Progression toward Vertebral Collapse of Vertebral Metastases Treated with Percutaneous Vertebroplasty: Rate and Risk Factors.

PURPOSE: To evaluate of the rate of and risks for progression toward collapse in vertebral metastases (VMs) treated with percutaneous vertebroplasty (PV). MATERIALS AND METHODS: A total of 151 PVs were performed in 81 patients with vertebral metastases and were retrospectively analyzed. Follow-up imaging was performed at 12 months to measure vertebral body height and to report vertebral collapse at the level of the treated vertebrae. Vertebral characteristics (spine instability neoplastic score [SINS], number of lysed cortices, and prior radiotherapy) and procedural parameters (Saliou score, cortical contact with cement, and intradiscal cement leakage) were compared between the group of patients with and without collapse of the treated vertebrae. RESULTS: Of the vertebrae treated with PV, 41 of 151 (27%) progressed toward collapse. Vertebral collapse was influenced by a high SINS (odds ratio [OR] = 1.27, P = .004), SINS value > 9 (OR = 2.96, P = .004), intradiscal cement leakage (OR = 2.18, P = .048), pre-existing spinal deformity (OR = 2.65, P = .020), and pre-existing vertebral fracture (OR = 3.93, P = .045). A high Saliou score (OR = 0.82, P = .011), more than 3 cortices in contact with the cement (OR = 0.38, P = .014), and preserved spinal alignment (OR = 0.38, P = .020) were associated with a lower incidence of collapse. CONCLUSIONS: Rate of vertebral collapse despite PV was influenced by vertebra-specific characteristics and by cement injection quality. Vertebrae with a SINS of ≤9 and with homogeneous cement filling had a lower incidence of collapse.

Solitary intraosseous myofibroma: a rare case diagnosed from mandibular fracture.

Intraosseous myofibroma is a rare tumor of benign nature, slow growth, and low morbidity. The aim of this article is to report a case of pathologic fracture associated with the incidental diagnosis of myofibroma in the mandible of an adolescent. A 15-year-old girl reported that she experienced a physical assault resulting in facial injuries 1 month previously and had since experienced severe pain, malocclusion, and chewing difficulty. The cone beam computed tomographic examination revealed multiple features suggestive of pathologic fracture associated with a hypodense lesion with lobulated limits, as well as expansion and thinning of the cortical bone in the left mandible. The histopathologic diagnosis of the lesion indicated myofibroma. Treatment consisted of enucleation and curettage of the lesion with reduction and internal fixation of the fracture. After 18 months, the osteosynthesis plates and an impacted mandibular third molar were removed. Curettage of the lesion in association with treatment of the mandibular fracture proved to be effective for both bone consolidation and absence of recurrence while restoring mandibular functionality.

Carbon-fibre plates for traumatic and (impending) pathological fracture fixation: Where do we stand? A systematic review.

BACKGROUND: Carbon-fibre (CF) plates are increasingly used for fracture fixation. This systematic review evaluated complications associated with CF plate fixation. It also compared outcomes of patients treated with CF plates versus metal plates, aiming to determine if CF plates offered comparable results. The study hypothesized that CF plates display similar complication rates and clinical outcomes as metal plates for fracture fixation. METHODS: The study adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The following databases were searched from database inception until June 2023: PubMed, MEDLINE, Embase, Web of Science, Cochrane Library, Emcare, Academic Search Premier and Google Scholar. Studies reporting on clinical and radiological outcomes of patients treated with CF plates for traumatic fractures and (impending) pathological fractures were included. Study quality was assessed, and complications were documented as number and percentage per anatomic region. RESULTS: A total of 27 studies of moderate to very low quality of evidence were included. Of these, 22 studies (800 patients, median follow-up 12 months) focused on traumatic fractures, and 5 studies (102 patients, median follow-up 12 months) on (impending) pathological fractures. A total of 11 studies (497 patients, median follow-up 16 months) compared CF plates with metal plates. Regarding traumatic fractures, the following complications were mostly reported: soft tissue complications (52 out of 391; 13%) for the humerus, structural complications (6 out of 291; 2%) for the distal radius, nonunion and structural complication (1 out of 34; 3%) for the femur, and infection (4 out of 104; 4%) for the ankle. For (impending) pathological fractures, the most frequently reported complications were infections (2 out of 14; 14%) for the humerus and structural complication (6 out of 86; 7%) for the femur/tibia. Comparative studies reported mixed results, although the majority (7 out of 11; 64%) reported no significant differences in clinical or radiological outcomes between patients treated with CF or metal plates. CONCLUSION: This systematic review did not reveal a concerning number of complications related to CF plate fixation. Comparative studies showed no significant differences between CF plates and metal plates for traumatic fracture fixation. Therefore, CF plates appear to be a viable alternative to metal plates. However, high-quality randomized controlled trials (RCTs) with long-term follow-up are strongly recommended to provide additional evidence supporting the use of CF plates. LEVEL OF EVIDENCE: III, systematic review.

Intramedullary nailing versus cemented plate for treating metastatic pathological fracture of the proximal humerus: a comparison study and literature review.

BACKGROUND: Pathological fracture of the humerus causes severe pain, limited use of the hand, and decreased quality of life. This study aimed to compare the outcomes of intramedullary nailing and locking plate in treating metastatic pathological fractures of the proximal humerus. METHODS: This retrospective comparison study included 45 patients (22 male, 23 female) with proximal humerus metastatic pathological fractures who underwent surgical treatment between 2011 and 2022. All data were collected from medical records and were analyzed retrospectively. Seventeen cases underwent intramedullary nailing plus cement augmentation, and 28 cases underwent locking plate plus cement augmentation. The main outcomes were pain relief, function scores, and complications. RESULTS: Among 45 patients with mean age 61.7 ± 9.7 years, 23 (51.1%) had multiple bone metastases, and 28 (62.2%) were diagnosed with impending fractures. The nailing group had significantly lower blood loss [100 (60-200) versus 500 (350-600) ml, p < 0.001] and shorter hospital stay (8.4 ± 2.6 versus 12.3 ± 4.3 days, p < 0.001) than the plating group. Average follow-up time of the nailing group was 12 months and 16.5 months for the plating group. The nailing group had higher visual analog scale (VAS) scores than the plating group, indicating greater pain relief with nailing [7 (6-8) versus 6 (5-7), p = 0.01]. Musculoskeletal Tumor Society functional scores [28 (27-29) versus 27 (26.5-28.5), p = 0.23] were comparable between groups. No complications, local recurrence, or revision surgery were reported until the last follow-up in either group. However, one case in the plating group had a humeral head collapse and fragmentation without needing revision surgery. CONCLUSIONS: Intramedullary nailing with cement augmentation is a viable option for treating proximal humerus metastatic pathological fracture, providing rigid fixation and better pain relief resulting in earlier mobility to optimize functional outcomes. Less invasive procedure with less blood loss and shorter hospital stay also benefits patients. Level of evidence Level II. Trial registration statement Not applicable.

Risk factors of fracture following curettage for bone giant cell tumors of the extremities.

BACKGROUND: Following curettage of giant cell tumor of bone (GCTB), it is common to fill the cavity with polymethylmethacrylate (PMMA) bone cement, bone allograft, or artificial bone to maintain bone strength; however, there is a 2-14% risk of postoperative fractures. We conducted this retrospective study to clarify the risk factors for fractures after curettage for GCTB of the extremities. METHODS: This study included 284 patients with GCTBs of the extremities who underwent curettage at our institutions between 1980 and 2018 after excluding patients whose cavities were not filled with anything or who had additional plate fixation. The tumor cavity was filled with PMMA bone cement alone (n = 124), PMMA bone cement and bone allograft (n = 81), bone allograft alone (n = 63), or hydroxyapatite graft alone (n = 16). RESULTS: Fractures after curettage occurred in 10 (3.5%) patients, and the median time from the curettage to fracture was 3.5 months (interquartile range [IQR], 1.8-8.3 months). The median postoperative follow-up period was 86.5 months (IQR, 50.3-118.8 months). On univariate analysis, patients who had GCTB of the proximal or distal femur (1-year fracture-free survival, 92.5%; 95% confidence interval [CI]: 85.8-96.2) presented a higher risk for postoperative fracture than those who had GCTB at another site (100%; p = 0.0005). Patients with a pathological fracture at presentation (1-year fracture-free survival, 88.2%; 95% CI: 63.2-97.0) presented a higher risk for postoperative fracture than those without a pathological fracture at presentation (97.8%; 95% CI: 95.1-99.0; p = 0.048). Patients who received bone grafting (1-year fracture-free survival, 99.4%; 95% CI: 95.7-99.9) had a lower risk of postoperative fracture than those who did not receive bone grafting (94.4%; 95% CI: 88.7-97.3; p = 0.003). CONCLUSIONS: For GCTBs of the femur, especially those with pathological fracture at presentation, bone grafting after curettage is recommended to reduce the risk of postoperative fracture. Additional plate fixation should be considered when curettage and cement filling without bone grafting are performed in patients with GCTB of the femur. This should be specially performed for those patients with a pathological fracture at presentation.

Synthetic Bone Graft Substitute for Treatment of Unicameral Bone Cysts.

BACKGROUND: Treatments for unicameral bone cysts (UBCs) have high documented failure rates (27% to 63%) because of recurrence or persistence of the cyst, similar to nonoperative management. Recent evidence suggests that filling of the defect with a synthetic bone graft substitute (SBGS) supports the weakened cortex and promotes new bone growth. A calcium sulfate, brushite, calcium phosphate, composite graft material (PRODENSE, Wright Medical, Memphis, TN) has been evaluated as a substitute for autogenous or allogenous graft in animal and human studies. The purpose of this study was to compare the rates of revision surgery in patients treated for UBCs with an SBGS compared with historical treatments with allograft or autologous bone marrow aspirate. METHODS: The authors reviewed 27 of 33 patients (age, 6 months to 21 years) an average of 121 months (range, 32 to 228) after filling of a UBC with an injection of SBGS (n=18) versus allograft or autologous bone marrow aspirate (n=9) between June 2008 and December 2017. Six patients with no follow-up were excluded. Groups did not differ in age at surgery, sex (19/27 male), history of pathologic fracture (22/27), or previous treatments (11/27). The primary outcome was the rate of revision surgery. Secondary outcomes included revision surgery-free survival as evaluated by the log-rank test, rate of postoperative fracture, persistent cysts, continued pain, and/or growth disturbance at the final follow-up. RESULTS: Seven of 9 patients treated with allograft or autograft underwent revision surgery for postoperative pathologic fracture (n=2) or resorption of the graft (n=5) compared with 2 of 18 patients injected with the SBGS, both treated for graft resorption. The use of SBGS was associated with a decreased need for revision surgery over all time periods (hazard ratio, 0.14; 95% confidence interval, 0.03-0.05). There was no significant difference between postoperative fracture (2/18 vs. 2/9), persistent cyst (7/18 vs. 5/9), pain (0/18 vs. 2/9), or growth disturbance (1/18 vs. 3/9). CONCLUSIONS: Treatment of UBCs with SBGS may decrease reoperation rates. Initial radiographic appearance after SBGS treatment shows solid structural support, followed by new bone formation. This appearance may lead to a less aggressive approach in considering revision surgery. LEVEL OF EVIDENCE: Level III-retrospective comparative study investigating the results of treatment.

Management of an Imminent Pathological Fracture of a Fibular Neomandible Via a Minimally Invasive Approach-A Case Report.

Fibular free flap reconstruction remains the workhorse of postmandibulectomy reconstruction. Dental implantation to support a dental prosthesis is a sought-after outcome when the area of resection involves tooth-bearing zones. Chronic perisoft tissue pedicle hyperplasia with secondary infection leading to gradual bone loss is a simple complication to manage in the general population, but it becomes a serious issue in the fibula mandibular reconstruction patient in that it can lead to pathological fracture of the fibula. A case of a patient with a near fracture of his fibula mandibular reconstruction, and its management via a minimally invasive approach is presented.

Denosumab versus zoledronic acid in bone disease treatment of newly diagnosed multiple myeloma: an international, double-blind, double-dummy, randomised, controlled, phase 3 study.

BACKGROUND: Multiple myeloma is characterised by monoclonal paraprotein production and osteolytic lesions, commonly leading to skeletal-related events (spinal cord compression, pathological fracture, or surgery or radiotherapy to affected bone). Denosumab, a monoclonal antibody targeting RANKL, reduces skeletal-related events associated with bone lesions or metastases in patients with advanced solid tumours. This study aimed to assess the efficacy and safety of denosumab compared with zoledronic acid for the prevention of skeletal-related events in patients with newly diagnosed multiple myeloma. METHODS: In this international, double-blind, double-dummy, randomised, active-controlled, phase 3 study, patients in 259 centres and 29 countries aged 18 years or older with symptomatic newly diagnosed multiple myeloma who had at least one documented lytic bone lesion were randomly assigned (1:1; centrally, by interactive voice response system using a fixed stratified permuted block randomisation list with a block size of four) to subcutaneous denosumab 120 mg plus intravenous placebo every 4 weeks or intravenous zoledronic acid 4 mg plus subcutaneous placebo every 4 weeks (both groups also received investigators' choice of first-line antimyeloma therapy). Stratification was by intent to undergo autologous transplantation, antimyeloma therapy, International Staging System stage, previous skeletal-related events, and region. The clinical study team and patients were masked to treatment assignments. The primary endpoint was non-inferiority of denosumab to zoledronic acid with respect to time to first skeletal-related event in the full analysis set (all randomly assigned patients). All safety endpoints were analysed in the safety analysis set, which includes all randomly assigned patients who received at least one dose of active study drug. This study is registered with ClinicalTrials.gov, number NCT01345019. FINDINGS: From May 17, 2012, to March 29, 2016, we enrolled 1718 patients and randomly assigned 859 to each treatment group. The study met the primary endpoint; denosumab was non-inferior to zoledronic acid for time to first skeletal-related event (hazard ratio 0·98, 95% CI 0·85-1·14; pnon-inferiority=0·010). 1702 patients received at least one dose of the investigational drug and were included in the safety analysis (850 patients receiving denosumab and 852 receiving zoledronic acid). The most common grade 3 or worse treatment-emergent adverse events for denosumab and zoledronic acid were neutropenia (126 [15%] vs 125 [15%]), thrombocytopenia (120 [14%] vs 103 [12%]), anaemia (100 [12%] vs 85 [10%]), febrile neutropenia (96 [11%] vs 87 [10%]), and pneumonia (65 [8%] vs 70 [8%]). Renal toxicity was reported in 85 (10%) patients in the denosumab group versus 146 (17%) in the zoledronic acid group; hypocalcaemia adverse events were reported in 144 (17%) versus 106 (12%). Incidence of osteonecrosis of the jaw was not significantly different between the denosumab and zoledronic acid groups (35 [4%] vs 24 [3%]; p=0·147). The most common serious adverse event for both treatment groups was pneumonia (71 [8%] vs 69 [8%]). One patient in the zoledronic acid group died of cardiac arrest that was deemed treatment-related. INTERPRETATION: In patients with newly diagnosed multiple myeloma, denosumab was non-inferior to zoledronic acid for time to skeletal-related events. The results from this study suggest denosumab could be an additional option for the standard of care for patients with multiple myeloma with bone disease. FUNDING: Amgen.

Surgical Approach to Bone Metastases.

PURPOSE OF REVIEW: The purpose of this review was to examine the recent changes in the surgical treatment of bone metastases and how the treatment paradigm has shifted with the improvement of adjuvant therapies. How surgery fits into the local and systemic treatment was reviewed for bone metastases in different areas. RECENT FINDINGS: The more common use of targeted chemotherapies and focused high-dose radiation have altered the treatment paradigm of bone metastases. Overall changes in the surgical treatment of bone metastases have been driven by an increased multidisciplinary approach to metastatic cancer and the awareness that one type of surgery does not work for all patients. The individual patient treatment goals dictate the surgical procedures used to achieve these goals. Advancements in adjuvant therapy-like radiation and more targeted chemotherapies have allowed for less invasive surgical approaches and therefore faster recoveries and reduced surgical morbidity for patients.

Navigating chronic pain: how taking a few wrong turns can miss sinister pathology.

Bone sarcomas are rare in childhood, and their presentation can often mimic more benign complaints or chronic musculoskeletal pain. Ewing sarcomas in particular are often diagnosed after a significant delay from the onset of symptoms. At a population level, a long diagnostic delay is not necessarily associated with worse survival , as tumours that display slow growth also tend to be less aggressive. For any specific individual however, a delayed diagnosis can result in a larger tumour that is more difficult to treat. We explore a case of Ewing sarcoma and discuss how the presenting features, approach to imaging and the role of clinician cognitive bias may have led to diagnostic delay.Ewing sarcoma is treated with chemotherapy and surgery and/or radiotherapy based on the initial site of disease, size of tumour and response to initial treatment. With current UK treatments, overall survival is approximately 70% for localised tumours and up to 20% in those with metastatic disease. Bone sarcomas usually present with deep-seated mechanical bone pain akin to toothache. The pain can be intermittent over the course of days or weeks, but pain occurring at night should be considered a red flag. Swelling may also present. On plain X-ray, bone sarcomas can demonstrate areas of bone destruction, new bone formation, periosteal inflammation and soft tissue swelling, but in some cases the changes are very subtle. Persistent unexplained symptoms require MRI to exclude tumours and detect potential benign causes that are amenable to treatment.

Molecular diagnosis in children with fractures but no extraskeletal signs of osteogenesis imperfecta.

In 26 of 94 individuals (28%) below 21 years of age who had a significant fracture history but did not have extraskeletal features of osteogenesis imperfecta (OI), we detected disease-causing mutations in OI-associated genes. INTRODUCTION: In children who have mild bone fragility but do not have extraskeletal features of OI, it can be difficult to establish a diagnosis on clinical grounds. Here, we assessed the diagnostic yield of genetic testing in this context, by sequencing a panel of genes that are associated with OI. METHODS: DNA sequence analysis was performed on 94 individuals below 21 years of age who had a significant fracture history but had white sclera and no signs of dentinogenesis imperfecta. RESULTS: Disease-causing variants were detected in 28% of individuals and affected 5 different genes. Twelve individuals had mutations in COL1A1 or COL1A2, 8 in LRP5, 4 in BMP1, and 2 in PLS3. CONCLUSIONS: DNA sequence analysis of currently known OI-associated genes identified disease-causing variants in more than a quarter of individuals with a significant fracture history but without extraskeletal manifestations of OI.

Clinical, radiographic and biochemical characteristics of adult hypophosphatasia.

In this study, we report on clinical, radiographic and biochemical characteristics of 38 patients with adult hypophosphatasia. High-resolution peripheral quantitative computed tomography showed alterations of bone microstructure in a subgroup of 14 patients. Pyridoxal-5-phosphate levels correlated with the occurrence of fractures and the number of symptoms. INTRODUCTION: Hypophosphatasia (HPP) is a rare disorder with a wide range of clinical manifestations. A reduced enzymatic activity of alkaline phosphatase (ALP) is the key marker of the disease, causing an accumulation of ALP substrates such as pyridoxal-5-phosphate (PLP). The purpose of this retrospective study was to further characterize adult onset HPP. METHODS: We assessed clinical, radiographic and laboratory characteristics of 38 adult patients with HPP. Diagnosis of HPP was established by the combination of low-serum ALP, raised PLP levels and typical symptoms and was genetically confirmed in 32 patients. Dual-energy X-ray absorptiometry (DXA) and laboratory data were available in most patients. High-resolution peripheral quantitative computed tomography (HR-pQCT) was performed in 14 patients. RESULTS: Clinical characteristics included a wide spectrum of symptoms. A history of fracture was present in 15 patients (39%). Twenty-one patients (55%) complained about recurring headaches, 23 patients (61%) had recurring muscle pain, 4 patients (11%) suffered from severe muscle weakness and 18 patients (47%) showed dental abnormalities. Z-scores assessed by DXA were only slightly reduced in most adult HPP patients. HR-pQCT of 14 patients showed microstructural changes of trabecular and cortical bone compared to reference values of healthy subjects. The occurrence of fractures and multiple symptoms (>2 typical HPP symptoms) were associated with significantly elevated levels of PLP. CONCLUSION: Adult HPP presents with a wide range of clinical symptoms and is not associated with low bone mass in general. PLP seems to be a good marker for disease severity in adult patients as its level is correlated with the occurrence of fractures and number of symptoms.

Comparison of percutaneous long bone cementoplasty with or without embedding a cement-filled catheter for painful long bone metastases with impending fracture.

OBJECTIVE: To compare the efficacy of percutaneous long bone cementoplasty (PLBC) with and without embedding a cement-filled catheter in the medullary canal (ECFC) for painful long bone metastases with impending fracture. METHODS: A retrospective study was conducted in 36 consecutive patients undergoing PLBC and ECFC combination (n = 17, group A) or PLBC alone (n = 19, group B). All patients had a high risk of impending fracture in the long bone based on Mirels' scoring system. Clinical effects were evaluated using both a pre- and a postoperative visual analogue scale (VAS) and Karnofsky performance scale (KPS). RESULTS: Overall pain relief rate with excellent (VAS 0-2) and good (VAS 2.5-4.5) results during follow-up was significantly higher in group A than in group B (88.2 % vs. 57.9 %, P<0.05). The average VAS and KPS changes in group A were significantly higher than those in group B at 1, 3 and 6 months postoperatively (P<0.05). Also, the rate of fractures of the treated long bone in group A was significantly lower than that in group B (P<0.05). CONCLUSIONS: Combined PLBC and ECFC is a safe and effective procedure for long bone metastases with impending fracture. KEY POINTS: • Metastases in long bones may cause pain and subsequent pathological fractures. • Cementoplasty resulted in significant pain relief in patients with long bone metastases. • Combination of PLBC and ECFC may reduce the incidence of fractures.

Absence of recognition of low alkaline phosphatase level in a tertiary care hospital.

SUMMARY: Low serum total alkaline phosphatase level (ALP), the hallmark for hypophosphatasia (HPP), must be recognized to provide appropriate care of the patients and to avoid antiresorptive treatment. The prevalence of persistent low ALP in a clinical setting is 0.13% and the recognition is very low (3%). INTRODUCTION: A low serum total alkaline phosphatase level is the hallmark for the diagnosis of hypophosphatasia. Although very rare, HPP must be recognized to provide appropriate treatment of non-union fractures and to avoid potentially harmful drugs, such as antiresorptive treatments. The aim of this study was to assess the recognition of persistent low ALP in a tertiary care hospital. METHODS: Between the 1st of January and the 31st of December 2013, 48,755 patients had ALP assessment in the Biochemistry Department of our hospital. Sixty-eight patients had all serum ALP values persistently below 40 IU/l. Among them, six had potential causes of secondary hypophosphatasia. We consulted the summary discharges of the 62 patients in order to check for the notation of low ALP. Patients from the departments of rheumatology and internal medicine were contacted to fulfill a questionnaire about clinical manifestations potentially related to HPP. RESULTS: 0.13% of hospitalized patients had persistently low value. They were 46.5 ± 17.7 years old, and 73% were females. The low ALP value was notified in the discharge summary for two patients (3%), without any comment. Twenty-four patients (46 + /-16 years old) were contacted. Eight patients had fractures; two had a diagnosis of rickets in the childhood; two had symptomatic chondrocalcinosis. Nine had dental abnormalities. Three were receiving a bisphosphonate; two of them had a fracture while being treated with bisphosphonate. CONCLUSION: Our study shows that low ALP is not recognized in a clinical setting in adults hospitalized in a tertiary care hospital.

Safety and Efficacy of Minimally Invasive Acetabular Stabilization for Periacetabular Metastatic Disease with Thermal Ablation and Augmented Screw Fixation.

PURPOSE: To evaluate minimally invasive acetabular stabilization (MIAS) with thermal ablation and augmented screw fixation for impending or minimally displaced fractures of the acetabulum secondary to metastatic disease. MATERIALS AND METHODS: Between February 2011 and July 2014, 13 consecutive patients underwent thermal ablation, percutaneous screw fixation, and polymethyl methacrylate augmentation for impending or nondisplaced fractures of the acetabulum secondary to metastatic disease. Functional outcomes were evaluated before and after the procedure using the Musculoskeletal Tumor Society (MSTS) scoring system. Complications, hospital length of stay, and eligibility for chemotherapy and radiation therapy were assessed. RESULTS: All procedures were technically successful with no major periprocedural complications. The mean total MSTS score improved from 23% ± 11 before MIAS to 51% ± 21 after MIAS (P < .05). The mean MSTS pain scores improved from 0% (all) to 32% ± 22 after MIAS (P < .05). The mean MSTS walking ability score improved from 22% ± 19 to 55% ± 26 after MIAS (P < .05). Two complications occurred; a patient had a minimally displaced fracture of the superior pubic ramus at the site of repair but remained ambulatory, and septic arthritis was diagnosed in another patient 12 months after repair. The average length of hospital stay was 2 days ± 3.6; six patients were discharged within 24 hours of the procedure. All patients were eligible for chemotherapy and radiation therapy immediately after the procedure. CONCLUSIONS: MIAS is feasible, improves pain and mobility, and offers a minimally invasive alternative to open surgical reconstruction.

Advances in the Classification and Treatment of Osteogenesis Imperfecta.

Osteogenesis imperfecta (OI) is a rare disorder of type 1 collagen with 13 currently identified types attributable to inherited abnormalities in type 1 collagen amount, structure, or processing. The disease is characterized by an increased susceptibility to bony fracture. In addition to the skeletal phenotype, common additional extraskeletal manifestations include blue sclerae, dentinogenesis imperfecta, vascular fragility, and hearing loss. Medical management is focused on minimizing the morbidity of fractures, pain, and bone deformities by maximizing bone health. Along with optimizing Vitamin D status and calcium intake and physical/occupational therapy, individualized surgical treatment may be indicated. Pharmacological therapy with bisphosphonate medications is now routinely utilized for moderate to severe forms and appears to have a good safety profile and bone health benefits. New therapies with other anti-resorptives as well as anabolic agents and transforming growth factor (TGF)ß antibodies are in development. Other potential treatment modalities could include gene therapy or mesenchymal cell transplant. In the future, treatment choices will be further individualized in order to reduce disease morbidity and mortality.

Hypophosphatasia.

Hypophosphatasia is a rare disorder due to a mutation in the ALPL gene encoding the alkaline phosphatase (ALP) leading to a diminished activity of the enzyme in bone, liver, and kidney. Hypophosphatasia is a heterogeneous disease, ranging from extreme life-threatening forms revealed at birth in young infants presenting with severely impaired bone mineralization, seizures, and hypercalcemia, to young adults with premature exfoliation of their teeth without any other symptom. We will review the challenges of the clinical, biochemical, radiological, and genetic diagnosis. Schematically, the diagnosis relies on low ALP levels and, in most cases, on the genetic defect in the ALPL gene. An enzyme replacement therapy is now developed for hypophosphatasia; early results in the severe form of the disease are extremely encouraging. However, multidisciplinary care remains the core of treatment of hypophosphatasia encompassing nutritional support, adjustment of calcium and phosphate intake, monitoring of vitamin D levels, careful and personalized physical therapy, and regular dental monitoring and care.

Pathological Mandibular Fracture Associated With Diffuse Large B-Cell Lymphoma in HIV-Positive Patient.

This article describes the occurrence of diffuse large B-cell lymphoma in a 39-year-old human immunodeficiency virus-positive patient. The patient sought medical care complaining of increased volume in the right mandibular angle and imaging tests showed an extensive radiolucency with undefined boundaries compromising the mandibular border. After the incisional biopsy, the patient had a pathological fracture in the region, which was properly treated in a second surgical procedure using a 2.4-mm reconstruction plate. Immunohistochemical analysis revealed positive marking for CD3, CD79a, Ki67, and Epstein-Barr virus-encoded RNA. The treatment consisted of concurrent antiretroviral therapy with chemotherapy with rituximab combined with cyclophosphamide, doxorubicin, vincristine, and prednisone. Examinations of images (2 years postoperatively) revealed complete bone repair and absence of injury recurrence. This work is important because it describes an unusual location of diffuse large B-cell lymphoma and shows the importance of diagnosis and treatment of the injury at an early stage in order to promote the prognosis and survival of patients.

A 5-year perspective on a removable overdenture appliance for a patient with osteonecrosis of the jaw, a mandibular resection, and rampant caries.

A 78-year-old man with a history osteonecrosis of the jaw and 2 subsequent surgeries to repair a fractured mandible had a condition that contraindicates most dentoalveolar procedures to restore his failing dentition. A Snap-On-Smile overdenture restored his opportunity for natural speech, occlusal function, and esthetics.

Treatment outcome in 60 children with pathological fractures of the humerus caused by juvenile or aneurysmal bone cysts.

The treatment of pathological fractures of the humerus caused by juvenile or aneurysmal bone cysts (JBC/ABC) should be a single approach with a high success rate and low complication rate. This study evaluates how day by day treatment concepts fulfil these aims. Children below 15 years of age with a pathological fracture of the humerus caused by a JBC or ABC between 01.01.2001 and 31.12.2010, were investigated by chart review in four major paediatric trauma centres. Age, gender, fracture localisation, X-ray findings, treatment and outcome - assessed by the Capanna classification (I to IV), were analysed. 60 children [41male, 19 female; mean age: 9 years (4-14 years)] with 43 JBC and 12 ABC were included as well as five cysts, who could not be classified definitively. First treatment was non-operatively in 33 children. Of these 27 cysts did not improve; likewise the supportive installation of cortisone in six patients did not change the outcome. The first treatment consisted of elastic stable intramedullary in 13 children; up to three nail exchanges included. But only six of these reached (nearly) complete resolution (I/II). Overall the combined mechanical and biological treatment with curettage, elastic stable intramedullary nailing, (artificial) bone substitute and in some cases growth factors was performed as the 1st-line treatment in nine patients and further in 2nd or 3rd-line treatments in 13 humeral cysts. More than half of these reached a complete or nearly complete resolution of the cyst (12x I, 5x II, 1x III, 4x IV). Major complications in all operated patients were six nails not removable and two children with upper extremities length differences. Healing rates are low for non-operative treatment, elastic stable intramedullary nailing alone and by using cortisone for cysts resolution in pathological fractures of the humerus. Data support a combined mechanical and biological treatment with curettage, elastic stable intramedullary nailing, (artificial) bone substitute and the use of growth factors.