Prognostic factors in high-grade pediatric osteosarcoma among children and young adults: Greek Nationwide Registry for Childhood Hematological Malignancies and Solid Tumors (NARECHEM-ST) data along with a systematic review and meta-analysis.

The 5-year overall survival of children and adolescents with osteosarcoma has been in plateau during the last 30 years. The present systematic review (1976-2023) and meta-analysis aimed to explore factors implicated in the prognosis of children and young adults with high-grade osteosarcoma. Original studies including patients ≤30 years and the Nationwide Registry for Childhood Hematological Malignancies and Solid Tumors (NARECHEM-ST) data (2010-2021) referred to children ≤14 years were analysed. Individual participant data (IPD) and summary estimates were used to assess the n-year survival rates, as well as the association of risk factors with overall survival (OS) and event-free survival (EFS). IPD and the n-year survival rates were pooled using Kaplan-Meier and Cox regression models, and random effects models, respectively. Data from 8412 patients, including 46 publications, NARECHEM-ST data, and 277 IPD from 10 studies were analysed. The summary 5-year OS rate was 64% [95% confidence interval (95%CI): 62%-66%, 37 studies, 6661 patients] and the EFS was 52% (95%CI: 49%-56%, 30 studies, 5010 patients). The survival rates generally differed in the pre-specified subgroups. Limb-salvage surgery showed a higher 5-year OS rate (69%) versus amputation (47%). Good responders had higher OS rates at 3 years (94%) and 5 years (81%), compared to poor responders at 3 years (66%), and 5 years (56%). Patients with metastatic disease had a higher risk of death [Hazard Ratio (HR): 3.60, 95%CI: 2.52, 5.15, 11 studies]. Sex did not have an impact on EFS (HR females/males: 0.90, 95%CI: 0.54, 1.48, 3 studies), whereas age>18 years seems to adversely affect EFS (HR 18+/<10 years: 1.36, 95%CI: 1.09, 1.86, 3 studies). Our results summarize the collective experience on prognostic factors of high-grade osteosarcoma among children and young adults. Poor response to neoadjuvant chemotherapy and metastatic disease at diagnosis were confirmed as primary risk factors of poor outcome. International collaboration of osteosarcoma study groups is essential to improve survival.

Surgical Removal of Bone Bridge and Interposition of the Extensor Digitorum Brevis in the Treatment of Calcaneonavicular Coalition in Pediatric Patients: A Case Series, Short Review, and Commentary.

This study retrospectively presents a single-clinic case series of pediatric patients with calcaneonavicular coalition treated by surgical removal of the bone bridge and interposition of the extensor digitorum brevis. This technique is currently the most cited and utilized protocol in operative treatment. Clinical, functional, and radiological results are analyzed and compared with related research. This is a single-clinic retrospective study, conducted for a period of 15 years. One independent investigator reviewed medical records and conducted a de-identified preoperative, inpatient, and postoperative assessment focused on demographic data, history and clinical evaluation, imaging assessment, American Orthopedic Foot and Ankle Society (AOFAS) scoring, and complication analysis. Of 34 patients, 13 met the inclusion criteria. Mean patient age was 11.2 years; 10 were males and 3 were females. Pathology concerned the right lower limb in eight cases and the left in five. Mean time between diagnosis and surgical intervention was 4.3 months and mean postoperative follow-up was 27.2 months. Thorough overview of reported symptoms, identified signs, imaging evaluation, functional outcomes, and adverse effects was performed. Bone bridge removal and interposition of the extensor digitorum brevis is an effective method of treating the condition. Despite drawbacks, results are comparable or even, to some degree, superior to other techniques.

Clinical Radiological and Molecular Profile of a Patient Affected With Multicentric Osteolysis Nodulosis and Arthropathy.

Multicentric Osteolysis Nodulosis and Arthropathy (MONA) is an ultra-rare multisystem autosomal recessive disorder characterized by progressive osteolysis, subcutaneous nodules and developing arthropathy. The characteristic radiological signs combined with symptoms resembling juvenile idiopathic arthritis (JIA) set the diagnosis, which is established either by measuring matrix metalloproteinase-2 (MMP-2) enzyme activity through electrophoresis (zymography) or genomic testing. We report the clinical and radiographic findings of a 14-year-old girl with molecularly proven MONA, who presented with painless osteolytic changes of the feet and upper extremities and developed hip arthritis. To this day, no specific therapy has been identified with proven long term relief and control of the disease progression.

Derotational Subtrochanteric Osteotomy and External Fixation for the Treatment of Neurogenic Hip Dislocation in Children with Cerebral Palsy: Could This Be a Viable Method of Treatment?

Purpose The treatment of painful and chronic dislocated hip in children with severe cerebral palsy (CP) is particularly demanding and controversial. Numerous surgical techniques have been described, and their outcomes vary a lot. The purpose of the present study is to evaluate a new method, which combines varus derotational subtrochanteric osteotomy (VDSO) and external osteosynthesis: (VDSOEO). Methods Six non-ambulatory children with spastic quadriplegia and chronic dislocated painful hips were treated. The technique involved a small incision on the subtrochanteric site of the osteotomy, followed by retention with a single-sided external osteosynthesis with rotational correction capability [swiveling clamp (SC)] for the reduction of the femur head in the acetabulum, and finally by the osteotomy. Hardware was removed without a second intervention four-six months postoperatively and after the osteotomy was healed. Evaluation of the method was based on clinical, functional, and radiological criteria. Results  Four patients achieved improved radiological scores. Two patients demonstrated resubluxation during the period of the osteotomy's healing process. However, no patients experienced pain, and all were able to sit post-surgery, while caregivers reported improved capacity for nursing care. Conclusions It is our strong belief that this approach can improve the quality of life in children with severe CP and painful and chronic dislocated hips. It is a viable and definitely less invasive procedure than classic pelvic or femur osteotomies.

Pediatric Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) Syndrome: Diagnostic Challenges and Treatment Approach.

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare disease; however, more and more case reports have been published that increase the awareness of this disorder, especially in children. Clinically it presents as a combination of chronic recurrent multifocal osteomyelitis symptoms and skin manifestations. SAPHO treatment remains a challenge. In most cases, non-steroidal anti-inflammatory drugs are initially used, although a combination with other drugs is preferred. In addition, antibiotics, corticosteroids, bisphosphonates, and disease-modifying anti-rheumatic drugs are usually administered with varied success. There are also promising results from novel biological therapy. This paper emphasizes some non-specific symptoms of the disease, in order to increase the suspicion of SAPHO in all pediatric clinical doctors. We present the case of a 13-year-old boy with severe acne, who was admitted to our hospital due to fever of unknown origin, accompanied by arthralgia of the right ankle and left knee.

Salmonella Osteomyelitis of the Proximal Tibia in a Previously Healthy Adolescent: A Case Report.

Salmonella osteomyelitis is an uncommon pathological condition. Usually, it is associated with hemoglobinopathies or other underlying disorders. Osteomyelitis due to Salmonella is extremely rare in a previously healthy patient. We present a case of a 12-year-old previously healthy male who suffered Salmonella osteomyelitis of the proximal tibia as well as the diagnostic algorithm and therapy protocol. In our case, Salmonella osteomyelitis was identified by direct inoculation of the sample in blood culture bottles. Traditional cultures were negative. The practice of blood culture bottles seems to be essential for diagnosis, so the appropriate treatment is performed.

Multifocal Οsteomyelitis Localization after Pyomyositis in Children: Importance of Timely Response.

Pyomyositis is a rare bacterial infection that used to prevail in tropical areas for the past century. Nowadays though, more and more cases are reported in high-temperature climate areas. Diagnosis is often delayed due to the variance in clinical presentation, the challenging nature of physical examination of a child, and lack of specific laboratory investigating tools. When the diagnosis is delayed, the outcome may be unpredictable. Multifocal localization through hematogenous or direct spread that may affect the skeletal bone tissue is common. Timely diagnosis and response is a race against septic shock. We present a case series of seven children diagnosed with pyomyositis due to Staphylococcus aureus. High or less clinical suspicion has obviously affected the final outcome since two patients who were not treated in time were subjected to a life-threatening hazard. Five patients who were diagnosed and treated within the first three days after initiation of their symptoms had a predictable and good outcome without complications.

Strangling Congenital Constriction Ring Band of the Forearm with Fracture: A Rare Case Report.

Congenital constriction ring syndrome (CCRS) is a well-described pathological entity that is caused by fibrous bands that entrap parts of the fetus. The manifestations of this syndrome may vary a lot. We present a case of an almost intrauterine amputation of a fetus's upper limb. Our case is infrequent because the constriction band caused a fracture of the fetus's forearm during pregnancy. Both the band and the fracture resulted in ischemia to the hand and a salvage procedure was applied after birth. Not many authors have reported fractures due to constriction ring bands and even less have reported fractures of the upper limb. A literature review of this rare entity was conducted.

Pathological Hip Fractures in Children and Adolescents Due to Benign Tumor or Tumor-Like Lesions.

Hip fractures are uncommon in childhood and adolescence. A high-energy injury is the most common cause. Low-energy trauma may result in a pathological hip fracture or stress fracture of the femoral neck, on the grounds of a preexisting skeletal disease, a tumor, or a tumor-like lesion. Surgical or conservative treatment may be provided based on underlying disease and/or age, Delbet and/or Colona classification, and displacement of the fracture. We present a case series of 13 children with pathological hip fracture. Mean age of patients was 7 years, and average follow-up was 3 years. There were types I and II, 3 type III, and 10 type IV fractures according to Delbet/Colona classification. Patients were treated conservatively, by open reduction and internal fixation or by minimally invasive surgery. In 11 children (84.5%), the outcome was satisfactory; one child (7.75%) presented with nonunion and refracture after minimally invasive surgery, and another (7.75%) presented with refracture after conservative treatment. Both patients were treated with open reduction and internal fixation. Pathological hip fractures are rare in children and should be treated properly. Surgical treatment with open reduction, internal fixation, and bone grafting is preferred in displaced fractures. In nondisplaced fractures, the choice between conservative and surgical treatment should be based on Delbet/Colona classification and patient profile.

The role of negative pressure wound therapy in salvaging a six-year-old child's crushed foot - A case report.

Crushed extremity is an infrequent injury in children and represents a major challenge for the orthopaedic surgeon. Sometimes the decision making process, whether to amputate or save a limb, is very difficult. Several scoring systems have been developed in order to determine the fate of crushed extremities, but they are not always predictive when patients are children. In the past few years, advances in bone and soft tissue reconstruction techniques have improved outcomes, even in the most extreme cases. Negative Pressure Wound Therapy has become an accepted option in the treatment of crushed limbs. We report a 6-year-old child with a crushed left foot from the running chain of his father's motorcycle. Early revascularization and stabilization along with multiple debridement and the application of Negative Pressure Wound Therapy leaded to salvage of the child's limb. At the end of one year follow up, he presented a very good functional and aesthetic result. LEVEL OF CLINICAL EVIDENCE: "4".

Riddles in the diagnosis and treatment of osteoid osteoma in child foot: A concisive study.

BACKGROUND: Osteoid osteoma in child foot is very unusual. We investigate its polymorphism and difficulties in approach. METHODS: We retrospectively studied 12 children (7 girls, 5 boys) treated in our department from February 1995 until February 2010. Mean age was 12 years (range 8-16 years) and average follow up time 5 years (3-8 years). The lesion affected mostly the talus (8 cases), calcaneus in 3 and once the fourth metatarsal. En bloc excision of lesion was the surgical method of choice. RESULTS: Children's symptoms lasted average 2 years (14 months to even 4 years). Nocturnal pain and relief with NSAIDs occurred only in half cases. Atypical symptoms were hip referring pain, tenosynovitis of foot extensors and achilles' tendon atrophy. Complete cure and no recurrencies were noted. CONCLUSIONS: Diversity of symptoms and delay in diagnosis still remain troublesome and the absolute modality of treatment for child foot osteoid osteomas urges further investigation. Minimally invasive surgical techniques have recently emerged but orthopaedic surgeons must not forget that open excision remains the most safe and documented method for pediatric foot osteoid osteomas.

Osteomyelitis of the calcaneus due to atypical Mycobacterium.

World wide tuberculous infection of bone remains common but there are few case reports of atypical mycobacteria causing bone infection. A case is reported of recurrent osteomyelitis of the calcaneus due to such an atypical Mycobacterium and associated with interferon-gamma receptor deficiency (IFN-gamma) receptor deficiency.