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Outcomes for adults with soft tissue sarcoma are better when managed at referral centers. Care guidelines advise for 5 main criteria: 1-Imaging before biopsy; 2-Tumor biopsy before surgery; 3-Multidiscipinary team discussion (MTD) before biopsy; 4-Biopsy in "expert centers"; 5-Somatic molecular biology feasible. The aim is to describe and assess the prognostic impact of initial management of STS according to the type of referring centers and the number of optimal criteria.Monocentric retrospective analysis of the management of 127 youths (0-25 years) with localized STS treated from 2006 to 2015.Median age at diagnosis was 9.6 years (range: 025). Overall, only 41% patients had 5/5, 28% 3-4, 31% ≤2. No adequate imaging was performed before surgery/biopsy for 18% patients, no biopsy before treatment for 29%. Patients referred by "expert centers" had higher compliance to guidelines (P = 0.025). Upfront surgery was performed in 59/127 patients. Immediate re-operation was inversely related to the number of criteria (0% when 5 criteria vs. 14% for 3-4, 46% if ≤ 2; P 0.001). For malignant tumors, outcome was better when 5 criteria were reached: 5 year EFS 90.8% (81.4-100.0%) vs. 71.6 for (60.4-84.9%; ≤4 criteria; p = 0.033), OS 93.6% (85.5-100%) vs. 79.5% (68.9-91.8%; p = 0.11), and LRFFS 90.6% (81.0-100.0) vs. 73.1% (62.0-86.3%; p = 0.047).Less than half of the youths with STS are initially managed according to international guidelines, highlighting the need for better information about optimal management. These results plead for immediate management in reference centers to reduce initial burden of therapy.

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

Pseudotumoral soft tissue masses in children and adolescents are a frequent reason for consultation and a diagnostic dilemma. Soft tissue malignancies are relatively uncommon, unlike the large number of benign lesions that may be seen in the superficial tissue and that can be diagnosed with clinical characteristics.This retrospective study concerns 161 children and adolescents less than 20 years old, referred for a soft tissue mass between 2007 and 2011. It describes their epidemiology, clinical characteristics, and course of care to validate a diagnostic strategy for such masses.Final diagnoses were malignant tumors (44%), benign tumors (32%), and pseudotumoral lesions (24%). Clinical features were similar between these three groups except for age and tumor location, with more benign thoracic masses in younger children. Clinical and radiological association led to an accurate diagnosis for 50% of benign masses and with cytological analysis contribution in 79% of benign tumors and 86% of pseudotumoral lesions. Malignant tumors were suspected in only 39% of cases with radiological exams and in 89% after fine-needle aspiration, an essential additional diagnostic tool. Final diagnoses were formally established through simple standard clinical and radiological evaluation in 19 patients (11.8%; benign tumors, seven patients; malformations, eight patients; post-traumatic lesions, two patients; infection and inflammation, one patient each); ultrasound exam in five patients (3.1%; hemangioendotheliomas, two patients, fascial dehiscence, hemangioma, and vascular malformation, one patient each); MRI in four patients (2.5%; three vascular malformations and one lipoma); CT in two cases (1.2%; vascular malformation and myositis ossificans), and radiological examinations associated with cell aspiration in 15 cases (9.3%; ten benign tumors and five malignant tumors).A multidisciplinary approach should be requested from oncological, radiological, and pathologic experts to optimize soft tissue mass management as soon as initial investigations start. The authors advise a diagnostic strategy for children with pseudotumoral soft tissue masses.

This study was concluded on paraplegic and tetraplegic patients of all aetiologies except neoplasic, where paralysis developed within 48 hours. All patients were admitted to the rehabilitation department within 90 days after the onset of paralysis. In a preliminary review of 328 files, there were 27 cases of clinical deep vein thrombosis (DVT) and 10 with pulmonary embolism (PE), 6 of which were fatal. A prospective study was conducted, based on systematic detection of asymptomatic DVT with phlebography. Among the 147 patients, 20 previously presented with DVT. The 127 others underwent phlebography which showed 39 DVT in 29 patients. Eighty seven patients with negative phlebography underwent a second study a month later which showed 14 DVT in 12 patients. Only one minor pulmonary embolism occurred in these 147 patients. The incidence of DVT after acute spinal cord injury and the frequent absence of clinical manifestations were confirmed. Prophylactic anticoagulant therapy is useful but insufficient. This study demonstrates that systematic and repeated detection of DVT by phlebography may reduce the incidence of PE.

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.

Fibrous dysplasia is a rare benign bone tumor which occurs preferentially in the proximal femur. In children, there is a risk of repeated fractures and coxa vara deformity, particularly in the polyostotic form. The most common orthopedic problem is fibrous dysplasia of the proximal femur which generally requires surgical treatment. The purpose of this study was to analyze clinical and radiological outcome after surgical treatment.All children who underwent surgical treatment for fibrous dysplasia of the proximal femur between 1979 and 2001 were reviewed retrospectively. The study cohort included 22 children (11 boys and 11 girls). Eight patients had a monostotic form and 14 a polyostotic form of the disease. For the monostotic forms, the type of treatment depended on the size of the tumor and its localization but curettage was used in all cases. For the polyostotic forms, treatment consisted in valgus osteotomy with "humeralization" in the event of associated coxa vara in combination with internal fixation, generally with a centromedullary nail.In the monostotic forms, the clinical outcome was considered good in all cases. Nearly total involution of the tumor was noted in 75% of patients. In the polyostotic forms, osteotomy with "humeralization" and centromedullary nailing provided stable correction of the deformation. Outcome was less satisfactory because of fractures and deformities.In light of our results and those reported in the literature, the prognosis of the monostotic form can be considered good after surgical treatment. For the polyostotic form, preventive fixation is necessary. Osteotomy with "humeralization" appears to correct the deformity and prevent coxa vara in certain cases. For the more severe forms, medical treatment with biphosphonates may be a useful complement to the surgical treatment.

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.

To describe bacteriologic epidemiology of bone and joint infections, a total of 52 osteomyelitis, 52 arthritis and 20 osteoarthritis of children aged one month to 15 years during a one-year period (2001) were included in a retrospective unicentric review. The mean age was 3,9 +/-3,6 years. Fever and pain were the most common clinical symptoms. The site of infection was single in 95%, involving lower extremities in 80%. Bone scintigraphy was abnormal in 71% of osteomyelitis. Positive cultures was obtained in 29% of all cases (blood cultures: 20%, aspiration cultures: 29%), but in 42% of cases which have both blood and aspiration cultures. Thirty-six bacteria were identified: 19 Staphylococcus (14 aureus), ten Streptococcus (four pneumoniae), three Salmonella, three Kingella kingae, one Moraxella. All the isolates were susceptible to the empiric antibiotic therapy. Outcome was good in 100% of osteomyelitis and in 96% of arthritis.

A prototype 'telethesis', a telemanipulator for high level tetraplegic and similarly disabled persons, has been developed in the French Spartacus project. The system has a modular control structure, both in the choice of transducers and in the microprocessor programmes assuring the ergonomic link with the individual user. A special training procedure has been developed and tested both in the laboratory and in the hospital. Six tetraplegic patients have used the system in the laboratory and seven in hospital. The experience of 6 months of experimentation in the occupational therapy department with the seven patients is reported. The telethesis has been well accepted by four of them, two of whom have used it for prolonged periods of time. One case has not been adapted with great success prior to his departure, and in two others the use of the system has been rejected, largely for psychological reasons.

Among giant tumours of the cauda equina, apart from ependymomas, neurinomas merit a special place. Depending on their site, they give rise to severe lumbar pain or root pain, with, almost constantly, aggravation during the night. X ray suggests the diagnosis when one finds displacement of the bone without inflammation, a regular bony defect, well-limited and asymmetric, and when the tumour is frequently prolonges in several directions across the bony orifices, sometimes giving a very large paravertebral mass which requires a double approach during surgery. The prognosis of isolated neurinomas is excellent, with disappearance of the pain immediately after operation, regression of the neurological signs or, at any rate, stabilisation when the latter are important. As far as the problem of spinal stability is concerned, this has been solved by sufficient spontaneous bony reconstruction.

Arthrogryphosis, a disease of unknown nature, is accompanied by radiological manifestations which involve essentially the limbs and spine. A distribution involving all four limbs is the most common. The commonest radiological abnormalities are equinovarus clubfoot, clubhand in ulnar deviation, scoliosis and dislocation of the hip. The radiologist examines such children in order to assess the extent of their disease and follow such orthopaedic problems as they may develop.

The authors have reviewed 35 cases in the literature and reported on 12 cases seen personally of late onset syringomyelia in paraplegics. Initial pain was found to be present in only slightly more than half the cases and an insidious onset particularly with sensory disturbance led sometimes to delayed recognition. In the fully developed case, it can closely simulate idiopathic syringomyelia with arthropathy but impairment of touch and proprioception sometimes occurs in addition. Neurosurgical treatment to the fluid-filled cavity, does not seem to modify obviously the spontaneous evolution of the condition. In 5 cases, the paraplegia was not of traumatic origin. Possible mechanisms are discussed.

The authors made a detailed clinical analysis of 119 stabilized total paraplegics in order to define, as precisely as possible, the limits of the medullary segment which, for each patient, had lost all its functional value. The relationships between the affected segments and the sexual capabilities of these patients show that the segment must: --exclude the sacral spinal cord if reflex erections are to occur; --underly Th. IX at least, or better still Th. XI, for psychogenic turgescence to appear; the efferent pathways seem therefore, to emerge from the cord quite close to the level which receives testicular afferent pathways; --underly or overly Th. XII, L I, and L II for at least the greater part, for emission of sperm to occur; a true abrupt ejaculation probably requires the complementary integrity of the sacral spinal cord.

The authors report 9 cases of para and tetraplegia due to decompression sickness following deep sea diving. Poor technique was the cause of 8 of these cases, but one remained totally unexplained. The sequelae were serious; 3 dorsal paraplegias, which were functionally complete, 4 incomplete tetraplegias giving rise to permanent disability. In 2 cases there remained some spasticity of the lower limbs, in some cases associated with genito-urinary disorders. The authors review recent physiopathological theories. -- Blood disturbances may be due to the presence of gas bubbles which aggress the organism and give rise to coagulation disorders. -- The formation of the bubbles may be the cause of the spinal lesions, e.g. liberation in situ, gas embolism; the bubbles may form in various parts of the circulation and may cross the pulmonary barrage. Bubbles probably form in the lung itself, in the pulmonary veins and in the aorta, including the spinal capillaries. There is some slowing of the circulation, secondary to increased pressure, and pulmonary stasis may also play a harmful role. Finally, the dorsal spinal segments, which are the most poorly vascularised, are particularly exposed. This corresponds to the clinical findings. As far as treatment is concerned, the authors emphasize that recompression with hyperbaric equipment, should be carried out as an emergency, especially in unconsciuos subjects, together with other appropriate treatment. Such facts are important now that deep sea diving is becoming more and more commonly practised.