Fracture in Irradiated Rat Femur: A Description of an Experimental Model and Evaluation of its Effectiveness / Fratura em fêmur irradiado de rato: Descrição de um modelo experimental e avaliação de sua eficácia

Abstract Objective We aim to describe an experimental model for studying femoral fractures in rats after exposure to ionizing radiation, demonstrating a way to apply a substance for analysis, the method for patterning fracture and irradiation, and how to evaluate its effectiveness based on radiographic studies. Methods We used 24 rats divided into 2 groups of 12 animals each. The STUDY group was exposed to ionizing radiation and treated with saline solution, and the CONTROL group was not exposed to radiation and was treated with saline solution. All animals were subjected to standardized fracture of the right femur that was fixed with intramedullary wire. The efficiency of the bone union was assessed by radiographic exam. Results Fracture healing was more efficient in bones not exposed to ionizing radiation (p = 0.012). All fractures met the criteria of being simple, diaphyseal, transverse or short oblique. Conclusion The experimental model presented is an efficient alternative for the study of fractures in irradiated bones in rats.

Resumo Objetivo Nosso objetivo é descrever um modelo experimental para estudo de fraturas de fêmur em ratos após exposição a radiação ionizante, demonstrando uma forma de aplicação de uma substância para análise, o método de padronização de fratura e irradiação e a forma de avaliação de sua eficácia com base em estudos radiográficos. Métodos Utilizamos 24 ratos divididos em dois grupos de 12 animais cada. O grupo ESTUDO foi exposto à radiação ionizante e tratado com soro fisiológico, enquanto o grupo CONTROLE não foi exposto à radiação e foi tratado com soro fisiológico. Todos os animais foram submetidos à fratura padronizada do fêmur direito e sua fixação com fio intramedular. A eficácia da consolidação óssea foi determinada por exame radiográfico. Resultados A cicatrização de fraturas foi mais eficiente em ossos não expostos à radiação ionizante (p = 0,012). Todas as fraturas atenderam aos critérios de serem simples, diafisárias, transversas ou oblíquas curtas. Conclusão O modelo experimental apresentado é uma boa alternativa para o estudo de fraturas em ossos irradiados em ratos.

Multiple Myeloma (Part 1) - Update on Epidemiology, Diagnostic Criteria, Systemic Treatment and Prognosis / Mieloma múltiplo (Parte 1) - Atualização sobre epidemiología, critérios diagnósticos, tratamento sistêmico e prognóstico

Abstract Multiple myeloma (MM) is a hematological malignancy characterized by unregulated and clonal proliferation of plasma cells in the bone marrow; these cells produce and secrete an anomalous monoclonal immunoglobulin, or a fragment of this, called M protein. The clinical manifestations of MM result from the proliferation of these plasmocytes, the excessive production of monoclonal immunoglobulin and the suppression of normal humoral immunity, leading to hypercalcemia, bone destruction, renal failure, suppression of hematopoiesis and humoral immunity, increasing the risk for the development of infections. The increase in life expectancy of the world population led to a concomitant increase in the prevalence of MM, a pathology that usually affects the elderly population. The aim of this review is to update the reader on epidemiology, diagnostic criteria, differential diagnosis with other monoclonal gam-mopathies, systemic treatment and prognosis of MM.

Resumo O mieloma múltiplo (MM) constitui neoplasia maligna de origem hematológica caracterizada pela proliferação desregulada e clonal de plasmócitos na medula óssea; estas células produzem e secretam imunoglobulina monoclonal anômala, ou um fragmento desta, denominado proteína M. As manifestações clínicas do MM decorrem da proliferação destes plasmócitos, da produção excessiva de imunoglobulina monoclonal e da supressão da imunidade humoral normal, levando à hipercalcemia, destruição óssea, insuficiência renal, supressão da hematopoiese e da imunidade humoral,aumentandooriscoparaodesenvolvimento de infecções. O aumento na expectativa de vida da população mundial levou a concomitante incremento na prevalência do MM, patologia que habitualmente acomete a população idosa. O objetivo desta revisão é atualizar o leitor sobre a epidemiologia, critérios diagnósticos, diagnóstico diferencial com outras gamopatias monoclonais, tratamento sistêmico e prognóstico do MM.

Multiple Myeloma (Part 2) - Update on The Approach to Bone Disease / Mieloma múltiplo (Parte 2) - Atualização sobre a abordagem da doença óssea

Abstract The increase in life expectancy of the world population has led to a concomitant increase in the prevalence of multiple myeloma (MM), a disease that usually affects the elderly population. Bone lesions are frequent in patients with this condition, demanding an early approach, from drug treatment, through radiotherapy to orthopedic surgery (prophylactic or therapeutic) with the objective of preventing or delaying the occurrence of fracture, or, when this event has already occurred, treat it through stabilization or replacement (lesions located in the appendicular skeleton) and/or promote stabilization and spinal cord decompression (lesions located in the axial skeleton), providing rapid pain relief, return to ambulation and resocialization, returning quality of life to patients. The aim of this review isto update the reader on the findings of pathophysiology, clinical, laboratory and imaging, differential diagnosis and therapeutic approach of multiple myeloma multiple myeloma bone disease (MMBD).

Resumo O aumento da expectativa devida da população mundial levou a incremento concomitante na prevalência de mieloma múltiplo (MM), patologia que geralmente afeta a população idosa. Lesões ósseas são frequentes nos portadores desta condição, demandando abordagem precoce, desde o tratamento medicamentoso, passando pela radioterapia até a cirurgia ortopédica (profilática ou terapêutica) com os objetivos de prevenir ou retardar a ocorrência de fratura, ou, quando este evento já ocorreu, tratá-la mediante estabilização ou substituição (lesões situadas no esqueleto apendicular) e/ou promover estabilização e descompressão medular (lesões situadas no esqueleto axial), proporcionando rápido alívio da dor, retorno à deambulação e ressocialização, devolvendo a qualidade de vida aos pacientes. O objetivo desta revisão é atualizar o leitor sobre a fisiopatologia, a clínica, exames laboratoriais e de imagem, diagnóstico diferencial e abordagem terapêutica da doença óssea no mieloma múltiplo (DOMM).

Axial Axis Metastasis / Metástase do eixo axial

Abstract Axial axis metastasis remains a challenge for surgical as well as other treatment modalities, like chemotherapy, immunotherapy, and radiotherapy. It is unequivocal that surgery provides pain improvements and preservation of neurological status, but this condition remains when associated with radiotherapy and other treatment modalities. In this review, we emphasize the current forms of surgical treatment in the different regions of the spine and pelvis. The evident possibility of percutaneous treatments is related to early or late cases, and in cases in which there are greater risks and instability to conventional surgeries associated with radiotherapy and have been shown to be the appropriate option for local control of metastatic disease.

Resumo As metástases no eixo axial permanecem um desafio para o tratamento cirúrgico bem como para outras modalidades, como quimioterapia, imunoterapia e radioterapia. É inequívoco que a cirurgia proporciona melhorias na dor e na preservação do status neurológico; porém, há permanência desta condição quando associada à radioterapia e a outras modalidades de tratamento. Nesta revisão damos ênfase às formas de tratamento cirúrgico atuais nas diferentes regiões da coluna vertebral e pelve. A evidente possibilidade de tratamentos percutâneos está relacionada a casos iniciais ou tardios, bem como a casos em que há maiores riscos e instabilidade às cirurgias convencionais associadas à radioterapia, e tem se mostrado a opção adequada para o controle local da doença metastática.

Diagnostic challenges in hypophosphatasia in adult patients / Desafios do diagnóstico da hipofosfatasia em adultos

Introdução: Hipofosfatasia é um distúrbio metabólico que afeta a mineralização óssea e dentária, causada por mutações no gene ALPL, levando à deficiência enzimática da fosfatase alcalina tecido não-específica. A forma adulta caracteriza-se por fraturas atípicas do fêmur, osteomalácia, osteoporose, grave osteoartropatia, condrocalcinose e artralgia. Objetivo: Demonstrar desafios diagnósticos relacionados à hipofosfatasia através do relato de dois casos. Paciente 1: feminino, 59 anos, encaminhada para avaliação clínica devido às fraturas patológicas de difícil consolidação e osteoporose generalizada de causa genética. Relata perda dentária precoce da arcada superior, fraturas na coluna, em ombro esquerdo e no fêmur. Atualmente, queixa-se de dor crônica intensa, com uso de múltiplos medicamentos. Achados clínicos, laboratoriais e radiológicos foram compatíveis com o diagnóstico de hipofosfatasia. Paciente 2: masculino, 31 anos, filho da paciente 1, encaminhado para avaliação clínica por fratura patológica precoce em fêmur esquerdo e osteoporose não esclarecida. Atualmente relata dor e claudicação importante em membro inferior esquerdo, associado à lombalgia crônica. Confirmação do diagnóstico de hipofosfatasia por exames laboratoriais e radiológicos e sequenciamento do gene ALPL, aliados ao diagnóstico da sua genitora. Discussão: Hipofosfatasia é uma doença rara de herança autossômica dominante e recessiva. Pacientes acometidos apresentam fraturas constantes, densidade mineral óssea baixa, cicatrização óssea deficitária. É comum a hipofosfatasia ser diagnosticada erroneamente como osteopenia e/ou osteoporose primária, acarretando prejuízos ao paciente. Ressalta-se a importância da história clínica completa e dos antecedentes familiares a fim de se obter um diagnóstico precoce, garantindo, por sua vez, o adequado acompanhamento e manejo terapêutico (AU)

Introduction: hypophosphatasia is a metabolic disorder affecting bone and tooth mineralization, caused by mutations in the ALPL gene leading to enzymatic deficiency of tissue non-specific alkaline phosphatase. The adult form is characterized by atypical femur fractures, osteomalacia, osteoporosis, severe osteoarthropathy, chondrocalcinosis, and arthralgia. Objective: to demonstrate diagnostic challenges related to hypophosphatasia through the report of two cases. Patient 1: female, 59 years old, referred for clinical evaluation due to pathological fractures of difficult consolidation and generalized osteoporosis of genetic cause. She reports early tooth loss in the upper arch, fractures in the spine, left shoulder and femur. Currently, he complains of severe chronic pain, with use of multiple medications. Clinical, laboratory, and radiological findings were compatible with the diagnosis of hypophosphatasia. Patient 2:male, 31 years old, son of patient 1, referred for clinical evaluation due to an early pathological fracture in the left femur and unclear osteoporosis. He currently reports pain and significant claudication in the left lower limb, associated with chronic low back pain. Confirmation of the diagnosis of hypophasatasia by laboratory and radiological tests and sequencing of the ALPL gene combined with the diagnosis of his mother. Discussion: hypophosphatasia is a rare disease of autosomal dominant and recessive inheritance. Affected patients have constant fractures, low bone mineral density, and impaired bone healing. It is common for hypophosphatasia to be misdiagnosed as osteopenia and/or primary osteoporosis, which can be harmful to the patient. The importance of a complete clinical history and family history is emphasized in order to obtain an early diagnosis, ensuring adequate follow-up and therapeutic management (AU)

Desafios do diagnóstico da hipofosfatasia em adultos / Diagnostic challenges in hypophosphatasia in adult patients

RESUMO:Introdução: Hipofosfatasia é um distúrbio metabólico que afeta a mineralização óssea e dentária, causada por mutações no gene ALPL, levando à deficiência enzimática da fosfatase alcalina tecido não-específica. A forma adulta caracteriza-se por fraturas atípicas do fêmur, osteomalácia, osteoporose, grave osteoartropatia, condrocalcinose e artralgia. Objetivo: Demonstrar desafios diagnósticos relacionados à hipofosfatasia através do relato de dois casos. Paciente 1: feminino, 59 anos, encaminhada para avaliação clínica devido às fraturas patológicas de difícil consolidação e osteoporose generalizada de causa genética. Relata perda dentária precoce da arcada superior, fraturas na coluna, em ombro esquerdo e no fêmur. Atualmente, queixa-se de dor crônica intensa, com uso de múltiplos medicamentos. Achados clínicos, laboratoriais e radiológicos foram compatíveis com o diagnóstico de hipofosfatasia. Paciente 2: masculino, 31 anos, filho da paciente 1, encaminhado para avaliação clínica por fratura patológica precoce em fêmur esquerdo e osteoporose não esclarecida. Atualmente relata dor e claudicação importante em membro inferior esquerdo, associado à lombalgia crônica. Confirmação do diagnóstico de hipofosfatasia por exames laboratoriais e radiológicos e sequenciamento do gene ALPL, aliados ao diagnóstico da sua genitora. Discussão: Hipofosfatasia é uma doença rara de herança autossômica dominante e recessiva. Pacientes acometidos apresentam fraturas constantes, densidade mineral óssea baixa, cicatrização óssea deficitária. É comum a hipofosfatasia ser diagnosticada erroneamente como osteopenia e/ou osteoporose primária, acarretando prejuízos ao paciente. Ressalta-se a importância da história clínica completa e dos antecedentes familiares a fim de se obter um diagnóstico precoce, garantindo, por sua vez, o adequado acompanhamento e manejo terapêutico. (AU)

ABSTRACT: Introduction: hypophosphatasia is a metabolic disorder affecting bone and tooth mineralization, caused by mutations in the ALPL gene leading to enzymatic deficiency of tissue non-specific alkaline phosphatase. The adult form is characterized by atypical femur fractures, osteomalacia, osteoporosis, severe osteoarthropathy, chondrocalcinosis, and arthralgia. Objective: to demonstrate diagnostic challenges related to hypophosphatasia through the report of two cases. Patient 1: female, 59 years old, referred for clinical evaluation due to pathological fractures of difficult consolidation and generalized osteoporosis of genetic cause. She reports early tooth loss in the upper arch, fractures in the spine, left shoulder and femur. Currently, he complains of severe chronic pain, with use of multiple medications. Clinical, laboratory, and radiological findings were compatible with the diagnosis of hypophosphatasia. Patient 2:male, 31 years old, son of patient 1, referred for clinical evaluation due to an early pathological fracture in the left femur and unclear osteoporosis. He currently reports pain and significant claudication in the left lower limb, associated with chronic low back pain. Confirmation of the diagnosis of hypophasatasia by laboratory and radiological tests and sequencing of the ALPL gene combined with the diagnosis of his mother. Discussion: hypophosphatasia is a rare disease of autosomal dominant and recessive inheritance. Affected patients have constant fractures, low bone mineral density, and impaired bone healing. It is common for hypophosphatasia to be misdiagnosed as osteopenia and/or primary osteoporosis, which can be harmful to the patient. The importance of a complete clinical history and family history is emphasized in order to obtain an early diagnosis, ensuring adequate follow-up and therapeutic management. (AU)

Revisión narrativa: unidad de ortogeriatría, la propuesta para reducir los desenlaces adversos en adultos mayores con fracturas de cadera por fragilidad / Narrative review: Orthogeriatrics unit, the proposal to reduce adverse outcomes in older adults with fragility fractures

Introducción Las fracturas de cadera son un problema de salud pública debido a su asociación con altas tasas de mortalidad a un año, deterioro funcional y empeoramiento de la calidad de vida. Un enfoque multidisciplinario mejora los resultados de los pacientes adultos mayores con fracturas por fragilidad. Objetivo Realizar una revisión narrativa para evaluar la evidencia de las unidades de ortogeriatría como estrategia para reducir desenlaces adversos en adultos mayores con fracturas de cadera por fragilidad. Materiales y métodos Se realizó una búsqueda de la literatura en las siguientes bases de datos: PubMed,Cochrane y Scielo, con los términos: "hip fracture" or "fragility fracture" AND "multidisciplinary team" or "Geriatric co management" or AND "orthogeriatric" or "ortho-geriatric". Resultados El manejo por ortogeriatría reduce el tiempo de evaluación pre-quirúrgico, complicaciones postoperatorias y la estancia hospitalaria. La valoración interdisciplinaria ha demostrado ser eficaz para disminuir el deterioro funcional, la mortalidad intrahospitalaria, la mortalidad a los 30 días y el riesgo de institucionalización en comparación con otros modelos de atención. Estos factores impactan sobre la optimización de recursos disminuyendo los costos en salud. Conclusión Los servicios de ortogeriatría son el modelo ideal para tratar las fracturas de cadera por fragilidad en adultos mayores. En Colombia se deben implementar más unidades de ortogeriatría para mejorar la atención intrahospitalaria, crear programas de seguimiento y rehabilitación.

Introduction Hip fracture is a public health problem due to its association with high mortality rates at one year, functional decline and worsening of quality of life. A multidisciplinary approach improves outcomes for elderly patients with fragility fracture. Objective To carry out a narrative review to evaluate the evidence of orthogeriatric units as a strategy to reduce adverse outcomes in older adults with fragility hip fractures. Material and Methods A literature search was carried out in the following databases: PubMed, Cochrane and Scielo, with the terms: "hip fracture" or "fragility fracture" AND "multidisciplinary team" or "Geriatric co management" or AND "orthogeriatric" or "ortho-geriatric". Results Care by orthogeriatrics reduces pre-operative surgical time, postoperative complications and hospital stay. Interdisciplinary assessment has been shown to be effective in reducing functional decline, in-hospital mortality, 30-day mortality, and the risk of institutionalization compared to other models of care. These factors impact on the optimization of resources, reducing health costs. Conclusion Orthogeriatric services are the ideal model to treat fragility fractures in older adults. In Colombia, more orthogeriatric units should be implemented to improve in-hospital care, create follow-up and rehabilitation programs.

Evaluation of Intramedullary Methods with Polymethylmethacrylate for Fixation of Bone Lesions of the Extremities / Avaliação de métodos intramedulares com polimetilmetacrilato para fixação em lesões ósseas de extremidades

Abstract Objective To evaluate the better potential and functional results in pain control in the treatment of pathological fractures and prophylactic fixation with an intramedullary nail associated with polymethylmethacrylate, compared with the utilization of an intramedullary nail in long bone tumor lesions. Methods From January 2012 to September 2017, 38 patients with 42 pathological lesions (fractures or impending fractures according to the Mirels criteria) were treated surgically. Sixteen patients allocated to the control group underwent a locked intramedullary nail fixation, and 22 patients with pathological lesions were allocated to treatment with an intramedullary nail associated with polymethylmethacrylate. Postoperatively, the patients were submitted to the Musculoskeletal Tumor Society (MSTS) rating scale, radiographic assessment, and to the assessment of events and complications related to the treatment. Results The evaluation using the MSTS questionnaire showed better functional results in the group associated with polymethylmethacrylate, in comparison with the control group, which obtained an average score of 16.375 out of a maximum of 30 points (54.6%). The group studied with association with polymethylmethacrylate obtained a mean of 22.36 points (74.5%). The procedure proved to be safe, with similar complication and severity rates, and with no statistical difference in comparison with the standard treatment. Conclusion Stabilization of tumor lesions with an internal fixation associated with the polymethylmethacrylate demonstrated early rehabilitation and improved the quality of life, allowing rapid functional recovery. The use of polymethylmethacrylate has advantages such as reduced bleeding, tumor necrosis and higher mechanical stability.

Resumo Objetivo Avaliar o potencial melhor resultado funcional e controle álgico no tratamento de fraturas patológicas e fixações profiláticas tratadas com haste intramedular associada ao polimetilmetacrilato (PMMA) em comparação com o uso de haste intramedular em lesões tumorais em ossos longos. Métodos De janeiro de 2012 a setembro de 2017, 38 pacientes com 42 lesões patológicas (fraturas ou iminência segundo os critérios de Mirels) foram tratados cirurgicamente. Dezesseis pacientes submetidos a fixação com haste intramedular bloqueada foram alocados ao grupo controle e 22 pacientes com lesões patológicas foram alocados para tratamento com haste intramedular associada ao PMMA. No pósoperatório, foi realizada a submissão dos pacientes ao escore da Musculoskeletal Tumor Society (MSTS, na sigla em inglês) e à avaliação radiográfica do tratamento realizado, assim como à avaliação de intercorrências e complicações relacionadas ao tratamento. Resultados A avaliação através do questionário MSTS demonstrou melhor resultado funcional do grupo associado com PMMA quando comparado com o grupo controle, o qual obteve uma pontuação média de 16,375 em um máximo de 30 pontos (54,6%), enquanto o grupo em estudo com associação do PMMA obteve uma média de 22,36 pontos (74,5%). O procedimento mostrou-se seguro, taxas de complicações e gravidade semelhantes e sem diferença estatística quando comparado com o tratamento padrão. Conclusão A estabilização de lesões tumorais com fixação associada ao PMMA demonstrou reabilitação precoce e melhora na qualidade de vida, permitindo rápida recuperação funcional. A utilização do PMMA apresenta vantagens como diminuição do sangramento e da necrose tumoral e maior estabilidade mecânica.

Osteopetrosis autosómica dominante benigna: Reporte de caso / Autosomal dominant osteopetrosis: Case report

La osteopetrosis es una enfermedad infrecuente, se caracteriza por el incremento de la densidad ósea observada en las radiografías, resultado de anormalidades en la diferen- ciación y función de los osteoclastos que les incapacita para la resorción ósea y cartilaginosa, formándose huesos más densos, pero más frágiles. Objetivo: describir la Osteopetrosis Auto- sómica Dominante mostrando nuestra experiencia el método de tratamiento. Con un amplio conocimiento de esta patología, los hallazgos radiográficos característicos y los manejos tera- péuticos adecuados podremos lograr un diagnóstico precoz certero y una mejor sobrevida de los pacientes. Reporte de caso: Paciente femenina de 13 años, con historia de fracturas espontáneas a repetición en los antebrazos principalmente, la madre niega antecedentes de trauma; asimismo refiere observar retraso en el crecimiento de la paciente, por lo cual acude al hospital regional de occidente, Quetzaltenango, Guatemala, para evaluación. Se le realizan radiografías en proyección anteroposterior (AP) y lateral de cráneo, de extremidades superio- res e inferiores y de columna dorsal evidenciando en las radiografías de cráneo aumento de la densidad ósea y aumento de grosor de la misma, en la columna dorsal se observó aumento de la esclerosis a nivel de las placas terminales superiores e inferiores de los cuerpos vertebrales, dando la típica apariencia de "vertebra en sándwich", signo patognomónico de esta enferme- dad. La paciente recibió tratamiento con prednisolona, vitamina D y calcio en dosis de acuerdo a las medidas antropométricas de la paciente y control médico por año para evaluar estado clínico...(AU)

Enfermedad de Chester Erdheim costal: reporte de caso y revisión de la literatura / Chester Erdheim costal disease: case report and literature review

Introducción. La enfermedad de Erdheim Chester es un trastorno infrecuente, de etiología no clara y edad de presentación variable, que se caracteriza por la proliferación anormal de histiocitos no Langerhans. Puede presentarse de forma asintomática, con manifestaciones locales o como complicaciones sistémicas. El diagnóstico se basa en las condiciones clínicas del paciente, estudios imagenológicos y el estudio anatomopatológico, mediante el cual se confirma la enfermedad. Caso clínico. Paciente masculino con una lesión lítica en el séptimo arco costal, inicialmente asintomático y posteriormente con dolor torácico persistente. Se realizó resección y reconstrucción de la pared torácica, con resolución de la sintomatología y adecuada evolución posoperatoria. El resultado de la patología confirmó el diagnóstico de enfermedad de Erdheim Chester. Discusión. Se presenta el caso de un paciente con compromiso costal y fractura patológica como localización y manifestación inusual de la enfermedad de Erdheim Chester, tratado quirúrgicamente de forma adecuada, y sin aparición de recidiva. Debido a la diversidad de síntomas que pueden presentar y a la afectación de múltiples órganos, además de los estudios imagenológicos, la clínica debe orientar los exámenes complementarios, como electro o ecocardiograma, resonancia nuclear magnética, o angioTAC. En los pacientes asintomáticos se ha recomendado un manejo expectante y en los sintomáticos el tratamiento médico con glucocorticoides, inhibidores de BRAF e interferón alfa. El papel de la cirugía no ha sido estudiado a profundidad

Introduction. Erdheim Chester disease is a rare disorder of unclear etiology and variable age of presentation, characterized by abnormal proliferation of non-Langerhans histiocytes. It can present asymptomatic, with local manifestations or as systemic complications. The diagnosis is based on the clinical conditions of the patient, imaging studies, and the pathological study, through which the disease is confirmed.Clinical case. A male patient with a lytic lesion in the seventh costal arch, initially asymptomatic and later with persistent chest pain, underwent resection and reconstruction of the chest wall, with resolution of the symptoms and adequate postoperative evolution. The pathology report confirmed the diagnosis of Erdheim Chester's disease.Discussion. The case of a patient with rib involvement and pathological fracture is presented as an unusual location and manifestation of Erdheim Chester's disease, treated surgically appropriately, and without recurrence. Due to the diversity of symptoms that they can present and the involvement of multiple organs, in addition to imaging studies, the clinic should direct complementary tests such as electrocardiogram or echocardiogram, magnetic resonance imaging, or CT angiography. Expectant management has been recommended in asymptomatic patients, and medical treatment with glucocorticoids, BRAF inhibitors, and interferon alfa in symptomatic patients. The role of surgery has not been studied in depth

Result analysis of percutaneous core needle biopsy for bone tumors in upper limbs with pathological fracture / 中国骨伤

OBJECTIVE@#To analyze the results of percutaneous core needle biopsy for bone tumors in upper limbs with pathologic fracture and to find the possible factors that could impact the results.@*METHODS@#The including criteria for this study was the patients who had received percutaneous core needle biopsy and definitive surgery, whose tumor was located at upper limb with pathologic fracture. From January 2015 to December 2019, seventy-seven patients were enrolled. There were 55 males and 22 females. The median age was 27 years old (range:5 to 88 years old). The tumor located at humerus in 67 cases, radius in 8 cases and ulna in 2 cases. If the pathologic diagnosis of core needle biopsy was the same with the definitive surgery, it was defined as "correct". If the pathologic diagnosis of biopsy for benign or malignant was right but the exact diagnostic name was not the same with definitive surgery, it was defined as "supportive". If the pathologic diagnosis of biopsy for benign or malignant was not correct, it was defined as "wrong". We retrospectively analyzed the accuracy and impact factors for core needle biopsy.@*RESULTS@#The result was "correct" in 63 cases(81.8%), "supportive" in 14 cases(18.2%), and "wrong" in 0 cases. We analyzed the gender, age, location, fracture displacement, the destroyed type for bone tumor, soft tissue mass, fluid area in the tumor as the factors. The results showed the rate for "correct" was significantly higher when the tumor had soft tissue mass (@*CONCLUSION@#The accuracy of percutaneous core needle biopsy for upper limb bone tumor with pathologic is high and acceptable. The biopsy chosen the soft tissue mass area can increase the accuracy.

Tratamiento médico exitoso de una osteomielitis multifocal neonatal producida por la bacteria Staphylococcus aureus meticilina resistente (SAMR). Revisión de caso / Successful medical treatment of neonatal multifocal osteomyelitis caused by methicillin-resistant Staphylococcus aureus (MRSA). Case review

RESUMEN La osteomielitis (OM) en el periodo neonatal es una condición rara y trascendental. Su incidencia estimada corresponde al 0,1 de casos por cada 100 admisiones hospitalarias. El subdiagnóstico es frecuente dada la presentación paucisintomática de este grupo etario, que lleva a demoras en el inicio del tratamiento y puede conducir a secuelas permanentes. Presentamos un caso de osteomielitis multifocal con fracturas asociadas, causada por la bacteria Staphylococcus aureus meticilina resistente (SAMR), secundaria a una infección asociada a catéter en un neonato nacido a las 37 semanas de gestación, con antecedente de asfixia perinatal y encefalopatía hipóxica isquémica. El caso debutó con sepsis, edema y artritis, pero el diagnóstico fue confirmado con ultrasonografía y radiografías que evidenciaron múltiples fracturas patológicas. Al no ser candidato para el manejo quirúrgico, se concertó una adecuación del esfuerzo terapéutico dirigiendo el manejo a la terapia antibiótica de amplio espectro y al control del dolor con adecuada respuesta clínica.

SUMMARY Osteomyelitis in the neonatal period is a rare but serious condition. Its estimated incidence corresponds to 0.1 case per 100 hospital admissions. Underdiagnosis is frequent given the paucisymtomatic presentation of this age group, leading to delays in the beginning of treatment and potential permanent sequelae. We present a case of osteomyelitis with associated fractures, caused by meticillin resistant Staphylococcus aureus secondary to catheterrelated bloodstream infection, in a term neonate with history of perinatal asphyxia and hipoxicischaemic encephalopathy. The newborn debuted with sepsis associated with edema and arthritis and the diagnosis was confirmed with ultrasonography and radiographs that showed multiple pathologic fractures. Not being a candidate for surgical management, the therapeutic effort was adapted, directing management to broadspectrum antibiotic therapy and pain control, with adequate clinical response.

Caso Clínico: PlasmocitomaÓseo Solitario / Clinical Case: Solitary Bone Plasmacytoma

Introducción: Aproximadamente un 5% de las neoplasias de células plasmáticas corresponden al Plasmocitoma óseo solitario (POS)con una edad de presentación después de la 5ta década de la vida, por lo que su aparición en edades tempranas es muy rara, siendo este el motivo principal de la presentación de este caso. Caso clínico: El caso corresponde a una mujer de 19años de edad con antecedente fractura patológicade fémur derecho. Labiopsia óseareportó un Plasmocitoma Solitariocon Inmunohistoquímica Plasma Cell positivo, Kappa positivo, CD38 y CD138 positivo.La biopsia de Medula ósea fue normal asícomo las concentraciones de los diferentes tipos de inmunoglobulinay beta 2 microgobulina. Calcio sérico normal. La electroforesis de proteínas gamma fue de 0.98 g/dl (14.3 %).La serie ósea reportó la presencia de lesión osteolítica de aproximadamente 8 cm a nivel de diáfisis proximal de fémur derecho con fractura patológica. El resto del esqueleto sin lesiones. Evolución: La paciente recibió 4 ciclos de quimioterapia con el protocolo VISTA (Bortezomib 1.8 mg, Ciclofosfamida 500 mg, Dexametasona 20 mg, Doxopeg 15 mg), luego del primer ciclo, fue intervenida quirúrgicamente con Osteosíntesis con sistema de compresión dinámica (DCS) y un relleno de fémur proximal derecho.La paciente terminó su tratamiento de Quimioterapia en noviembre del 2015 y concomitantemente recibió radioterapia a dosis de 50 Gys, la misma que culminóen octubredel mismo año. Desenlace: La paciente ha permanecido en controles con periodos de dolor a nivel de cadera que han sido manejados con analgesia y con controles radiológicos sin evidencia de recidiva. Actualmente paciente seencuentra asintomática, recibiendo Bifosfonatos por 24 meses. Conclusión: Este caso pone de relieve la importancia de la búsqueda de patología oncológica en un a mujer joven con fractura patológica

Introduction: Approximately 5% of plasma cellneoplasms correspond to solitary bone plasmacytoma (POS) with an age of presentation after the 5th decade of life, so its appearance at an early age is very rare, this being the reason of the presentation of this case. Clinical case: The case corresponds to a 19-year-old woman with a history of a pathological fracture of the right femur. The bone biopsy reported a Solitary Plasmacytoma with positive Plasma Cell Immunohistochemistry, positive Kappa, CD38 and positive CD138. The bone marrow biopsy was normal as well as the concentrations of the different types of immunoglobulin and beta 2 microgobulin. Normal serum calcium. Gamma protein electrophoresis was 0.98 g / dl (14.3%). The bone series reported the presence of an osteolytic lesion of approximately 8 cm at the proximaldiaphysis of the right femur with a pathological fracture. The rest of the skeleton without injuries. Evolution: The patient received 4 cycles of chemotherapy with the VISTA protocol (Bortezomib 1.8 mg, Cyclophosphamide 500 mg, Dexamethasone 20 mg, Doxopeg 15 mg), after the first cycle, she underwent surgery with Osteosynthesis with a dynamic compression system (DCS) and a right proximal femur filling. The patient finished her chemotherapy treatment in November 2015 and concomitantly received radiotherapy at a dose of 50 Gys, which ended in October of the same year. Outcome: The patient has remained in controls with periods of pain at the hip level that have been managed with analgesia and with radiological controls without evidence of recurrence. Currently the patient is asymptomatic, receiving bisphosphonates for 24 months. Conclusion: This case highlights the importance of searching for oncological pathology in a young woman with a pathological fracture

Osteogénesis imperfecta: revisión de la literatura actual / Osteogenesis imperfecta: review of current literature

La osteogénesis imperfecta (OI) abarca un grupo de enfermedades de origen genético, caracterizadas por un aumento de la fragilidad ósea, debido a una alteración cualitativa y cuantitativa de la masa ósea, que conlleva un riesgo mayor de recurrencia de fracturas y produce deformidades de diversa magnitud, especialmente en los huesos largos. La incidencia en el ámbito mundial es de aproximadamente 1 en 12 000 a 15 000 nacidos vivos. En nuestro país esta patología es poco conocida y además se lleva un sub-registro de los casos que se presentan. Conclusión: La OI es el trastorno hereditario más común del tejido conectivo; en el 90 % de los casos, se debe a las mutaciones de colágeno tipo I. Los tipos I a V son autosómicos dominantes y del VI al XIII son autosómicos recesivos. Las intervenciones terapéuticas existentes no son curativas. El manejo con bifosfonatos puede mejorar significativamente la historia natural de la enfermedad de tipo III y IV.

Osteogenesis imperfecta (OI) covers a group of diseases of genetic origin, is characterized by an increase in fragility of the bones due to a qualitative and quantitative alteration of bone mass, which entails a higher risk of fractures and produce deformities specially of long bones. The incidence worldwide is approximately 1 in 12,000 to 15,000 live births. In our country this pathology is little known and there is an under-reporting of OI cases. Conclusion: OI is the most common inherited disorder of connective tissue. 90% is due to mutations of type I collagen. Type I to type V are autosomal dominant and type VI to type XIII are autosomal recessive. The existing therapeutic interventions are not curative. Management with bisphosphonates can improve the natural history of type III and type IV disease.

Treatment of Pathological Humerus-Shaft Tumoral Fractures with Rigid Static Interlocking Intramedullary Nail-22 Years of Experience / Tratamento de fraturas patológicas tumorais diafisárias do úmero com haste intramedular rígida bloqueada estática - Experiência de 22 anos

Abstract Objective This was a retrospective observational study in patients submitted to intramedullary nail fixation after established or impeding pathological humerus-shaft tumoral fracture in the context of disseminated tumoral disease along 22 years of experience at the same institution. Methods Sample with 82 patients and 86 humeral fixations with unreamed rigid interlocking static intramedullary nail by the antegrade or retrograde approaches. Results The most prevalent primary tumors were breast carcinoma (30.49%), multiple myeloma (24.39%), lung adenocarcinoma (8.54%), and renal cell carcinoma (6.10%). The average surgical time was 90.16 ± 42.98 minutes (40-135 minutes). All of the patients reported improvement in arm pain and the mean Musculoskeletal Tumor Society (MSTS) score rose from 26% in the preoperative period to 72.6% in the evaluation performed in patients still alive 3 months after the surgery. The overall survival was 69.50% 3 months after the surgery, 56.10% at 6 months, 26.70% at 1 year, and 11.90% at 2 years. No death was related to the surgery or its complications. There were only 4 surgery-related complications, 1 intraoperative and 3 late, corresponding to a 4.65% complication risk. Conclusion Closed unreamed static interlocking intramedullary nailing (both in the antegrade or retrograde approaches) of the humerus is a fast, safe, effective, and low morbidity procedure to treat pathological fractures of the humerus shaft, assuring a stable arm fixation and consequently improving function and quality of life in these patients during their short life expectation.

Resumo Objetivo Estudo retrospectivo observacional em pacientes submetidos à fixação com haste intramedular de fratura patológica tumoral consumada ou iminente da diáfise do úmero em contexto de doença tumoral disseminada ao longo de 22 anos na mesma instituição. Métodos Amostra com 82 pacientes e 86 fixações do úmero com haste intramedular rígida bloqueada estática não fresada anterógrada ou retrógrada. Resultados Os tumores primários mais prevalentes foram carcinoma da mama (30,49%), mieloma múltiplo (24,39%), adenocarcinoma do pulmão (8,54%) e carcinoma das células renais (6,10%). O tempo médio de intervenção cirúrgica para fixação com haste foi 90,16 ± 42,98 minutos (40-135). Todos os pacientes referiram melhoria das queixas álgicas no nível do braço e velicou-semelhoria do scoreMSTSmédio de 26% no pré-operatório para 72,6% na avaliação efetuada nos pacientes ainda vivos aos três meses de pós-operatório. A taxa de sobrevivência aos três meses após a cirurgia foi de 69,50%, 56,10% aos seis meses, 26,70% em um ano e 11,90% em dois anos. Nenhuma dasmortes decorreu da cirurgia ou de complicações dela. Apenas se registaramquatro complicações relacionadas com a cirurgia, uma intraoperatória e três tardias, corresponderam a risco de complicações de 4,65%. Conclusão O uso de haste intramedular não fresada estática bloqueada (anterógrado ou retrógrado) no úmero é um método rápido, seguro, eficaz e com baixa morbilidade no tratamento das fraturas patológicas da diáfise umeral, garante fixação estável do braço e consequentemente melhora a funcionalidade e a qualidade de vida desses pacientes durante a sua curta expectativa de vida.

Percutaneous Vertebroplasty versus Conservative Treatment Using a Transdermal Fentanyl Patch for Osteoporotic Vertebral Compression Fractures

OBJECTIVE: Although surgical intervention, such as percutaneous vertebroplasty (PVP), is the standard treatment for osteoporotic vertebral compression fractures (OVCFs), its effectiveness and safety are unclear. Therefore, this study compared the safety and efficacy of conservative treatment with that of PVP for acute OVCFs.METHODS: Patients with single-level OVCFs who were treated conservatively with a transdermal fentanyl patch (TFP) or with PVP between March 2013 and December 2017 and followed-up for more than 1 year were retrospectively evaluated. Patients with pathologic fractures, fractures of more than two columns, or a history of PVP were excluded. Clinical outcomes (visual analog scale [VAS] scores) and radiographic factors were evaluated, including changes in the compression rate of the corresponding vertebral body at onset and after 12 months, sagittal Cobb angle at onset and after 6 and 12 months, and the incidence of adjacent compression fractures.RESULTS: Of the 131 patients evaluated, 75 were treated conservatively using TFPs and 56 underwent PVP. We divided the patients into TFP and PVP groups. Their baseline characteristics (including sex, level of fracture, and bone mineral density T-scores) were similar, but the TFP group was significantly younger. The overall VAS score for pain showed a greater decrease during the first month (1 week after PVP) in the PVP group but remained similar in the two groups thereafter. The compression rate after 12 months increased in the TFP group but decreased in the PVP group. Five patients in the PVP group, but none in the TFP group, experienced adjacent compression fractures within 12 months.CONCLUSION: We compared clinical and radiological outcomes between the TFP and PVP groups. The immediate pain reduction effect was superior in the PVP group, but the final clinical outcome was similar. Although the PVP group had a better-preserved compression rate than the TFP group for 1 year, the development of adjacent fractures was significantly higher. Although TFPs seemed to be beneficial in reducing the failure rate of conservative treatment, the possibility of side effects (22.6%, 17 out of 75 patients, in this study) should be carefully monitored.

Percutaneous Vertebroplasty versus Conservative Treatment Using a Transdermal Fentanyl Patch for Osteoporotic Vertebral Compression Fractures

OBJECTIVE: Although surgical intervention, such as percutaneous vertebroplasty (PVP), is the standard treatment for osteoporotic vertebral compression fractures (OVCFs), its effectiveness and safety are unclear. Therefore, this study compared the safety and efficacy of conservative treatment with that of PVP for acute OVCFs. METHODS: Patients with single-level OVCFs who were treated conservatively with a transdermal fentanyl patch (TFP) or with PVP between March 2013 and December 2017 and followed-up for more than 1 year were retrospectively evaluated. Patients with pathologic fractures, fractures of more than two columns, or a history of PVP were excluded. Clinical outcomes (visual analog scale [VAS] scores) and radiographic factors were evaluated, including changes in the compression rate of the corresponding vertebral body at onset and after 12 months, sagittal Cobb angle at onset and after 6 and 12 months, and the incidence of adjacent compression fractures. RESULTS: Of the 131 patients evaluated, 75 were treated conservatively using TFPs and 56 underwent PVP. We divided the patients into TFP and PVP groups. Their baseline characteristics (including sex, level of fracture, and bone mineral density T-scores) were similar, but the TFP group was significantly younger. The overall VAS score for pain showed a greater decrease during the first month (1 week after PVP) in the PVP group but remained similar in the two groups thereafter. The compression rate after 12 months increased in the TFP group but decreased in the PVP group. Five patients in the PVP group, but none in the TFP group, experienced adjacent compression fractures within 12 months. CONCLUSION: We compared clinical and radiological outcomes between the TFP and PVP groups. The immediate pain reduction effect was superior in the PVP group, but the final clinical outcome was similar. Although the PVP group had a better-preserved compression rate than the TFP group for 1 year, the development of adjacent fractures was significantly higher. Although TFPs seemed to be beneficial in reducing the failure rate of conservative treatment, the possibility of side effects (22.6%, 17 out of 75 patients, in this study) should be carefully monitored.

Assessment of Bone Mineral Density of Patients with Spinocerebellar Ataxia Type 3

OBJECTIVE: Machado-Joseph disease (MJD) is a spinocerebellar ataxia, and osteoporosis is a multifactor disease that may affect patients with neurologic conditions. The frequency of osteoporosis among MJD patients, however, has not been studied. The purpose of this study is to evaluate bone mineral density (BMD) and identify correlations between clinical factors and frequency of vertebral fractures in patients with MJD. METHODS: Clinical data, lumbar X-rays and BMD data were obtained in 30 patients with MJD. RESULTS: Ten patients (33.3%) showed low BMD in at least one of the sites studied based on Z-scores. The Z-score correlated directly with body mass index, and the femoral neck Z-score was inversely correlated with cytosine-adenine-guanine (CAG) expansion. There was no correlation between BMD and other clinical factors. Forty-three percent of the patients reported previous pathologic fractures. Five patients (16.7%) had at least one fracture detected by lumbar X-ray. CONCLUSION: Low BMD and fractures are frequent among MJD patients, and careful management of BMD may be beneficial for these patients.

Acute and Delayed Epidural Hematoma After Total Spondylectomy for a Metastatic Spinal Tumor: A Case Report / 대한척추외과학회지

STUDY DESIGN: Case report. OBJECTIVES: We report a case of recurrent spinal epidural hematoma after total spondylectomy for a metastatic spinal tumor. SUMMARY OF LITERATURE REVIEW: Postoperative epidural hematoma is rare, and no case of delayed epidural hematoma after hematoma removal has been reported. MATERIALS AND METHODS: A 74-year-old woman experienced a ninth thoracic vertebral (T9) pathologic fracture caused by a metastatic spinal tumor and underwent total spondylectomy. Immediate postoperative epidural hematoma occurred and neurological symptoms appeared. After hematoma removal, the symptom improved. Ten days after surgery, the neurological symptoms worsened again. Spine magnetic resonance imaging showed delayed epidural hematoma. Hematoma removal was done again. RESULTS: The patient's neurological symptoms improved after delayed hematoma removal. CONCLUSIONS: Delayed hematoma that cause neurological symptoms may occur after primary hematoma removal. If neurological symptoms recur after hematoma removal, the surgeon should consider the possibility of hematoma recurrence. Before total spondylectomy surgery, preoperative embolization is recommended.

The Clinical Outcome of Dedifferentiated Chondrosarcoma / 대한정형외과학회잡지

PURPOSE: A dedifferentiated chondrosarcoma is a rare lethal tumor characterized by a low grade chondrosarcoma juxtaposed with a high grade dedifferentiated sarcoma, such as osteosarcoma, fibrosarcoma. The aim of our study was to document the clinical manifestation and oncologic outcomes of a dedifferentiated chondrosarcoma. MATERIALS AND METHODS: This study identified 11 patients who were diagnosed and treated for dedifferentiated chondrosarcoma between January 2007 and December 2016. The identified cohort was then reviewed regarding age, sex, symptom onset, tumor location, magnetic resonance imagings (MRIs), surgical margin, and pathologic diagnosis. The time to local recurrence and/or metastasis, follow-up duration, and the patients' final status were analyzed. RESULTS: The patients were comprised of 7 males and 4 females with a mean age of 54 years (range, 33–80 years). The location of the tumor was in the femur in 6 cases, pelvis in 4 cases, and metatarsal in 1 case. The average tumor diameter was 12.7 cm (range, 6.0–26.1 cm). At the time of diagnosis, 2 patients showed pathologic fracture; 1 patient was Enecking stage IIA, 9 patients were stage IIB, and 1 patient was stage III. Eight patients were classified as a primary dedifferentiated chondrosarcoma and 3 patients were secondary. One of the primary lesions was misinterpreted initially as a low grade chondroid lesion by MRI and underwent curettage. Local recurrence occurred in 8 cases and distant metastasis occurred in 10 cases with a mean duration of 8 months (range, 2–23 months) and 7 months (range, 1–32 months), respectively. The three-year overall survival of patients with dedifferentiated chondrosarcoma was 18%, and 10 patients died due to disease progression. CONCLUSION: Dedifferentiated chondrosarcoma developed lung metastases in the early period of the clinical courses and the prognosis was dismal.