Clinical management of adolescents and young adults suffering from sarcoma in the French Rhône-Alpes region: A prospective exhaustive cohort with 10 years follow up.

INTRODUCTION: Survival of adolescents and young adults (AYA) with sarcoma is lower than in younger patients. The objective of this study was to describe the regional healthcare circuits, the differences in the management between adult, paediatric and mixed units and to assess the prognostic impact of compliance with clinical practice guidelines (CPGs) on overall survival (OS) and on relapse free survival (RFS). MATERIALS AND METHODS: Retrospective analysis of the management and long term follow-up of all 13-25 year old patients with a sarcoma diagnosed in the Rhône-Alpes area between 2000 and 2005. RESULTS: 140 patients satisfied inclusion criteria and were selected. The majority of 13-25 year old patients were treated in paediatric units. Joint management resulted in a higher rate of discussion in multidisciplinary tumour board, inclusion in clinical trials, and fertility preservation. Non-compliance with guidelines was observed in 65% of cases. Overall compliance was not reported to correlate to survival. Compliance of radiotherapy with CPG's seemed associated with a better prognosis for OS (HR = 0.20, 95% CI = [0.10-0.40]; p < 0.0001) and RFS (HR = 0.18, 95% CI = [0.09-0.37; p < 0.0001) as well as compliance of surgery for OS (HR = 0.43, 95% CI = [0.23-0.81]; p = 0.01). Multivariate Cox regression analysis revealed other independent predictors of OS like age at diagnosis, stage and histological subtype. CONCLUSIONS: Management of AYA in joint units seems to improve the quality of care. Compliance of surgery and radiotherapy with CGP's seems to improve survival.

Voluntary femoro-tibial subluxations: a benign differential diagnosis in the snapping knee of a child.

PURPOSE: Voluntary femoro-tibial subluxation is a rare entity predominantly found in pretoddlers. It presents as a dynamic phenomenon with uni- or bilateral audible snapping of the knee, often in a context of fatigue or irritation at the end of the day. The aim of the study was to observe the evolution and recovery in these patients. METHODS: Ten children were included. Other causes of dislocating joints and pathologies with snapping of the lateral meniscus were excluded from this study. Six-week immobilisation with a splint at 70° of knee flexion was primarily recommended to all patients. RESULTS: The mean age at onset of symptoms was 10 months. Forty percent of the patients presented with bilateral symptoms. Clinically, four patients were hyperlax. In all patients, subluxations could be reproduced passively by rotating the foot externally and advancing the internal tibial plateau anteriorly. Three of the patients were treated with a dorsal splint and experienced significantly less or cessation of symptoms. Two patients underwent surgery, one for a concomitant anterior cruciate ligament (ACL) rupture and meniscus tear that worsened the symptoms, another for concomitant patella dislocation and a meniscus tear. One patient's parents refused treatment and four patients experienced less symptoms at the time of consultation and were not immobilized. Except for the two patients undergoing surgeries, no sequelae were observed. In general, the symptoms got less frequent when the patient began to walk. CONCLUSION: Voluntary femoro-tibial subluxation in children is a rare and benign condition that often resolves spontaneously without sequelae. The risk of meniscus tear should, however, be considered if subluxations do not cease. LEVEL OF EVIDENCE: IV.

Vascularised fibula or induced membrane to treat congenital pseudarthrosis of the Tibia: A multicentre study of 18 patients with a mean 9.5-year follow-up.

BACKGROUND: The objective of this study was to compare outcomes of two surgical techniques used to treat congenital pseudarthrosis of the tibia (CPT), the induced membrane technique (IM) and the transfer of the contralateral vascularised fibula (VF). HYPOTHESIS: The IM technique produces similar outcomes to those of VF grafting in terms of healing and function, while being simpler and having a lower complication rate. MATERIAL AND METHOD: This retrospective multicentre study included 18 patients with a mean age of 2.8 years at surgery. Among them, 11 had neurofibromatosis type 1 (NF1). The IM technique was used in 10 patients and VF grafting in 8 patients. Mean follow-up was 9.5 years (range: 5-15 years). RESULTS: The two groups showed no significant differences for healing or the occurrence of complications such as limb length discrepancy and residual malalignment. Two patients required amputation, one in each group. The mean number of surgical procedures per patient was 4.7 in the IM group and 5 in the VF group. DISCUSSION: Outcomes are similar with the two techniques. Although VF grafting theoretically involves a single stage, the mean number of surgical procedures was not lower than after the IM technique. The IM technique was associated with lower risks of complications and residual donor site abnormalities. Regardless of the reconstruction technique, the quality of the initial bone resection and internal fixation, particularly regarding alignment, is of the utmost importance. LEVEL OF EVIDENCE: IV, comparative retrospective study.

Diagnostic value of ultrasonography in elbow trauma in children: Prospective study of 34 cases.

INTRODUCTION: Among the various elbow injuries in children that initially have normal radiographs, a certain number of occult fractures are only diagnosed correctly after the fact, during a follow-up visit. PURPOSE: This study evaluated the diagnostic contribution of ultrasonography in the treatment of acute elbow injuries in children and the strategic and economic impact of using this tool alongside radiography. MATERIALS AND METHODS: During this prospective study performed between January 1 and April 1 2014, elbow ultrasonography was performed within 6 days in all children under 15 years of age with a suspected occult fracture. The ultrasonography exam looked for lipohemarthrosis, the posterior fat pad sign and cortical disruption. If no fracture was visible on ultrasonography, a removable splint was given to the patient to relieve pain, and no radiological or clinical follow-up was scheduled. The patients were contacted again at least 15 days later to determine whether an undetected fracture was present. Lastly, we evaluated the cost of treatment with and without ultrasonography in the cases where no fracture was diagnosed. RESULTS: In 13 cases, ultrasonography revealed lipohemarthrosis and a fat pat sign, with cortical disruption also present in 11 of these cases. In two cases, the diagnosis was made based solely on the presence of lipohemarthrosis and a fat pat sign. There were seven lateral condyle fractures, two medial epicondyle fractures and two supracondylar fractures. Among the 21 patients with normal ultrasonography, no fracture was diagnosed later on. In patients without a fracture, using ultrasonography resulted in a cost savings of €29.10 per patient versus not using it. CONCLUSION: In our study, ultrasonography is a sensitive examination for the diagnosis of occult elbow fractures in children. When the radiography and ultrasonography are both normal, the possibility of fracture can be rule out definitively, which reduces the need for immobilization, follow-up and treatment costs. The findings of this preliminary study should be validated with a larger prospective study.

Osteoid osteoma of the proximal femur: treatment by percutaneous bone resection and drilling (PBRD). A report of 44 cases.

INTRODUCTION: Osteoid osteoma is a benign osteogenic tumor that is mainly located in the lower limbs. According to Campanacci the proximal femur is involved in 25% of cases. We present a series of 44 cases of osteoid osteoma located in the neck of the femur or the lesser trochanter treated by the minimally invasive method, CT-guided percutaneous bone resection and drilling (PBRD). MATERIALS AND METHODS: This series included 44 patients, 20 girls and 24 boys, treated between 1987 and 2012. The average age at surgery was 12.7 years old (range 4-34). The diagnosis was based on the "association" of scintigraphy (hyperfixation) - CT scan (nidus located on the femoral neck or near the lesser trochanter). These patients underwent CT-guided PBRD under general anesthesia. Specific ancillary material was used to reach and remove the nidus and a cylinder of bone was sent to the pathologist for assessment. A lateral or anterior approach was used in all cases except one in which a posterior incision was made. Histological confirmation was obtained in 23 cases (the bone fragment was damaged in 21 cases). RESULTS: Forty-two patients were reviewed after a minimum follow-up of one year (12-56 months). Two patients were lost to follow-up. Results were evaluated clinically and on CT scan 1 year after surgery: there were 35 cures with complete and permanent pain relief. There were 5 failures and 1 case of recurrence requiring a second CT-guided PBRD procedure as well 2 complications involving femoral fracture (one associated with failure). DISCUSSION: The proximal femur is a common location of osteoid osteoma. Treatment requires careful preoperative planning to determine the surgical approach for safe removal. PBRD is a minimally invasive technique, allowing complete resection with suitable ancillary equipment. This method should be compared with thermoablation, which is a similar technique. CONCLUSION: CT-guided PBRD is a therapeutic option in case of osteoid osteoma of the proximal femur. LEVEL OF EVIDENCE: Level IV.

Patellar instability in children and adolescents.

Paediatric patellar instability encompasses many anatomic entities located along a continuum of knee extensor mechanism abnormalities. Major or minor clinical manifestations may occur at a variable age. In major forms with irreducible patellar dislocation or habitual patellar dislocation during knee flexion, shortness of the quadriceps is a consistent feature. A comprehensive aetiological work-up is in order, as syndromic conditions are common. Early surgical treatment is mandatory and should be performed by an experienced paediatric orthopaedic surgeon, as the procedure is technically challenging. Minor forms are more common; they are characterised by patellar dislocation or subluxation near terminal knee extension. The diagnosis may be difficult, particularly at the acute phase. Surgery is needed in patients with recurrent dislocation or functional impairments. The semiology of patellar instability has undergone considerable development in recent years, and a three-dimensional evaluation of patellar position can now be obtained using magnetic resonance imaging. Individually tailored surgical treatment "à la carte" remains a valid approach in 2013. However, new techniques for medial patello-femoral ligament reconstruction have modified the management strategies for adults and superseded many stabilisation procedures. Adapting these new techniques to paediatric patients and developing new procedures constitute major challenges.

The introduction of a new MRI index to evaluate sagittal patellofemoral engagement.

INTRODUCTION: Patella alta is one of the primary factors of patellofemoral instability and its importance lies in the reduced engagement between patella and trochlea during the early degrees of flexion. The evaluation of patellar height is based on conventional x-rays, CT scan and, more recently, MRI. The objective of this multicentric prospective study is to describe a novel index to assess in the sagittal plane the functional engagement between patella and trochlea. MATERIALS AND METHODS: One hundred and thirty-five patients with objective patellar dislocation were prospectively enrolled between April 2010 and September 2011 and were compared with a second group of 45 controls. All patients underwent a standard MRI and a complete radiographic study. Sagittal engagement was measured as the ratio between the articular cartilage of the patella and the trochlear cartilage length measured on two different MRI slices. RESULTS: The mean Sagittal Patellofemoral Engagement (SPE) index was 0.43 ± 0.18 and ranged from 0.02 to 0.913 in the Objective Patellar Dislocation group versus 0.42 ± 0.11 range 0.22 to 0.55 in controls. In the Patellar Dislocation group the mean Caton-Deschamps index was 1.18 ± 0.21 (range 0.71 to 1.91). There were 58 patients with patella alta, in whom the mean SPE was 0.39 ± 0.18 (range 0.02 to 0.87). Sagittal engagement was significantly higher when compared with patients in the Patellar Dislocation group who had no patella alta (mean 0.46 ± 0.16, range 0.1-0.913). DISCUSSION: The present study introduces a new method to measure the SPE with the use of MRI. The evaluation of the functional engagement of the patella with the femoral trochlea in the sagittal plane can serve as a supplementary tool to the existing methods of evaluating patellar height, and may help to better identify the cases where inadequate engagement is recorded despite the absence of patella alta, so that the need for tibial tuberosity osteotomy may be re-assessed.

Axial MRI index of patellar engagement: a new method to assess patellar instability.

INTRODUCTION: The aim of this study was to define a new index to measure lateral patellar displacement (LPD) using nuclear magnetic resonance imaging (MRI), an axial index of engagement of the patella (AEI) obtained from two different axial MRI views then to validate its use in a prospective series of patients presenting an objective patellar instability (OPI). MATERIALS AND METHODS: One hundred and thirty-five patients with OPI and no history of surgery of the patella were included in a prospective study organized by the French Society of Arthroscopy performed between June 2010 and August 2012. All patients underwent axial and sagittal MRI. The AEI was obtained by projecting predefined patellar and trochlear landmarks (cartilaginous landmarks) on 2 different axial MRI views (one trochlear and one patellar). The results were compared with a series of controls (n=45). RESULTS: The preoperative AEI of the patella was 0.94 ± 0.09 for the control group and 0.84 ± 0.16 for OPI group (P=0.000016). The AEI could be obtained in 100% of the cases if it was measured on 2 MRI views while it could not be measured in 38.5% of the cases if the measurement was only obtained from one MRI view or whenever the widest part of the patella was not across from the femoral trochlea. The AEI did not significantly depend on dysplasia or the presence of a supratrochlear spur. The lowest AIE values were associated with trochlear dysplasia with a supratrochlear spur (P=0.0023) and a more prominent trochlea (P=0.0016). The AEI was correlated with patellar tilt (P<0.000001) and TT-TG on MRI (P<0.000001). DISCUSSION: AEI is a new index to measure LPD. It can be obtained in all cases because it is obtained from two different MRI views. The normal value is close to 1. It can be used to measure patellar instability on the axial plane in patients with OPI, especially in the most severe cases.

Induced-membrane femur reconstruction after resection of bone malignancies: three cases of massive graft resorption in children.

Bone reconstruction after surgical resection of bone malignancies in children remains a difficult challenge. Induced-membrane reconstruction as described by Masquelet et al. was originally reported in traumatic or septic bone defects and is now adapted to this field. We report here three cases of massive femoral graft resorption requiring surgical revision in two boys aged 3 and 6 years and a 9-year-old girl. Hypotheses include the long delay between the two stages, nature of the bone graft, high varus loads specific to this location, and lack of stability of the fixation. This technique has recently provided promising preliminary results when applied to the field of bone tumours. However, reconstruction of the femur seems to be specifically associated with a risk of graft resorption. Identification of the origin of this major complication is needed to amend the technique or its indications.

Osteoid osteoma of the acetabular fossa: five cases treated with percutaneous resection.

BACKGROUND: Osteoid osteoma is a painful benign osteogenic tumour for which the treatment objective is surgical resection of the nidus. The acetabular fossa is an uncommon site of involvement where surgical access can prove challenging. MATERIALS AND METHODS: We report a case-series composed of five patients with osteoid osteoma of the acetabular fossa treated with percutaneous bone resection and drilling under computed tomography guidance. RESULTS: All five patients had an uneventful postoperative course with immediate pain relief that was sustained over time. DISCUSSION: The outcomes achieved using our percutaneous technique compare favourably with those of other percutaneous methods, most notably regarding pain relief and patient tolerance of the procedure. CONCLUSION: Percutaneous bone resection and drilling under computed tomography guidance proved effective for the treatment of osteoid osteoma involving the acetabular fossa. LEVEL OF EVIDENCE: Level IV.

Osteoid osteoma transformation into osteoblastoma: fact or fiction?

BACKGROUND: Osteoid osteoma and osteoblastoma are rare, benign, bone-forming tumours. The clinical presentation, imaging study findings, and course indicate clearly that these two tumours are distinct entities. CLINICAL REPORTS: We report two cases suggesting transformation of osteoid osteoma into osteoblastoma and therefore inviting a discussion of the links between these two tumours. An 11-year-old girl with a small metaphyseal lesion of the proximal tibia was given a diagnosis of osteoid osteoma. Over the next few weeks, worsening pain and marked tumour growth prompted a biopsy, which was consistent with an aggressive osteoblastoma. A review of the case suggested primary osteoblastoma at the earliest stage of development. In a 14-year-old boy, en-bloc excision was performed to remove a 1cm defect located within the femoral shaft cortex and typical for osteoid osteoma. An asymptomatic recurrence measuring 20mm along the long axis was removed 18 months later. Reassessment of the histological slides indicated recurrence of an incompletely excised osteoid osteoma. DISCUSSION: The histological similarities between osteoid osteoma and osteoblastoma, together with the lesion size criterion, may result in confusion. Collaboration between the clinician and pathologist is crucial and should take the tempo of evolution into account. CONCLUSION: The histopathological differences between these two tumour types deserve to be emphasized. The data reported here challenge the concept that osteoid osteoma can transform into osteoblastoma. These two tumours are distinct entities that should no longer be differentiated based on size, as was long done in the past.

Early surgical anterior release for congenital and isolated elbow contracture in flexion: a case report of a 16-month-old child.

Isolated congenital elbow contracture is a rare upper-extremity disorder and there are few data about management of this condition. Authors report their experience after aggressive management of children with isolated congenital elbow contracture in flexion. Because of total absence of range of motion (ROM) improvement despites physical therapy (ROM 90-120°) and bone deformity, an anterior surgical release of the elbow was performed through an extensive lateral approach, at sixteen months of age. After surgery, this child was treated by three casts at maximal gained extension followed by sequential Turnbuckles splints. After five years of follow-up, the result was excellent with ROM 5-135°, normal function and absence of growth disturbance. The limiting factor of this protocol was excessive traction in elbow extension on the neurovascular structures, especially the radial nerve. This treatment represents an aggressive management with multiple general anaesthesia, but was found to be a valid option.

Induced membrane technique for reconstruction after bone tumor resection in children: a preliminary study.

AIM: Segmental long-bone defect due to tumor resection remains a challenge to treat. The induced membrane technique is a new alternative for biological reconstruction. During the first stage, a cement spacer is inserted after bone resection and stabilisation. The cement spacer is removed during a second stage procedure performed after chemotherapy, and cortico-cancellous bone autograft was placed in the biological induced chamber. The aim of this study was to assess preliminary results in eight children. PATIENTS AND METHODS: This prospective study included six girls and two boys, with a mean age of 12.1 years (range 9.5 to 18) and treated for a mean 15 cm defect (range 10 to 22 cms) due to resection of osteosarcoma (n=4), Ewing sarcoma (n=3) and low grade sarcoma. All patients except one, were given pre- and postoperative chemotherapy. Surgery was performed for three patients with a distal femur tumor, two patients with a proximal tibial tumor and three patients who had proximal humerus, shaft of humerus and fibular tumors. Fixation was mainly performed with locking compression plate (n=4) and locked nail (n=2). The mean operating times for first and second step procedures were 4.8 and 4h respectively. The healing process was radiologically assessed. RESULTS: After a mean follow-up of 21.6 months (15 to 30), all patients were free of disease and seven had bony union. For the lower limb reconstructions, full weight bearing was possible after a mean of 116 days (range 90 to 150) following the second stage. Mean time to bone union was 4.8 months (1.5 to 10). The early Musculoskeletal Tumor Society (MSTS) score was 25.2/30 (range 20-30). Complications were: non-union (n=1), paradoxical graft resorption (n=1) requiring graft revision. CONCLUSION: This two stage procedure reduces the operating time during the first stage and it also reduces early complications. Rapid bone union is objectively obtained despite major bone resection and the patients receiving chemotherapy. SIGNIFICANCE: The induced membrane technique could be an excellent alternative for biological reconstruction after tumor resection in children.

Knee osteochondral fractures in skeletally immature patients: French multicenter study.

INTRODUCTION: Femoral or patellar osteochondral fractures complicating patellar dislocation are more frequently observed in adolescents than in adults. These incidental lesions of vulnerable joint cartilage are often neglected in the initial phase, which is regrettable given their good capacity for healing after repositioning. The objective of this study was to investigate the characteristics and analyze the results of repositioning these fractures in skeletally immature patients. MATERIAL AND METHODS: This retrospective multicenter study grouped 14 patients and knees (seven females). The mean age at the time of the accident was 12.9 years (range, 11.2-14.9 years) for the girls and 14 years (range, 12.2-15 years) for the boys. These fractures involved the lateral condyle in nine cases and the patella in five cases. The injury mechanism was secondary to demonstrated patellar dislocation (n=9) or a direct impact (n=4). In nine cases out of 14, a leisure sports accident was the cause. The injury was treated a mean 5.2 days (range, 0-20 days) after the accident. All of the detached fragments were repositioned surgically with screw fixation (n=5), resorbable pins (n=5), or pull-out suture (n=4). Biological glue was added for six patients. Patellar stabilization was associated during the same procedure in two cases. RESULTS: No postoperative complications were observed. The results at the mean follow-up of 30 months (range, 15-89 months) showed no revision for failure, with all of the fractures demonstrating union at the final examination. The mean IKDC 2000 subjective score was 88±6 (range, 79-98) out of 100 points. The subjective satisfaction level was very satisfied in two cases and satisfied in the 12 others. The final IKDC score was A for eight patients, B for five patients, and C for one patient. Three patients underwent secondary patellar stabilization surgery. CONCLUSION: Better knowledge of this fracture and attentive reading of the radiographic images of a knee with hemarthrosis should result in more frequent diagnosis of this condition and adapted treatment. Unexplained hemarthrosis in a context of trochlear dysplasia should be considered to be associated with an OCF until proof of the contrary. A fragment released in a weightbearing zone should ideally be repositioned within 10 days but remains possible at 2 months. It regularly provides bone union and good results in children.

[PNET/Ewing tumours: current treatments and future perspectives]. / Tumeurs PNET/Ewing : prise en charge actuelle et perspectives.

Ewing tumours are characterised as tumours consisting of small, blue, round malignant cells that may exhibit varying degrees of neural differentiation. Most of them arise in bony sites, and they represent the second commonest primary osseous malignancy in and adolescence and young adults. During the past 30 years, chemotherapy has increased survival from less than 5% to 65-70% in localized tumours and to 25-30% in primary metastatic tumours. Surgery is a major tool, whereas advances in imaging techniques have improved treatment indication and optimization. Radiotherapy remains useful, either alone or in addition to surgery, and new techniques (conformational RT and IMRT) will reduce short-term toxic effects. However, long-term toxic effects are also of major concern. Clinical and biological prognostic factors has been clearly identified and should guide the therapeutic choice for these patients. The metastatic Ewing tumours are of extremely poor prognosis, and impose the development of new therapeutic agents. This article is a review of the data available in 2009 concerning Ewing's sarcoma either as biologic aspects or as therapeutic aspects.

Recurrent Legg-Perthes-Calvé disease.

INTRODUCTION: Legg-Perthes-Calvé disease (LPC), or primitive hip osteochondritis, is a frequent pathology but in which recurrence is extremely rare. WE REPORT: The case of a girl diagnosed with bilateral LPC at the age of 4.5 years. Containment in a Scottish-Rite (Atlanta) brace was prescribed. X-ray follow-up found complete right-hip healing at 1.5 years' evolution (Catterall group 2). The patient was seen again at the age of 8 years, for limp and functional disorder associated with recurrence of right-hip LPC (Catterall group 3). Evolution was satisfactory: the girl was assessed at the time of bone maturity, with good clinical and radiological findings. DISCUSSION: Children contracting LPC are commonly thought to be thereby "vaccinated" against it. This is wrong, and a literature search found 10 cases similar to the present one, making 11 children in all (two girls, nine boys) presenting with recurrent LPC. Mean age at initial onset was 4 years (range, 2.5-6 yrs). Five of the 11 had initially been presented with bilateral LPC. Mean age at recurrence was 9.4 years (range, 6-12 yrs). The recurrences were more severe than the initial episodes, but final prognosis after recurrence would not seem to be worse than normal. CONCLUSION: This exceptional case of recurrent LPC was well documented up to bone maturity. It does not support the notion of Meyer's disease at the initial episode, as suggested by certain authors, but rather that of true recurrence of the primitive LPC.

Variability in the presentation of synovial sarcoma in children: a plea for greater awareness.

We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.