[Chimeric Antigen Receptor T-cells -  Gene Therapy of the Future for Malignant Diseases?]. / Chimérický antigenní receptor T lymfocytu -  genová terapie budoucnosti u nádorových onemocnení?

Chimeric antigen receptors are synthetic, genetically modified receptors of T-cells. The introduction of chimeric antigen receptors into autologous patient T-cells can redirect the lymphocytes to specific antigen targets on the surface of malignant cells. This has recently emerged as an intriguing therapy approach in both hematologic malignancies and later also in solid tumors. Various chimeric antigen receptor designs and manufacturing processes were developed and seem to have a strong impact on the activity of chimeric antigen receptor T-lymphocytes and thereby the therapy success. The individual variables are currently being tested in numerous clinical trials. In this review, I will briefly describe the principle, basic structure and construction of chimeric antigen receptor T-lymphocytes.

Soft-tissue necrosis complicating tibial osteotomy in a child with Proteus syndrome.

INTRODUCTION: Proteus syndrome is a rare congenital hamartomatous disease frequently responsible for musculoskeletal deformities. The results and complications of surgical treatment are not well documented owing to the scarcity of reported cases. CASE REPORT: The authors report a case of poor evolution of valgus proximal tibial osteotomy in a 6-year-old girl with Proteus syndrome. The surgery was complicated by extensive deep wound necrosis exposing the tibial bone, necessitating surgical excision, antibiotherapy and controlled wound healing. At 1 year postoperatively, the deformity recurred. DISCUSSION: The possibility of serious wound complications and of recurrence must be kept in mind when operating on a limb deformity in patients with Proteus syndrome. Potential complications should be taken into account in selecting the surgical correction technique: epiphysiodesis may be preferable to osteotomy.

[Intravenous antibiotic therapy for acute hematogenous osteomyelitis in children: short versus long course]. / Antibiothérapie intraveineuse des ostéomyélites aiguës : traitement long versus court.

The aim of this study was to evaluate the results of two antibiotic therapy protocols for osteomyelitis with different durations of intravenous treatment. This was a prospective randomized study of children treated for acute hematogenous osteomyelitis. Patients in group 1 (G1) received 7 days of intravenous antibiotics, whereas patients in group 2 (G2) received 14 days. Treatment was deemed effective if there were no signs of chronic osteomyelitis at the last follow-up. Fifty-three patients were included in the study (G1=27, G2=26). After a mean follow-up of 11.5 months, none of the patients in either group showed signs of chronic osteomyelitis. In conclusion, a shortened treatment of 7 days of intravenous antibiotic therapy is as effective as a longer treatment.

Dysplasia epiphysealis hemimelica: a report of four cases.

UNLABELLED: Dysplasia epiphysealis hemimelica (DEH), also known as Trevor's disease, is a rare congenital skeletal developmental disorder in childhood. It is characterized by an asymmetric osteocartilaginous overgrowth arising from epiphyses or epiphyseal equivalents. Lesions have hemimelic topography, usually occur in the lower limbs, most commonly in the ankle and the knee and affect either the medial or lateral part of one epiphysis. OBJECTIVE: The purpose of this study is to describe the imaging features of DEH by reporting four cases. MATERIALS AND METHODS: We present four cases of DEH in one female and three males aged between 7 and 15 years. Lower limb is involved in all cases and patients suffer from pain, limited function and deformity. Radiographs and CT findings were reviewed. All patients were treated by surgical excision. RESULTS: Plain X-ray revealed in all cases an irregular ossification arising from the affected epiphysis. The CT scan revealed an irregular fragmented osteocartilaginous mass involved from the epiphysis, with enlargement of epiphyses and intra-articular extension. CONCLUSION: The early diagnosis and treatment of DEH is necessary in preventing articular function, CT assists in defining the anatomic relationship between the mass and its parent epiphysis and in evaluating the condition of the articular cartilage and soft tissue. The distinct clinical and radiographic features should enable to differentiate the osteochondroma and the DHE.

[An unusual cause of hand nodule: peri-tendon dirofilariasis]. / Une étiologie inhabituelle de nodule de la main : la dirofilariose péritendineuse.

Dirofilariasis is a zoonosis affecting dogs and cats. It was transmitted to man by mosquito bites. Human dirofilariasis is rare. We report a case of 4-year-old girl presented with a subcutaneous palmar nodule of the hand. Surgical excision shows a nodule encircling the forth flexor tendon. The histological examination established the diagnosis by the presence of an adult worm identified as Dirofilaria repens. Surgical excision was curative.

[Hematogenous osteomyelitis of the calcaneus in children: 26 cases]. / L'ostéomyélite hématogène du calcanéum chez l'enfant: à propos de 26 cas.

PURPOSE OF THE STUDY: Osteomyelitis is rarely observed in the calcaneus; about 3 to 10% of the bone infections in children. The diagnosis is often established late because of the less pronounced symptoms in long-bone localizations. We report a series of 26 cases of osteomyelitis of the calcaneus observed in children. MATERIAL AND METHODS: We studied the clinical history, the diagnostic process and the treatments delivered. Outcomes were assessed in terms of complications, anatomy and function at mean two years follow-up (range one to seven years). RESULTS: There were 15 boys and 11 girls, mean age was seven years (range one month to 13 years). Mean time from symptom onset to consultation was 13 days and mean time from consultation to hospital admission was four days, range one to 29 days. The clinical presentation was not specific. Body temperature was not above 38.5 degrees C in 45% of patients. Symptoms were fever, pain in the rear foot and functional impotency of the lower limb. Eight patients (30%) complained of moderate pain, 18 (70%) of intense pain. The pain was focused far from the calcaneus in six patients, retarding the diagnosis. Laboratory tests did not always reveal signs of inflammation. White cell counts above 10,000 were noted in only 61% of patients. The diagnosis of osteomyelitis of the calcaneus was based on: the plain X-ray, which revealed a defect in the calcaneus (n=12), ultrasound (performed in 19 patients) which revealed calcaneal subperiosteal detachment (n=6), collections in the rear foot (n=3) and soft-tissue thickening (n=4). Bone scintigraphy was performed in one child and showed intense uptake in the calcaneus. Magnetic resonance imaging, performed in one patient, demonstrated an anomalous signal in the calcaneus (high-intensity T(2) and low-intensity T(1) with presence of a subperiosteal abscess). Bacteriology was positive in 53% of the children. Medical treatment was delivered for all patients and 23 underwent a surgical procedure. For one of the three patients treated medically, the diagnosis of osteomyelitis of the calcaneus was clinical, since the plain X-ray was normal, the ultrasound yielded no evidence of abscess formation and the bacteriology was negative; but after two months of antibiotic treatment, bone remodelling was in favour of osteomyelitis of the calcaneus. For the two other patients treated medically, the plain X-ray showed a defect in the calcaneus, which had filled after two months of antibiotics. For the 23 patients treated surgically, the procedure was an evacuation of a subperiosteal abscess for 13 (n=6 nonruptured and 7 ruptured). Surgery revealed a bone lesion in nine children allowing curettage of the defect. Articular involvement was noted in eight cases: subtalar osteoarthritis (n=6) and tibiotarsal arthritis (n=2). Two surgical explorations failed to find any abscess formation; blood cultures confirmed the diagnosis and enabled isolation of the causal germ. Outcome was assessed with a mean follow-up of two years, range one to seven years. Nineteen patients (73%) were free of sequelae. Seven patients (27%) presented poor outcome with significant limitation of motion in the rear foot and ankle ankylosis. Seven patients developed chronic fistules, with persistent discharge at last follow-up. The poor results were observed in patients treated late with mean 17 days before consultation. Six of the seven cases of poor outcome were associated with arthritis involving a calcaneal joint (subtalar and tibiotarsal in two patients and subtalar in four). DISCUSSION: The same pathophysiological phenomenon as observed in long-bone localizations is noted for osteomyelitis of the calcaneus; the calcaneus has an apophysis, which is equivalent to the metaphyseal region of long bones, leading to the bone's vulnerability to hematogenous infection. Late diagnosis can be related to the notion of trauma, the manifestations of osteomyelitis being attributed to ligament injury. The positive diagnosis of osteomyelitis of the calcaneus is often established late because of late consultation (13 days in our series) or the minimal expression of general signs. Magnetic resonance imaging contributes significantly to diagnosis by showing an abnormal bone signal; it can also disclose associated abscess formation. Authors differ in their descriptions of the complications. The analysis of our results shows that the prognosis of osteomyelitis of the calcaneus is related to early diagnosis and management. Associated septic arthritis is an element of poor prognosis.

[Acute hematogenous osteomyelitis of the obturator rim in seven children]. / L'ostéomyélite aiguë hématogène du pourtour du foramen obturé chez l'enfant : à propos de sept cas.

PURPOSE OF THE STUDY: Acute osteomyelitis of the rim of the obturator foramen is rarely reported. The clinical presentation is atypical, making diagnosis a difficult task. We report a series of seven cases of osteomyelitis of the obturator rim which illustrate the different features of this localization. MATERIAL AND METHODS: The series included seven boys with osteomyelitis involving the obturator foramen. We reviewed the clinical history, the diagnostic approach and management. Complications and anatomic outcome were noted at mean three years follow-up (range one to 10 years). RESULTS: Mean age was 9.5 years (five to 12 years). Bilateral involvement was noted in one boy. Mean time from symptom onset to consultation was five days and mean time from consultation to hospitalization was eight days. No specific clinical presentation could be identified. Fever was not a constant feature. Mean body temperature was 38.6 degrees C and was not greater than 38.5 degrees C in four children. Symptoms were limited to hip pain in five cases and abdominopelvic pain was noted in two. Physical examination failed to trigger exquisite ischial or pubic pain in two patients. The osteomyelitis involved the ischiopubic ramus in four cases, the ischium in two with one bilateral case, and the pubis in one. Certain diagnosis was established as follows: MRI findings plus isolation of the pathogenic agent (n=4) ; the plain x-ray showed a defect in the ischiopubic ramus, MRI showed signs favoring osteomyelitis of the ischiopubic ramus and surgery evacuated a purulent collection (n=1) ; strong uptake of the obturator rim on scintigraphy (n=2). Medical treatment was given in all cases and surgery was performed in six patients. Outcome was noted at three years follow-up, range one to 10 years. All patients recovered normal function. There were no complications. DISCUSSION: Hematogenous osteomyelitis of the pelvis is exceptional (2-11 % of cases of osteomyelitis). Localization in the obturator rim is rare. We discuss the specific diagnostic and therapeutic features of this localization. Despite the controversy concerning the treatment of pelvic osteomyelitis and in particular cases involving the obturator rim, prognosis is generally good. No complications have been described in the literature specifically associated with osteomyelitis of the obturator rim.

[Acute hematogenous osteomyelitis of the neck of the femur in children: 28 cases]. / L'ostéomyélite aiguë hématogène du col du fémur chez l'enfant : à propos de 28 cas.

PURPOSE OF THE STUDY: Osteomyelitis of the neck of the femur is uncommon, often with a misleading clinical presentation. We report a series of 28 cases of osteomyelitis of the femoral neck to illustrate the particular clinical and imaging findings related to this localization. MATERIAL AND METHODS: This was a series of 28 children treated in our unit from 1990 through 2004: 17 boys and 11 girls, mean age eight years (range one month to 14 years). We analyzed the data in this series using a standard checklist, which noted the diagnostic and therapeutic measures. Results were analyzed by studying the complications, anatomic and functional outcome at mean follow-up of 3.5 years. RESULTS: Time from symptom onset to consultation was five days on average with a delay of 4.5 days from consultation to hospitalization. Intense pain was noted for eight patients (28%) and total functional incapacity of the limb was noted for 15 (53%). Hip stiffness was observed in 11 patients (39%). The diagnosis of osteomyelitis of the femoral neck was established on the basis of imaging (MRI or scintigraphy) in three patients with bacteriological proof in two, of operative findings which confirmed the neck localization in 19, and on changes in the X-ray image of the neck in six. A positive bacteriology was noted in 71%. The germ was isolated from blood cultures and local samples obtained by arthrotomy in five patients (same germ), only in blood cultures for four, and only in local samples in 11. Met-S Staphylococcus aureus was isolated in 18 patients, Met-R S. aureus in one and a Streptococcus in one. All patients were given medical treatment and 25 underwent surgery. There were five thromboembolic complications and five patients who developed femoral pandiaphysitis. Results were analyzed at mean 3.5 years (range four months to 14 years). Complete hip motion was recovered in 78%. There were four cases of hip stiffness and two cases of ankylosis. Partial cephalic necrosis was noted in two hips and total necrosis of the head and neck in two. DISCUSSION: Little data is available in the literature on isolated osteomyelitis of the femoral neck. Based on the pathogenic mechanisms known for osteomyelitis, an isolated localization in the neck of the femur, with no other site in the hip joint, is quite possible in an early stage of infection. We discuss the specific clinical and imaging features of this localization. Analysis of our findings show that the prognosis of femoral neck osteomyelitis is directly related to time to management. Outcome is poorer when treatment is started late. Prognosis is poor if pandiaphysitis develops.

Osteosarcoma with rhabdomyosarcomatous component or so-called malignant mesenchymoma of bone.

BACKGROUND: Primary malignant mesenchymoma of the bone is a rare neoplasm consisting of two or more unrelated malignant mesenchymal components. The literature reports fewer than 20 cases, most of which were composed of osteosarcoma and liposarcoma. OBSERVATION: We report an exceedingly rare case of primary malignant mesenchymoma of bone composed of rhabdomyosarcoma, osteosarcoma, and a minor chondrosarcoma component, arising in the right proximal humerus of a 15-year-old girl. The rhabdomyosarcomatous component was present in the initial biopsy and persisted in surgical specimen following chemotherapy. CONCLUSION: Effect of chemotherapy is enigmatic since rhabdomyosarcomatous component could appear, persist or disappear after chemotherapy according to literature.

Combined fracture of the distal radius and scaphoid in children. Report of 2 cases.

The authors report 2 cases of concomitant scaphoid and distal radial fractures, a rare combination of lesions in children. The first case was in a 13-year-old boy who presented a transverse midscaphoid fracture associated with a Salter type II distal radial fracture. The second concerned a 10-year-old boy who presented a bilateral wrist injury. On the left side, a distal forearm fracture with anterior displacement was associated with a transverse midscaphoid fracture. On the right side, there was only a distal forearm fracture. Scaphoid fracture associated with distal radial fracture seems to have been more frequently reported in the literature during the last decade, probably because it is more frequently recognised. In fact, children with distal forearm injury should be well examined both clinically and radiologically in order to search for an associated scaphoid fracture. Reduction of the radius fracture should be done carefully to avoid possible displacement of the scaphoid fracture.

[Juxtacortical osteosarcoma of the radius in a child]. / Ostéosarcome juxtacortical du radius chez un enfant.

The authors report a rare case of juxtacortical osteosarcoma of the radius in a child. The diagnosis and treatment were delayed because of misleading and wrongly reassuring radiological and histological aspects. The 12-year-old boy was admitted for a tumefaction of the proximal extremity of the right radius noticed after trauma. Physical examination showed a hard and slightly painful swelling. Xrays showed an irregular osseous mass developed from the superior metaphysis of the radius and encircling it. MRI showed a juxtacortical tumor with medullary involvement. A biopsy concluded to a fracture callus. Ten months later, the tumor became painful and homogeneous, regular and well defined on Xrays. A repeat biopsy showed juxtacortical osteosarcoma. Enbloc resection of the tumor was done. After a follow-up of 2 years, the child is alive without recurrence and metastasis and with a satisfactory function of the upper limb.

[Brachial plexus paralysis of infectious origin: report of four cases]. / Paralysie du plexus brachial d'origine infectieuse: à propos de quatre cas.

BACKGROUND: Brachial plexus palsy (BPP) may occur after septic osteoarthritis of the shoulder. BPP is a rare, well recognized complication. The pathophysiology is thought to be either ischemic nerve damage secondary to occlusion of vasa nervorum or extrinsic compression. Early diagnosis and treatment of the infection are needed. CASE REPORTS: We report the cases of three infants aged less than three months and one six-day-old newborn infant who presented with an authentic BPP due to an osteoarthritis of the shoulder. The diagnosis of osteoarthritis relied on clinical, biological and radiological signs. In one case, the causal germ (Enterobacter) was isolated. The diagnosis of the palsy was based on clinical signs. EMG performed on one patient, showed signs of abnormal motor pattern. Following intravenous antibiotics and percutaneous drainage, all infants completely recovered.

[Calcifying aponeurotic fibroma: the case of a 15-year old boy]. / Fibrome aponévrotique calcifiant: a propos d'une observation chez un adolescent de 15 ans.

Calcifying aponeurotic fibroma is a rare benign soft tissue tumor that usually involves distal extremities in children and young adults. It is characterized by a high rate of recurrence. We report a case in a 15-year-old boy who complained of painless swelling of his right foot. Tomodensitometry showed a dense well-circumscribed tumor with peripheral calcification. The tumor was completely removed. The patient is well at 28 months follow-up. Diagnosis relied on microscopic analysis showing fibroblastic proliferation with focal calcification and chondroid differentiation.

[Unusual radiologic signs of bone echinococcosis in children]. / Signes radiologiques inhabituels de l'échinococcose osseuse chez l'enfant.

The authors report 2 cases of 2 children aging 11 and 12-years-old presenting hydatitosis in the upper extremity of 2 long bones femur and tibia. In 2 cases, radiological appearances were atypical. In the femur, plain radiographs revealed multiple lucencies, irregular, located in the superior extremity with a periostal reaction. The CT scan confirmed these data and showed a cortical rupture, extension to soft tissue with contrast enhancement. In the tibia, plain radiographys showed delimitated lucencies, confluent with a rim condensation in the proximal epiphyseal-metaphyseal area. Echography and CT are helpful when radiological appearances are unusual.

Parturient's posture during epidural puncture affects the distance from skin to epidural space.

STUDY OBJECTIVE: To assess the factors affecting the distance from skin to epidural space. DESIGN: Prospective observational study of consecutive cases over a 2-year period. SETTING: Inpatient obstetric unit in a French university hospital. PATIENTS: 2,123 consecutive term parturients who received epidural anesthesia for cesarean section or epidural analgesia for labor and vaginal delivery. INTERVENTIONS: At the time of epidural puncture, the interspace used and the patient's posture (sitting or left lateral decubitus) were recorded, and the distance from the skin to the epidural space (DS-ES) was measured to the nearest 0.5 cm using a marked epidural needle. MEASUREMENTS AND MAIN RESULTS: The relationship between patient factors [height, weight, body mass index (BMI; weight/height2), presence of scoliosis] and technical factors (interspace, patient's posture at puncture) versus DS-ES was investigated using multiple regression analysis. DS-ES correlated positively with the parturient's weight and BMI. In addition, DS-ES was significantly greater when epidural puncture was performed in the lateral position as compared with the sitting position. CONCLUSION: Both the patient's weight and position during epidural needle placement are important factors influencing DS-ES. A change from the sitting to the lateral position may increase DS-ES, causing catheter dislodgment and consequent inadequate analgesia. Clinical studies relating DS-ES to inadequate analgesia must take these factors into account.

[Xanthogranulomatous pyelonephritis. Apropos of 42 cases]. / La pyélonéphrite xanthogranulomateuse. A propos de quarante-deux cas.

The authors report their experience of forty-two cases of xanthogranulomatous pyelonephritis. They point out the various difficulties of preoperative identification of such diseases by the imaging modalities. They also discuss the main pathogenic hypotheses proposed in the literature.

[Xanthogranulomatous pyelonephritis in children. Apropos of 8 cases]. / La pyélonéphrite xanthogranulomateuse de l'enfant. A propos de huit cas.

Eight pediatric cases of the diffuse form of xanthogranulomatous pyelonephritis are reported. These patients were selected among 17 with destruction of one kidney. Clinical symptoms were suggestive of a tumor in some instances. However, an infectious etiology was strongly suggested by the presence of a renal lithiasis in seven cases (including five staghorn stones) and of a urinary tract infection in six cases (usually caused by a Proteus). Nephrectomy was performed in every case.

[Calculous anuria in children. Apropos of 12 cases]. / L'anurie calculeuse de l'enfant. A propos de douze cas.

The authors report their experience of calculus anuria in children. They based their study on a series of 12 cases. They point out, in particular, the diagnostic difficulties and prognostic risks which could be encountered. They also emphasize, the role of prompt renal drainage in the management of such an emergency and recommend percutaneous nephrostomy followed by cold nephrolithotripsy.