Search

Resume Introduction Le choix de la technique chirurgicale des spondylolisthesis de haut grade (SPLHG) associe a une cyphose lombo-sacree reste controverse. Malgre la multiplicite et la modernite des ancillaires, les techniques non instrumentees sont-elles toujours d’actualite ? Hypothese Notre hypothese etait que l’arthrodese circonferentielle non instrumentee apres une periode de reduction progressive dans les SPLHG, associee a une cyphose lombo-sacree, de l’enfant et de l’adolescent permet d’obtenir des resultats fonctionnels et radiographiques satisfaisants a long terme tout en minimisant le risque de complication. Materiel et methodes Trente-et-un SPLHG L5-S1 associes a une cyphose lombo-sacree operes par une arthrodese circonferentielle non instrumentee apres une periode de traction-suspension ont ete inclus. La technique comportait un temps de reduction progressive par traction puis immobilisation en position de correction, un second temps chirurgicale posterieur puis anterieur et une periode d’immobilisation plâtree de 4 mois. L’âge moyen de la chirurgie etait de 13,9 ± 2,3 ans (6–18) et le suivi moyen de 10,3 ± 4,5 ans (2,1–17,8). Resultats Le taux de complications etait de 26 % (n = 8/31) soit 13 % de complications neurologiques, 10 % de defauts de fusion osseuse et 3 % de complications cutanees. Le taux de re-operation etait de 13 % (n = 4/31). L’ODI moyen (/50) etait de 3 ± 4,6 (0–22) et le SRS-30 de 126,7 ± 15 (72–143). L’index de glissement de Taillard a diminue de 25 % (p Conclusion Cette technique induit une moindre reduction de l’angle lombo-sacre mais reduit au minimum d’un facteur trois le taux de complications neurologiques et conduit a des resultats fonctionnels satisfaisants par rapport aux series instrumentees et correction extemporanee. Niveaux de preuve IV.

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

La epifisiolisis femoral proximal (EFP) consiste en un deslizamiento de la epifisis femoral proximal respecto al cuello femoral. La zona de crecimiento es la localizacion de este deslizamiento, cuyo desplazamiento se produce en la mayoria de los casos en sentido posteromedial, por el efecto del peso del cuerpo. Esta afeccion de produce la mayoria de las veces durante la pubertad. Se han identificado varios factores de riesgo epidemiologicos y metabolicos, a la cabeza de los cuales se situa el sobrepeso. Hay que insistir en el retraso frecuente del diagnostico en las formas cronicas. La evolucion espontanea esta dominada por el aumento del deslizamiento y el riesgo de aparicion de un deslizamiento «agudo», que provocan complicaciones precoces (condrolisis, osteonecrosis, etc.) y a largo plazo (conflicto femoroacetabular, artrosis secundaria. El tratamiento tiene como finalidad detener la progresion del deslizamiento para evitar las complicaciones, cuya frecuencia aumenta con la importancia del desplazamiento. En la actualidad, la clasificacion clinica y radiologica que tiene en cuenta la estabilidad de la epifisis es la mas utilizada para guiar las indicaciones terapeuticas y parece la que mejor correlaciona con el pronostico. En las formas poco desplazadas y estables, la fijacion in situ mediante un tornillo canulado es el metodo de eleccion y proporciona buenos resultados en la mayoria de los pacientes. En las formas inestables o con gran desplazamiento, que plantean mas problemas, las diversas opciones quirurgicas y el momento en el que realizar la intervencion son motivo de controversia. Un diagnostico precoz es el mejor factor pronostico en esta afeccion.

Resume Introduction Les resultats a long terme des arthrodeses rachidiennes anterieures dans le traitement des scolioses idiopathiques de l’adolescent (SIA) sont peu documentes dans la litterature. Cette etude retrospective a pour objectif d’evaluer les resultats cliniques et radiologiques d’une serie monocentrique utilisant une technique d’arthrodese en un temps a l’aide de plaques vissees en titane preformees. Materiel et methodes Cent onze patients ont beneficie de cette intervention entre 1975 et 1993. Tous les patients inclus devaient disposer d’une evaluation clinique et radiologique complete au recul minimum de 15 ans. Le questionnaire SRS-30 et le score d’Oswestry ont ete utilises pour l’evaluation des resultats fonctionnels. Les grands cliches de face et de profil preoperatoires, postoperatoires et au dernier recul ont ete utilises pour l’evaluation radiographique. Resultats Trente-cinq patients ont ete revus a un recul moyen de 21 ans (15–31). L’angle de Cobb moyen preoperatoire etait de 44°. Les patients ont ete operes a un âge moyen de 14,7 ans. Le score SRS-30 moyen etait de 3,65/5 et le score d’Oswestry de 14,9 %. Au dernier recul, l’angle de Cobb moyen etait de 14,7°. Vingt-cinq patients presentaient un desequilibre frontal, en moyenne de 12 mm. Dans le plan sagittal, la « C7 plumbline » moyenne au dernier recul etait de −28 mm avec une translation anterieure de 8 mm par rapport a la valeur postoperatoire. On a retrouve une correlation statistiquement significative entre les resultats fonctionnels (SRS-30) et deux parametres : version pelvienne et translation anterieure de la « C7 plumbline ». Conclusion L’arthrodese anterieure donne de bons resultats fonctionnels a long terme dans le traitement des SIA. Cette technique permet l’obtention d’une correction satisfaisante et perenne. La reequilibration du rachis en avant semble etre correlee a de meilleurs resultats fonctionnels. Niveau de preuve IV. Etude retrospective.

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.

Las fracturas de la columna vertebral son infrecuentes en el nino (1-10% de las lesiones traumaticas segun los autores). Difieren de las fracturas del adulto por sus causas, localizaciones, anatomia patologica de las lesiones observadas, su potencial evolutivo y su tratamiento. Afectan a un esqueleto en crecimiento que consta de zonas cartilaginosas vertebrales aun no osificadas, que son zonas de debilidad en los ninos y, en ocasiones, dificiles de evaluar en las radiografias. Estas particularidades desaparecen a medida que se produce la maduracion esqueletica, hasta la adolescencia, donde las lesiones son superponibles a las del adulto. Se debe conocer la semiologia radiologica de estas lesiones, sobre todo la de las lesiones de la columna cervical superior. Para completar la informacion de las radiografias, las exploraciones complementarias como la resonancia magnetica (RM) o la tomografia computarizada (TC) estan indicadas si existen fracturas o lesiones neurologicas asociadas. Estas exploraciones son las mas eficaces para establecer un estudio preciso de las lesiones oseas, cartilaginosas y del contenido raquideo. El tratamiento ortopedico es la regla en los ninos en caso de lesion osea o cartilaginosa sin lesion ligamentaria asociada. El tratamiento quirurgico tiene indicaciones indudables y se debe limitar a las lesiones para evitar los trastornos del crecimiento secundarios al tratamiento quirurgico (laminectomia, desperiostizacion, etc.). El seguimiento prolongado de estas lesiones es indispensable, debido al riesgo de trastornos del crecimiento a largo plazo. Las deformaciones postraumaticas se observan en la mayoria de los casos cuando la gravedad de la lesion inicial se ha pasado por alto o cuando el tratamiento ortopedico o quirurgico ha sido inadecuado. Por lo tanto, la prevencion de estas deformaciones requiere el conocimiento de los elementos anatomicos responsables de la estabilidad y del crecimiento raquideos.

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

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

Background/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 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.

Resume Le but de cette etude est de rapporter les cas de synovites villonodulaires pigmentees de l’enfant pris en charge a l’hopital des Enfants-Malades, d’etudier leur specificite afin de proposer une conduite therapeutique adaptee permettant de prevenir le risque de recidive tout en preservant le potentiel de croissance de l’enfant. Cette etude retrospective portait sur six patients, avec un âge moyen de 11,5 ans au moment du diagnostic. Le genou etait atteint dans cinq cas (trois formes diffuses et deux localisees) et la cheville dans un cas, de forme diffuse. Une hemarthrose etait retrouvee dans toutes les localisations au genou. Le diagnostic a ete suspecte dans tous les cas a l’imagerie par resonance magnetique (IRM) et confirme par la biopsie. Le delai entre le debut des symptomes et le diagnostic histologique a ete de huit mois. Le traitement a ete chirurgical dans cinq cas et medical dans une forme diffuse du genou. Une synoviorthese a l’Hexatrione® a ete associee de facon systematique dans trois formes diffuses du genou. Dans quatre cas, les resultats ont ete satisfaisants avec une absence de recidive clinique ou sur les IRM de controle. Deux formes diffuses ont recidive, l’une au genou a dix mois de la synovectomie arthroscopique et l’autre a la cheville a 24 mois de la synovectomie chirurgicale necessitant des reprises chirurgicales iteratives.

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).

Resume Les fractures de Tillaux chez l’adolescent sont des fractures de type III de Salter et Harris emportant la partie anterolaterale de l’epiphyse tibiale. Ce sont des fractures articulaires. Les auteurs ont revu une serie de 19 fractures de Tillaux dont 10 ont eu un traitement chirurgical et 9 ont eu un traitement orthopedique. Le but etait de determiner et de preciser les circonstances de survenue, la semiologie radiologique, l’attitude therapeutique et le devenir a long terme sur le plan clinique et radiologique. Cette fracture survient le plus souvent chez l’adolescent lors d’un traumatisme du pied en rotation externe. La fermeture du cartilage de croissance distal du tibia se fait de dedans en dehors et le secteur antero-lateral reste plus longtemps ouvert. Lors d’un mouvement force en rotation externe le ligament tibio-fibulaire anterieur va arracher un fragment antero-lateral de l’epiphyse tibiale distale. Le traitement a ete chirurgical pour les fractures dont le deplacement etait superieur a 2 mm ou de deplacement vertical et a consiste en une reduction a foyer ouvert avec une osteosynthese par vis. Le traitement a ete orthopedique pour les fractures non deplacees. Les resultats sont bons avec un recul de 33,8 mois comme cela a ete rapporte dans la litterature.

Purpose of the study 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. Material and method 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. Results 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. Discussion 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. Conclusion 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.

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.

Resume Cette etude retrospective analyse le traitement de 10 arthrites septiques de hanche chez les patients drepanocytaires par prothese totale de hanche. La destruction de la hanche s’est produite malgre le traitement medical et la mise au repos de l’articulation par traction suspension. Le traitement a consiste en une resection tete et col suivie d’une traction suspension pendant 45 jours et la pose d’une prothese totale de hanche. Le recul actuel est compris entre 2 et 12 ans. Au recul moyen de 6 ans, deux recidives infectieuses ont ete observees sur les protheses implantees. L’arthroplastie a permis a ces patients de retrouver une activite fonctionnelle satisfaisante, alors qu’en preoperatoire ils etaient impotents du fait de la hanche touchee par l’arthrite infectieuse, et d’autant plus que leur hanche controlaterale etait porteuse d’une osteonecrose.

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.

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

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

Introduction Les infections osteo-articulaires pediatriques (IOAP) en France, bien que rares (22/106 en 2008), peuvent entrainer troubles de croissance et sequelles articulaires. Des etudes recentes suggerent l’efficacite d’un traitement intraveineux court (2–5 jours), autorisant une sortie d’autant plus rapide qu’elle a lieu majoritairement au domicile (93 % en 2008). L’objectif etait d’etudier les facteurs associes aux hospitalisations longues (> 5 j) pour les IOAP, a partir de la base PMSI 2013. Methodes Les sejours d’enfants ( 5 j, par regression logistique. Resultats Une IOAP etait retrouvee chez 2717 patients. L’incidence nationale etait de 22/106, plus elevee chez les garcons (26/106) et les enfants de 1 an (80/106). Les arthrites representaient 50 % des cas et les osteomyelites 46 %. Un microorganisme etait code pour 28 % des sejours (50 % Staphylococci) et une comorbidite pour 4 % des sejours. La moitie des patients (49 %) etait hospitalisee plus de 5 jours, sans difference en termes de rehospitalisation. Les caracteristiques patient associees a un sejour long etaient : nourrisson ( 5 j (2,2 [1,4-3,4]), alors qu’aucune difference n’etait observee avec les osteomyelites (0,9 [0,8-1,1]). Comparativement au CHRU, une hospitalisation en CH etait associee plus frequemment a un sejour > 5 j (1,6 [1,4-1,9]). Conclusion Cette etude montrait une prevalence inchangee d’IOAP en France. Cependant, la moitie des sejours hospitaliers etaient plus longs qu’attendu au regard des etudes recentes en faveur de durees raccourcies de traitement hospitalier. Un effort d’information doit etre fourni pour ameliorer la connaissance et l’utilisation des traitements courts.

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.

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

In the last years, much progress has been achieved in the treatment of lysosomal storage disorders. Until recently only symptomatic treatment was available for the affected patients. Progressively enzyme replacement treatments have been developed for several diseases, namely Gaucher disease, Fabry disease, mucopolysaccharidoses type I, II and VI and Pompe disease. In this review we will summarize the efficacy and safety of these treatments and describe new therapeutic trials for other lysosomal storage disorders or perspectives in the use of currently available treatments.

Scoliosis is a 3D deformity of the spine resulting in aesthetic and functional discomfort, sometimes leading to respiratory insufficiency due to abnormal thoracic cage development (Canavese and Dim...

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.

Scoliosis is the result of progressive deformations with torsion and wedging of vertebrate leading to lateral deviations, thoracic torsions and hypokyphosis. No spontaneus animal model of scoliosis are knowned. Although reported models mimics idiopathic scoliosis deformities, extensive violation of spine and chest wall elements are required; making these models suboptimal for implant testing and pulmonary parameters analysis. The purpose of the study is to create a reliable porcine scoliosis model, class as an infantile or juvenile scoliosis, without chest wall injury.

Lors de la formation de la clavicule, il existe un noyau d'ossification primaire pour chaque extremite, sternale et acromiale de la clavicule. Se developpent ainsi deux ebauches distinctes qui finissent par fusionner .

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.

Septic hip arthritis is a recognized complication of sickle-cell disease. The incidence is difficult to assess but is significant since certain authors estimate that 11% of children with sickle-cell disease develop orthopedic complications. We report our experience with hip joint infection in adults with sickle-cell disease.We diagnosed ten cases of hip joint infection in seven adults with sickle-cell disease. The characteristic feature of the septic arthritis was the development of a septic focus in a zone of osteonecrosis of the femoral head. Diagnosis was difficult due to the presence of prior hip disease and also the circumstances of development: other infectious foci, septicemia, distant osteomyelitis. The diagnosis was confirmed by joint puncture and isolation of the causal germ. Despite adapted antibiotics and immobilization with traction-suspension, hip joint destruction could not be prevented and all patients became bedridden. Surgery was therefore undertaken to remove the head and neck and institute local antibiotic treatment. A total hip prosthesis was implanted in all patients.At 2 to 12 years follow-up, all seven patients had nearly normal hip function (all 10 hips). Recurrent infection nevertheless developed in 2 hips, demonstrating the limitations of this technique.

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.

Introduction Le traitement des spondylolisthesis (SPL) lombo-sacres a grand deplacement reste controverse. L’objectif de cette etude retrospective est de rapporter les resultats a long terme de l’arthrodese circonferentielle non instrumentee sans reduction peroperatoire chez l’adolescent. Materiel et methode Vingt deux SPL de grade III ou IV ont ete revus retrospectivement. Ils ont ete pris en charge de 1993 a 2009 avec une preparation par traction bipolaire et hamac preoperatoire de 7 jours et arthrodese circonferentielle in situ non instrumentee (postero-laterale L4-S1 avec greffe iliaque par voie de Wiltse et anterieure L5–S1 avec greffon tibial encastre par abord retroperitoneal). L’immobilisation etait assuree par un plâtre en hyperlordose. La verticalisation etait reprise au quatrieme mois postoperatoire. Une laminectomie a ete associee dans 4 cas en raison d’une stenose lombaire preoperatoire sur l’IRM. Les patients ont ete evalues radiologiquement avant la chirurgie, en postoperatoire ( Resultats L’âge moyen lors de la chirurgie etait de 13,71 ans. Le suivi moyen radiologique etait de 6,56 ans. Le suivi clinique moyen etait de 11,21 ans. Au dernier recul, l’Oswestry moyen etait de 5, le SRS-30 moyen de 4,26. L’incidence pelvienne moyenne etait de 72,90°. La version pelvienne moyenne preoperatoire, postoperatoire et au dernier recul etait respectivement de 29,14°, 19,56° et 34,24°. La pente sacree etait de 43,75°, 53,80° et 54,43°. La lordose lombaire moyenne etait de 55,91°, 54,33° et 56,42°. La correction moyenne de l’angle lombo-sacre etait de 40° et stable dans le temps. En postoperatoire immediat, une ecchymose d’anse grele, une occlusion et un deficit des extenseurs ont ete constates. Trois retards de fusion dont un sur fracture du greffon tibial, un trouble vesico-sphincterien rapidement resolutif et 3 cas de sciatalgies occasionnelles ont ete retrouves a distance. Une reprise par laminectomie a 14 mois de recul a ete necessaire sur un deficit radiculaire. Discussion et conclusion L’arthrodese circonferentielle in situ permet d’obtenir de bons resultats fonctionnels et une correction acceptable et stable dans le temps des parametres pelviens et rachidiens. Le taux de fusion est tres bon (> 95 %) avec un faible taux de complications, notamment neurologiques.