An Early Onset of Myocardial Infarction Patient with Syphilis and Familial Hypercholesterolemia / 罕见病研究

Syphilis may affect the cardiovascular system, in which coronary arteries are less commonly involved. Familial hypercholesterolemia (FH) is an autosomal dominant inherited disease with elevated low-density lipoprotein-cholesterol (LDL-C) levels due to impaired LDL-C clearance. We report a young male patient with syphilis and FH. The clinical manifestations were acute myocardial infarction and high LDL-C levels. Coronary angiography and intracoronary imaging showed multiple aneurysmal ectasia and stenosis. A drug-eluting stent was implemented when recurrent restenosis occurred after two percutaneous coronary drug-eluting balloon angioplasties.

Quistes óseos aneurismáticos. Terapia celular con células mononucleares y seguimiento imagenológico en dos pacientes adolescentes / Aneurysmal bone cysts. Mononuclear cell therapy and imaging follow-up in two adolescent patients

RESUMEN Los quistes óseos aneurismáticos son frecuentes en la edad pediátrica. Para su determinación se cuenta con diversos estudios imagenológicos como la radiografía y la tomografía axial computarizada que pueden colaborar al diagnóstico diferencial con otras lesiones. Existen disímiles opciones terapéuticas, el uso de factores de crecimiento autólogos se ha considerado como alternativa eficaz. Se presentan dos pacientes consultados en el servicio de Ortopedia del Hospital Provincial Pediátrico Universitario «José Luis Miranda¼, de Villa Clara, con diagnóstico clínico e imagenológico de quiste óseo aneurismático que recibieron tratamiento mediante terapia celular con células mononucleares con buena evolución clínica y radiográfica. Esta técnica es aplicable en nuestro medio ya que no requiere de estimables recursos materiales lo que constituye una fortaleza para su implementación. Las radiografías permiten reconocer la evolución posterior al tratamiento.

ABSTRACT Aneurysmal bone cysts are common in children. For its determination, various imaging studies are available, such as radiography and computerized axial tomography, which can collaborate in the differential diagnosis with other lesions. There are dissimilar therapeutic options, the use of autologous growth factors has been considered as an effective alternative. We present two patients seen in the Orthopedics service at "José Luis Miranda" University Pediatric Hospital in Villa Clara, with a clinical and imaging diagnosis of aneurysmal bone cyst who received treatment with mononuclear cell therapy having a good clinical and radiographic evolution. This technique is applicable in our environment since it does not require considerable material resources, which constitutes a strength for its implementation. X-rays allow us to recognize the evolution after treatment.

Mandibular Aneurysmal Bone Cyst: About One Case

ABSTRACT: Aneurysmal bone cyst (ABC) is a benign multilocular expansive osteolytic pseudotumor with hematic content whose etiopathogeny is very controversial and whose histopathological diagnosis is sometimes difficult. The case of a 34 year old patient is reported, with no previous history, consulted for a right the mandibular angle and ramus swelling increasing in size, progressively hard, painless, cold, an orthopantomogram was performed in our patient completed by a facial CT scan in coronal and 3D axial sections. In view of our strong suspicion of aneurysmal cyst, we performed a fine needle puncture under general anesthesia before any operative gesture, which brought back a pure hematic liquid. We scheduled our patient for radical surgery: interrupting hemimandibulectomy of the horizontal branch at the right mandibular angle, with immediate reconstruction by maxipl ate given the extent of the tumor. Aneurysmal bone cyst was described in 1940 by Ewing and individualized by Jaffe and Lichtenstein (1942) who gave it their names. Clinically, the painless facial swelling, predominantly mandibular, is the main symptom and responsible for facial asymmetry. The paraclinical examinations requested are mainly orthopantomogram and facial CT scan. The treatment of ABC is exclusively surgical, either conservative or radical. The positive diagnosis of aneurysmal cyst is brou ght by imaging and the confirmation is anatomopathological. Regular clinical and laboratory follow-up is required to detect any recurrence. Many minimally invasive treatments are currently available.

RESUMEN: El quiste óseo aneurismático (QA) es un pseudotumor osteolítico expansivo multilocular benigno con contenido hemático cuya etiopatogenia es controvertida de difícil diagnóstico histopatológico Se reporta el caso de un paciente de 34 años, sin antecedentes previos, que consultó por aumento de tamaño del ángulo y rama mandibular derecha, progresivamente duro, indoloro, frío. Al paciente se le realizó ortopantomografía completada con tomografía computarizada facial en secciones coronal y axial 3D. Ante nuestra sospecha de quiste aneurismático, realizamos una punción con aguja fina bajo anestesia general antes de cualquier gesto operatorio, que devolvió un líquido hemático puro. Programamos al paciente para cirugía radical: hemimandibulectomía interrumpida de la rama horizontal en el ángulo mandibular derecho, con reconstrucción inmediata mediante maxiplaca dada la extensión del tumor. El quiste óseo aneurismático fue descrito en 1940 por Ewing e individualizado por Jaffe y Lichtenstein (1942) quienes le dieron sus nombres. Clínicamente, la tumefacción facial indolora, de predominio mandibular, es el síntoma principal y responsable de la asimetría facial. Los exámenes clínicos solicitados son principalmente la ortopantomografía y tomografía computarizada facial. El tratamiento del ABC es exclusivamente quirúrgico, ya sea conservador o radical. El diagnóstico positivo de quiste aneurismático se da por imagen y la confirmación es anatomopatológica. Se requiere un seguimiento clínico y de laboratorio regular para detectar cualquier recurrencia. Muchos tratamientos mínimamente invasivos están disponibles actualmente.

Tratamiento endovascular a malformación aneurismática de la Vena de Galeno / Endovascular treatment of aneurysmal malformation of the Vein of Galen

Gestante de 19 años sin antecedentes de embarazos previos, presenta a las 27 semanas en ecografía del segundo trimestre hallazgo sugestivo en el feto de MAVG; a las 38 semanas se realiza parto por cesárea sin complicaciones, se planificó angiografía cerebral con embolización. Se abordó por la arteria femoral derecha utilizando lavado heparinizado continuo y sistema de navegación coaxial se estudió la MAVG de tipo mural, se canalizo de forma selectiva a las arterias coroideas postero medial y postero lateral derecha y a la arteria coroidea posteromedial izquierda y se embolizó bajo control radiológico utilizando espirales de coils y líquido embolizante Onix. Control inmediato mostro cierre completo de conexiones fistulosas y preservación de ramas sanas.

A 19-year-old pregnant woman with no history of previous pregnancies, presented at 27 weeks in a second-trimester ultrasound suggestive finding in the fetus of MAVG; At 38 weeks, cesarean delivery was performed without complications, cerebral angiography with embolization was planned. It was approached through the right femoral artery using continuous heparinized lavage and a coaxial navigation system. The mural-type AVM was studied, it was selectively channeled to the right posteromedial and posterolateral choroidal arteries and to the left posteromedial choroidal artery and it was embolized under control. radiological using coil spirals and Onix embolizing liquid. Immediate control showed complete closure of fistulous connections and preservation of healthy branches.

Soft tissue aneurysmal bone cyst of left hemithorax: An extremely rare case

Aneurysmal bone cyst (ABC) is a benign expansile cystic lesion that can affect any bone of the skeleton, especially the femur, tibia, and humerus. Lesions with histologic features of an ABC can be originated within soft tissue in exceedingly rare cases. Extra-skeletal ABC may mimic a variety of benign and malignant lesions and can be confused with other common or rare giant cell-rich tumors of soft tissue. Clinical, radiological and histologic correlation are crucial in reaching the correct diagnosis. Here we report a case of an extra-skeletal ABC arising in left hemithorax in a 13-year-old girl and discuss the common differential diagnosis of this rare entity.

Estudio radiológico simple de quiste óseo simple y aneurismático: diagnóstico diferencial / Plain radiologic assessment of unicameral and aneurysmal bone cyst: differential diagnosis

OBJETIVO Dar a conocer nuestra experiencia en el diagnóstico diferencial de los quistes óseos simple y aneurismático mediante estudio radiológico simple. MATERIALES Y METODOS Se incluyeron pacientes menores de 20 años con radiografía de quiste óseo simple o aneurismático histológicamente confirmado pertenecientes al Registro Nacional de Tumores Óseos. Las radiografías fueron analizadas por dos radiólogos experimentados. Se compararon las variables demográficas de los pacientes, y las variables clínicas y radiológicas de ambos quistes. RESULTADOS Un total de 97 pacientes cumplieron los criterios de inclusión (65% presentaba quistes óseos simples y 35%, aneurismáticos). No se observaron diferencias en cuanto a la edad, al hueso comprometido, al tamaño de la lesión, a la expansión ósea, ni al adelgazamiento cortical. El género, la forma de presentación, la presencia de interrupción de la cortical, y la localización de la lesión en los ejes longitudinal y transversal del hueso son parámetros que podrían ser útiles en el diagnóstico diferencial de ambos quistes. DISCUSIÓN Los quistes óseos simple y aneurismático son lesiones benignas frecuentes que, de acuerdo con la literatura, serían difíciles de diferenciar únicamente con radiografía simple. La resonancia magnética permite una mejor caracterización anatómica, y aporta sensibilidad y especificidad al diagnóstico. Sin embargo, debe ser precedida por la radiografía simple, y su disponibilidad es limitada. CONCLUSIÓN Aun en centros con disponibilidad de resonancia magnética, la radiografía simple sigue siendo el estudio inicial de elección en el diagnóstico de tumores óseos. Ciertas características demográficas y radiográficas permiten orientar el diagnóstico diferencial inicial entre el quiste óseo simple y el aneurismático

OBJETIVE To describe our experience in the differential diagnosis of unicameral and aneurysmal bone cysts using plain radiography. PATIENCES AND METHODS We included patients under 20 years of age with radiographs of histologically-confirmed unicameral or aneurysmal bone cysts found on the Chilean National Bone-Tumor Registry. The radiographs were evaluated by two experienced radiologists. We compared the demographic variables of the patients, and the clinical and radiological variables of both tumors.. RESULTADOS A total of 97 patients met the inclusion criteria, 65% of whom had simple bone cysts, and 35%, aneurysmal bone cysts. No differences were found regarding age, the bone affected, the size of the lesion, bone expansion, nor cortical thinning. Gender, clinical presentation, cortical interruption, and location of the lesion on the longitudinal and transverse bone axes are parameters that could be used in the differential diagnosis of both cysts. DISCUSSION According to the literature, unicameral and aneurysmal bone cysts are frequent benign lesions that are difficult to differentiate merely through plain radiographs. Magnetic resonance imaging enables a better anatomical characterization and provides sensitivity and specificity to the diagnosis. However, its availability is limited, and it should be preceded by plain radiography. CONCLUSION Plain radiography is still the initial imaging study of choice in patients with clinical suspicion of bone tumor, even in those centers where magnetic resonance imaging is available. Certain demographic and radiological characteristics guide physicians in the differential diagnosis of unicameral and aneurysmal bone cysts.

Long-term use of denosumab in giant cell tumors and vertebral aneurysmal bone cysts / Uso prolongado de denosumab no tumor de células gigantes e cistos ósseos aneurismáticos vertebrais / Uso prolongado de denosumab en tumor de células gigantes y quistes óseos aneurismáticos vertebrales

ABSTRACT Introduction: Denosumab is a human monoclonal antibody that binds to the receptor activator of nuclear factor kB (RANKL), it is used in the treatment of Osteoporosis. The Giant Cell Tumor (GCT) and the Aneurysmal Bone Cyst (ABC) use the same RANKL, and for this reason this drug began to be used for its treatment. There is consensus on the use, dose-time and 12-month duration for Denosumab treatment of GCT. Not so for ABC. In unresectable, disabling or recurrent tumors, its use could be for life. The adverse events of the habitual use of the drug are known, but it is not known if these increase with time. The objective of the present work is to identify the possible adverse events of treatment with Denosumab for more than 12 months. Material and Method: Series of cases with a diagnosis of GCT or ABC in spine, treated with Denosumab for more than 12 months. Adverse events are: arthralgia, fatigue, spinal pain, pain in extremities, headache, hypokalaemia, hypocalcemia, osteonecrosis of the jaw, malignant transformation, pathological fractures. Results: Eight patients, 6 TCG and 2 ABC, with a mean age at diagnosis of 25,6 years; presenting a mean treatment of 4.18 years (range 1.7 - 8.7). Of 6 operated patients, 4 had recurrence (2 to 36 months after surgery). One patient had to suspend treatment due to necrosis of the jaw, another hypocalcemia, both returned to treatment when stabilized. Conclusions: A minor adverse event (hypocalcemia) and a major adverse event (jaw bone necrosis) were observed. Level of Evidence IV; Original.

RESUMO Introdução: O denosumab é um anticorpo monoclonal humano que se liga ao receptor ativador do fator nuclear kB (RANKL), sendo utilizado no tratamento da Osteoporose. O Tumor de Células Gigantes (TCG) e o Cisto Ósseo Aneurismático (CAO) utilizam o mesmo RANKL, por isso esse medicamento passou a ser utilizado para seu tratamento. Há consenso sobre o uso, o tempo de dosagem e a duração de 12 meses para o tratamento com Denosumabe de TCG. Não é assim para CAO. Em tumores irressecáveis, incapacitantes ou recorrentes, seu uso pode ser vitalício. Os eventos adversos do uso habitual do medicamento são conhecidos, mas não se sabe se aumentam com o tempo. O objetivo do presente trabalho é identificar os possíveis eventos adversos do tratamento com Denosumabe por mais de 12 meses. Material e Método: Série de casos com diagnóstico de TCG ou CAO na coluna, tratados com Denosumabe por mais de 12 meses. Os eventos adversos são: artralgia, fadiga, dor na coluna, dor nas extremidades, cefaleia, hipocalemia, hipocalcemia, osteonecrose da mandíbula, transformação maligna, fraturas patológicas. Resultados: Oito pacientes, 6 TCG e 2 LRA, com média de idade ao diagnóstico de 25,6 anos; apresentando um tratamento médio de 4,18 anos (variação 1,7 - 8,7). Dos 6 pacientes operados, 4 tiveram recorrência (2 a 36 meses após a cirurgia). Um paciente teve que suspender o tratamento por necrose da mandíbula, outro hipocalcemia, ambos voltaram ao tratamento quando estabilizados. Conclusões: Um evento adverso menor (hipocalcemia) e um evento adverso maior (necrose óssea da mandíbula) foram observados. Nível de Evidência IV; Original.

RESUMEN Introducción: El Denosumab es un anticuerpo humano monoclonal que se une al receptor activador del factor nuclear kB (RANKL), se lo utiliza en el tratamiento de Osteoporosis. El Tumor de Células Gigantes (TCG) y el Quiste Óseo Aneurismático (QOA), utilizan los mismos RANKL, y por ello se comenzó a utilizar esta droga para su tratamiento. Existe consenso en la utilización, dosis-tiempo y 12 meses de duración para el tratamiento con Denosumab del TCG. No así para el QOA. En tumores irresecables, incapacitantes o con recidiva, su uso podría ser de por vida. Se conocen los eventos adversos de la utilización habitual de la droga, pero no se sabe si estas aumentan con relación al tiempo. El objetivo del presente trabajo, es identificar los posibles eventos adversos del tratamiento con Denosumab por más de 12 meses. Material y Método: Serie de casos con diagnóstico de TCG o QOA de columna, tratados con Denosumab por más de 12 meses. Los eventos adversos son: artralgias, fatiga, raquialgia, dolor en extremidades, cefalea, hipopotasemia, hipocalcemia, osteonecrosis de mandíbula, transformación maligna, fractura patológica. Resultados: Ocho pacientes, 6 TCG y 2 QOA, con promedio de edad al diagnóstico de 25,6 años; presentando una media de tratamiento de 4.18 años (rango 1,7 - 8,7). De 6 pacientes operados, 4 presentaron recidiva (2 a 36 meses después de la cirugía). Un paciente se debió suspender el tratamiento al presentar una necrosis de mandíbula, otro hipocalcemia, ambos retornaron al tratamiento al estabilizarse. Conclusiones: Se observa un evento adverso menor (hipocalcemia) y un evento adverso mayor (necrosis ósea de mandíbula). Nivel de Evidencia IV; Original.

Characteristics and postoperative recurrence factors of aneurysmal bone cyst in children′s limbs / 中华实用儿科临床杂志

Objective:To analyze the characteristics of aneurysmal bone cysts (ABC) and related factors of postoperative recurrence, so as to improve the clinical diagnosis and treatment level of ABC in children.Methods:The clinical data of children pathologically diagnosed as ABC after surgery in the Affiliated Hospital of Zunyi Medical University from January 2010 to December 2018 were retrospectively analyzed.Clinical characteristics of the patients, including age, gender, lesion sites, and main clinical manifestations (pain, swelling, local tenderness, joint dysfunction and pathological fracture) were analyzed and summarized.Before operation, imaging Enneking staging was carried out, cyst volume was estimated, and the distance from cysts to the epiphyseal plate was measured.During surgery, patients received lesion curettage, local cautery and bone grafting, and the pathological fractures were treated with auxiliary internal fixation.Results were determined by Neer imaging grading after surgery, and grades Ⅰ and Ⅱ were postoperative recurrence.Possible recurrence factors were analyzed statistically by Fisher′ s exact test. Results:A total of 29 cases meeting the criteria were included, including 19 males and 10 females.The age ranged from 3.6 to 14.0 years old, averaging 9.2 years old; 12 patients were smaller than 10 years old and 17 patients were older than 10 years old.The cysts of 9 cases were located in proximal femur (31.0%), 5 cases in proximal humerus (17.2%), and 4 cases in proximal fibula (13.8%); The other 11 cases (37.9%) occurred in the middle and distal end; 26 cases (89.7%) had local tenderness, 25 cases (86.2%) showed varying degrees of pain, 18 cases (62.1%) presented local swelling, 15 cases (51.7%) were accompanied by joint dysfunction, and 12 cases (41.4%) were combined with pathological fractures.According to Enneking staging results, 18 cases (62.1%) were at rest stage, 7 cases (24.1%) at active stage, and 4 cases (13.8%) at invasive stage.Cyst volume was estimated to be 3.3-172.0 cm 3, with a median of about 50.8 cm 3.The distance from cysts to the epiphyseal plate was 0-85.0 mm, with a median of 20.8 mm.All children were followed up for 2.2-10.1 years (averaging 3.8 years). There were 6 cases (20.7%) of grades Ⅰ and Ⅱ according to Neer grading standard, and they suffered from recurrence about 2.5-20.3 months after surgery (averaging 12.5 months). The recurrence rate was higher in patients with cyst volume >50.8 cm 3 (42.9%, 6/14 cases) as well as in patients at active stage and invasive stage (45.5%, 5/11 cases) ( P<0.05). There was no statistical difference between the recurrence rate of ABC in different gender, age, the distance from cysts to the epiphyseal plate and pathological fractures. Conclusions:ABC is prone to occur in the proximal metaphysis of the long bones of children′s extremities.Main manifestations are pain, swelling, local tenderness and joint dysfunction, and ABC is frequently accompanied by pathological fractures.A higher postoperative recurrence rate is related to a larger cyst size and the active and invasion phases of the cyst, but gender, age, the distance from the cyst to the epiphyseal plate and pathological fractures are not significantly related to the postoperative recurrence rate.

Niños andinos con quiste óseo aneurismático tratados con polidocanol (uso fuera de etiqueta) / Andean children with aneurysmal bone cyst treated with polidocanol (use off-label)

Resumen: Introducción: El quiste óseo aneurismático es un raro tumor osteolítico benigno, pero agresivo para el que aún no existe tratamiento ideal. Los comunicados sobre tratamiento mediante escleroterapia en población pediátrica son escasos y en nuestra región más aún. El objetivo es comunicar la experiencia del tratamiento del quiste óseo aneurismático con polidocanol 3%. Material y métodos: Estudio retrospectivo, descriptivo y transversal. Período: Junio de 2017 a Junio de 2021. Inclusión: pacientes con diagnóstico histológico de quiste óseo aneurismático. Bajo anestesia general y guía fluoroscópica se realizó punción intralesional con aguja 16G a través de la cual se administró lentamente el polidocanol 3%. Datos: historia clínica. Las variables cuantitativas se expresarán en medidas de tendencia central y de dispersión; las variables cualitativas se expresarán como frecuencias o porcentajes. Resultados: Se incluyeron nueve pacientes consecutivos, todos presentaban dolor y tumoración en alguna de las extremidades. Género: tres femeninos y seis masculinos. Edad: mediana 10.5 años (rango: 2-15.1). Peso: mediana 32.8 kg (rango: 11-44.5). Total de procedimientos: 44, procedimientos por paciente: promedio 4.9 (DE: ± 2.0). Tiempo de procedimiento: promedio 33.9 minutos (DE: ± 18.3); radiación: promedio 1.34 mGy (DE: ± 1.55). Hospitalización: un día, excepto un paciente. Complicaciones: Daño de piel en un caso, no recidivas. Seguimiento: de 12 a 50 meses. Conclusión: En esta serie pediátrica el polidocanol 3% fue útil y efectivo para el tratamiento del quiste óseo aneurismático con escasas complicaciones. Una desventaja es que requiere varias sesiones y además, no se ha demostrado una diferencia significativa entre otras formas de tratamiento en términos de la tasa de recurrencia.

Abstract: Introduction: The aneurysmal bone cyst is a rare benign but aggressive osteolytic tumor for which there is still no ideal treatment, the reports on treatment by sclerotherapy in the pediatric population are scarce and in our region even less. The objective is to communicate the experience of the treatment of aneurysmal bone cyst with polydocanol 3%. Material and methods: Retrospective, descriptive and cross-sectional study. Period: June/2017 to June/2021. Inclusion: patients with histological diagnosis of aneurysmal bone cyst; Under general anesthesia and fluoroscopic guidance, intralesional puncture with 16G needle was performed through which 3% polydocanol was slowly administered. Data: medical history. Quantitative variables shall be expressed in measures of central tendency and dispersion; qualitative variables shall be expressed as frequencies or percentages. Results: Nine consecutive patients were included, all of whom had pain and tumor in one of the extremities. Gender: 3 female and 6 male. Age: median 10.5 years (range: 2-15.1). Weight: median 32.8 kg (range: 11-44.5). Total procedures: 44; procedures per patient: mean 4.9 (SD: ± 2.0). Procedure time: mean 33.9 minutes (SD: ± 18.3); radiation: mean 1.34 mGy (SD: ± 1.55). Hospitalization: one day, except one patient. Complications: skin damage in one case, no recurrences follow-up: 12 to 50 months. Conclusion: In this pediatric series, polydocanol 3% was useful and effective for the treatment of aneurysmal bone cyst, with few complications. One disadvantage is that it requires several sessions and in addition, no significant difference has been demonstrated between other forms of treatment in terms of the recurrence rate.

Cirugía de preservación valvular como alternativa en enfermedad de la raíz aórtica / Aortic Valve Sparing Surgery as Alternative in Aortic Root Disease

RESUMEN Introducción: Desde 1968, la enfermedad aneurismática de la raíz aórtica ha sido tratada mediante el remplazo con tubo valvulado. En las últimas décadas la cirugía de preservación valvular surgió y evolucionó como una opción al remplazo protésico. Objetivo: Reportar la experiencia institucional en la técnica de preservación valvular y sus resultados a largo plazo. Material y métodos: Revisión de 116 casos consecutivos con criterios de reparabilidad, intervenidos entre 2005 y 2019. Previo ecocardiograma transesofágico (ETE) y angiotomografía (AngioTC), se procedió quirúrgicamente acorde a la clasificación anatomofuncional, con la combinación de técnicas. Se realizó control intraoperatorio y conversión a remplazo según el criterio del cirujano interviniente. Se reportan las variables intraoperatorias, la morbimortalidad intrahospitalaria y la mortalidad, la libertad de insuficiencia valvular significativa y la reoperación en el seguimiento clínico y ecocardiográfico. Resultados: La edad media era 56 ± 15,6 años, varones 73%, 59% asintomáticos, intervenidos por diámetro aórtico (52 ± 11,7 mm) o progresión de valvulopatía. En el posprocedimiento, 4% de los casos resultó con insuficiencia leve o nula y 2 conversiones (1,7%); mortalidad hospitalaria 0,9%. A 10 años de seguimiento, sobrevida actuarial del 88% y libertad de insuficiencia significativa (moderada/grave) 79%. Se reintervinieron 5 casos, a un intervalo promedio de 9,1 años, libertad de reoperación de 90% a 10 años. No se registraron eventos tromboembólicos ni hemorrágicos mayores. Conclusión: las técnicas de preservación valvular aórtica, en contexto de enfermedad de la raíz, resultan una opción factible, segura y estable en el tiempo.

ABSTRACT Background: Since 1968, ascending aorta replacement with a valved conduit has been the standard practice for aortic root aneurysm. By the end of the 20th century, aortic valve sparing operation emerged and evolved as an alternative to aortic valve replacement. Objective: The aim of this study was to report our experience with aortic valve sparing technique and its long -term outcomes. Methods: A total of 116 consecutive cases with criteria of repairability operated on between 2005 and 2019 were analyzed. Preopera- tive transesophageal echocardiography (TEE) and computed tomography angiography (CTA) were used in combination to determine the aortic phenotype based on a previous anatomical and functional classification. Perioperative control was performed and conver- sion to aortic valve replacement was left to the discretion of the attending surgeon. Intraoperative variables, in-hospital morbidity and mortality, freedom from significant aortic regurgitation (AR) and reoperation in the clinical and echocardiographic follow-up were reported. Results: Mean age was 56±15.6 years and 73% were men; 59% were asymptomatic, and the reason for the intervention was the aortic diameter (52±11.7 mm) or progression of AR. After the procedure, 4% of the cases presented mild or trivial AR and 2 patients required conversion to aortic valve replacement (1.7%). In hospital mortality was 0.9%. Actuarial survival was 88% at 10 years, and 79% were free from significant (moderate/severe) AR. Five cases underwent reoperation after a mean interval of 9.1 years and free- dom from reoperation at 10 years was 90%. There were no major thromboembolic or bleeding events. Conclusion: Aortic valve sparing technique in the setting of aortic root disease is a feasible and safe option, and stable over time.

Quistes óseos de localizacion inusual como causa de lumbalgia crónica en adultos / Bone cyst of unusual location as a cause of chronic back pain in adults

Se presenta el caso de un paciente con quistes en el hueso iliaco como causa atípica de lumbalgia crónica a la cual se puede enfrentar el reumatólogo, y se hará una revisión de la literatura sobre los tipos de quistes óseos y sus diagnósticos diferenciales. También se hará mención de sus opciones de tratamiento.

The case of a patient with cysts in the iliac bone is presented as an atypical cause of chronic low back pain that the rheumatologist may face, and a review of the literature on the types of bone cysts and their differential diagnoses will be made. Mention will also be made of your treatment options.

Relevancia clínica del diagnóstico ecográfico de la malformación aneurismática en la vena de Galeno / Clinical relevance of ultrasound diagnosis of Vein of Galen aneurysmal malformation

Introducción: La malformación aneurismática en la vena de Galeno es infrecuente, pero tiene alta mortalidad en neonatos. Objetivo: Examinar la relevancia clínica del diagnóstico ecográfico de la malformación aneurismática en la vena de Galeno Métodos: Investigación observacional, prospectiva y transversal realizado en el Cardiocentro William Soler. (noviembre 1999-diciembre 2016) La muestra la conformaron 18 neonatos con diagnóstico de malformación aneurismática en la vena de Galeno variedad coroidea mediante ecografía doppler. Se configuraron dos grupos de referencia: 1) 70 niños supuestamente sanos. 2) 73 niños con malformación de la vena Galeno de diferente naturaleza que la variante coroidea estudiada. El procesamiento de la información incluyó elementos de estadística inferencial y herramientas de la medicina basada en la evidencia. Resultados: La presencia de fallo cardíaco neonatal, signos electrocardiográficos de isquemia miocárdica y detección de soplo continuo transcraneal, fueron significativamente diferentes en el grupo de estudio en relación con el grupo de referencia (p= 0,000001), con predominio en el número de pacientes del grupo estudio en todas las variables. Los resultados anteriores conjugados con diversos hallazgos ecográficos: la dilatación anómala de la vena, exceso de vasculatura aferente, reducción de los índices circulatorios encefálicos e incremento en los diámetros vasculares supraaórticos, identificaron de forma precisa la malformación aneurismática en la vena de Galeno. La evaluación del riesgo relativo reafirmó la documentación de los hallazgos expuestos. Conclusiones: La ecografía doppler, por su relevancia clínica y vínculo con otros elementos diagnósticos, es mandatoria en la detección de la malformación aneurismática en la vena de Galeno(AU)

Introduction: Vein of Galen aneurysmal malformation is not frequent but it has high mortality rates in newborns. Objective: To assess the clinical relevance of ultrasound diagnosis of Vein of Galen aneurysmal malformation. Methods: Observational, prospective and cross-sectional research conducted in William Soler Cardiocentro (November 1999- December 2016). The sample was formed by 18 newborns with diagnosis of Vein of Galen aneurysmal malformation (choroidal variety) through doppler echocardiography. Two reference groups were formed: 1) 70 supposedly healthy children; 2) 73 children with Vein of Galen malformation with a nature different to the studied choroidal variety. Processing of the information included elements of inferential statistics and tools from medicine based in evidences. Results: The presence of neonatal heart failure, electrocardiographic signs of myocardial ischemia and detection of transcranial continuous murmur were significantly different in the study group in relation with the reference group (p= 0,000001), with predominance in the number of patients of the study group in all the variables. The previous results combined with different ultrasound findings as the anomalous dilation of the vein, the excess of afferent vasculature, the reduction of encephalic circulation indexes and the increase of the supraaortic vascular diameters identified in a precise way the vein of Galen aneurysmal malformation. The assessment of the relative risk reaffirmed the information on the exposed findings. Conclusions: Doppler echography, due to its clinical importance and its links with other diagnostic elements, is mandatory in the detection of the vein of Galen aneurysmal malformation(AU)

Quiste Óseo Aneurismático en Clavícula: Reporte de un caso / Clavicle Aneurysmal Bone Cyst, Case Report

Introducción:El quiste óseo aneurismático es una neoplasia benigna poco común de aparición en edad temprana. Tiene mayor incidencia en huesos largos y en la columna vertebral. Su etiología es incierta, aunquesuele asociarse a traumatismo, probablemente debido a obstrucción venosa o a la formación de fístulas que se producen tras la contusión. Caso:En este estudio se presenta el caso de un paciente de 15 años sin antecedentes de trauma que presenta un quiste óseo aneurismático en clavícula, localización poco habitual para esta patología. Evolución: Se le brindótratamiento con terapia esclerosante con Polidocanol al 3% por 8 ocasiones con respuesta favorable.No ha requerido cirugía hasta el momento. Conclusión:El tratamientoesclerosante fue exitoso en este informe de casos

Introduction: The aneurysmal bone cyst is a rare benign neoplasm that appears at an early age. It has a higher incidence in long bones and in the spine. Its etiology is uncertain, although it is usually associated with trauma, probably due to venous obstruction or the formation of fistulas that occur after contusion. Case: This study presents the case of a 15-year-old patient with no history of trauma who presents with an aneurysmal bone cyst in the clavicle, an unusual location for this pathology. Evolution: Treatment with sclerosing therapy with 3% Polidocanol was given 8 times with a favorable response. He has not required surgery so far. Conclusion: Sclerosing treatment was successful in this case report

Emergencias hipertensivas neurológicas Aproximación diagnóstica y terapéutica con base en imágenes por resonancia magnética / Hypertensive neurological emergencies Diagnostic approach and treatment based on magnetic resonance imaging

RESUMEN El diagnóstico y el tratamiento de las complicaciones cerebrales de la emergencia hipertensiva constituyen un reto para los médicos de urgencias y terapia intensiva. Mediante una aproximación diagnóstica basada en las diferentes secuencias de las imágenes por resonancia magnética se llega al diagnóstico de encefalopatía hipertensiva, isquemia cerebral, hemorragia intracraneal, hemorragia subaracnoidea aneurismática, síndrome de encefalopatía posterior reversible y a su inmediato tratamiento ajustado a nuestro medio según las últimas guías de práctica clínica de la AHA/ASA (Asociación Americana del Corazón y Asociación Americana de Ataque Cerebrovascular).

SUMMARY The diagnosis and treatment of brain injury in patients with hypertensive emergencies is a challenge for the emergency department and intensive care doctors. The diagnosis of hypertensive encephalopathy, ischemic stroke, intracranial hemorrhage, aneurysmal subarachnoid hemorrhage and posterior reversible encephalopathy syndrome is achieved with different MRI sequences as well as their appropriate treatment according to the last AHA/ASA clinical practice guidelines.

Long-term clinical outcomes of patients with aneurysmal subarachnoid hemorrhage in Yunnan Province / 南方医科大学学报

OBJECTIVE@#To investigate the clinical outcomes of patients with aneurysmal subarachnoid hemorrhage (aSAH) after surgeries in Yunnan Province.@*METHODS@#We retrospectively analyzed the demographic features, vascular risk factors, severity at admission, and aneurysm locations in 85 patients with aSAH receiving surgical interventions in Yunnan Province. All the patients were treated by aneurysm clipping or coiling and followed up for clinical outcomes and recovery of daily activities evaluated by modified Rankin Scale (mRS) and Activities of Daily Living (ADL) scale, respectively.@*RESULTS@#Thirty-four of the patients (40.0%) underwent aneurysm clipping and 51 (60.0%) underwent aneurysm coiling. During a median follow- up period of 66.23 months (IOR, 12.03 months), 84.7% of the patients had low mRS scores, and 78.8% lived independently. The WFNS grade at admission was significantly correlated with the follow-up mRS scores (95%: 1.48-19.09, =0.011) and ADL (95%: 2.55-28.77, 0.05), but the cost of hospitalization was significantly higher in coiling group than in the clipping group ( < 0.001).@*CONCLUSIONS@#Both aging and a high WFNS grade at admission are associated with a poor prognosis of aSAH, for which aneurysm clipping and coiling have similar long- term outcomes, but for patients with a high WFNS score, aneurysm clipping is favored over coiling in terms of health economics.

Aneurysmal Patellar Bone Cyst: Case Reportt / Cisto Ósseo Aneurismático de Patela: Relato de Caso

Abstract Patellar tumors are rare. Commonly benign, giant-cell tumors and chondroblastomas are the most frequent types of this tumor. Aneurysmal bone cysts are a less common type, corresponding to less than 1% of the cases. The authors present a case of a 23-year-old male patient who presented left patellar pain and swelling for two years. The radiographic images suggested tumoral causes, and the biopsy was negative for neoplasm. The treatment approach was a complete patellectomy, without complications. The imaging follow-up showed no recurrence.

Resumo Os tumores patelares são uma condição rara. Comumente benignos, o tumor de células gigantes e o condroblastoma são os tipos mais frequentes. O tipo menos comum entre os tumores patelares é o cisto ósseo aneurismático, que corresponde a menos de 1% dos casos. Os autores relatam o caso de um paciente do sexo masculino, de 23 anos, com dor e aumento do volume da patela esquerda havia 2 anos. A radiologia sugeriu causas tumorais, e a biópsia foi negativa para neoplasia. A abordagem terapêutica escolhida foi uma patelectomia total, feita sem intercorrências. O acompanhamento com imagens não mostrou recorrência.

Aneurysmal fibrous histiocytoma

Aneurysmal fibrous histiocytoma is rare clinicopathological variant of Cutaneous Fibrous Histiocytoma. The clinical manifestation of Aneurysmal Fibrous Histiocytoma is often confusing to distinguish from other skin lesions. Most of the cases showed rapid increase in size or a history of recurrence, however histologically all are almost similar. Rarity of aneurysmal fibrous histiocytoma and high numbers of recurrence rate poses a big diagnostic challenge. Late treatment will result in a decrease until loss of function of the affected region.  In this article, author reported the case of aneurysmal fibrous histiocytoma of the hand in 7 years old girl with restricted at 2nd-3rd metacarpophalangeal joints. The patient had undergone a series of investigations until finally a wide excision was carried out. Excision tissue was performed CD 68 and CD 34  immunocytochemical smear to establish the diagnosis. It was not simple to make diagnosis aneurysmal fibrous histiocytoma. While it is benign, the lesion can appear malignant, and one should consider an excisional biopsy to rule out malignant conditions. The diagnosis had to be confirmed by histopathological and performed immunocytochemical smear. It was often necessary to take aggressive actions with wide excision and reconstruction.

Malformación aneurismática de la vena de Galeno / Aneurysms malformation of vein of Galen

Introducción: La malformación aneurismática de la vena de Galeno es una infrecuente variedad de fístula arteriovenosa cerebral de origen embriológico. Se localiza en la línea media de la fisura coroidal y tiene morfología de amplio espectro. Objetivo: Exponer información actualizada acerca del tema. Método: La información se obtuvo de la búsqueda automatizada realizada fundamentalmente en bases de datos MEDLINE, Current Contents y Scielo. Resultados: La malformación aneurismática de la vena Galeno se manifiesta con síntomas y signos derivados de la insuficiencia cardiaca severa refractaria al tratamiento médico y del daño neurológico que provoca. La entidad implica morbilidad grave y mortalidad en el periodo neonatal. La angiorresonancia es la técnica de referencia, pero la ecografía doppler es buen método de valoración. El tratamiento de elección es la embolización endovascular. Consideraciones finales: Debe existir índice elevado de sospecha para no fracasar en el diagnóstico precoz de la enfermedad. Es importante definir la anatomía de la lesión, por las implicaciones clínicas, terapéuticas y pronósticas que ello acarrea. El tratamiento exitoso sigue siendo un reto terapéutico complejo(AU)

Introduction: Vein of Galen aneurysm is a rare and congenital cerebral arteriovenous abnormality with reported incidence of 1:25 000 live births. It represents the 30 percent of the vascular congenital cerebral malformations that harm the pediatric population. Objective: To show updated information about the topic. Method: The information was obtained from the automated search mostly done in MEDLINE, CurrentContents and Scielo databases. Development: Vein of Galen malformation presents with symptoms and signs derived from the severe refractary heart failure to medical treatment and the neurologic damage it causes. That entity implies severe morbidity and mortality on the neonatal period. Angioresonance is the reference technique, but the Doppler ecography is a good valuation method. The election treatment is the endovascular embolization. Final considerations: There must be an elevated rate of suspect to do not fail on the early diagnostic of the disease. It is important to define the lesion's anatomy, because of the clinic, therapeutic and prognostic implications this represent. The successful treatment is still a complex therapeutic challenge(AU)

Monostotic craniofacial fibrous dysplasia: report of two cases with interesting histology

Fibrous dysplasia (FD) is a relatively rare osseous disease of unknown etiology, wherein the normal bone is replaced by collagen-rich tissue, comprising of fibroblasts and variably abundant immature woven bone. Clinically, it may involve a single bone or multiple bones. It commonly arises in the jaw bone, skull, rib, and proximal femur. Those arising in the skull and the jaw are together termed "craniofacial fibrous dysplasia." The differential diagnosis at this location includes meningioma and metastatic carcinoma. In this report, we highlight two diagnostically challenging cases presenting with orbital swelling and headache as the main complaints. Our first case was misinterpreted as meningioma on intraoperative squash smear, and paraffin sections revealed characteristic features of FD. The second case highlights the morphological feature of non-specific cystic degeneration occurring in FD. Radiographs in such cases show cystic swelling, which is indicative of a secondary aneurysmal bone cyst.

Aneurysmal Bone Cyst of the Skull Base ­ Case Report

Introduction Aneurysmal bone cysts (ABCs) are pseudotumoral bone lesions of unknown etiology that are also hypervascularized, benign, and locally destructive. They are rare in the base of the skull. The present case report describes a case of aneurysmal bone cyst in the sella turcica. Case Report The present study was developed at the department of neurosurgery of the Hospital Universitário Professor Alberto Antunes of the Universidade Federal de Alagoas (HUPAA-AL, in the Portuguese acronym), Maceió, state of Alagoas, Brazil, and is accompanied by a review of the literature from the PubMed database. A 17-year-old female patient with bitemporal hemianopia and intense left hemicranial headache associated with symptoms from the cranial nerves contained in the cavernous sinus. Neuroimaging evidenced a large lesion in the suprasellar region with calcification foci, sellar erosion, and extension to the cavernous sinus. The patient was submitted to a partial lesion resection and the histopathological analysis showed an aneurysmal bone cyst. Conclusion A rare case of intracranial aneurysmal bone cyst, with the important differential diagnosis from pituitary adenoma.