An updated review of idiopathic osteosclerosis of the jaws / Una revisión actualizada de la osteoesclerosis idiopática en los maxilares

Objective: The aim of this study was to update the concepts of the diagnosis of idiopathic osteosclerosis (IO) of the jaws by digital panoramic radiographs and cone beam tomography and describe the impact of this disease on oral and general health. Methods: A search of the main databases of dental medical research was carried out using the search terms "osteosclerosis, panoramic radiography, cone beam computed tomography, jaws". Articles without language restriction until September 30, 2020 were identified. The prevalence and clinical and radiographic characteristics of IO of the jaws were examined in 2D and 3D imaging studies, as well as the interaction during treatments in the various dental specialties. Results: We analyzed the current situation regarding the diagnosis of IO, with an update of the diagnostic criteria used to accurately identify IO in the latest generation imaging studies, as well determine its possible interactions in oral an general health. Conclusions: It is important to have a clear differential diagnosis of IO and be able to distinguish different radiopacities in the maxilla. Accurate reporting and monitoring of the morphometric characteristics are necessary taking into account the impact the presence of IO of the jaws has on future dental treatments. (AU)

Objetivo: El propósito de esta investigación fue actualizar las consideraciones para el diagnóstico de la osteoesclerosis idiopática en radiografías panorámicas digitales y tomografías de haz cónico, a fin de determinar sus implicancias en la salud oral y general. Métodos: Se realizó las pesquisas en las principales bases de datos de investigación médica estomatológica, utilizando las palabras "osteoesclerosis", "radiografía panorámica", "tomografía computarizada de haz cónico" y "mandíbula". Se identificaron artículos sin restricción de idioma, desde las primeras publicaciones hasta el 30 de septiembre del 2020. Se examinaron la prevalencia, las características clínicas y radiográficas en estudios imagenológicos de dos y tres dimensiones, así como su interacción durante los tratamientos realizados en las diversas especialidades estomatológicas. Resultados: La información obtenida nos permitió analizar la situación actual con respecto al diagnóstico de la OI y actualizar los criterios diagnósticos para una identificación certera de la OI en los estudios imagenológicos de última generación, así como sus posibles interacciones en la salud oral y general. Conclusiones: Es importante tener un criterio diagnóstico diferencial claro al distinguir las diferentes radiopacidades como la OI, que se pueden presentar en los maxilares, mediante un registro preciso de sus características morfométricas y seguimiento en el tiempo, teniendo en cuenta su existencia y sus implicancias en los tratamientos dentales a futuro. (AU)

Frecuencia de aparición de la osteoesclerosis idiopática y la osteítis condensante en maxilares inferiores en radiografías panorrámicas / Frecuency of the appearance of idiopathic osteoesclerosis and condensing osteotis in lower jaws in panoramic X-Rays

Objetivo: Determinar la frecuencia de la osteoesclerosis idiopática (OI) y la osteítis condensante (OC) en radiografías panorámicas. Metodología: Se estudiaron 1500 radiografías panorámicas de pacientes de ambos sexos, con edades cronológicas entre 20 y 88 años. Se identificó la presencia de condensaciones óseas (CO); la distribución de acuerdo con la edad, el sexo, la localización y la relación con los dientes; y si eran solitarias o múltiples. Resultados: Se observaron 183 CO, con una prevalencia del 12,4%. Del total de casos, 113 correspondieron al sexo femenino (61,7%) y 70 al masculino (38,2%). La OI fue verificada en 127 sujetos (8,5% del total de la muestra), mientras que 56 presentaron OC (3,7%). La OI fue más frecuente entre la tercera y cuarta décadas de vida, mientras que se verificó un mayor número de OC por encima de los 40 años (p = 0,002). Respecto del sexo, ambos tipos de CO fueron más frecuentes en las mujeres, lo que fue estadísticamente significativo (p = 0,005). Las CO se localizaron con mayor frecuencia en la zona molar derecha, molar izquierda y premolar derecha; en cuanto a la relación con los dientes, fueron observadas en un número más alto en apical, separadas y en la zona apical e interradicular. La presentación única de las CO fue más prevalente según el sexo o el grupo etario. Conclusiones: Las características radiográficas de las CO estudiadas permiten distinguirlas de otras patologías. Su alta prevalencia indica que deben ser consideradas en el manejo clínico del paciente para orientar un plan de tratamiento adecuado. (AU)

Objective:To determine the frequency of idiopathic osteoesclerosis (IO) and condensing osteitis (CO) in panoramic radiographs. Methodology: 1500 panoramic radiographs of patients of both sexes, with chronological ages between 20 and 88 years, were studied. The presence of bone condensations (BC), distribution according to age, sex, location, relationship with the teeth, and whether they were solitary or multiple were evaluated. Results: 183 BC (12.4%) were observed with 113 in women (61.7%) and 70 in men (38.2%). IO was verified in 127 subjects (8.5% of the total sample), while 56 presented CO (3.7%). IO was more frequent between the third and fourth decade of life, while a greater number of CO was found over 40 years of age (p = 0.002); regarding sex, both types of BC were significantly more frequent in women (p = 0.005). BC were more frequently located in the right molar, left molar, and right premolar zone; Regarding the relationship with the teeth, a higher number of BC were observed in the apical, separated and in the apical and interradicular areas. The single presentation of BC was more prevalent considering sex or age. Conclusions: BC can be differentiated from other pathologies based on the radiographic characteristics. Taking into account, the high prevalence of BC, they should be considered in the clinical management of patients in order to guide adequate treatment planning. (AU)

Consideraciones para la colocación de implantes dentales en pacientes con osteosclerosis idiopática. Una revisión de la literatura / Considerations for the placement of dental implants in patients with idiopapthic osteosclerosis. A review of the literature

Esta investigación realizó una revisión sobre la colocación de implantes dentales en pacientes con osteoesclerosis idiopática, para lo cual algunos investigadores proponen que se debe tener en cuenta un protocolo específico, el cual habla de un fresado con irrigación externa con suero fisiológico a 5 °C. La estabilidad del implante dental es importante para una buena osteointegración, la cual depende de la calidad ósea, la técnica quirúrgica y la superficie de contacto entre hueso e implante. La fuerza de torque para una buena estabilidad primaria, según una revisión sistemática, es de 20 Ncm a 45 Ncm. Para el éxito de la colocación de implantes dentales, se debe tener un buen diagnóstico de la zona edéntula y una correcta osteointegración. La identificación correcta de los hallazgos clínicos, como la osteoesclerosis idiopática, es importante para diferenciarla de otras patologías óseas benignas y malignas. La osteoesclerosis idiopática se caracteriza por ser asintomática, no tener etiología conocida y no tener tratamiento indicado. El lugar más común donde se presenta esta patología ósea es en el maxilar superior y la zona más prevalente es la zona premolar, aunque algunos autores han reportado la zona molar como la más prevalente para esta condensación ósea. En la actualidad, no existe suficiente investigación sobre la colocación de implantes dentales en pacientes con osteoesclerosis idiopática para determinar la fuerza de torque de inserción con la que se deberían colocar los implantes para una buena estabilidad primaria en una zona con condensación ósea. (AU)

The present review is on the placement of dental implants in patients with idiopathic osteoesclerosis. Some studies have proposed the need for a specific protocol which involves milling with external irrigation with physiological serum at 5 degrees Celsius. The stability of the dental implant is important for good osseointegration, which depends on bone quality, surgical technique and contact surface between the bone and the implant. According to a systematic review, the torque force for good primary stability is 20 to 45 Ncm. Successful placement of dental implants requires accurate diagnosis of the edentulous area and correct osseointegration. It is important to differentiate clinical findings such as idiopathic osteoesclerosis from other benign and malignant bone pathologies. Idiopathic osteosclerosis is characterized as being asymptomatic, having no known etiology, and having no indicated treatment. The most common localization of this bone pathology is in the upper jaw, with the most prevalent area being the premolar area, while some authors have reported the molar area as the most prevalent for this bone condensation. At present, there are few studies on to the force of the insertion torque with which implants should be made to achieve good primary stability in an area with bone condensation in patients with idiopathic osteosclerosis. (AU)

Síndrome POEMS: um diagnóstico desafiador / POEMS Syndrome: a challenging diagnosis

A síndrome POEMS é um distúrbio multissistêmico. Sua patogênese não está totalmente estabelecida, mas sabe-se que tem relação com fator de crescimento vascular endotelial, interleucinas e fator de necrose tumoral alfa. A idade média de incidência é 50 anos, com maior prevalência em homens. Neuropatia periférica e gamopatia monoclonal estão presentes em todos os pacientes e são consideradas critérios maiores; quando associadas a pelo menos um critério menor, estabelecem diagnóstico da síndrome. As opções de tratamento são radioterapia, corticosteroides e quimioterapia, além de transplante autólogo de células-tronco hematopoiéticas. (AU)

POEMS syndrome is a multisystem disorder. Its pathogenesis isn't fully established, but it is known to be related to endothelial vascular growth factor, interleukins, and tumoral necrosis factor alpha (TNF-α). The mean age at incidence is 50 years, with a higher prevalence in men. Peripheral neuropathy and monoclonal gammopathy are present in all patients, and are considered major criteria; when associated with at least one minor criterium, they establish the diagnosis of the syndrome. Treatment options are radiotherapy, corticosteroids, chemotherapy, as well as autologous hematopoietic stem cell transplantation. (AU)

Extensive hyperdense zone in the mandible: a case report

Hyperdense zones are considered a generic term to define an area of increased density regardless of its cause. Idiopathic hyperdense zones are referred in literature as enostosis, focal osteosclerosis, periapical osteopetrosis or bone scar and are found as imaging finding during a rutine radiograph. They have greater predilection for long bones, but can also appear in the maxillary bones in certain occasions, often located in the jaw, especially in the molar region, with an informed incidence rate that varies from 2,3 to 9,7% depending on the population in which the study is being applied. In 40% of the cases, in spite of being of idiopathic origin, they seem to be associated with patients with occlusal trauma or can be a result of a predominant development of isolated bone during bone growth. The case of a 36-year-old female patient is described, who presents hyperdensity that differs in form, location and imaging features from the commonly documented in this type of anatomical variations. It was diagnosed as idiopathic osteosclerosis, periodic imaging controls were established. The purpose of this case report is to emphasize the importance of performing an appropriate differential diagnosis among hyperdense lesions at maxillofacial level.

Maxillomandibular giant osteosclerotic lesions

Abstract Giant Osteosclerotic Lesions (GOLs) are a group of rarely reported intraosseous lesions. Their precise diagnosis is important since they can be confused with malignant neoplasms. Objective This retrospective study aimed to record and analyze the clinical and radiographic Giant Osteosclerotic Lesions (GOLs) detected in the maxillomandibular area of patients attending to our institution. Materials and Methods: Informed consent from the patients was obtained and those cases of 2.5 cm or larger lesions with radiopaque or mixed (radiolucid-radiopaque) appearance located in the maxillofacial bones were selected. Assessed parameters were: age, gender, radiographic aspect, shape, borders, size, location and relations to roots. Lesions were classified as radicular, apical, interradicular, interradicular-apical, radicular-apical or located in a previous teeth extraction area. Additionally, several osseous and dental developmental alterations (DDAs) were assessed. Results Seventeen radiopacities in 14 patients were found and were located almost exclusively in mandible and were two types: idiopathic osteosclerosis and condensing osteitis. GOLs were more frequent in females, and in the anterior and premolar zones. 94.2% of GOLs were qualified as idiopathic osteosclerosis and one case was condensing osteitis. All studied cases showed different osseous and dental developmental alterations (DDAs). The most common were: Microdontia, hypodontia, pulp stones, macrodontia and variations in the mental foramina. Conclusions GOLs must be differentiated from other radiopaque benign and malignant tumors. Condensing osteitis, was considered an anomalous osseous response induced by a chronic low-grade inflammatory stimulus. For development of idiopathic osteosclerosis, two possible mechanisms could be related. The first is modification of the normal turnover with excessive osseous deposition. The second mechanism will prevent the normal bone resorption, arresting the osseous breakdown process.

Tratamiento de la osteítis condensante en molar: informe de caso / Molar condensing osteitis treatment: Case report

En la actualidad, la principal causa por la que acuden los pacientes al odontólogo es el dolor dental, endonde la mayoría presenta un padecimiento pulpar o periapical irreversibles, que pueden estar asociados a factores traumáticos e irritativos. Sin embargo, pocosde ellos son asintomáticos, como la osteítis condensante que es escasamente mencionada en elámbito de la Endodoncia; por lo tanto, el objetivo de este caso clínico es el de proporcionar información acerca de la osteítis condensante siguiendo los lineamientos internacionales de Case Report (CARE). La osteítis condensante tiene una incidencia muy baja en pacientes y se debe diagnosticar correctamente al momento de tratar este tipo de lesiones con las diferentes herramientas de diagnóstico que se conocen. En este caso, se presenta un paciente del sexo femenino de 58 años de edad con un estado prediabético, que refiere un fractura del segundo molar inferior derecho, al cual radiográficamente se le encontróuna lesión periapical radiopaca en la raíz distal. Se muestra la secuencia del tratamiento, el manejo clínico y la rehabilitación.

At present, the main reason for patients to visit adentist is dental pain, where most of them presenta pulp or periapical irreversible condition, whichmay be associated with traumatic and irritative factors. However, few of them are asymptomatic as osteitiscondensing that is barely mentioned in thefield of endodontics. The aim of this case report isto provide information about the condensing osteitisfollowing international Case Report (CARE)guidelines. Condensing osteitis has a very low incidence in patients and should be correctly diagnosed with the different available diagnostic tools. In thiscase a 58-years-old female patient, with prediabeticstate, referred of a right lower second molar fracturewhich radiographically showed a radiopaque periapicallesion in the distal root of the molar. The sequence of treatment, clinical management and rehabilitation is presented.

Otoesclerosis: revisión de aspectos etiopatogénicos, clínico-demográficos e imagenológicos / Otosclerosis: a review of aetiopathogenic, clinical-demographic and imaging aspects

Abstract. Otosclerosis is a common cause of progressive hearing loss in the young adult population. Most of the time the diagnosis is suspected based on consistent clinical symptoms and physical examination. The role of imaging in this pathology consists of confirmation of doubtful cases, ruling out by differential diagnoses, level of affection, and the pre-postoperative evaluation of patients. High-resolution computed tomography of the temporal bone is the imaging method of choice.

Resumen. La otoesclerosis corresponde a una causa común de hipoacusia progresiva en población adulta joven. La mayoría de las veces el diagnóstico es sospechado en base a un cuadro clínico y examen físico concordantes. El rol de las imágenes en esta enfermedad consiste en la confirmación de los casos dudosos, el descarte de diagnósticos diferenciales, la graduación del compromiso y la evaluación pre- y posquirúrgica de los pacientes. La tomografía computada de alta resolución del hueso temporal es el método de estudio imagenológico de elección.

Características imaginológicas de la osteosclerosis idiopática de los maxilares evaluada con tomografía computarizada de haz cónico / Imaginological idiopathic osteosclerosis characteristic of the jaws evaluated with cone beam computed tomography

Determinar las características imaginológicas de la osteoesclerosis idiopática (OI) de los maxilares evaluada con tomografía computarizada de haz cónico (TCHC). Materiales y métodos: Se revisó 454 TCHC bimaxilares del Servicio de Radiología Oral y Maxilofacial de la sede de San Isidro del Posgrado de la Facultad de Estomatología Roberto Beltrán de la Universidad Peruana Cayetano Heredia, periodo 2011-2012. Se analizaron las variables sexo, edad, número, localización, tamaño, forma, límites, posición céfalo-caudal, posición vestíbulo-lingual y relación a la pieza dentaria de la OI de los maxilares. Resultados: La frecuencia de OIs fue del 28,6%. No encontrando diferencias entre los porcentajes de edad y sexo. El mayor porcentaje fue la región molar inferior 56,7%. No se encontró diferencias estadísticamente significativas entre las arcadas por lado. El tamaño promedio de las OIs fue de 6,84mm ± 3,1. La forma irregular fue la predominante con 80,3%, de límites definidos con 93,3%. La posición media se halló con mayor porcentaje tanto para la posición céfalocaudal como para la posición vestíbulo-lingual. Con relación a las piezas dentarias, se hallaron en zona edéntula 36,5% y separado del diente 33,1%. Conclusión: Las características imaginológicas de las OIs nos ayudan a diferenciarlas de verdaderas patologías óseas. El alto porcentaje encontrado con respecto a la frecuencia, en comparación a otras poblaciones, nos indica que los especialistas involucrados deben tenerlo en cuenta, durante el plan de tratamiento...

To determine the imaging features of idiopathic osteosclerosis (IO) characteristics of the jaws evaluated cone beam computed tomography (CBCT). Methods: 454 CBCT bimaxillary of the Department of Oral and Maxillofacial Radiology, located at the San Isidro, Post in the Dentistry Universidad Peruana Cayetano Heredia, period 2011-2012 was reviewed. The variables sex, age, number, location, size, shape, boundaries, cephalo-caudal position, bucco-lingual position and relation to the tooth of the maxillary IO were analyzed. Results: The frequency was 28.6% of Ios. No differences were found between the percentages of age and sex. The highest percentage was 56.7% the lower molar region. No statistically significant differences between the arches on each side was found. The average size of IOs was 6,84mm ± 3.1. The irregular shape was predominant with 80.3%, and the defined limits with 93.3%. The middle position with the highest percentage was found for both cephalo-caudal position to the bucco-lingual position. With relation to the tooth, were found in 36.5% edentulous area and 33.1% away from the teeth. Conclusion: The imaging features of IOs, to be able to differentiate true bone disease. Furthermore, its high percentage found with respect to frequency, compared to other populations, indicates that specialists involved should take into account during the treatment plan...

A comparative assessment of avoidable blindness and visual impairment in seven Latin American countries: prevalence, coverage, and inequality / Una evaluación comparativa de la ceguera y la deficiencia visual evitables en siete países latinoamericanos: prevalencia, cobertura y desigualdades

Objective. To conduct a comparative analysis of social inequalities in eye health and eye health care and generate baseline evidence for seven Latin American countries as a benchmarking exercise for monitoring progress toward three goals of the regional Plan of Action for the Prevention of Blindness and Visual Impairment: increasing eye health service coverage, minimizing barriers, and reducing eye health-related disease burden. Methods. Results from cross-sectional eye health surveys conducted in six Latin American countries (Argentina, El Salvador, Honduras, Panama, Peru, and Uruguay) from 2011 to 2013 and recently published national surveys in Paraguay were analyzed. The magnitude of absolute and relative inequalities between countries in five dimensions of eye health across the population gradient defined by three equity stratifiers (educational attainment, literacy, and wealth) were explored using standard exploratory data analysis techniques. Results. Overall prevalence of blindness in people 50 years old and older varied from 0.7% (95% CI: 0.4-1.0) in Argentina to 3.0% (95% CI: 2.3-3.6) in Panama. Overall prevalence of visual impairment (severe plus moderate) varied from 8.0% (95% CI: 6.5-11.0) in Uruguay to 14.3% (95% CI: 13.9-14.7) in El Salvador. The main reported cause of blindness was unoperated cataract and most cases of visual impairment were caused by uncorrected refractive error. Three countries had cataract surgical coverage of more than 90% for blind persons, and two-thirds of cataract-operated patients had good visual acuity. Conclusions. Blindness and moderate visual impairment prevalence were concentrated among the most socially disadvantaged, and cataract surgical coverage and cataract surgery optimal outcome were concentrated among the wealthiest. There is a need for policy action to increase services coverage and quality to achieve universality.

Objetivo. Realizar un análisis comparativo de las desigualdades sociales en materia de salud ocular y atención oftálmica, y generar datos probatorios de referencia de siete países latinoamericanos como un ejercicio de evaluación comparativa para vigilar el progreso hacia tres metas del Plan de Acción para la Prevención de la Ceguera y la Deficiencia Visual Evitables: el aumento de la cobertura de los servicios de salud ocular, la reducción al mínimo de las barreras y la disminución de la carga de morbilidad relacionada con la salud ocular. Métodos. Se analizaron los resultados de las encuestas transversales de salud ocular realizadas en seis países latinoamericanos (Argentina, El Salvador, Honduras, Panamá, Perú y Uruguay) desde el 2011 al 2013, y las encuestas nacionales del Paraguay recientemente publicadas. Mediante el empleo de técnicas ordinarias de análisis exploratorio de datos, se investigó la magnitud de las desigualdades absolutas y relativas entre países en cinco dimensiones de la salud ocular a través del gradiente poblacional definido por tres variables de estratificación de equidad (logro educativo, alfabetización y riqueza). Resultados. La prevalencia general de la ceguera en personas de 50 años de edad o mayores varió de 0,7% (intervalo de confianza (IC) de 95%: 0,4-1,0) en Argentina a 3,0% (IC95%: 2,3-3,6) en Panamá. La prevalencia general de la deficiencia visual (grave y moderada) varió de 8,0% (IC95%: 6,5-11,0) en Uruguay a 14,3% (IC95%: 13,9-14,7) en El Salvador. La principal causa notificada de ceguera fue la catarata no operada, mientras que la mayor parte de los casos de deficiencia visual fueron causados por un error de refracción no corregido. Tres países tenían una cobertura quirúrgica de la catarata de más de 90% para las personas ciegas, mientras que dos terceras partes de los pacientes operados de cataratas mostraban una buena agudeza visual. Conclusiones. Las prevalencias de la ceguera y la deficiencia visual moderada se concentraban en las personas más desfavorecidas socialmente, mientras que la cobertura quirúrgica de la catarata así como los resultados óptimos de esta intervención se concentraban en los más adinerados. Son necesarias acciones políticas para aumentar la cobertura y la calidad de los servicios con objeto de alcanzar la universalidad.

Could idiopathic osteosclerosis have correlations with palatally impacted maxillary canines?

Aim: To investigate the association between palatally impacted maxillary canines (PIC) andidiopathic osteosclerosis. Methods: A sample of 54 subjects (28 females and 26 males, mean ageof 12.98±1.59 years) with PIC was selected from the records of 1,650 orthodontic patients treatedat the Discipline of Orthodontics clinics at the Dental School of the Pontifical Catholic University ofParaná (PUCPR), in Curitiba, PR, Brazil. A control group of 54 subjects with normally eruptedcanines was also selected from the same files (mean age of 12.93±1.58 years). Panoramic,lateral skull, postero-anterior skull, periapical and occlusal radiographs, as well as stone casts ofthe patients were examined. The Kolmogorov-Smirnov test revealed a normal distribution ofgender and age in the groups. The results were analyzed with the Chi-square test (α=0.05).Results: There were no statistically significant differences (p>0.05) between the groups. Fourpatients from each group had idiopathic osteosclerosis (7.41%), a rate that falls in the prevalencerange reported in the literature. Conclusions: No correlation was observed between palatallyimpacted maxillary canines and idiopathic osteosclerosis.

Melorheostosis: A case report / Melorreostose - apresentacao de um caso

Melorheostosis is a rare disease (0.9/million habitants), characterized by linear hyperostosis along the cortex bone. It can affect any bone, being more frequent in long bones. The lesions tend to be segmental and unilateral. The etiology remains unknown although several theories proposed over the past year (vascular, inflammatory processes, embryonic defects or genetic). Show no significant difference between sexes or heredity. Clinical manifestations are mainly pain, deformity and joint stiffness. The diagnosis is obtained by combining the clinical findings with imaging studies (mainly radiography with typical image in "candle wax"). There is no definitive or specific treatment, being always palliative. We describe a case of a patient of twenty-four years, followed in Orthopedic consultation since age eight, with a deformity of the right side of the body. X-rays showed hyperostosis of the bones of the limbs in the right side of the body (image in "candle wax"). The patient is in physical therapy program and has a positive response to analgesia with ibuprofen.

A melorreostose é uma doença rara (0,9/milhão de habitantes), caraterizada por hiperostose linear ao longo do córtex ósseo. Pode afetar qualquer osso, mas é mais frequente nos ossos longos. As lesões tendem a ser segmentares e unilaterais. A etiologia permanece desconhecida, apesar de várias teorias propostas ao longo dos últimos anos (alterações vasculares, processos inflamatórios, defeitos embrionários ou genéticos). Não apresenta diferença significativa entre sexos ou hereditariedade. As manifestações clínicas são principalmente a dor local, a deformidade e a rigidez articular. O diagnóstico é obtido pela conjugação da clínica com os exames imagiológicos (principalmente radiografia com imagem típica em "cera derretida"). Não existe tratamento definitivo ou específico, é sempre paliativo. Descreve-se um caso clínico de um doente de 24 anos, seguido em consulta externa de ortopedia desde os 8 anos, por deformidade do hemicorpo direito. O RX revelou hiperostose dos ossos dos membros do hemicorpo direito (imagem em "cera derretida"). O doente encontra-se em vigilância clínica e em programa de fisioterapia com resposta positiva à analgesia com ibuprofeno.

Osteopetrosis: reporte de un caso en paciente de 20 años / Osteopetrosis: a case report in a 20 years old patient

Introducción: El concepto de Osteopetrosis agrupa un conjunto de enfermedades óseas caracterizadas por aumento de la densidad ósea, debido a una disfunción osteoclástica. Esto determina una insuficiente resorción ósea, provocando un aumento en la densidad ósea, osteoesclerosis generalizada, disminución de la resistencia ósea e incluso supresión de la médula ósea. Presentación del caso: Paciente de sexo masculino, de 20 años de edad, antecedentes de consanguinidad, con diagnóstico de osteopetrosis. Se destacan hidrocefalia, retraso del desarrollo psicomotor, anopsia bilateral, fracturas reiteradas, osteomielitis maxilar bilateral crónica e hipoplasia medular. Consulta frecuentemente por episodios reiterados de diátesis hemorrágica (epistaxis y gingivorragia). Radiografías revelan aumento de la densidad ósea, deformidades y esclerosis ósea difusa. Discusión: Llama la atención la prolongada sobrevivencia de este paciente, mantenido con transfusiones de eritrocitos y plaquetas frente a cada episodio hemorragíparo. Conclusiones: El diagnóstico precoz y tratamiento oportuno evitarán alteraciones irreversibles, optimizando la calidad de vida de los pacientes y sus familias.

Introduction: Osteopetrosis refers to a group of bone diseases characterized by increased bone density due to osteoclast dysfunction. This leads to insufficient bone resorption, leading to increased bone density, generalized osteosclerosis, decreased bone strength and even bone marrow suppression. Case report: Man 20 years old, history of consanguinity, diagnosticated with osteopetrosis. Stands out hydrocephalus, psychomotor developmental delay, bilateral anopsia, repeated fractures, chronic bilateral maxillary osteomyelitis, and bone marrow hypoplasia. Frequently consult by repeated episodes of hemorrhagic diathesis (epistaxis and gingivorrhagia). X-rays revealed increased bone density, bone deformities and diffuse osteosclerosis. Discussion: Calls the attention the prolonged survival of this patient, maintained with red cell and platelets transfusions after each bleeding episode. Conclusions: Early diagnosis and prompt treatment prevent irreversible damage, optimizing quality of life of patients and their families.

Melorreostosis de la mano en un paciente pediátrico / Melorheostosis of the hand in a pediatric patient

La melorreostosis es una rara displasia ósea benigna esclerosante.Su etiología es desconocida y ambos sexos pueden verse afectados. Los huesos largos de las extremidades inferiores y los tejidos blandos adyacentes son los más frecuentementeinvolucrados. La enfermedad se presenta raramente en los miembros superiores y se han comunicado muy pocos casos localizados en la mano. Comunicamos el caso de una niña de 7años de edad que presenta características típicas de melorreostosisen la mano derecha. El diagnóstico se realizó mediante radiografía convencional y gammagrafía ósea. La resonancia magnética contribuyó a la evaluación de las lesiones en partesblandas. El tratamiento médico permitió aliviar el dolor y mejorar la movilidad. Como la melorreostosis tiene una tendencia variable hacia la progresión de las lesiones y asociación conpatologías tumorales, se recomienda un estricto control periódicode estos pacientes.

Melorheostosis is a rare benign sclerosing bone dysplasia. Its etiology is unknown. Both sexes can be affected. Sites most frequently involved are the long bones of the lower limbs andthe adjacent soft tissue structures. The disease is unusual in the upper limbs, and few cases have been reported in the hand. We report the case of a 7-year-old girl who had typical featuresof melorheostosis in the right hand. Diagnosis was made by conventional radiography and bone scintigraphy. Magnetic resonance contributed to the assessment of soft tissue lesions.Medical treatment allowed pain relief and range of motion improvement. Because melorheostosis has a variable tendency towards progression and association with tumors, a close follow-up of these patients is recommended.

Prevalence of radiological findings among cases of severe secondary hyperparathyroidism / Prevalência de achados radiológicos em pacientes com grave hiperparatireoidismo secundário

CONTEXT AND OBJECTIVE: Patients with end stage renal disease (ESRD) and secondary hyperparathyroidism (HPT2) are prone to develop heterotopic calcifications and severe bone disease. Determination of the sites most commonly affected would decrease costs and patients' exposure to X-ray radiation. The aim here was to determine which skeletal sites produce most radiographic findings, in order to evaluate hemodialysis patients with HPT2, and to describe the most prevalent radiographic findings. DESIGN AND SETTING: This study was cross-sectional, conducted in one center, the Hospital Universitário Clementino Fraga Filho (HUCFF), in Rio de Janeiro, Brazil. METHODS: Whole-body radiographs were obtained from 73 chronic hemodialysis patients with indications for parathyroidectomy due to severe HPT2. The regions studied were the skull, hands, wrists, clavicles, thoracic and lumbar column, long bones and pelvis. All the radiographs were analyzed by the same two radiologists, with great experience in bone disease interpretation. RESULTS: The most common abnormality was subperiosteal bone resorption, mostly at the phalanges and distal clavicles (94 percent of patients, each). "Rugger jersey spine" sign was found in 27 percent. Pathological fractures and deformities were seen in 27 percent and 33 percent, respectively. Calcifications were presented in 80 percent, mostly at the forearm fistula (42 percent), abdominal aorta and lower limb arteries (35 percent each). Brown tumors were present in 37 percent of the patients, mostly on the face and lower limbs (9 percent each). CONCLUSION: The greatest prevalence of bone findings were found on radiographs of the hands, wrists, lateral view of the thoracic and lumbar columns and femurs. The most prevalent findings were bone resorption and ectopic calcifications.

CONTEXTO E OBJETIVO: Pacientes com hiperparatireoidismo secundário (HPT2) à insuficiência renal crônica são propensos a desenvolver calcificações ectópicas e grave doença óssea. A determinação dos sítios mais revelantes pode diminuir o custo e a exposição do paciente a radiação desnecessária. O objetivo foi determinar quais locais radiológicos apresentam mais achados radiográficos para avaliar o HPT2 em pacientes em hemodiálise, assim como os achados mais prevalentes. TIPO DE ESTUDO E LOCAL: Estudo transversal, realizado no Hospital Universitário Clementino Fraga Filho (HUCFF), no Rio de Janeiro, Brasil. MÉTODOS: Radiografias de corpo inteiro foram obtidas de 73 pacientes em hemodiálise crônica que tiveram indicação de paratireoidectomia devido a HPT2 grave. As regiões estudadas foram crânio, mãos e punhos, clavículas, coluna torácica e lombar, ossos longos e pélvis. Todas as imagens foram analisadas pelos mesmos dois radiologistas, com grande experiência na interpretação de doenças do osso. RESULTADOS: A alteração mais comum foi reabsorção óssea subperiosteal, principalmente nas falanges distais e clavículas (ambos em 94 por cento de pacientes). Sinal de "rugger jersey" foi descoberto em 27 por cento. Fratura patológica e deformidades foram visualizadas em 27 por cento e 33 por cento, respectivamente. As calcificações foram encontradas em 80 por cento dos pacientes, principalmente na fístula de antebraço (42 por cento), aorta abdominal e artérias dos membros inferiores (ambos 35 por cento). Tumores marrons estavam presentes em 37 por cento dos pacientes, principalmente na face e nos membros inferiores (ambos 9 por cento). CONCLUSÃO: As radiografias com achados mais prevalentes foram mãos e punhos, radiografia lateral da coluna torácica e lombar e fêmur. Os achados mais prevalentes foram reabsorção óssea e calcificação ectópica.

Enfermedades osteocondensantes: una nueva visión clínico-radiológica soportada en la genética y la inmunoosteología / Osteocondensant diseases: A new clinical-radiological vision supported in genetics and osteoimmunology

Las alteraciones en el remodelado óseo llevan al incremento o disminución de la masa ósea, generando daño de la microarquitectura ósea, lo cual incrementa el riesgo de fractura. Las patologías con incremento de la densidad conducen a diversos procesos osteocondensantes genéticamente dirigidos. La osteocondensación es explicada actualmente por alteración en la función del osteoclasto asociada a una deficiente función de la resorción ósea, alteración en la función del osteoblasto que genera un incremento anormal en la formación ósea, o un imbalance homeostático entre los dos procesos; la expresión clínica y radiológica de estas entidades puede darse en etapas tempranas del desarrollo o en la vida adulta dependiendo del componente autosómico recesivo o dominante respectivamente. En esta revisión, se discute la clasificación basada en el desorden funcional de las células óseas y las principales características clínicas y radiológicas que permiten un abordaje diagnóstico sencillo y aplicable en la práctica clínica.

The alterations in osseous remodeling lead to the increase or decrease of the osseous mass, generating damage to the osseous micro-architecture, which increases the risk of fracture. The pathologies with increase in osseous density lead to different genetically directed osteocondensing processes. The osteocondensing is currently explained by alteration in the function of the osteoclast, associated with a deficient function of the osseous resorption, an alteration in the osteoblast function, which generates an abnormal increase in the osseous formation, or a homeostatic imbalance between the two processes; the clinical and radiological expression of these diseases can take place in early stages of the development, or in the adult life, depending on the recessive or dominant autosomic component, respectively. In this review, the classification based on the functional disorder of the bone cell is discussed, as well as the main clinical and radiological characteristics than permit a simple and applicable diagnostic approach in the clinical practice.

Osteoesclerosis axiales. Propuesta para una nueva aproximación diagnóstica / Axial osteoesclerosis. Proposal for a new diagnostic approach

Las enfermedades osteoesclerosantes que comprometen el esqueleto axial son un grupo de enfermedades poco frecuentes que se caracterizan por aumento en la densidad ósea, producto de alteraciones primarias en la osificación endocondral o membranosa. En este artículo, realizamos un análisis crítico de la clasificación de las osteoesclerosis axiales y proponemos una nueva clasificación con el objetivo de simplificar el abordaje de estas patologías. Así mismo, describimos las características clínicas, radiológicas y de laboratorio que permiten hacer su diagnóstico diferencial.

Osteosclerosant diseases with axial involvement are a rare group of disorders characterized by a high bone mineral density secondary to impairment in the endochondral and membranous bone formation process. In this article, we analyzed the current classification of these diseases and propose a new and simple approach. In addition, we describe the main radiologic, laboratory and clinical keys to arrive to the differential diagnosis.

Conceptos actuales sobre osteopetrosis / Current concepts in osteopetrosis

Este artículo revisa los conceptos actuales en la osteopetrosis, expone la utilidad de los métodos de imagen para diagnóstico, clasificación y seguimiento. Se presentan las características de la enfermedad y la evaluación de sus complicaciones con base en casos producto de la experiencia de los autores. La osteopetrosis es una entidad poco común, con una variabilidad clínica en razón a su causa genética. La expresión fenotípica va desde un individuo asintomático hasta la muerte en la primera década de la vida. El diagnóstico está basado en los hallazgos por imagen y la tipificación genética, que es¬tablece las pautas de pronóstico y tratamiento. La radiología simple valora la afectación, la escanografía permite evaluar complicaciones y la resonancia magnética desempeña un papel importante en la mejor caracterización de la cavidad medular afectada en esta entidad. En casos graves, la terapia con transplante de medula ósea ha mostrado un notable impacto en la calidad de vida del paciente.

Derrame pleural quiloso na melorreostose: relato de caso com revisão da literatura / Chylous pleural effusion in melorheostosis a case with literature review

O objetivo desse estudo foi fazer uma revisão dos sinais clínicos e radiológicos de um caso atípico de melorreostose: uma mulher de 62 anos com alterações hiperostóticas irregular, de envolvimento bilateral, nos ossos raramente afetados como clavícula, escápula, costelas e crânio. Adicionalmente, a paciente tem múltiplos nevos e o diagnóstico foi feito devido a um quilotórax resistente a terapia e com evolução rápida para insuficiência respiratória.