Haemophilic arthropathy: Diagnosis, management, and aging patient considerations.

Gene therapy and universal use of safer, more effective, and personalised prophylactic regimens (factor, and nonfactor) are expected to prevent joint bleeding and promote joint health in persons with haemophilia (PwH). Growing evidence suggests that subclinical bleeding, with active and inactive synovial proliferation, continues and haemophilic arthropathy remains a major morbidity in PwH despite early institution of joint prophylaxis. Joint health assessment is evolving with physical examination scores complementing imaging scores. Point-of-care ultrasound is emerging as a safe, cost-effective, and readily available tool for acute determination of musculoskeletal abnormalities, serial evaluation of joints for sonographic markers of haemophilic arthropathy, and in providing objective insight into the efficacy of new therapies. In acute haemarthrosis, arthrocentesis expedites recovery and prevent the vicious cycle of bleed-synovitis-rebleed. When synovial proliferation develops, a multidisciplinary team approach is critical with haematology, orthopaedics, and physiotherapy involvement. Synovectomy is considered for patients with chronic synovitis that fail conservative management. Non-surgical and minimally invasive procedures should always be offered and considered first. Careful patient selection, screening and early intervention increase the success of these interventions in reducing bleeding, pain, and improving joint function and quality of life. Chemical synovectomy is practical in developing countries, but radioactive synovectomy appears to be more effective. When surgical synovectomy is considered, arthroscopic/minimally invasive approach should be attempted first. In advanced haemophilic arthropathy, joint replacement and arthrodesis can be considered. While excited about the future of haemophilia management, navigating musculoskeletal challenges in the aging haemophilia population is equally important.

An original preoperative orthopaedic management of a complex spinal deformity in a 16-year-old patient with spondyloepiphyseal dysplasia congenita.

PURPOSE: Spinal deformities in patients with Spondyloepiphyseal Dysplasia congenita are particularly challenging to treat. Addressing these deformities requires a holistic surgical strategy. The aim of this case report is to highlight an original preoperative protocol combining atlantoaxial instability stabilization by halo jacket with progressive correction of a thoracic kyphoscoliosis using continuous elongation by tension plaster cast as described by Stagnara. METHOD: A 16-year-old patient with spondyloepiphyseal dysplasia congenita presenting a severe thoracic kyphoscoliosis associated with atlantoaxial instability causing cervical myelopathy was managed through a preoperative protocol combining a halo jacket with a Stagnara elongation cast allowing progressive correction of the kyphoscoliosis while stabilizing the cervical instability. RESULTS: The preoperative protocol allowed a safer and a more effective surgical spine fusion, reducing neurological risks, improving the final correction, and offering better postoperative recovery. Clinical and radiological solid fusion was observed at 3-year-follow-up with significant improvement of the patient's quality of life. CONCLUSION: This case report highlights the effectiveness of the preoperative preparation combining a halo jacket with a Stagnara elongation cast to safely stabilize the cervical spine while correcting the thoracic kyphoscoliosis. This strategy can serve as a valuable tool for spine teams when addressing complex spinal deformities particularly in this patient with SEDc. LEVEL OF EVIDENCE: Level IV.

Posterior cruciate ligament avulsion associated with an epiphyseal fracture of the proximal fibula: a case report of a 13-year-old child.

Posterior cruciate ligament (PCL) avulsion fractures are rare among the pediatric population. Reported PCL injury incidence varies from 1 to 40% depending on the study population. PCL lesions can be isolated or associated with other ligamentous lesions, which makes their management challenging. Knee ligament reconstruction is essential to restore knee stability and, thus, prevent future menisci and cartilage degeneration. However, surgical management of these injuries can lead to subsequent iatrogenic growth disturbances. Case presentation: The authors describe the case of a 13-year-old child involved in a sports accident causing a PCL avulsion fracture associated with an epiphyseal fracture of the proximal fibula resulting from an incomplete peel-off injury of the lateral collateral ligament. The patient was scheduled for open reduction and internal fixation on the same day of the presentation. A long-leg cast was then applied for 6 weeks. By 3 months postoperatively, the patient regained full range of motion, and return to sport was possible 6 months after the surgery. Conclusion: In children and adolescents, PCL avulsion fractures are often associated with other occult lesions. Although good functional and clinical outcomes using operative management for these lesions are reported, specific treatment recommendations are lacking in skeletally immature patients.

Ankle sprain injury in child and adolescent: Diagnostic pitfalls.

Introduction and importance: Sprained ankles in children and adolescents are rare. Certain diagnostic pitfalls must arise in the mind of the clinician because many differential diagnoses must be confirmed or refuted urgently. Among them, the infectious osteoarticular pathology including acute hematogenous osteomyelitis of the tibia or distal fibula and septic arthritis of the subtalar joint.The main purpose of this work, through four clinical cases, we will discuss diagnostic pitfalls in the course of ankle trauma in children and adolescents. Case presentation clinical: In this work, we have demonstrated through four cases some diagnostic errors in the context of ankle trauma in children and adolescents. Infectious and malignant tumor pathologies are real diagnostic and therapeutic emergencies. For all these cases, the initial diagnosis was a sprained ankle following a gambling or sports accident. Discussion: Ankle trauma in children and adolescents is a frequent reason for consultation in emergencies. The fear of the orthopedic surgeon is to miss a serious pathology and wrongly make the simple diagnosis of a sprained ankle. Temperature measurement must be systematic. The clinical examination must be meticulous. The interpretation of the radiograph must be cautious. Acute hematogenous osteomyelitis and malignant tumor pathology should be on the surgeon's mind. Conclusion: We have shown through these four examples that a serious pathology can simulate a sprain. Hence, a careful analysis of clinical, biological and radiological elements is the only guarantee of adequate care.

Acute hematogenous osteomyelitis in children: Management of pandiaphysitis with extensive bone destruction: A case series of thirteen child.

Introduction: and Importance: Acute hematogenous osteomyelitis (AHO) poses a public health problem in severe forms from the outset or with delayed diagnosis. The aim of this work is to describe the management of pandiaphysitis with extensive bone destruction by the Ilizarov external fixator and antibiotics and to evaluate the results. Methods: This is a retrospective, descriptive, cross-sectional and single-center study. It was performed over a seven-year period. We included children with acute hematogenous osteomyelitis complicated by pandiaphysitis of the long bones with extensive bone necrosis. All patients underwent stabilization with an Ilizarov external fixator and antibiotic therapy adapted to the antibiogram in ten cases. One patient had the induced membrane technique. All the patients underwent dynamization of the Ilizarov external fixator followed by immobilization with a cast or a splint for the lower limb. Outcomes: 13 medical files were collected during the study period. The sex ratio was 1.6, the average age was 7.56 years. Three localization sites: humerus for 3 cases, femur for 3 cases and tibia for 7 cases. The germ has been identified in ten cases. Methicillin-resistant staphylococcus aureus was the most common. Bone consolidation is obtained in all cases with an average delay of ten months. Conclusion: The Ilizarov external fixator associated with antibiotics has proven its effectiveness in the management of pandiaphysitis with extensive bone destruction. This method is an alternative in the therapeutic arsenal available to pediatric orthopedic surgeons. Level of evidence: IV, Case series.

Calcaneal lengthening osteotomy in the management of idiopathic flatfoot in children: cCase series of twenty-one feet.

INTRODUCTION: Flatfoot is a frequent reason for consultation in pediatric orthopedics. The calcaneal lengthening osteotomy according to the EVANS technique is a therapeutic alternative. The objective of this work was to evaluate the short and medium term clinical and radiological results of calcaneal lengthening osteotomy in children with idiopathic flat foot valgus. METHODS: This study concerned 12 children and 15 ft treated surgically by calcaneal lengthening osteotomy by an orthopedic surgeon in a pediatric orthopedic surgery center. The evaluation of the results was clinical according AOFAS score and radiological. RESULTS: The deformity was reducible in all of our patients. The mean preoperative AOFAS score was 61, postoperatively 90. The overall result was excellent in 11 cases (11 ft) and good in 4 cases. The postoperative radiological result was close to normal values. CONCLUSION: Calcaneal lengthening osteotomy is a reliable and recommended technique for the correction of symptomatic idiopathic flatfoot. LEVEL OF EVIDENCE: IV, Case series.

The "Patient management problem" as a formative evaluation during the internship.

INTRODUCTION: The evaluation of the internship in pediatric orthopedics is based on a clinical examination in the form of ECOS, after the internship period involving all surgical specialties. The validation of the clinical examination test does not necessarily mean that the student has achieved his internship objectives and vice versa. This discrepancy between the evaluation test and the field of the internship prompted us to question the need to establish a formative evaluation method during the clinical internship. METHODS: Our study is a prospective, evaluative, longitudinal study, from October 2019 until September 2020, in the infantile orthopedics and traumatology department of the "Béchir HAMZA" children's hospital in Tunis. We carried out a formative evaluation using the "Patient Management Problem" (PMP), with two different subjects, one on acute hematogenous osteomyelitis, the other on malignant bone tumors, with a Pre-test and a Post-test for each PMP. RESULTS: We had 18 interns, during the period between October 2019 and September 2020, including 13 who had medical training in Tunisian faculties. The score for the acute osteomyelitis pretest was 14.56 on average, the PMP score was 16.33 on average, with one interne scoring zero. The posttest mean was 18.11 [13.75-20], with a statistically significant difference from the pretest. The pre-test for the bone tumors' PMP had an average of 9.7 [3.75-15], that of PMP was 12.6 [0-20]. There was an improvement in post test scores with an average of 16.45. Comparing the two tests we noticed that the scores for the PMP osteomyelitis were higher than the scores for the PMP bone tumors with a significant difference between the two groups (p = 0.04). CONCLUSIONS: The results of this pilot project are encouraging for the establishment of a continuous evaluation by the PMP method, intended for interne during the pediatric orthopedic surgery internship. This method allows learning and formative evaluation of learners by evaluating clinical and therapeutic reasoning.

Torticollis as a sign of spinal tuberculosis.

Bone localization of tuberculosis mainly affects the thoracolumbar spine. The cervical spine is rare. Its diagnosis is often late which exposes to great instability and potentially serious complications. We report the case of a 12-year old girl with no medical history, showing torticollis and high temperature without neurological complication. In the physical examination, he had torticollis and pain in the third, fourth and fifth cervical vertebra. When the biopsy was performed, we find an inter apophysis (between C7 and D1) collection. The histological examination confirmed the diagnosis of apophysis tuberculosis. The management based on tuberculosis chemotherapy and immobilization started as soon as possible.

Isolated tuberculosis of the navicular bone in a child.

Tuberculosis is a topical issue in endemic countries. Foot involvement is rare and accounts for 5-10% of all osteoarticular tuberculosis. Pediatric cases of osteoarticular tuberculosis are uncommon especially in well vaccinated children. The clinical presentation is not specific and can be confused with common germ like staphylococcus. Biopsy is mandatory in endemic zone, and if the evolution is not good with antibiotherapy. Debridement can be indicated but must be gentle, because of the risk of navicular necrosis.

Congenital pseudarthrosis of the forearm treated with induced membrane technique: A case report.

Congenital pseudarthrosis of the forearm (CPF) is an uncommon entity frequently described in association with neurofibromatosis type 1. Surgical treatment Is required. This is a challenging pathology in pediatric orthopedic surgery due to the difficulty of achieving a satisfying result in terms of bone defect healing. Many techniques are described such us free vascularized fibula, illizarov and induced membrane. We describe a first case of the forearm non union associated with neurofibromatosis type 1 successfully treated with 2 steps of induced membrane. Whatever the technique selected it's remain challenging problem in pediatric orthopaedic.

Kienböck's disease in an 11-year-old girl: a case report.

Kienböck disease is a pathology that remains uncommon in children, therefore the number of published cases of Kienböck's disease before skeletal maturity is limited. The etiology of Kienböck's disease is still controversial. Although many therapeutic methods are described in the literature. There is no consensus treatment for this pathology. We describe a case of Kienböck's disease of an 11-year-old girl who presented with avascular necrosis of the lunate bone confirmed by the radiologic pattern. And who was treated with 10 weeks of splinting with satisfying clinical outcome. Wrist pain and other symptoms resolved after 2 months. A magnetic resonance imaging (MRI) confirmed partial revascularization of the lunate. After 18 months of follow-up the patient remains asymptomatic.

[Epidemiology of acute hematogenous osteomyelitis in children: a prospective study over a 32 months period]. / Epidémiologie des ostéomyélites aiguës hématogènes communautaires de l'enfant : Etude prospective sur 32 mois.

AIM: To determine the demographic, clinical, biological and bacteriological profile of acute community acquired hematogenous osteomyelitis (AHO) in children. METHODS: Prospective study including children admitted for AHO. We noted the demographic parameters of patients and the clinical, biological and radiological characteristics of the infection. Blood cultures and local specimen in operated children were systematically performed. RESULTS: 70 patients were included. The mean age was 7.7 years. The mean time between onset of symptoms and admission was 3.2 days. Distal tibia was the most frequent localization (18.66%). Fever higher than 38° on admission was found in 92.8% of patients. Creactive protein (CRP) was superior to 20 mg / L in 95.8% and ESR superior to 20 mm in the first hour in 92% of cases. A deep venous thrombosis was found in 7 patients and a pleuropulmonary Staphylococcus infection in 4 patients. A micro organism was isolated in 64.7% of cases. Staphylococcus aureus methicillin susceptible (SAMS) was the predominant germ. Staphylococcus aureus methicillin resistant (SAMR) accounted for 15.7% of staphylococcus aureus infections. The importance of CRP on admission and time to resolution of fever after the start of treatment were significantly higher in SAMR infections. The existence of deep venous thrombosis and a pleuropulmonary Staphylococcus infection and the need for surgical drainage were significantly more frequent in SAMR infections. CONCLUSION: The existence of a severe form of AHO should lead to a high suspicion of SAMR infection and prompt the prescription of an appropriate antibiotiotherapy.

Progressive correction of severe spinal deformities with halo-gravity traction.

Treatment of rigid and severe spinal deformities is challenging and risky. Preoperative halo-gravity traction can be used to progressively reduce the deformity before spinal fusion. The aim of this study was to evaluate the effectiveness of halo-gravity traction for the correction of severe spinal deformities. Fifteen patients were reviewed retrospectively. Their mean age at the beginning of traction was 13.5 years. The mean duration of traction was 64 days. The main curve in the coronal plane improved from +/- 95 degrees to +/- 67 degrees, a gain of +/- 28 degrees (range 0 degrees-50 degrees) or +/- 30%. The curve in the sagittal plane improved from +/- 96 degrees to +/- 78 degrees, a gain of +/- 18 degrees (range 0 degrees-45 degrees) or +/- 19%. Other authors report gains up to 46% and 43%, respectively in the coronal and in the sagittal plane, but this might be due to different conditions, techniques, and evaluations. One patient with a pre-existing neurological deficit developed paraplegia. According to the literature congenital curves with associated kyphosis are exposed to paraplegia. Halo-gravity traction is effective and is usually tolerated better than other techniques of traction using the halo device.

Bilateral stress fractures of the femoral neck with no risk factor : a case report.

BACKGROUND: Stress fractures of the femoral neck are uncommon. They are caused by either excessive stress or bone fragility. AIM: To report a case of bilateral stress fracture of the femoral neck in a 15-year-old girl without any risk factors OBSERVATION: We report a case of bilateral stress fracture of the femoral neck in a 15-year-old girl in whom no risk factors were found. One of the fractures was displaced at first diagnosis and was successfully treated by closed reduction and valgus subtrochanteric osteotomy. The other fracture was initially undisplaced but displacement occurred later despite internal fixation with a dynamic hip screw. CONCLUSION: A high index of suspicion of stress fractures of the femoral neck must be kept in mind even without risk factors. Delays in diagnosis and displacement should therefore be avoided.

Magnetic resonance evaluation of acetabular residual dysplasia in developmental dysplasia of the hip: a preliminary study of 27 patients.

Thirty-one hips in 27 young girls, treated for developmental dysplasia of the hip in the authors' institute since 2003, showed persistent radiographic evidence of residual acetabular dysplasia. These hips were registered as candidates for pelvic osteotomy. A prospective study was conducted and these hips were evaluated by magnetic resonance imaging (MRI); the average age of the patients was 5 years. MRI measurement of acetabular angle and acetabular head index in 2 different landmarks (bone and cartilage) was performed. The results were correlated with plain radiographic film evolution. MRI studies revealed sufficient cartilaginous acetabular coverage in 27 hips, cartilaginous acetabular dysplasia in 2 hips, and short acetabulum in 2 others. The 27 hips with thick cartilage of the acetabular roof were subsequently followed up by plain radiographs. The average follow-up period was 2.1 years. The authors observed a spontaneous progressive ossification of the cartilaginous acetabular roof in all the 27 cases. In 4 cases, the correction of the acetabular angle was complete. They concluded that MRI promotes more accurate selection of patients for pelvic osteotomy and aids in the choice of the most appropriate type of osteotomy. Clinical imaging examples are presented and need to be further evaluated.

Early results of the Ponseti method using the Steenbek foot abduction brace: a prospective study of 95 feet.

The purpose of this study is to evaluate the early results of the Ponseti method and the effectiveness of the Steenbek foot abduction brace. A total of 74 patients with 110 idiopathic clubfeet were included in this prospective study. The feet were evaluated according to the Dimeglio-Bensahel classification, the Catteral-Pirani classification and the functional classification of the Hospital for Joint Diseases. Ninety-eight feet (89%) had a good result after the casting period. All the feet evaluated after the period of full-time bracing and during the period of part-time bracing showed a good correction. The Ponseti method using the Steenbek foot abduction brace is effective in correcting idiopathic clubfeet.