Displasia fibrosa monostótica / Monostotic fibrous displasia

Imágenes

Displasia fibrosa monostótica simulando proceso neoformativo intraoral en una paciente con sida. Primer caso reportado en esta población / MONOSTOTIC FIBROUS DYSPLASIA SIMULATING A NEOPROLIFERATIVE INTRAORAL NEOPLASM IN A FEMALE WITH AIDS. FIRST CASE REPORTED IN THIS POPULATION

Objetivo: Los pacientes con enfermedad por el virus de la inmunodeficiencia humana suelen presentar lesiones orales, hasta en un 50% de los casos con diagnóstico de sida. La displasia fibrosa es una lesión intra-ósea caracterizada por una alteración del crecimiento y diferenciación de los osteoblastos debida a una mutación genética. Clínicamente se caracteriza por presentar una tumoración de lento crecimiento con dolor, deformidad ósea y, en ocasiones, fracturas ante traumas mínimos. Caso clínico: Se presenta una paciente con sida y tuberculosis diseminada que desarrolló una lesión ósea tumoral , con compromiso de paladar y encía superior derecha, cuyo diagnóstico histopatológico fue de displasia fibrosa de paladar óseo y maxilar superior. Conclusión: La displasia fibrosa debe incluirse en el diagnóstico diferencial de las lesiones tumorales orales de los pacientes con enfermedad VIH/sida.

Aim: Patients with human immunodeficiency virus infection usually have oral lesions, including up to 50% of patients diagnosed with AIDS. Fibrous dysplasia is an intra-bone lesion, characterized by an alteration of the growth and differentiation of osteoblastes produced by a genetic mutation. Clinically it is characterized by presenting a tumor of slow growth with pain, bone deformity and sometimes fractures to minimal trauma. Clinical case: Here we describe a patient with AIDS and disseminated tuberculosis who developed a large tumor lesion that involve the hard palate and the maxilla. Final histopathological diagnosis was of fibrous dysplasia involving the hard palate and the upper maxilla. Conclusion: fibrous dysplasia should be included in the differential diagnosis of intraoral tumor lesions in HIV/AIDS patients.

Tratamiento multidisciplinario en un niño afectado por un quiste dentígero / Multidisciplinaty treatment in a child with dentigerous cyst

Se presenta el caso clínico de un paciente que a los 7 años de edad fue remitido a la Clínica Estomatológica Provincial Docente Mártires del Moncada de Santiago de Cuba por presentar un quiste dentígero y displasia fibrosa monostótica. Desde entonces, y durante casi 10 años, el paciente ha sido atendido por un equipo multidisciplinario odontopediátrico, que ha seguido su evolución y ha aplicado diferentes protocolos diagnósticos y terapéuticos; estos últimos han incluido intervenciones quirúrgicas, rehabilitaciones protésicas, quimioterapia, entre otros. Por la complejidad del caso y la poca frecuencia con que aparecen asociadas ambas entidades clínicas en la infancia, se decidió comunicar este artículo al gremio odontológico nacional y extranjero.

The case report of a seven years old child who was referred to Mártires del Moncada Provincial Teaching Stomatological Clinic in Santiago de Cuba, who had a dentigerous cyst and monostotic fibrous displasia is presented. Since then, and almost during 10 years, the patient has been assisted by a multidisciplinary odontopediatric team, which has followed his clinical course and has applied different diagnostic and therapeutic protocols, including surgical procedures, prosthetic rehabilitations, chemotherapy, among others. Due to the case complexity and the frequency with which both clinical entities are associated in childhood, it was decided to publish this work for the national anf foreing odontological community.

Displasia fibrosa monostótica craneofacial / Craniofacial monostotic fibrous dysplasia

La displasia fibrosa es una enfermedad benigna del hueso, de base genética y de progresión lenta, que se caracteriza por el reemplazo progresivo del hueso normal por tejido fibrótico. El proceso puede afectar un solo hueso o múltiples huesos (displasia fibrosa monostótica o poliostótica); esta última puede estar asociada con enfermedades sindrómicas, como el síndrome de McCune-Albright. Objetivo: revisar el diagnóstico y tratamiento de los pacientes con displasia fibrosa craneofacial. Materiales y métodos: se realizó una revisión de artículos científicos en revistas indexadas en línea como técnica para la recolección de información relevante en bases de datos como SciELO, Google Académico, Scopus, PubMed y DynaMed, con artículos publicados en inglés y español. El período de revisión tuvo como límite el 1 de junio de 2020. Los términos de búsqueda fueron: "displasia fibrosa", "displasia fibrosa monostótica" y "displasia fibrosa craneofacial". Se obtuvieron 33 artículos. Conclusión: la displasia fibrosa monostótica craneofacial es una enfermedad rara que afecta principalmente la mandíbula, el hueso maxilar y el cornete inferior. Puede cursar asintomática o presentar deformidades faciales, dolor y trastornos visuales. Su diagnóstico es principalmente por estudios de imágenes y la biopsia. El tratamiento quirúrgico debe ser bien planeado en cada uno de los pacientes y enfocado a deformidades faciales, se deben evitar los trastornos visuales y es necesario cuando se maligniza la lesión.

Fibrous dysplasia is a slowly progressive, genetically based, benign bone disease characterized by progressive replacement of normal bone with fibrotic tissue. The process can affect a single bone or multiple bones (monostotic or polyostotic fibrous dysplasia); the latter may be associated with syndromic diseases, such as McCune-Albright syndrome. Objective to review the diagnosis and treatment of patients with fibrous craniofacial dysplasia. Materials and methods a review of scientific articles was carried out in online indexed journals as a technique for collecting relevant information in databases such as SciELO, Google Scholar, Scopus, PubMed, and DynaMed, with articles published in English and Spanish. The review period was limited to June 1, 2020. The search terms were: "fibrous dysplasia", "monostotic fibrous dysplasia", and "craniofacial fibrous dysplasia". Obtaining 33 articles. Conclusion craniofacial monostotic fibrous dysplasia is a rare disease, mainly affecting the mandible, the maxillary bone, and the inferior turbinate. It can be asymptomatic or present facial deformities, pain and visual disorders. Its diagnosis is mainly by imaging studies and biopsy. Surgical treatment must be well planned in each of the patients, focused on facial deformities, avoiding visual disorders and is necessary when the lesion is malignant.

O Cripto-1 é um potencial biomarcador para o diagnóstico diferencial dos tumores ósseos benignos e malignos dos maxilares / Cripto-1 is a potential biomarker for the differential diagnosis of benign and malignant jaw bone tumors

O termo lesão fibro-óssea dos maxilares (LFOM) é uma designação inespecífica para um grupo de distúrbios caracterizados, morfologicamente, pela substituição do tecido ósseo por uma matriz de tecido conjuntivo fibrosa, a qual exibe neoformação de tecido ósseo com diferentes graus de mineralização. O diagnóstico preciso das LFOM não é fácil e só pode ser realizado após uma análise minuciosa dos aspectos clínicos, radiológicos e histológicos. No entanto, deve-se admitir que alguns casos desafiam a exatidão na emissão do diagnóstico. Considerando a diversidade do comportamento biológico das lesões e as pesquisas sobre a identificação de potenciais marcadores moleculares, o objetivo deste trabalho foi realizar uma análise imunohistoquímica do cripto-1 (CR-1) e da ß-catenina em uma série de casos diagnosticados microscopicamente como displasia fibrosa (DF) (n=30), fibroma ossificante central (FOC) (n=28) e osteossarcoma (OS) (n=5) armazenados nos arquivos do Serviço de Anatomia Patológica Oral de uma população brasileira. As expressões imuno-histoquímicas foram analisadas através de escore imunorreativo. Os dados obtidos foram inseridos em um arquivo do software Microsoft Excel® e, posteriormente, analisados no software Statistical Package for Social Science. Para todos os testes estatísticos utilizados, o nível de significância foi estabelecido em 5% (p<0,05). O CR-1 exibiu predominância de um padrão fortemente positivo para os casos de FOC e OS, e do padrão moderado para os casos de DF (p<0,001). A ß-catenina exibiu predominância do padrão negativo para os casos de FOC e DF, e do padrão fortemente positivo para os casos de OS (p=0,001). O teste de correlação de Spearman revelou correlação positiva entre os escores imunorreativos de CR-1 e ß-catenina. Os resultados desta pesquisa sugerem a participação do CR-1 na patogênese do FOC e OS, assim como o uso dessa proteína como potencial biomarcador molecular para o diagnóstico diferencial de LFOM (AU).

Fibro-osseous lesions of maxilar (FOLM) is a non-specific designation for a group of disorders characterized, morphologically, by replacement of bone tissue by a matrix of fibrous connective tissue, showing neoformation of bone tissue with varying degrees of mineralization. Precise diagnosis of FOLM is not easy, and requires careful analyisis of clinical, radiological and histological aspects. Even so, some cases still challenge accuracy in diagnosis. Considering the diversity of biological behaviour of the lesions and the research regarding identification of potential molecular markers, this study aims to perform immunohistochemical analysis of crypto-1 and ß-catenin in a series of cases diagnosed microscopically as fibrous dysplasia (FD) (n=30), central ossifying fibroma (COF) (n=28) and osteossarcoma (OS) (n=5), stored in archives of Oral Pathological Anatomy Service of a Brazilian population. Immunohistochemical expressions were analysed by imunorreactive score. All data obtained was inserted into a file of Microsoft Excel® software (Microsoft Corporation, USA) and then transferred to a database of SPSS® for Windows software (Statistical Package for Social Sciences; IBM, USA), version 20.0. For all statistical tests used, the significance level established was p ≤ 0.05. CR-1 showed a predominant pattern of strong positive in COF and OS cases, and a moderate positive in FD cases (p<0,001). ß-catenin showed a predominant negative pattern for COF and FD cases, and a predominant strong positive pattern for OS cases (p=0,001). Spearman correlation tests showed positive correlation of the imunoreative scores of CR-1 and ß-catenin. Those results suggests CR-1 could be involved in the pathogenesis of COF and OS, and this protein could be used as a potential molecular biomarker for diferential diagnosis of FOLM (AU).

Serie de Casos Clínicos: Displasia Osteofibrosa Craneofacial. Resolución Quirúrgica / Case Series: Craniofacial Fibrous Dysplasia. Surgical Treatment

INTRODUCCIÓN: La Displasia Fibrosa Ósea Craneofacial es una lesión ósea benigna en la que se da una sustitución de tejido óseo normal por tejido fibro-óseo. Desarrollada a partir de una mutación genética. Subordinada en variantes: monostósica y polistósica. Escasos casos son reportados sobre malignización de la patología. El Diagnóstico se realiza con la sospecha clínica y se confirma mediante exámenes de imagen y anatomopatológicos. CASO CLÍNICO: El presente trabajo presenta una serie de tres casos diagnosticados de Displasia Osteofibrosa Craneofacial en el Hospital José Carrasco Arteaga y Hospital del Río, Cuenca-Ecuador, en los que por el cuadro clínico de los pacientes se decidió tratamiento quirúrgico. EVOLUCIÓN: En todos los casos, la evolución fue favorable. Se logró resultados positivos, reducción de síntomas y mejoría estética general en todos los casos. CONCLUSIÓN: Se obtienen mejores resultados con los tratamientos quirúrgicos actuales (remodelado más congelamiento óseo), entre ellos: buenos resultados estéticos, mejor calidad de vida, menor riesgo de infecciones. En el presente trabajo los tres participantes sometidos a resolución quirúrgica señalaron que el tratamiento reflejó positivamente en el ámbito funcional, estético y emocional. (au)

BACKGROUND: Craniofacial Bone Fibrous Dysplasia is a benign bone lesion where normal bone tissue is replaced with fibrous tissue. Developed from a genetic mutation. Subordinated in variants: monostotic and polyistotic. Few cases are reported on malignancy of the pathology. The diagnosis is determined with clinical suspicion and confirmed by imaging and pathology tests. CASE REPORT: In this paper, we present a series of three cases diagnosed with Craniofacial Fibrous Dysplasia at José Carrasco Arteaga Hospital and Hospital del Río, Cuenca-Ecuador. All of patients were treated with surgery, because of the clinical features. EVOLUTION: In all the cases, the patient evolution was favorable. Reduction of symptoms and general aesthetic improvement were achieved in all cases. CONCLUSION: Better results are obtained with the current surgical treatments (bone remodeling plus bone freezing), among them: good aesthetic results, better quality of life, lower risk of infections. In the present paper, the three patients treated with the surgical procedure indicated that the treatment reflected positively in the functional, aesthetic and emotional aspects.(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.

Midfacial degloving approach for management of the maxillary fibrous dysplasia: a case report / 대한악안면성형재건외과학회지

BACKGROUND: Fibrous dysplasia (FD) is a benign bone lesion characterized by the progressive replacement of normal bone with fibro-osseous connective tissue. The maxilla is the most commonly affected area of facial bone, resulting in facial asymmetry and functional disorders. Surgery is an effective management option and involves removing the diseased bone via an intraoral approach: conservative bone shaving or radical excision and reconstruction. CASE PRESENTATION: This case report describes a monostotic fibrous dysplasia in which the patient’s right midface had a prominent appearance. The asymmetric maxillary area was surgically recontoured via the midfacial degloving approach under general anesthesia. Follow-up photography and radiographic imaging after surgery showed the structures were in a stable state without recurrence of the FD lesion. Furthermore, there were no visible scars or functional disability, and the patient reported no postoperative discomfort. CONCLUSIONS: In conclusion, the midfacial degloving approach for treatment of maxillary fibrous dysplasia is a reliable and successful treatment option. Without visible scars and virtually free of postoperative functional disability, this approach offers good exposure of the middle third of the face for treatment of maxillary fibrous dysplasia with excellent cosmetic outcomes.

Displasia fibrosa ósea / Fibrous dysplasia of bone

La displasia fibrosa ósea es un trastorno no hereditario del desarrollo esquelético caracterizado por una proliferación anormal de fibroblastos y diferenciación deficiente de osteoblastos que conduce a un reemplazo del tejido óseo esponjoso por tejido conectivo fibroso. Es producida por una mutación somática activadora del gen GNAS1 que induce una activación y proliferación de células mesenquimales indiferenciadas con formación de tejido fibroso y trabéculas óseas anómalas. Existen formas monostóticas, poliostóticas y craneofaciales con diversos grados de dolor, deformidades y fracturas óseas, aunque muchos casos son asintomáticos. En ocasiones se producen quistes óseos aneurismáticos, hemorragias, compromisos neurológicos y raramente osteosarcomas. Algunos casos se asocian a síndrome de McCune-Albright, síndrome de Mazabraud y a osteomalacia por hipofosfatemia por pérdida tubular renal inducida por el FGF23 producido por el tejido displásico. Los hallazgos en las radiografías convencionales son característicos, aunque variables y de carácter evolutivo. La gammagrafía ósea es la técnica de imagen con mayor sensibilidad para determinar la extensión de la enfermedad. El diagnóstico diferencial incluye múltiples lesiones óseas de características similares y en raras ocasiones se requiere biopsia ósea o estudio genético para confirmarlo. No existe un consenso unánime acerca del abordaje terapéutico de estos pacientes, razón por la cual es necesario un enfoque multidisciplinario. La conducta puede ser expectante o quirúrgica según el tipo de lesiones y es importante el manejo del dolor y de las endocrinopatías asociadas. La mayor experiencia publicada se refiere al uso de bifosfonatos y, más recientemente, denosumab. Los tratamientos actuales son insuficientes para modificar el curso de la enfermedad y es necesario el desarrollo de nuevas moléculas que actúen específicamente en el gen GNAS1 o sobre las células mesenquimales afectadas. (AU)

Fibrous dysplasia of bone is a noninherited developmental anomaly of bone characterized by abnormal proliferation of fibroblasts and differentiation of osteoblasts that cause a replacement of trabeculous bone by fibrous connective tissue. It is caused by a somatic mutation in the GNAS1 gene, which induces an undifferentiated mesenquimal cells activation and proliferation with formation of fibrous tissue and abnormal osseous trabeculae. There are monostotic, polyostotic and craniofacial variants with different grades of bone pain, deformities and fractures, although many cases remain asymptomatic. Aneurysmal bone cysts, bleeding, neurological compromise and infrequently osteosarcoma are possible complications. Some cases are associated to McCune-Albright syndrome, Mazabraud syndrome or hypophosphatemia and osteomalacia due to to renal tubular loss induced by FGF23 produced by dysplastic tissue. The findings on conventional radiography are characteristic although variable and evlolve with time. Bone scintigraphy is the most sensitive technique to evaluate the extent of disease. Differential diagnosis include several osseous lesions of similar appearance and, in some cases, bone biopsy or genetic testing may be necessary. Today, there is no consensus regarding the therapeutic approach for these patients and it is necessary a multidisciplinary medical team. Watchful waiting or surgical interventions can be indicated, depending on the type of bone lesions. Bone pain and associated endocrinopathies management are very important. Most published experience refers to the use of bisphosphonates and, more recently, denosumab. Current treatments are insufficient to modify the natural curse of the disease and therefore, new molecules with specific action on GNAS1 gene or affected mesenchymal cells are necessary. (AU)

Fibrous Dysplasia in the Epiphysis of the Distal Femur

Fibrous dysplasia is a common benign skeletal lesion that may involve a single bone or multiple bones. Although fibrous dysplasia can affect any bone, monostotic fibrous dysplasia of the long bone typically occurs in the diaphysis or metaphysis. We report a very rare case of monostotic fibrous dysplasia involving the epiphysis of the distal femur in a young man.

Displasia fibrocartilaginosa focal de femur. Un caso de correccion espontanea / Focal fibrocartilaginous dysplasia of the femur. A case with spontaneous resolution

La displasia fibrocartilaginosa focal es una enfermedad rara que genera una deformidad localizada en los huesos largos de los niños. Algunos autores recomiendan la cirugía como tratamiento inicial. El objetivo de este trabajo es comunicar un caso de displasia fibrocartilaginosa en el fémur que tuvo una resolución espontánea y revisar la bibliografía. Se comunica el caso de una niña de 2 meses de edad con deformidad en varo del fémur izquierdo y discrepancia de longitud de los miembros inferiores. La radiografía mostró una imagen en la diáfisis femoral radiolúcida de bordes escleróticos y deformidad en varo de 30º. En un período de 22 meses, se observó la corrección espontánea completa de la deformidad. Se han publicado 20 casos de localización femoral, 17 tratados con cirugía y tres, con corrección espontánea. Se aconseja mantener una conducta expectante durante un período más prolongado que el recomendado en la literatura para evitar procedimientos invasivos innecesarios.

Focal fibrocartilaginous dysplasia is a condition of unknown etiology associated with long bone deformities in children. Some authors recommend surgery as the initial treatment. The aim of this paper is to report a case of focal fibrocartilaginous dysplasia of the femur with spontaneous resolution and to review the literature. A case of a 2-month-old girl with varus deformity of the left femur and leg length discrepancy is reported. Radiographs showed a radiolucent area with marginal sclerosis and a varus deformity of 30º. Over a period of 22 months, complete spontaneous correction of the deformity was noticed. Twenty cases of femoral focal fibrocartilaginous dysplasia have been reported in the literature, 17 were treated surgically, three showed spontaneous correction. Patients should be monitored during a period longer than that recommended to avoid unnecessary invasive procedures.

Fibrous dysplasia of the maxilla in an elderly female: Case report on a 14-year quiescent phase

Fibrous dysplasia (FD) is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibro-osseous tissue. Mainly, FD is found in children, and by adulthood it usually becomes quiescent. Our case showed FD of more than 14-year duration in the left maxilla. Our evaluation was that growth ceased in adulthood and had achieved the static stage. Because FD cases in elderly patients are rarely reported, we hereby present a monostotic FD case in a 65-year-old female. We presented sequential radiographic images and scintigraphic images of this case, and combined them with a literature review that emphasized the progression of the disease.

A Rare Case of Monostotic Spinal Fibrous Dysplasia Mimicking Solitary Metastatic Lesion of Thyroid Carcinoma

Monostotic fibrous dysplasia of the vertebra is a rare entity. A case of a 53-year-old lady who presented with an 8 months history of pain in the thoracic spine region with paraparesis is discussed. She had a history of papillary thyroid carcinoma and had undergone total thyroidectomy one year prior to her current problem. Magnetic resonance imaging revealed isolated osteolytic lesion over the posterior element of the T12 vertebra with narrowing of the spinal canal causing compression of the cord. The diagnosis of fibrous dysplasia was made histologically. Fibrous dysplasia rarely occurs in axial bones compared with peripheral bones. This case illustrates that osteolytic lesion of the vertebrae should be evaluated with detailed radiological and histopathological examination before an empirical diagnosis of spinal metastasis is made in an adult with a background history of primary malignancy well-known to spread to the bone.

Epidermal Nevus Syndrome Associated with Ipsilateral Monostotic Fibrous Dysplasia: An Uncommon Skeletal Abnormality / 대한피부과학회지

Epidermal nevus syndromes are a group of congenital syndromes comprising epidermal nevi in conjunction with the central nervous system, ocular, musculoskeletal, and other organ anomalies. Skeletal manifestations in epidermal nevus syndromes are common, ranging from 50% to 66% of patients. However, fibrous dysplasia of the bone has been rarely described in association with epidermal nevus syndromes. Herein, we report a 46-year-old-male patient who presented with extensive epidermal nevi associated with monostotic fibrous dysplasia of the rib which is located ipsilateral to the skin findings.

Displasia fibrosa óssea extensa em coluna cervical de paciente jovem e assintomático: Relato de caso / Extense cervical fibrous dysplasia of bones in young Assymptomatic Patient. Case Report

Introdução: A Displasia Fibrosa Óssea (DFO) é uma lesão benigna, representando 5 a 7% dos tumores ósseos benignos. Temsua etiologia relacionada a mutações somáticas. Classifica-se em monostótica, quando atinge apenas um osso ou poliostóticano envolvimento de mais de um. O acometimento cervical é raro, sendo mais comum na apresentação poliostótica. Os casoscom comprometimento funcional ou estético excessivo constituem-se como principais indicações para o tratamento cirúrgico.Objetivos: Os autores mostram o tratamento da lesão destrutiva do corpo vertebral cervical, quinto segmento, causada porDFO monostótica, por meio de somatectomia associada a artrodese anterior com substituto de corpo vertebral, placa detitânio anterior, laminectomia e fixação posterior com cabos de titânio sublaminares. Métodos e Resultados: Relato de caso,inicialmente de trauma automobilístico, que após estudo por imagem da coluna cervical confirmou diagnóstico de DFO. Devidoà instabilidade da região cervical indicou-se cirurgia, sobre a qual foram comentadas suas principais etapas...

Introduction: Fibrous Dysplasia of bones (FDB) is a benign lesion, representing 5-7% of all benign bone tumors. Its etiology isassociated to somatic mutations. It is classified as monostotic only when it reaches a bone, polyostotic when there is involvementof two or more bones. The cervical involvement is rare and is more common in polyostotic presentation. Cases with excessivefunctional or aesthetic impairment constitute the main indications for surgical treatment. Objectives: The authors present thetreatment of destructive lesions of the cervical vertebral body, segment of the fifth vertebra, caused by FDB monostotic usingsomatectomia associated to anterior arthrodesis with vertebral body replacement, titanium plate anterior, laminectomy andposterior fixation with sublaminares tittanium system. Methods and Results: Initially detected as an automobile trauma, afterimaging study of the cervical spine confirmed diagnosis of FDB. Due to the instability of the cervical region, surgery wasindicated...

Prosthetic reconstruction after surgical resection of fibrous dysplasia of the maxillary and palatine bone / Reconstrucción prostética tras la resección quirúrgica de la displasia fibrosa del hueso maxilar y el palatino

Fibrous dysplasia is a rare disorder of the bone. It is seen in two main forms ofpresentation: monostotic and the polyostotic. A case of monostotic fibrous dysplasia of the maxillary and palatine bones in a 22-year old man who received prosthetic reconstruction is presented with a review of the literature.

La displasia fibrosa es un trastorno raro del hueso. Se le ve en dos formas principales: la monostótica y la poliostótica. Junto con la correspondiente revisión de la literatura, se presenta un caso de displasia fibrosa monostótica de los huesos maxilar y palatino en un hombre de 22 anos que recibió una reconstrucción prostética.

Displasia fibrosa monostótica: um desafio terapêutico / Monostotic fibrous Dyspalsia: a therapeutic challenge

A displasia fibrosa monostótica é uma condição benigna que se caracteriza pela proliferação e substituição do tecido ósseo por tecido fibroso. Essa doença geralmente inicia-se na infância e progride até a puberdade e adolescência. Relata-se um caso de Displasia Fibrosa Monostótica em um paciente de 15 anos de idade, com aumento de volume assintomático no lado esquerdo do corpo da mandibular que foi tratado cirurgicamente. Por meio de revisão de literatura procurou-se estabelecer critérios de diagnóstico, planejamento e tratamento cirúrgico que norteassem a exérese da displasia fibrosa monostótica nos ossos maxilares.

Monostotic Fibrous Dysplasia is a benign fibro-osseous developmental anomaly where only one bone is involved. This disease usually begins in childhood and progresses throughout puberty and adolescence. The present case is a 15 years-old male patient. The patient was concerned about asymptomatic swelling in his left mandible body. The literature review was used to establish diagnostic criteria, planning and treatment in order to guide the monostotic fibrous dysplasia exeresis in the jaws.

Displasia fibrosa: a doença e a imagem / Fibrous dysplasia: the disease and imageologic findings

A displasia fibrosa é uma lesão benigna que afeta um ou mais ossos, caracterizada pela substituição gradativa de tecido ósseo por tecido fibra-ósseo. Radiograficamente, as lesões apresentam-se com o aspecto de "vidro despolido" ou de "casca de laranja". A tomografia óssea é o método ideal para estabelecimento de diagnóstico adequado e acompanhamento de pacientes com esta patologia. Este artigo relata um caso clínico em que a displasia fibrosa foi diagnosticada a partir da documentação radiográfica com a finalidade de colocação de implantes dentários. O objetivo desse relato é apresentar os aspectos clínicos e imaginológicos da displasia fibrosa e alertar o cirurgião-dentista para a identificação desta patologia na rotina diária.

Fibrous dysplasia is a benign disease that affects one ar more banes, characterized by the gradual replacement of the normal bane for a fibro-osseous tissue. Lesions are observed radiographically with a "ground qlass" appearance or orange peel. Computed tomography images proved to be the best way for diagnosis and for the evaluation of fibrous dysplasia lesions. This is a case of patient which fibrous dysplasia had been diagnosed during documentation for implants replacement. The objective of this report is to alert the dentist to make the identifications of this lesion in daily routine, presenting the clinic and imageologic findings of this lesion.

Displasia fibrosa da mandíbula / Fibrous dysplasia of mandible

A displasia fibrosa da mandíbula é uma patologia óssea benigna que representa cerca de 2% de todos os tumores ósseos. Relatamos o caso de uma paciente de 11 anos de idade, com queixa de dor em região mandibular esquerda há 1 semana, com história de abaulamento da mesma região há 3 meses. Após realização de exames de imagem, foi diagnosticado displasia fibrosa da mandíbula. O objetivo deste relato é revisar as principais características clínicas, radiológicas e possíveis tratamentos para o caso, dando a devida importância para o seguimento ambulatorial pós-tratamento

Fibrous dysplasia is a benign bone disease which represents about 2% of all bone tumors. Here we report the case of an 11-year-old who complained of pain at the left mandible area for a week, with history of arching in this area for 3 months. Imaging examinations led to the diagnosis of fibrous dysplasia of the mandible. The aim of this work is to review the main clinical and radiological characteristics and possible treatments for the case, giving due importance to ambulatory follow-up care