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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.

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: 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: 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.)

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.

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.

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.)

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

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.)

Introduction: An original classification of congenital dislocation of the knee (CDK) was drawn up, based on neonatal semiology. The objective of the present study was to assess impact on treatment decision-making and prognosis., Material and Methods: Fifty-one CDKs in 40 patients were classified neonatally into 3 types: I, reducible (n=28); II, recalcitrant (n=16); and III, irreducible (n=7). Number of anterior skin grooves, range of motion (RoM), flexion deficit and reduction stability were recorded. Depending on reducibility, treatment comprised: physiotherapy with splints, traction with cast immobilization, or surgery. At follow-up, knees were assessed in terms of RoM and stability., Results: Mean age at first consultation was 5.6 days (range: 0-30). Mean age at follow-up was 9 years (range: 1-26). Physiotherapy with splinting achieved stable reduction in all type-I knees. Five type-II knees (31%) required traction, none of which needed surgery. Four type-III knees (57%) required surgery. Outcome was good or excellent in 82% of type-I knees, good in 68% of type II and poor in all type-III knees., Conclusion: The study confirmed the relevance of the present neonatal classification to treatment, with increasing rates of surgical indication and decreasing rates of satisfactory outcome from types I to III. Therapeutic attitude can be graded according to severity of CDK., Level of Evidence: IV, single-center retrospective series., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

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.)

We report a case of Bartonella henselae, an agent of cat scratch disease, C1-C2 osteoarthritis with osteolysis of the lateral mass of C2 in a 14-year-old boy. Oral antibiotics did not successfully treat the infection and surgery was necessary to treat the septic arthritis. The case opens discussion about bacterial osteoarthritis of the cervical spine and bone involvement in disseminated bartonellosis., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Unlabelled: Developmental dislocation of the hip (DDH) is frequently, even after reduction, associated with residual acetabular dysplasia. Various surgical techniques are used to correct this, one of which is Dega acetabuloplasty. This osteotomy technique has, however, rarely been assessed in this particular indication. The present study therefore sought to describe the technical details, report clinical and radiological results, and present limitations., Hypothesis: Unlike reorientation osteotomy in children, Dega acetabuloplasty does not lead to a high rate of acetabular retroversion at the end of growth., Patients and Methods: Sixteen Dega acetabuloplasties in 15 patients were assessed on joint range of motion, limp, lower limb length discrepancy and impaired everyday activity, pre-operatively and at end of follow-up. Hips were classified following Wicart et al. (2003). Radiologic assessment comprised Wiberg angle and acetabular index, pre- and post-operatively and at follow-up. Acetabular retroversion was analyzed by crossover sign, and hips were classified following Severin., Results: Median age at surgery was 3 years (range, 1.1-12.2 years) and 10 years (6.4-17.8) at end of follow-up. At end of follow-up, all hips were pain-free and classified as Wicart A, and all activities were allowed. Radiologically, hips were classified as Severin I, II or IV, in 11 (68.5%), 4 (25%) and 1 (6.5%) cases respectively. Wiberg angle rose from a mean 3.3° (-30° to 30°) to 23° (10° to 38°) and acetabular index fell from a mean 31° (25° to 45°) to 20° (5° to 30°) with surgery, and both continued to improve over follow-up: 26° (12-45°) and 13° (3-24°) respectively (P<0.05). Acetabular retroversion was found in 2 of the 10 hips with Y cartilage fusion., Discussion: Modified Dega acetabuloplasty was effective in correcting acetabular dysplasia in DDH. Functional and radiological results were good, with a low rate of acetabular retroversion (2/10), unlike with other techniques., Level of Evidence: Level IV. Therapeutic study., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Purpose: The purpose of this study was to evaluate the two-stage surgical technique combining induced membrane, spongy autograft and intramedullary fixation for the treatment of congenital pseudarthrosis of the tibia (CPT)., Methods: Three boys and two girls were treated by this technique between 2003 and 2008. All patients had type IV CPT in Crawford's classification. Four of them had a limited dystrophic form, whereas one case presented an extensive tibia bone dystrophy. The average age of patients at the time of surgery was 23 months (range 10-30 months), with an average follow-up of 5.8 years (range 2.4-8.1 years)., Results: Satisfactory tibial bony union was achieved in all cases at the last follow-up. Bone healing was obtained in the four limited forms after an average term of 4 months. One patient suffered from a non-displaced fracture that healed by casting in a usual period of time. The patient with an extensive dystrophic bone had to undergo a secondary inter-tibiofibular bone graft to finally achieve bone union., Conclusions: The preliminary results show that this technique is successful in CPT. It may be used even in young children and offers a good alternative to other treatments available, avoiding external fixation and the technical difficulties of microvascular surgery.

The epidemiology of acute paediatric osteoarticular infections (OAI) has recently evolved, mainly due to the improvement of microbiological diagnosis. We conducted a prospective study to analyse the recent epidemiology and the clinical evolution of paediatric OAI in order to validate the adequacy of our probabilistic first-line antibiotic treatment (intraveinous cefamandole + gentamicin). All children suspected of community acquired OAI were included and followed-up for 3 years. The etiologic diagnosis was based on blood cultures, joint aspirations and bone punctures. All osteoarticular (OA) samples were systematically inoculated into blood culture bottles. Real-time universal 16S rRNA and PCR targeted on Staphylococcus aureus, Kingella kingae, Streptococcus pneumoniae and Streptococcus pyogenes were performed twice a week. From 17 March 2007 to 26 February 2009, 98 septic arthritis, 70 osteomyelitis, 23 osteoarthritis and six spondylodiscitis were analysed. A portal of entry was suspected in 44% of cases, including 55% of otorhinolaryngological infections. C reactive protein was the most sensitive inflammatory marker. PCR increased by 54% the performance of bacteriological diagnosis. Among the patients completely investigated (blood culture and OAI samples), there were 63% documented OAI. The main pathogens found were K. kingae (52%), S. aureus (28%), S. pyogenes (7%), S. pneumoniae (3%) and Streptococcus agalactiae (2%). All isolated bacteria were sensitive to the probabilist treatment and outcome was favorable. PCR has significantly improved the performance and the delay of IOA diagnosis in children, for which K. kingae turned out to be the first causative agent. The probabilistic treatment was active against the main bacteria responsible for paediatric OAI., (© 2012 The Authors Clinical Microbiology and Infection © 2012 European Society of Clinical Microbiology and Infectious Diseases.)

Introduction: Pes planovalgus (PPV) is a complex three-dimensional deformity of which routine radiographs provide only a two-dimensional analysis., Hypothesis: Angles and other radiographic parameters of the foot in children and adolescents, when studied on both the dorsoplantar and the lateral view, can be used to establish a radiographic classification system for PPV that provides useful therapeutic guidance in clinical practice., Materials and Methods: A retrospective single-centre study was conducted on 65 feet in 35 patients aged 7 to 18 years and having adequate ossification. All patients had a clinical diagnosis of idiopathic or neurologic PPV and available weight-bearing dorsoplantar and strict lateral radiographs. We excluded pes planus due to tarsal coalition, congenital bone deformities, or overcorrection of talipes equinovarus (n=25). All possible axes were drawn and angles measured after an evaluation of interindividual agreement., Results: We identified four patterns of PPV: subtalar pes planus (n=16) with marked subtalar valgus and longitudinal sag predominating at the talonavicular joint, midtarsal pes planus (n=12) without subtalar valgus but with marked midtarsal abduction and sag predominating at the cuneonavicular joint, mixed pes planus (n=28) with subtalar valgus, midtarsal abduction, and sag at both the talonavicular and cuneonavicular joints, and pes planocavus (n=9) with sag of the medial arch and cavus deformity of the lateral arch., Conclusion: This original classification system provides therapeutic guidance by helping to match the surgical procedure to the nature and location of the deformities., Level of Evidence: Level IV., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background. Only limited data are available concerning desmoid tumor in children. Methods. Fifty-nine children and adolescents with desmoid tumor treated in 2 French cancer centers with a very long followup were retrospectively reviewed. Results. Median age was 6 years (range, 0-15). Tumors mainly involved the limbs (42%). Five cases occurred in a context of genetic disorder. Surgery was first-line treatment in 80% of cases. Resection was microscopically complete in 3 patients (pts), with a microscopic residue in 19 pts and a macroscopic residue in 35 cases. Various adjuvant therapies were used. Overall response to all systemic therapies was 33%. Thirty-eight patients developed one or more recurrences or progressions. After a median followup of 8.5 years, 34 patients were alive in complete remission (CR), including 16 first CR. Seven patients died, 6 from refractory disease and 1 from colorectal carcinoma in a genetic context. Ten-year progression-free survival (PFS) and overall survival were 31% and 88%, respectively. In univariate analysis, age less than 10 years and head-neck site were favorable prognostic factors for PFS. Conclusions. When surgery is required, surgical margins must be negative. Low-dose chemotherapy can be proposed as adjuvant therapy. Prospective trials must be developed to evaluate long-term response and side effects.

Introduction: Ankle valgus deformity is rare in children. It generally leads to difficulties wearing shoes, walking instability and mechanical pain. No medical treatment is effective and the only option is surgical correction of the deformity. Two main options are available: supramalleolar osteotomy and medial malleolar epiphysiodesis. We report our experience with epiphysiodesis using a transphyseal medial malleolar screw., Patients and Methods: This is a retrospective study of all children followed for ankle valgus and treated by transphyseal medial malleolar screw epiphysiodesis in our department. The study included 10 cases of ankle valgus deformity in seven children (four with multiple extostoses, two type 1 neurofibromatosis, one Larsen's syndrome) who completed skeletal maturity. At surgery, median bone age was 12 years (10 to 13 years and 6 months) and the median tibiotalar angle was 17.5° (10° to 30°)., Results: At skeletal maturity, preoperative valgus was corrected in six patients (9/10 ankles). The median tibiotalar angle was 5° (0° to 25°). Valgus was not corrected in one patient (30° to 25°). No postoperative complications occurred., Discussion: Epiphysiodesis by transphyseal medial malleolar screw is a simple, efficient and safe procedure to correct a significant or symptomatic ankle valgus deformity in children before skeletal maturity., Level of Evidence: Level IV, retrospective study., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Neurofibromatosis 1 (NF1), a common autosomal dominant disorder, was shown in one study to be associated with a 15-year decrease in life expectancy. However, data on mortality in NF1 are limited. Our aim was to evaluate mortality in a large retrospective cohort of NF1 patients seen in France between 1980 and 2006., Methods: Consecutive NF1 patients referred to the National French Referral Center for Neurofibromatoses were included. The standardized mortality ratio (SMR) with its 95% confidence interval (CI) was calculated as the ratio of observed over expected numbers of deaths. We studied factors associated with death and causes of death., Results: Between 1980 and 2006, 1895 NF1 patients were seen. Median follow-up was 6.8 years (range, 0.4-20.6). Vital status was available for 1226 (65%) patients, of whom 1159 (94.5%) survived and 67 (5.5%) died. Overall mortality was significantly increased in the NF1 cohort (SMR, 2.02; CI, 1.6-2.6; P < 10-4). The excess mortality occurred among patients aged 10 to 20 years (SMR, 5.2; CI, 2.6-9.3; P < 10-4) and 20 to 40 years (SMR, 4.1; 2.8-5.8; P < 10-4). Significant excess mortality was found in both males and females. In the 10-20 year age group, females had a significant increase in mortality compared to males (SMR, 12.6; CI, 5.7-23.9; and SMR, 1.8; CI, 0.2-6.4; respectively). The cause of death was available for 58 (86.6%) patients; malignant nerve sheath tumor was the main cause of death (60%)., Conclusions: We found significantly increased SMRs indicating excess mortality in NF1 patients compared to the general population. The definitive diagnosis of NF1 in all patients is a strength of our study, and the high rate of death related to malignant transformation is consistent with previous work. The retrospective design and hospital-based recruitment are limitations of our study. Mortality was significantly increased in NF1 patients aged 10 to 40 years and tended to be higher in females than in males.

Background: Numerous reports of THAs in patients younger than 30 years indicate a high risk of revision. Although risk factors for revision have been reported for older patients, it is unclear whether these risk factors are the same as those for patients younger than 30 years., Questions/purposes: We therefore (1) determined function and survivorship of revision THAs performed in patients younger than 30 years, and (2) assessed the risk factors for revision THAs in this younger population by comparison with a group of patients younger than 30 years who did not undergo revision., Patients and Methods: We retrospectively reviewed the clinical records and radiographs of 55 patients younger than 30 years (average age at revision, 24.3 years; range, 14-30 years) who underwent 77 hip revisions. Revision was performed, on average, 4.6 years (range, 0.4-12 years) after the primary THA. The results for these 55 patients (77 revision THAs) were compared with results for a nonrevised group, including 819 THAs in patients younger than 30 years. Minimum followup of the revision group was 1 year (mean, 6.2 years; range, 1-15 years)., Results: At followup after the revision, the Merle d'Aubigné-Postel score improved from 12.2 to 14.6. The rates of dislocation, neurologic lesions, and fractures were 15%, 7.8%, and 14%, respectively. The 10-year survival rate was 36% (95% confidence interval [CI], 21%-51%). Compared with the nonrevised group, the independent revision risk factors were young age at primary THA (OR 1.14 [1.07-1.19]), high number of previous surgeries (OR 5.41 [2.67-10.98]), and occurrence of at least one dislocation (OR 3.98 [1.74-9.07]). Hard-on-soft bearings had a higher risk (OR 3.42 [1.91-6.1]) of revision compared with hard-on-hard bearings., Conclusions: Revision THAs are likely in patients younger than 30 years, and the complication rate is high. The survivorship of hip revision in this population is low and alternative solutions should be advocated whenever possible., Level of Evidence: Level III, therapeutic study, case control study. See the Guidelines for Authors for a complete description of levels of evidence.

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.

Objectives: Procalcitonin (PCT) is an accurate marker for differentiating bacterial infection from non-infective causes of inflammation or viral infection. However, there is only one study in children which tested procalcitonin as a diagnostic aid in skeletal infections. With this study we sought to evaluate the sensitivity, specificity and predictive values of procalcitonin for identifying bone and joint infection in children evaluated in the emergency department for non traumatic decreased active motion of a skeletal segment., Methods: Patients aged 1 month to 14 years were prospectively included in the emergency department when suspected for osteomyelitis or septic arthritis. Procalcitonin levels, C reactiv protein, white blood cell count were measured and bacteriological samples were collected before initiation of antibiotic treatment. Patients were assigned to 3 groups according to the degree of suspected infection: group 1 confirmed infection, group 2 presumed infection and group 3 non infected patients., Results: Three hundred thirty nine patients were included (118 girls and 221 boys). Group 1 comprised 8 patients (2 had PCT levels > 0.5 ng/ml). Two had osteomyelitis and 6 septic arthritis. Forty children were incuded in group 2 (4 had PCT levels > 0.5 ng/ml). Eighteen had presumed osteomyelitis and 22 presumed septic arthritis. Group 3 comprised 291 children (9 PCT levels > 0.5 ng/ml) who recovered without antibiotic treatment. The specificity of the PCT as a marker of bacterial infection (comparing Group 1 and Group 3) was 96.9% [95% CI, 94.2-98.6], the sensitivity 25% [95% CI, 3.2-65.1], the positive predictive value (PPV) 18.2% [95% CI, 2.3-51.8] and the negative predictive value (NPV) 97.9% [95% CI, 95.5-99.2]., Conclusion: PCT is not a good screening test for identifying skeletal infection in children. Larger studies are needed to evaluate still more the place of PCT measurements in the diagnosis of osteomyelitis and septic arthritis.

There are a few case reports in the literature on mechanical compressions of the spinal cord associated with Scheuermann's disease and the treatment is not codified. We describe a case of spinal cord compression at the apex of the kyphosis, which disappeared with the reduction of the deformity. Given the skeletal immaturity, anterior and posterior fusions had to be performed together in order to obtain a good result.

Numerous options exist for intercalary segmental reconstruction after bone tumor resection. We present the extension of a recently developed surgical two-stage technique that involves insertion of a cement spacer, induction of a membrane, and reconstruction of the defect with cancellous and cortical bone autograft in a 12-year-old child. The boy was referred to our center for treatment of a right femoral diaphyseal Ewing's sarcoma. The first stage involved resection of the tumor and reconstruction with a locked intramedullary nail and a polymethylmethacrylate cement spacer. Seven months after the initial procedure during which adjuvant chemotherapy was given, the second-stage procedure was performed. The cement was removed and cancellous and cortical bone autograft was grafted in the membrane created around the cement spacer. Touchdown weightbearing was allowed immediately, partial weightbearing was resumed 6 weeks after the operation, and full weightbearing was allowed 4 months later. Successive plain radiographs showed rapid integration of the autograft to the host bone with bone union and cortical reconstitution. The principle of the induced membrane reconstruction seems applicable to intercalary segmental reconstruction after bone tumor resection in children.

Background: Langerhans cell histiocytosis (LCH) is a rare disease that affects mainly young children, and which features granulomas containing Langerhans-type dendritic cells. The role of several human herpesviruses (HHV) in the pathogenesis of LCH was suggested by numerous reports but remains debated. Epstein-barr virus (EBV, HHV-4), & Cytomegalovirus (CMV, HHV-5) can infect Langerhans cells, and EBV, CMV and HHV-6 have been proposed to be associated with LCH based on the detection of these viruses in clinical samples., Methodology: We have investigated the prevalence of EBV, CMV and HHV-6 infection, the characters of antibody response and the plasma viral load in a cohort of 83 patients and 236 age-matched controls, and the presence and cellular localization of the viruses in LCH tissue samples from 19 patients., Principal Findings: The results show that prevalence, serological titers, and viral load for EBV, CMV and HHV-6 did not differ between patients and controls. EBV was found by PCR in tumoral sample from 3/19 patients, however, EBV small RNAs EBERs -when positive-, were detected by in situ double staining in bystander B CD20+ CD79a+ lymphocytes and not in CD1a+ LC. HHV-6 genome was detected in the biopsies of 5/19 patients with low copy number and viral Ag could not be detected in biopsies. CMV was not detected by PCR in this series., Conclusions/significance: Therefore, our findings do not support the hypothesis of a role of EBV, CMV, or HHV-6 in the pathogenesis of LCH, and indicate that the frequent detection of Epstein-barr virus (EBV) in Langerhans cell histiocytosis is accounted for by the infection of bystander B lymphocytes in LCH granuloma. The latter observation can be attributed to the immunosuppressive micro environment found in LCH granuloma.

Background: Langerhans cell histiocytosis (LCH) is a rare clonal granulomatous disease that affects mainly children. LCH can involve various tissues such as bone, skin, lung, bone marrow, lymph nodes, and the central nervous system, and is frequently responsible for functional sequelae. The pathophysiology of LCH is unclear, but the uncontrolled proliferation of Langerhans cells (LCs) is believed to be the primary event in the formation of granulomas. The present study was designed to further investigate the nature of proliferating cells and the immune mechanisms involved in the LCH granulomas., Methods and Findings: Biopsies (n = 24) and/or blood samples (n = 25) from 40 patients aged 0.25 to 13 y (mean 7.8 y), were studied to identify cells that proliferate in blood and granulomas. We found that the proliferating index of LCs was low ( approximately 1.9%), and we did not observe expansion of a monocyte or dendritic cell compartment in patients. We found that LCH lesions were a site of active inflammation, tissue remodeling, and neo-angiogenesis, and the majority of proliferating cells were endothelial cells, fibroblasts, and polyclonal T lymphocytes. Within granulomas, interleukin 10 was abundant, LCs expressed the TNF receptor family member RANK, and CD4(+) CD25(high) FoxP3(high) regulatory T cells (T-regs) represented 20% of T cells, and were found in close contact with LCs. FoxP3(+) T-regs were also expanded compared to controls, in the blood of LCH patients with active disease, among whom seven out of seven tested exhibited an impaired skin delayed-type hypersensitivity response. In contrast, the number of blood T-regs were normal after remission of LCH., Conclusions: These findings indicate that LC accumulation in LCH results from survival rather than uncontrolled proliferation, and is associated with the expansion of T-regs. These data suggest that LCs may be involved in the expansion of T-regs in vivo, resulting in the failure of the host immune system to eliminate LCH cells. Thus T-regs could be a therapeutic target in LCH.

Lumbar hyperlordosis of neuromuscular origin is rare and requires surgical treatment in order to preserve a good sitting posture. We report twenty-seven cases of a preponderantly sagittal hyperlordosis deformity of the lumbar spine in patients with neuromuscular disorders and identify the indications and results of treatment. Seventeen males and ten females, aged 13 to 27 years, underwent operations for a lumbar hyperlordosis of neuromuscular origin responsible for major difficulties in sitting. In all patients, the sacrum was horizontal and associated in twenty-six cases with marked pelvic anteversion. Eleven patients were treated surgically by a posterior approach. The sixteen remaining patients had a preliminary discectomy, followed by posterior correction and fusion. Lumbar hyperlordosis was reduced from 8 degrees to 77 degrees between L1 and S1. The horizontal sacrum was partially reduced with an improvement from 8 degrees to 50 degrees . Consequently, patients recovered a comfortable sitting position. One patient died of respiratory complications six weeks after surgery. Surgical correction is a demanding procedure which can be performed by a posterior approach. It is mandatory to analyse the spino-pelvic balance to avoid iliac retroversion and the loss of the role of the ischia in the sitting position.

Between 1979 and 1996, 40 patients with high-grade lumbosacral spondylolisthesis were treated in our institution using a newly designed osteosynthesis device. The mean age was 13 years and 6 months, and the mean follow-up was 18 years. Combined posterior decompression and anterior reduction, instrumentation and fusion of the slippage were performed in all cases. The technique includes reduction of the slippage by means of an anteriorly placed plate that engages two screws, previously placed during the posterior approach, going through the S1 vertebra. Progressive compression applied on the plate by the screws achieves reduction. Complete fusion was obtained in all 40 patients. Twelve patients presented a postoperative radiculopathy, from which only ten recovered completely. There were six L4-L5 annulus lesions, responsible for instability, produced by the plate. We report five late infections. Thirty-five of the forty patients were asymptomatic at the latest follow-up. The double compressive plate technique proved to be effective in obtaining lumbosacral fusion and optimal slippage reduction. However, the high rates of neurological and infectious complications preclude recommendation of this technique in its present form.

The VATER association of vertebral anomalies (V), anal atresia (A), esophageal atresia and/or tracheo-esophageal fistula (TE), radial and renal anomalies (R) is a common congenital association of unknown origin with probably heterogeneous causes. Here, we report on a girl presenting with pre- and postnatal growth retardation, esophageal atresia, vertebral and costal anomalies and a unilateral radial defect, consistent with the diagnosis of VATER association. In the first month of life, she presented with failure to thrive, severe episodes of hypoglycemia, liver dysfunction and high levels of lactate, which prompted us to perform metabolic screening. A complex IV respiratory chain deficiency (RCD) was diagnosed on a liver biopsy. The respiratory chain defect was not observed in skin fibroblasts. No mtDNA point mutation or deletion was identified. The girl is now 9 years old and has a normal mental development but persistent feeding difficulties and moderate hyperlactatemia. To our knowledge, this is the second report of VATER association with mitochondrial disorder. In a previous report, a VACTERL association was observed in a girl with the mitochondrial A3243G point mutation. The association of VATER phenotype with a mitochondrial disorder may be coincidental but could also suggest that the presence of multiple malformations is the result of the antenatal expression of RCD.

We reviewed 5 cases of type I epiphyseal fracture with dislocation of the femoral head from the acetabulum in adolescent patients. All children had an open reduction and screw fixation. In all cases, the femoral head developed avascular necrosis. The clinical result after a mean of 3-9 years' follow-up was good according to the Merle d'Aubigné-Postel scale. Despite necrosis, 2 heads developed spherically after treatment: one which had a primary physeal resection and fixation, the other after an autogenous bone graft in the screw track following removal of the screw.