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

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. ispartof: PEDIATRIC BLOOD & CANCER vol:66 ispartof: location:United States status: published

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

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?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.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).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).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.IV.

International audience; Lipin-1 is a Mg2+-dependent phosphatidic acid phosphatase (PAP) that in mice is necessary for normal glycerolipid biosynthesis, controlling adipocytes metabolism and adipogenic differentiation. Mice carrying inactivating mutations in the Lpin1 gene display the characteristic features of human familial lipodystrophy. Very little is known on the roles of lipin-1 in human adipocyte physiology. Apparently fat distribution and weight is normal in humans carrying LPIN1 inactivating mutations, but a detailed analysis of adipose tissue appearance and functions in these patients has not been available so far. In this study, we performed a systematic histopathological, biochemical and gene expression analysis of adipose tissue biopsies from human patients harbouring LPIN1 biallelic inactivating mutations and affected by recurrent episodes of severe rhabdomyolysis. We also explored the adipogenic differentiation potential of human mesenchymal cell populations derived from lipin-1 defective patients. White adipose tissue from human LPIN1 mutant patients displayed a dramatic decrease in lipin-1 protein levels and PAP activity, with a concomitant moderate reduction of the adipocyte size. Nevertheless the adipose tissue develops without obvious histological signs of lipodystrophy and with a normal qualitative composition of the storage lipids. The increased expression of key adipogenic determinants such as SREBP1, PPARG and PGC1A shows that specific compensatory phenomena can be activated in vivo in human adipocytes under deficiency of a functional lipin-1.

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

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 12 mm. In the sagittal plane, the mean sagittal vertical axis (SVA) measured using the C7 plumb line was -28 mm, with 8 mm of anterior translation compared to the post-operative value (36 mm). 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).

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The hip is usually affected later, and its involvement is the most common cause of disability in JIA. Failure of medical and preventive treatment, significant joint destruction, or multiarticular involvement may necessitate total hip arthroplasty (THA) to restore good function. Related clinical trials show initially good THA results in most of the series, but long-term outcomes are uncertain due to a significant arthroplasty loosening rate in the initial years, particularly with cemented stems. The authors report the results of 62 noncemented THAs in 34 children with JIA after an average follow-up of 6 years (range 3-13). Mean age at surgery was 18.3 years (range 11.8-31) and 14 of the 34 children had active disease. Clinical results were good for hip function but less for global function. There were no infections. Two acetabular cups early in the series failed due to poor primary fixation and had to be revised. Survivorship analysis was performed with the Kaplan-Meier method. At 13 years, the survival rate was 100% for the femoral component and 90.1% for the acetabular component.

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.

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

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

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

Pigmented villonodular synovitis is an exceptional condition in children. The clinical, biological and imaging presentation is not specific and a histology sample is required for certain diagnosis. Because of this lack of specificity, together with the rare occurrence of the disease, late diagnosis is not uncommon, making correct management an even greater challenge in the growing child.Between 1995 and 2001, six children were treated for pigmentary villonodular synovitis, four girls and two boys, mean age 11.5 years. The knee was involved in five cases (three diffuse forms and two localized forms). One diffuse form involved the ankle. The diagnosis was suggested by the MRI findings in all patients and confirmed at the histological examination of a biopsy sample. Surgery was used in five cases and medical treatment in one patient with a diffuse form affecting the knee. A synoviorthesis (Hexatrione) was used systematically in three diffuse forms affecting the knee joint.Mean follow-up was 58 months. Recurrence was noted in two diffuse forms, one involving the knee at 10 months from arthroscopic synovectomy and one involving the ankle 24 months after surgical synovectomy. Systematic use of the synoviorthesis did not prevent recurrence in one case. The synoviorthesis was also used in the two cases of recurrence as a complement to surgical treatment but with no effect. Growth was not affected in any of the children.The etiopathogenic mechanism underpinning pigmented villonodular synovitis remains unclear. Genetic factors are suspected in childhood cases. MRI is the complementary examination of choice for diagnosis and follow-up. In children, treatment of pigmented villonodular synovitis depends on marginal excision of the lesion for localized forms and total synovectomy for the diffuse forms. The efficacy of the triamcinolone hexacetonide synoviorthesis remains open to debate and would require a larger series with longer follow-up for evaluation.

Tillaux fractures in adolescents correspond to Salter and Harris type III fractures involving the anterolateral portion of the tibial epiphysis. These are intra-articular fractures. The objective was to determine the circumstances of these fractures, the radiological signs, and the therapeutic modalities as well as the long-term clinical and radiological outcome.We reviewed 19 Tillaux fractures. Ten patients underwent surgical treatment and nine orthopedic treatment following importance of displacement.At mean follow-up of 33.8 months, results were rated good in 17 on 19 cases.This fracture is often observed in teenagers victims of trauma with external rotation of the foot. Closure of distal growth cartilage of the tibia occurs medially to laterally, the anterolateral portion remaining open longer. Forced external rotation of the anterior tibiofibular ligament pulls off an anterolateral fragment of the distal tibial epiphysis. Surgical treatment is indicated for fractures with a displacement of more than 2 mm or a vertical displacement to achieve open reduction and screw fixation. Orthopedic treatment is used for non-displaced fractures.The prognosis of Tillaux fractures is good as was observed in our series and in series reported in the literature.

Fracture of the anterior tibial tubercle is exceptional in adolescents. The purpose of this work was to better understand the circumstances of such fractures, identify potential complications, and assess outcome of different therapeutic options.This was a retrospective analysis of a consecutive series of 22 children with avulsion fracture of the anterior tibial tubercle. The circumstances of the fracture were noted. The Ogden classification was used to establish the radiological type. Associated lesions were also noted as were any complications. Two types of treatment were given: orthopedic or surgical (several types). The functional outcome was assessed at last follow-up.Mean age at fracture of the anterior tibial tubercle was 13 years (range 12-16) in 14 boys and 7 girls. It occurred after a jump or reception after a jump in 22 of the children. Five patients presented symptomatic homo- or contralateral anterior tibial apophysitis before the accident. The fracture was not displaced (type IA) in ten patients and was treated orthopedically. In twelve patients, the fracture was displaced (type IB, II, or III) and required osteosynthesis. Immobilization was maintained for four weeks on average (range 3-7). Associated lesions were observed in half of the twelve displaced fractures, with five cases of patellar ligament avulsion and three cases of meniscal injury. There were no complications with type IA fractures. For the twelve displaced fractures, complications were observed in seven children: hematoma (n = 2), infrapatellar hypoesthesia (n = 2), complete rupture of the patellar ligament 38 months after surgery (n = 1), recurvatum with leg length discrepancy (n = 1), and stiff knee (n = 1). Mean follow-up was two years (range 9 months-8 years). The functional outcome was excellent in all patients with a non-displaced fracture and in seven of the twelve patients who underwent surgical treatment.Non-displaced fracture of the anterior tibial tubercle I children (type IA) should be considered as a separate entity responding very well to orthopedic treatment. The other cases of displaced fracture generally require surgery which enables an assessment of often associated lesions. The prognosis of displaced fractures of the anterior tibial tubercle is more sever due to the associated lesions and potential complications. We describe the second case of recurvatum and leg length discrepancy following fracture of the anterior tibial tubercle.Displaced fracture of the anterior tibial tubercle in adolescents is often associated with soft tissue injury (patellar ligament, menisci). Surgery is indispensable and provides good functional results.

Traumatic hip dislocation in childhood is a rare consequence of violent trauma. After reduction, outcome is usually favourable although epiphyseal necrosis can occur. Reduction must be carried out as soon as possible and is achieved easily, although if the labrum is involved, surgery may be required to achieve complete reduction. To analyze a retrospective series of traumatic hip dislocations in children, describing the therapeutic and imaging strategy. A total of 42 patients were studied. Their mean age was 10 years 3 months. All relevant radiographic, CT, MRI and radionuclide bone scan examinations were reviewed. Special attention was paid to associated lesions. In 22 patients the dislocation was caused by low-energy trauma. Road traffic accidents accounted for 17 dislocations. An acetabular fracture was present in six patients and the femoral head was fractured in three. Reduction was easily achieved in 31 patients. In 11 patients the postreduction radiograph and CT showed joint space asymmetry secondary to labral entrapment. Only two patients developed epiphyseal necrosis. It has been difficult to define and evaluate accurate principles for a medical imaging strategy in this group of patients. Analysis of plain radiographs is essential before and after reduction of the joint, and it is important to perform postreduction CT in every patient whose joint space remains widened. A radionuclide bone scan should be performed between the second and third weeks after injury to assess epiphyseal vascularity. With the use of specific sequences, MRI may be an alternative modality to assess epiphyseal vitality.

Aneurysmal cyst is an uncommon benign primitive bone tumor generally observed in young subjects, in a spinal localization in 10% of the cases. We report the clinical, radiological, and therapeutic aspects of seven cases of aneurysmal cyst of the spine.This series included seven children, mean age 11.7 years (range 8-14) who were followed for 9 years on the average (range 2-24 years). Findings were compared with reports in the literature.Four cysts were located in the cervical spine (C1, C3, C4, C7), two in the thoracic spine (T4, T7) and one in the sacrum. Biopsy-resection was performed in six patients. The sacral cyst was treated by selective arterial embolization. Two complications were observed after surgical treatment. Bony filling of the lesion, evaluated by CT scan six months after surgery, was achieved in five cases, incomplete in two without recurrence of cystic cavities.Aneurysmal bone cysts account for 15% of all primitive tumors of the spine. They are more frequent in the cervical and thoracic spine than in a lumbar or sacral localization. One vertebra is involved in most cases, generally the vertebral body and the posterior arch with an asymmetrical extension to one pedicle in some cases. Neurological complications of variable severity are frequent and can be improved by surgical removal of the tumor. CT scan and MRI are the best exams to analyze the spinal cyst, to show bony walls and liquid-liquid levels suggestive of aneurysmal bone cyst, to evaluate the tumor extension and to search for cord compression. When the CT scan and MRI show characteristic signs of aneurysmal bone cyst of the spine, we do not perform a biopsy systematically if complete removal of the tumor appears possible. Surgical removal with biopsy is the treatment of choice. Reconstruction and stabilization of the spine must be complete using bone graft with or without instrumentation. In case of medullary involvement, decompression must be performed rapidly with the most complete tumor removal as possible. Selective arterial embolization can be used in some localizations or as preparation to surgery. Radiotherapy is contraindicated in children because of the risk of radio-induced tumors.