evolved in the last decade, with the emergence of fusionless surgical techniques that allow correction of the deformity before the end of growth spurt. These techniques are expected to delay definitive spine fusion and preserve trunk growth., Purpose: The aim was to evaluate long-term clinical, radiologic, and respiratory outcomes of a minimally invasive fusionless surgery (MIFLS) in SMA scoliosis., Methods: All children affected with SMA scoliosis who underwent MIFLS in our department from 2011 to 2019 were included. The instrumentation consisted in a bilateral sliding rod construct from T1 to the sacrum, anchored proximally by double-hook claws and distally by iliosacral screws. Clinical, genetic, respiratory and radiographic data were retrospectively reviewed. A patient's satisfaction survey was performed., Results: A total of 59 children with genetic confirmation of SMA (9SMA1c, 47SMA2, and 3SMA3) underwent MIFLS at a mean age of 11±1.9 years. All of them were nonwalker at the time of surgery. Twenty-six were treated with intrathecal Nusinersen. Mean follow-up was 5.2 years (2 to 9.6 y). Mean major coronal curve improved from 79±15 to 41±16 degrees and pelvic obliquity decreased from 24±11 to 5.9±4 degrees. Mean space available for lung improved from 77% to 93%. Mechanical or infectious complications occurred in 9 patients, with removal of the implant in 1. 6 children required unplanned surgeries. Postoperative bracing was needed in 13 children. Mean gain weight 3 years after the first surgery was 6 kg. 91.5% of patients had a positive satisfaction of the surgery. There was no significant impact in respiratory function postoperatively. Only 30 children required rod lengthening procedures, with a mean interval between procedures of 1.9 years (0.5 to 3.7 y). No arthrodesis was required at last follow-up in any patient., Conclusion: Bipolar MIFLS in SMA preserves spinal and thoracic growth without interference with respiratory function. It provides a significant correction of spinal deformity and pelvic obliquity, having a reduced rate of complications. The correction of spinal deformity was maintained at long term, not requiring definitive fusion at the end of growth., Level of Evidence: Level IV., Competing Interests: L.M. has intellectual property rights with Euros company. S.Q.-R. is consultant and belongs to advisory boards for Biogen, Avexis (Novartis Gene therapies), Roche, Sanofi-Genzyme. The remaining authors declare no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Pelvic fixation during procedures performed to treat spinal deformities in paediatric patients remains challenging. No computed tomography studies in paediatric have assessed the optimal trajectory of ilio- sacral screws to prevent screw malposition. We used pelvic computed tomography from 80 children divided into four groups : females <10 and ≥10 years and males <10 and ≥10 years. A secure triangular corridor parallel to the upper S1 endplate was delineated based on three fixed landmarks. The optimal screw insertion angle was subtended by the horizontal and the line bisecting the secure corridor. Student's t test was applied to determine whether the optimal screw insertion angle and/or anatomical parameters were associated with age and/or sex. Mean optimal angle was 32.3°±3.6°, 33.8°±4.7°, 30.2°±5.0°, and 30.4°±4.7° in the younger females, younger males, older females, and older males, respectively. The mean optimal angle differed between the two age groups (p=0.004) but not between females and males (p=0.55). Optimal mean screw length was 73.4±9.9 mm. Anatomical spinal canal parameters in the transverse plane varied with age (p=0.02) and with sex in the older children (p=0.008), and those in the sagittal plane varied with sex (p=0.04). Age affected ilio-sacral screw positioning, whereas sex did not. Several anatomical spinal canal parameters varied with age and sex. These results should help to ensure safe and easy ilio-sacral screw placement within a secure corridor.

Severe infant osteogenesis imperfecta requires osteosynthesis. Intramedullary tibia's osteosynthesis is a technical challenge given the deformity and the medullar canal's narrowness. We describe an extramedullary technique: 'In-Out-In' K-wires sliding. We performed an anteromedial diaphysis approach. The periosteum was released while preserving its posterior vascular attachments. To obtain a straight leg, we did numerous osteotomies as many times as necessary. K-wires ('In') were introduced into the proximal epiphysis, and the medial malleolus ('Out') bordered the cortical and ('In') reach their opposite metaphysis. K-wires were cut, curved and impacted at their respective epiphysis ends to allow a telescopic effect. All tibial fragments are strapped on K-wires, and the periosteum was sutured over it. Our inclusion criteria were children with osteogenesis imperfecta operated before 6 years old whose verticalization was impossible. Seven patients (11 tibias) are included (2006-2016) with a mean surgery's age of 3.3 ± 1.1 years old. All patients received intravenous bisphosphonates preoperatively. The follow-up was 6.1 ± 2.7 years. All patients could stand up with supports, and the flexion deformity correction was 46.7 ± 14.2°. Osteosynthesis was changed in nine tibias for the arrest of telescoping with flexion deformity recurrence and meantime first session-revision was 3.8 ± 1.7 years. At revision, K-wires overlap had decreased by 55 ± 23%. Including all surgeries, three distal K-wires migrations were observed, and the number of surgical procedures was 2.5/tibia. No growth arrest and other complications reported. 'In-Out-In' K-wires sliding can be considered in select cases where the absence of a medullary canal prevents the insertion of intramedullary rod or as a salvage or alternative procedure mode of fixation. It can perform in severe infant osteogenesis imperfecta under 6 years old with few complications and good survival time., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Multiple epiphyseal dysplasia (MED) and pseudoachondroplasia (PSACH) are congenital skeletal disorders characterized by irregular epiphyses, mild or severe short stature and early-onset osteoarthritis which frequently affect the hips. The current study evaluates the long-term results of the Chiari osteotomy in MED and PSACH patients., Methods: Twenty patients (14 MED and 6 PSACH) were retrospectively included. Clinical assessment used the Postel Merle d'Aubigné (PMA) score and the Hip disability and Osteoarthritis Outcome Score (HOOS). Risser index, Sharp angle, acetabular depth index, center-edge angle, Tönnis angle, and femoral head coverage were measured on the preoperative radiographs and at last follow-up. The Treble index, which identifies the hip at risk in MED patients, was also determined. Stulberg classification (grades I to V) was used to evaluate the risk of osteoarthritis in the mature hips.Statistical analyses determined differences between preoperative and postoperative data. The Kaplan Meier method was used to calculate the survival rate of the operated hips using total hip arthroplasty as the endpoint., Results: Thirty-three hips which underwent a Chiari osteotomy were reviewed. The average follow-up was 20.1 years. The PMA scores were significantly better at last follow-up than preoperatively. All radiographic parameters significantly improved. Moreover, the Sharp angle, center-edge angle, and femoral head coverage improved to a normal value at hip maturity. All of the operated hips had a Treble index of type I. At hip maturity, a majority of hip were aspherical congruent (Stulberg grades of III and IV). The survival rate of the operated hips was 80.7% at 24 years postoperative., Conclusions: The Chiari osteotomy is a satisfying solution for severe symptomatic hip lesions in MED and PSACH patients. At long-term follow-up, this procedure lessens pain and improves hip function, which delays total hip arthroplasty indication., Level of Evidence: Level IV., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: Multiple synostoses syndrome (MSS) is a rare genetic condition. Classical features consist of joint fusions which notably start at the distal phalanx of the hands and feet with symphalangism progressing proximally to carpal, tarsal, radio-ulnar, and radio-humeral joints, as well as the spine. Usually, genetic testing reveals a mutation of the NOG gene with variable expressivity. The goal was to present the anatomical, functional, and radiological presentations of MSS in a series of patients followed since childhood., Methods: Patients with more than 3 synostoses affecting at least one hand joint were included. When possible, genetic screening was offered., Results: A retrospective study was performed from 1972 to 2017 and included 14 patients with a mean follow-up of 18.6 years. Mutation of the NOG protein coding gene was seen in 3 patients. All presented with tarsal synostoses including 9 carpal, 7 elbow, and 2 vertebral fusions. Facial dysmorphia was seen in 6 patients and 3 were hearing-impaired. Surgical treatment of tarsal synostosis was performed in 4 patients. Progressing joint fusions were invariably seen on x-rays amongst adults., Conclusion: Long radiological follow-up allowed the assessment of MSS progression. Feet deformities resulted in a severe impact on quality of life, and neurological complications secondary to spine fusions warranted performing at least one imaging study in childhood. As there is no treatment of ankylosis, physiotherapy is not recommended. However, surgical arthrodesis for the treatment of pain may have reasonable outcomes.

Introduction: Vascularized fibular proximal epiphyseal transfer associated to a diaphyseal segment is used to treat childhood epiphyseal defect. The aim of the present study was to analyze surgical technique and long-term clinical and radiological results., Material and Method: Between 1997 and 2008, 7 patients with a mean age of 5.7 years (range, 2-8 years) were operated on for bone malignancy with vascularized fibular epiphyseal transfer: 5 Ewing sarcomas and 2 osteosarcomas, located in the proximal femur (n=3), proximal humerus (n=3) or distal radius (n=1). Mean transplant size was 13.8cm. Vascularization involved a single artery in 5 cases (3 peroneal, 2 anterior tibial) and both in 2 cases. Internal fixation used intramedullary nailing in 6 cases and screwed plate in 1. All patients underwent pre- and post-operative chemotherapy following French Pediatric Oncology Society (SFOP) protocols., Results: Mean follow-up was 11 years (range, 3 years 11 months to >17 years). All patients were alive and in tumor remission. Reconstructed joint function was satisfactory in 85% of cases. Graft thickening indicated integration in all cases. The transferred cartilage had recovered growth in 4 cases. Complications comprised postoperative infection (n =1), consolidation defects (n = 2), fractures (n = 8), malalignment requiring surgical revision (n = 1), and spontaneously resolving common peroneal nerve palsies (n = 2)., Discussion: In young children, vascularized fibular epiphyseal transfer fills bone defect, reconstructs a functional joint and allows continued growth in the resected segment. Growth prostheses, in the authors' experience, always give poor results in this age-group, and fusion fails to address the growth problem. Patients should be informed about the risk of fracture, persisting over the long term., Conclusion: Vascularized fibular epiphyseal transfer is a difficult technique, subject to complications, but enables reconstruction of a bone segment involving the epiphysis in young children, conserving function and growth., Level of Evidence: III, retrospective clinical study., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

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.)

Introduction: Aneurysmal bone cyst (ABC) is a benign bone lesion of childhood and adolescence. It can be locally aggressive, with risk of fracture. Management is controversial. The aim of the present study was to assess the efficacy, simplicity and tolerance of percutaneous alcohol-bases sclerotherapy in ABC., Hypothesis: Alcohol-based sclerotherapy for ABC under radiographic control is safe and effective., Material and Methods: A single-center retrospective study for the period 2008-2016 included all of the 55 ABCs, in 54 patients, confirmed on pathology and treated by alcohol-based sclerotherapy under radiographic control. Mean age at diagnosis was 9.6 years. ABC involved the humerus in 30 cases (54%), tibia in 7 (13%) and femur in 5 (9%). Mean follow-up was 50.9 months (range, 16-117 months). Mean number of applications was 1.7 (range, 1-4). Results were assessed clinically (pain, return to sport, limb length and alignment, revision surgery) and radiologically. The main endpoint was lesion volume reduction. The secondary endpoint was failure, defined by open revision surgery or pain preventing return to a sports activity., Results: Clinical progression was favorable in 36 patients (67%), and radiological progression in 45 (85%). Only 1 cyst required secondary resection. One patient experienced spontaneously resolving intraoperative bradycardia. Male gender and young age emerged as factors for poorer response., Discussion: ABC management in children can be made difficult by lesion size, aggressiveness, location, proximity to the growth plate and small bone stock. Alcohol-based sclerotherapy is simple, reliable and effective in childhood ABC, and may be a first-line attitude, avoiding recourse to invasive surgery., Level of Evidence: IV, retrospective study., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

The aim of the present study was to report results of direct circular suture after 1-stage circumferential resection of limb ring constriction in amniotic band syndrome. A multicentre retrospective study included 14 patients with amniotic band syndrome (mean age, 13.3 months) operated on between 2004 and 2019 by circumferential release of ≥1 ring constriction. Assessment was based on limb function and clinical scar aspect on the POSAS and Vancouver scales. Mean follow-up was 3.9 years. There were no scar-related, vascular or neurologic complications, postoperatively or at last follow-up. POSAS and Vancouver scores were satisfactory. One-stage circumferential release with direct closure is a simple technique that provides satisfactory functional and esthetic results., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

In Rubinstein-Taybi syndrome, patients may have a particularly severe clinodactyly of the thumb. We evaluated a new method for correction of these severe clinodactylies using non-vascularized toe phalanx transfer as a replacement for the abnormal delta phalanx. Results of the new technique are presented, together with those of an osteotomy technique. We retrospectively recorded the angle of the clinodactyly before and after surgery and at long-term follow-up of 11 osteotomies and five transfers in nine patients from 1990 to 2017. The pre-operative angle of clinodactyli was similar between the two groups with a mean of 59°. After surgery, the correction was equivalent (7° and 11°). At the last follow-up (7 and 18 years), the relapse of clinodactyly was 17° for osteotomies and 1° for phalanx transfers. We noticed growth of the transferred phalanx, resulting in an excellent thumb length. We conclude that non-vascularized toe transfer can be an effective correction of severe clinodactyly and may be more stable than osteotomy in the long-term. Level of evidence: IV.

Objective: Early-onset scoliosis (EOS) is not uncommon in patients with neurofibromatosis type 1 (NF1). Despite conservative treatment, spinal deformities progress and require early surgical intervention. To avoid potential interference with chest and trunk growth, growing rods (GRs) have been used effectively in EOS of various etiologies. In this study the authors sought to analyze the outcomes of GRs in EOS patients with NF1., Methods: This was a retrospective single-center cohort study that included consecutive EOS patients with NF1 who were treated with GRs and were followed up for a minimum of 2 years. Clinical and radiological analyses were performed preoperatively and until the last follow-up., Results: From to 2008 to 2017, 18 patients (6 male, 12 female) underwent GR surgery (14 single GRs, 4 dual GRs) at a mean age of 8 ± 2.1 years. Mean follow-up was 5 ± 2.4 years. Fifty-five lengthenings were performed at a mean rate of 3 lengthenings per patient (range 0-7). Ten of 14 single GRs (71%) were converted into dual GRs during treatment. No patient underwent definitive posterior spinal fusion (PSF) at GR treatment completion. The mean initial and last follow-up major curves were 57° and 36°, respectively (p < 0.001, 37% correction). The average T1-S1 increase was 13 mm/yr. Six of 9 hyperkyphotic patients had normal kyphosis at last follow-up. There were 26 complications involving 13 patients (72%), with 1 patient who required unplanned revision. The primary complications were instrumentation related, consisting of 17 proximal hook dislodgments, 6 distal pedicle screw pullouts, and 2 rod fractures. Only 1 patient experienced a mechanical complication after dual GR implantation. There were no wound infections., Conclusions: The GR technique provided satisfactory spinal deformity control in EOS patients with NF1 while allowing substantial spinal growth. Adequately contoured dual GRs with proximal hooks placed in nondystrophic regions should be used to minimize implant-related complications. Surgeons should not attempt to correct kyphosis at GR implantation.

Study Design: Biomechanical human cadaver study., Objective: To determine the three-dimensional intervertebral ranges of motion (ROMs) of intact and hook-instrumented thoracic spine specimens subjected to physiological loads, using an in vitro experimental protocol with EOS biplane radiography. Pedicle screws are commonly used in thoracic instrumentation constructs, and their biomechanical properties have been widely studied. Promising clinical results have been reported using a T1-T5 thoracic hook-claw construct for proximal rod anchoring. Instrumentation stability is a crucial factor in minimizing mechanical complications rates but had not been assessed for this construct in a biomechanical study., Methods: Six fresh-frozen human cadaver C6-T7 thoracic spines were studied. The first thoracic vertebrae were instrumented using two claws of supra-laminar and pedicle hooks, each fixed on two adjacent vertebrae, on either side of a single free vertebra. Quasi-static pure-moment loads up to 5 Nm were applied to each specimen before and after instrumentation, in flexion-extension, right and left bending, and axial rotation. Five steel beads impacted in each vertebra allowed 3D tracking of vertebral movements on EOS biplanar radiographs acquired after each loading step. The relative ranges of motion (ROMs) of each pair of vertebras were computed., Results: Mean ROMs with the intact specimens were 17° in flexion-extension, 27.9° in lateral bending, and 29.5° in axial rotation. Corresponding values with the instrumented specimens were 0.9°, 2.6°, and 7.3°, respectively. Instrumentation significantly (P < 0.05) decreased flexion-extension (by 92-98%), lateral bending (by 87-96%), and axial rotation (by 68-84%)., Conclusion: This study establishes the biomechanical stability of a double claw-hook construct in the upper thoracic spine, which may well explain the low mechanical complication rate in previous clinical studies., Level of Evidence: Not applicable, experimental cadaver study.

Forearm deformities are often observed in patients with hereditary multiple osteochondroma, resulting in functional disability and cosmetic impairment. The aim of this study was to assess clinical and radiological outcomes after corrective osteotomy of the radius (COR). We performed a retrospective analysis of clinical and radiologic data from patients with forearm deformities who underwent COR combined with osteochondroma resection between 1978 and 2015. Seventeen patients (17 forearms) were included. The mean (range) age at surgery was 11.8 years (3.2-14.4), and the mean interval between surgery and last follow-up was 8.2 years (2-34.2). Range of motion was moderately increased and postoperative radiological assessments found significant improvements in ulnar variance, radial articular angle, bowing of the radius, and carpal slip. At last follow-up, a loss of ulnar variance correction was noted in 11 cases (mean loss: 4mm). The mean score on the Quick Disabilities of the Arm, Shoulder and Hand self-administered questionnaire was 13.9. Our results show that a forearm deformity in a patient with hereditary multiple osteochondroma is an appropriate indication for COR combined with osteochondroma resection and should be performed at the end of growth. This simple, safe technique corrects bowing of the radius and radius-ulna length discrepancy and could limit the risk of radial head dislocation. LEVEL OF EVIDENCE: IV., (Copyright © 2019 SFCM. Published by Elsevier Masson SAS. All rights reserved.)

Background: Various techniques have been proposed for the treatment of cavovarus feet (CVF). The aim of this study was to report outcomes of the revisited Meary's dorsal closing wedge tarsectomy for fixed CVF secondary to Charcot-Marie-Tooth (CMT) disease., Methods: All CVF operated on between 1977 and 2011 were included. The tarsectomy design was modified from its original description and systemically combined with a plantar fascia release, a Dwyer osteotomy and a proximal extension osteotomy of the 1st metatarsal bone if required. Outcomes were assessed by 2 functional scores and radiographically., Results: Among the 26 feet (20 patients), the Wicart and Seringe score was very good or good, fair and poor in respectively 58%, 23% and 19% of the feet. Hindfoot and midfoot AOFASs were of 95.5 and 75 respectively. All radiographic measures were significantly improved., Conclusions: This complete revisited procedure is an efficient and safe surgical technique for the treatment of the CMT disease CVF., Level of Evidence: Level IV., (Copyright © 2018 European Foot and Ankle Society. Published by Elsevier Ltd. All rights reserved.)

Study Design: Retrospective case series., Objectives: To analyze the outcomes at skeletal maturity of patients treated with a single traditional growing rod (GR). To compare results of patients according to whether posterior spinal fusion (PSF) was performed at treatment completion., Summary of Background Data: Few studies examined the end results of GRs at skeletal maturity. There is no agreement on requirement of PSF at GR treatment completion., Methods: Clinical and radiological analysis of consecutive patients with severe and/or progressive scoliosis treated initially with traditional single GR. Group comparisons of patients with PSF and without fusion surgery at treatment completion., Results: Thirty-four patients underwent traditional single GR implantation at a median age of 11.7 years. Median follow-up was 6.5 years. At last follow-up, T1-S1 distance was increased by a median 116 mm (P < 0.001) and median major curve Cobb angle was changed from 55° preoperatively to 30° (P < 0.001). Complications included 26 rod fractures, 1 implant prominence, 4 proximal junctional kyphosis, 2 proximal hook dislodgments, and 2 wound infections. At the beginning our experience, PSF was performed systematically in 17 patients. Relying on spinal ankylosis, 17 patients were subsequently not fused at GR treatment completion (single GR removed N = 2, single GR retained N = 7, dual GR surgery N = 8). There were no statistical differences between groups in improvements of radiological parameters from preoperative GR insertion to last follow-up. No GR fracture occurred after dual GR surgery., Conclusion: Single GR allows curve control and promotes spinal growth. Dual GR is, however, recommended for rod fracture prevention and better correction maintenance. In patients with satisfactory deformity correction at skeletal maturity, one may consider retaining dual GR instead of performing PSF., Level of Evidence: 4.

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.)

Congenital clinodactyly known as "delta phalanx" is a congenital finger deviation caused by a middle phalanx abnormality. Progressive realignment can be achieved with normal growth through physiolysis according to Vickers. The purpose of this investigation was to identify the rate of correction in congenital clinodactyly following epiphyseal bar resection.We studied retrospectively 24 fingers in 14 patients aged from 14 months to 11 years old (y/o), operated on by two surgeons performing epiphyseal bar resection using a modified Vickers' method. Angle measurements were compared pre-operatively, at 1 year and at the final follow-up. The rate of correction was evaluated at each time point. The cohort was divided into two groups: surgery < 6 y/o and ≥ 6 y/o. Statistical analyses were conducted to compare the patient groups.The mean deviation decreased from 33° pre-operatively (20°-80°) to 17.2° (0°-40°) at 1 year after surgery. The deviation at the last follow-up decreased to 10.6° (0°-40°). Seventy percent of the operated fingers were completely corrected (angle ≤ 10°) at the latest follow-up visit. No difference was found regarding the age at surgery. There were two cases of premature fusion of the epiphyseal plates. Regardless of the patient's age, Vickers' procedure is a simple and effective solution for treating delta phalanx with early correction. Our data suggest that physiolysis is a successful surgery in patients up to 11 years old. LEVEL OF EVIDENCE: Level IV, therapeutic case series., (Copyright © 2019 SFCM. Published by Elsevier Masson SAS. All rights reserved.)

The "horseman" procedure is a surgical technique used to correct the talocalcaneal joint displacement of severe idiopathic flatfoot in children while maintaining the reduction with a temporary talocalcaneal screw. While this technique has been used since the early 1960s, very little has been reported on its results. Our objectives were to estimate the correction, functional results, and postoperative complications of the "horseman" procedure. We conducted a retrospective study on 23 consecutive patients (41 cases) who underwent the "horseman" procedure for a talocalcaneal joint displacement. Mean follow-up was 8.9 (range 1 to 28) years, and 8 patients (12 feet) had reached bone maturity at last follow-up. Mean age at surgery was 6.6 (range 4 to 9.5) years. At last follow-up, all the patients were asymptomatic except 2 [8.7%] (4 [9.8%] cases). The talocalcaneal divergence on anteroposterior and lateral radiographic views was reduced by 8.9° and 11.4°, respectively, after the surgery, and the correction was maintained with loss of 0.7° and 2.9°, respectively, at final follow-up. The talonavicular coverage angle was reduced by 25° without loss of correction at last follow-up. The calcaneal pitch angle did not change after the surgery. Mean American Orthopedic Foot and Ankle Society score increased from 88.7 of 100 (63 of 100 to 93 of 100) preoperatively to 99 of 100 (97 to 100 of 100) at last follow-up. No major complication occurred. The "horseman" procedure allows an immediate and lasting correction of severe idiopathic flatfoot in children., (Copyright © 2018 the American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: The objective of the present study was to evaluate the outcome of surgical treatment of hand lesions in Ollier disease (OD) carried out during childhood., Methods: A retrospective review was carried out of 10 pediatric patients with hand involvement of OD, who had undergone surgery for metacarpal or phalangeal enchondromas. The technique comprised curettage and cortical bone reconstruction with corticoplasty, to restore a near-normal phalangeal volume. The range of finger motion (pulp-to-palm distance), the shortened Disabilities of the Arm, Shoulder, and Hand (QuickDASH) questionnaire score, cosmetic improvement, radiological findings (according to Tordai's classification), and recurrence were recorded after a mean follow-up of 7.5 years (range, 4-11.3 years)., Results: The mean age at surgery was 10.7 years (range, 6-14 years). Curettage was performed on 35 enchondromas, and 9 cavities were filled with a bone graft. The mean pulp-to-palm distance was significantly lower after surgery (from 1.5 cm to 0.25 cm; P < .05). The mean QuickDASH score was 3.84 (range, 0-11.4). A marked cosmetic improvement was noted for 83% of the hands. Three enchondromas recurred in 1 patient, requiring a second curettage. Fifty-seven percent of the cavities were completely filled with bone (Tordai stage 1) at last follow-up. The outcome did not depend on the presence or absence of a bone graft., Conclusions: Our results suggest that early surgical treatment comprising curettage and corticoplasty leads to good clinical, cosmetic, and radiological outcomes. Early surgical treatment of well-developed and/or symptomatic enchondromas of the hand in OD should be considered., Type of Study/level of Evidence: Therapeutic V., (Copyright © 2018 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Purpose: The aim of the study was a review of the literature in order to evaluate the results and complications of closed reduction in late-detected developmental dysplasia of the hip (DDH)., Methods: This study consisted of an analysis of the literature relative to late-detected DDH treatment options considering hip congruency, rates of re-dislocation and of avascular necrosis., Results: Gradual closed reduction (Petit-Morel method) appears to be an effective method concerning joint congruency restitution. Dislocation relapse and avascular necrosis are more efficiently prevented with closed versus open reduction. The tendency for spontaneous correction of acetabular dysplasia decreases if closed reduction is performed after 18 months of age. Patient age at the beginning of traction should be considered for the prognosis, with a lower rate of satisfactory results showing after the age of 3 years., Conclusion: In our opinion, the Petit-Morel method is a suitable treatment option for children aged between six months and three years with idiopathic DDH.

Study Design: A retrospective review., Objective: To report the results of an alternative technique using a minimally invasive fusionless surgery. The originality is based on the progressive correction of the deformities with proximal and distal fixation and on the reliability of the pelvic fixation using iliosacral screws on osteoporotic bones., Summary of Background Data: Spinal deformities are common in neuromuscular diseases. Conventional treatment involves bracing, followed by spinal instrumented fusion. Growing rod techniques are increasingly advocated but have a high rate of complications., Methods: The technique relies on a bilateral double rod sliding construct anchored proximally by four hooks claws and distally to the pelvis by iliosacral screws through a minimally invasive approach. Hundred patients with neuromuscular scoliosis underwent the same fusionless surgery extended from T1 to the pelvis. The average age at initial surgery was 11 + 6 years. Diagnoses included cerebral palsy (61), spinal muscular atrophy (22), muscular dystrophy (10), and other neurological etiologies (7). Cobb angle and pelvic obliquity were measured before and after initial surgery, and at final follow-up. Complications were reviewed., Results: At latest follow-up 3 + 9 years (range 2 yr-6 + 3 yr), the mean Cobb angle improved from 89° to 35° which corresponds to 61% correction. Mean pelvic obliquity improved from 29° to 5°, which corresponds to 83% correction. Mean T1-S1 length increased from 30.02 to 37.28 cm. Mean preoperative hyper kyphosis was reduced from 68.44° to 33.29°. Complications occurred in 26 patients including mechanical complications (12) and wound infections (16). No arthrodesis was required at last follow-up., Conclusion: This original fusionless technique is safe and effective, preserving spinal and thoracic growth. It provides a significant correction of spinal deformities and pelvic obliquity with a reduced complications rate. The strength and stability of this modular construct over time allow the avoidance of final arthrodesis., Level of Evidence: 4.

Osteosarcoma is the most common malignant bone tumor in adolescents and young adults. Most osteosarcomas are sporadic but the risk of osteosarcoma is also increased by germline variants in TP53, RB1 and RECQL4 genes. ATRX germline variations are responsible for the rare genetic disorder X-linked alpha-thalassemia mental retardation (ATR-X) syndrome characterized by severe developmental delay and alpha-thalassemia but no obvious increased risk of cancer. Here we report two children with ATR-X syndrome who developed osteosarcoma. Notably, one of the children developed two osteosarcomas separated by 10 years. Those two cases raise the possibility that ATRX germline variant could be associated with an increased risk of osteosarcoma.

Multicentric carpotarsal osteolysis (MCTO) is a rare skeletal disorder characterized by progressive carpal and tarsal destruction. The upper and lower limbs may be involved, leading to deformities and joint limitation. These anatomic features may be associated with progressive renal failure. The radiographs obtained during childhood showed a carpal and tarsal osteolysis and an asymmetrical involvement. Here, we report on the long-term clinical and radiological findings of three patients with skeletal manifestations of MCTO.

Background: Triple pelvic osteotomy (TPO) is a treatment option in children and adolescents with residual acetabular dysplasia after developmental dislocation of the hip (DDH). However, TPO to redirect the acetabulum is often blamed for anterior and lateral overcoverage of the femoral head. The main objectives of this study were to assess the potential clinical impact, frequency, and radiological features of acetabular overcorrection. Evidence of post-operative remodelling and associations linking younger age at surgery and/or dysplasia severity to the existence and magnitude of overcorrection were sought., Hypothesis: Acetabular overcorrection has little or no clinical impact., Patients and Methods: TPO was performed on 41 hips in 31 patients at a mean age of 6.3 years (range, 3.0-15.2 years). Mean follow-up was 13.8 years (range, 5.4-28.7 years) and mean age at last re-evaluation was 22.1 years (range, 13-39 years). Clinical outcomes were assessed based on the Harris Hip Score (HHS) and Postel-Merle d'Aubigné (PMA) score. Radiographs were used to look for a cross-over sign (CO+) and to measure the vertical-centre edge (VCE) and vertical-centre anterior (VCA) angles and the acetabular index (AI). Overcorrection was defined as AI≤0° and/or VCE≥35° and/or VCA≥40° and/or CO+., Results: The HHS and PMA score values were good or excellent for 39 (94%) hips. One or more parameters indicated overcorrection of 33 (80.5%) hips. No significant differences were found between the overcorrected hips and the hips with normal parameters., Discussion: TPO effectively corrects residual acetabular dysplasia. Overcorrection is common in all three planes but has little clinical impact in young adults. The high frequency of overcorrection mandates a careful pre- and intra-operative evaluation of acetabular version., Level of Evidence: IV, retrospective study., (Copyright © 2018. Published by Elsevier Masson SAS.)

Objective: Juvenile dermatomyositis (JDM) is an inflammatory pediatric myopathy characterized by focal capillary loss in muscle, followed by progressive recovery upon adequate treatment with immunomodulating drugs, although some patients remain refractory to treatment. While the underlying mechanism of capillary depletion remains uncertain, recent studies have identified an up-regulation of type I interferon (IFN) expression specific to JDM. Given that myogenic precursor cells (MPCs) exert proangiogenic activity during normal skeletal muscle regeneration, we hypothesized that they may also modulate vascular remodeling/angiogenesis during JDM. The aim of this study was to investigate that hypothesis., Methods: Human cell cocultures were used to analyze angiogenic properties in patients with JDM, patients with Duchenne's muscular dystrophy (DMD) (control patients for vascular remodeling), and healthy control subjects. Transcriptome analysis was used to examine muscle-derived MPCs. Histologic analysis of type I IFN in muscle biopsy samples was also performed., Results: Using human cell cocultures, we showed highly angiogenic properties of MPCs from JDM patients in association with the expression of an angiogenic molecular signature. Transcriptome analysis of MPCs freshly isolated from muscle samples revealed type I IFN as the master regulator of the most up-regulated genes in JDM-derived MPCs. Functionally, treatment of normal MPCs with type I IFN recapitulated the molecular pattern and the proangiogenic functions of JDM-derived MPCs. In vivo histologic investigation showed that MPCs synthesized type I IFN and major proangiogenic molecules in JDM muscle. Moreover, MPCs derived from JDM muscles that were characterized by strong vasculopathy produced higher levels of type I IFN, confirming MPCs as a cellular source of type I IFN during JDM, and this correlated with the severity of the disease., Conclusion: These results demonstrate a new type I IFN pathway in JDM that activates the production of angiogenic effectors by MPCs, triggering their proangiogenic function to promote vessel recovery and muscle reconstruction., (© 2017, American College of Rheumatology.)

Background: X-linked hypophosphatemic rickets (XLHR) is due to mutations in PHEX leading to unregulated production of FGF23 and hypophosphatemia. XLHR is characterized by leg bowing of variable severity. Phosphate supplements and oral vitamin analogs, partially or, in some cases, fully restore the limb straightness. Surgery is the alternative for severe or residual limb deformities., Objective: To retrospectively assess the results of surgical limb correction in XLHR (osteotomies and bone alignment except for 3 transient hemiepiphysiodesis)., Methods: We analyzed the incidence of recurrence and post-surgical complications in 49 XLHR patients (29F, 20M) (mean age at diagnosis 6.0 years (± 7.1))., Results: At first surgery, the mean age was 13.4 years (± 5.0). Recurrence was observed in 14/49 (29%) patients. The number of additional operations significantly decreased with age (2.0 (± 0.9), 1.7 (± 1.0) and 1.2 (± 0.4) in children <11 years, between 11 and 15, and >15 years; P  < 0.001). Incidence of recurrence seemed to be lower in patients with good metabolic control of the rickets (25% vs 33%). Complications were observed in 57% of patients., Conclusion: We report a large series of surgical procedures in XLHR. Our results confirm that phosphate supplements and vitamin D analog therapy is the first line of treatment to correct leg bowing. Surgery before puberty is associated with a high risk of recurrence of the limb deformity. Such procedures should only be recommended, following multidisciplinary discussions, in patients with severe distortion leading to mechanical joint and ligament complications, or for residual deformities once growth plates have fused., (© 2017 The authors.)

Introduction: Ankle arthrodesis in patients with haemophilia is a joint-non-preserving treatment option often resulting in substantial pain relief and termination of haemarthrosis by osseous consolidating of the joint. However, limited data are available regarding ankle arthrodesis in young patients., Aim: The aim of this study was to evaluate the long-term clinical and radiographic results with a minimum follow-up of ten years in children with haemophilia who underwent ankle arthrodesis., Methods: We retrospectively reviewed the results of ankle fusions performed in young haemophiliacs in our department between 1980 and 2006. The Méary procedure was used for arthrodesis, performed on patients with closed growth plates. Only one patient had no fixation, due to being only six years old. The modified American Orthopaedic Foot & Ankle Society (AOFAS) hindfoot score was calculated at last follow-up, and standard radiographic evaluations were performed., Results: A total of 22 ankles were analysed from 17 patients. The mean follow-up was 19.7 ± 8 years. The mean age of the patients was 15.5 (6-23) years at the time of index surgery. There were no intra-operative or peri-operative complications related to ankle arthrodesis. The mean modified AOFAS score at last follow-up was 83 ± 10.5. Radiographic assessment demonstrated solid osseous fusion at the arthrodesis site, with no axis deformities. Two patients developed secondary subtalar arthrosis, treated by subtalar arthrodesis six years after initial ankle arthrodesis in one case., Conclusion: In our study, tibiotalar arthrodesis in young patients with haemophilia resulted in good long-term functional outcome with a low surgery-related complication rate.

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.

Most of tumours of the foot are tumour-like (synovial cyst, foreign body reactions and epidermal inclusion cyst) or benign conditions (tenosynovial giant cells tumours, planta fibromatosis). Malignant tumours of the soft-tissue and skeleton are very rare in the foot and their diagnosis is often delayed with referral to specialised teams after initial inappropriate procedures or unplanned excisions. The adverse effect of these misdiagnosed tumours is the increasing rate of amputation or local recurrences in the involved patients. In every lump, imaging should be discussed before any local treatment. Every lesion which is not an obvious synovial cyst or plantar fibromatosis should have a biopsy performed.After the age of 40 years, chondrosarcoma is the most usual malignant tumour of the foot. In young patients bone tumours such as osteosarcoma or Ewing's sarcoma, are very unusually located in the foot. Synovial sarcoma is the most frequent histological diagnosis in soft tissues. Epithelioid sarcoma or clear cell sarcoma, involve more frequently the foot and ankle than other sites. The classic local treatment of malignant conditions of the foot and ankle was below-knee amputation at different levels. Nowadays, with the development of adjuvant therapies, some patients may benefit from conservative surgery or partial amputation after multidisciplinary team discussions.The prognosis of foot malignancy is not different from that at other locations, except perhaps in chondrosarcoma, which seems to be less aggressive in the foot. The anatomy of the foot is very complex with many bony and soft tissue structures in a relatively small space making large resections and conservative treatments difficult to achieve. Cite this article: EFORT Open Rev 2017;2. DOI: 10.1302/2058-5241.2.160078. Originally published online at www.efortopenreviews.org., Competing Interests: Conflict of Interest: None

Metacarpal lengthening is a useful procedure to address hand deficiencies in children. In this study, we aimed to compare the results of three different techniques from one consecutive clinical series of hand deficiencies. A total of 15 metacarpal lengthenings have been performed in 12 children aged from 9 to 14 years. The callotasis technique was used in seven cases, the two-stage distraction-graft technique in four cases and the single-stage lengthening in four cases. All the metacarpals healed with bone. The lengthening obtained was a mean of 13 mm (range 8-21 mm), a mean of 22 mm (range 13-32 mm) and a mean of 12 mm (range 9-15 mm), respectively, in the three different techniques. The healing index was longer for callotasis (81 days/cm) compared with the other techniques (41 days/cm and 46 days/cm, respectively). We observed one case of fracture after callotasis and one after distraction-graft. One patient underwent tenolysis of the extensor mechanism after single-stage lengthening. In conclusion, distraction graft and single-stage lengthening may be valuable alternatives to callotasis., Level of Evidence: IV; therapeutic study; multi-case series.

The induced membrane technique was designed by Masquelet et al. to address segmental bone defects of critical size in adults. It has been used after bone defects of traumatic, infectious and tumoral origin with satisfactory results. Recently, it has been used in children but, after an initial enthusiasm, several cases of failure have been reported. The purpose of this study was to assess the success rate and the risk factors of failure of the induced membrane for children. We conducted a systematic review of all the studies reporting the results of the induced membrane technique to address bone defects of critical size in children. Our primary outcome was the success rate of the technique defined as a bone union before any iterative surgery. Our secondary outcomes were the complications and the risk factors of failure. We searched Medline via Pubmed, EMBASE and the Cochrane Library. Twelve studies, including 69 patients, met the inclusion criteria. There were 41 boys and 28 girls. Mean age at surgery was 10 years. Mean size of resection was 12.38 cm and the mean time between the two stages was 5.86 months. Mean rate of bone union after the two stages of the induced membrane technique was 58% (40/69) but this rate increased to 87% after revision surgeries (60/69). Main complications were non-unions (19/69), lysis of the graft (6/69) and fractures of the bone graft (6/69). Only 1/69 deep infection was reported. Other non specific complications were regularly reported such limb length discrepancies, joint stiffness and protruding wires. Risk factor of failure that could be suspected comprised the resection of a malignant tumour, a bone defect located at the femur, a wide resection, a long time between the two stages, an unstable osteosynthesis and a bone graft associating autograft to other graft materials. The induced membrane technique is suitable for bone defects of critical size in children. It is a reliable technique with no need of micro vascular surgery. However, we found several risk factors of failure for the use of the induced membrane technique to address segmental bone defect of critical size in children., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Introduction: Neurologic pes cavus is a progressive deformity that is difficult to treat during growth. The present study reports results of non-operative management, based on the pathophysiology of the deformity, by untwisting nocturnal splint, preceded in some cases by untwisting walking cast. The objective was to assess efficacy and impact on indications for surgery., Method: Twenty-three children (35 feet) were included. All had neurologic cavovarus foot, which was progressive in 24 feet (69%) (Charcot-Marie-Tooth disease). Mean age at initiation of treatment was 8.8 years. In 13 feet (38%), treatment began with a untwisting walking cast and in 22 (62%) began directly with the splint., Results: Mean follow-up was 4.5 years. Fifteen feet showed very good and 8 good clinical results (65%); 9 children (12 feet) had moderate or poor results, requiring renewed treatment in 11 feet at a mean 4.5 years after initiation of non-operative treatment. Thirteen patients (56.5%, 21 feet) had reached end of growth by last follow-up; 10 of these feet (48%) had good or very good results without surgery. No triple arthrodeses were required. Factors weighing against good outcome comprised young age at treatment initiation and poor compliance with the splint. Primary deformity severity did not affect outcome., Conclusion: The present study demonstrated efficacy for non-operative treatment of childhood neurologic cavovarus foot. Surgery was either avoided (in half of the cases followed up to end of growth) or delayed by a mean 4.5 years, allowing a single procedure before end of growth. We recommend initiating non-operative treatment of childhood cavovarus foot, associating untwisting walking cast and untwisting nocturnal splint, as soon as clinical progression is detected and/or Méary angle on lateral X-ray with block reaches 15°., Level of Evidence: IV., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Introduction: Congenital dislocation of the knee (CDK) is rare, and clinical semiology at birth is not always suitably analyzed. Existing classifications fail to guide treatment. The aim of the present study was to develop a CDK classification for the neonatal period., Hypothesis: A classification based on neonatal severity of clinical signs is easy to implement on simple criteria., Material and Methods: Fifty-one CDKs (40 patients) seen neonatally were included. Three types could be distinguished in terms of reduction and stability: type I, easily reducible CDK, with reduction snap when the femoral condyles pass in flexion, remaining stable in flexion; type II, "recalcitrant" dislocation, reducible by posteroanterior "piston" but unstable, with iterative dislocation once posteroanterior pressure on the condyles is relaxed; and type III, irreducible. The number of anterior skin grooves, global range of motion, flexion deficit and reduction stability were noted for each type., Results: Mean age at first consultation was 5.6 days (range: 0-30). CDK was type I, II and III in respectively 28, 16 and 7 cases. Number of skin grooves, flexion and baseline range of motion were greater in type I than types II and III., Conclusion: The present neonatal clinical classification is original, logical and simple. It may be useful for prognosis and guiding treatment., Level of Evidence: IV, single-center retrospective series., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)