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1.
Rev. med. Chile ; 150(10): 1275-1282, oct. 2022. ilus, tab
Article in Spanish | LILACS | ID: biblio-1431854

ABSTRACT

BACKGROUND: Fibrous Dysplasia/McCune-Albright Syndrome (FD/MAS) is characterized by a spectrum of manifestations that may include fibrous dysplasia of bone and multiple endocrinopathies. AIM: To describe the clinical spectrum, the study and follow-up of patients with FD/MAS cared at our institution. MATERIAL AND METHODS: Review of medical records of 12 pediatric and adult patients (11 women) who met the clinical and genetic diagnostic criteria for FD/ MAS. RESULTS: The patients' mean age at diagnosis was 4.9 ± 5.5 years. The most common initial clinical manifestation was peripheral precocious puberty (PPP) in 67% of patients and 75% had café-au-lait spots. Fibrous dysplasia was present in 75% of patients and the mean age at diagnosis was 7.9 ± 4.7 years. Ten patients had a bone scintigraphy, with an age at the first examination that varied between 2 and 38 years of age. The most frequent location of dysplasia was craniofacial and appendicular. No patient had a recorded history of cholestasis, hepatitis, or pancreatitis. In four patients, a genetic study was performed that was positive for the pathogenic variant of guanine nucleotide binding protein, alpha stimulating (GNAS). CONCLUSIONS: These patients demonstrate the variable nature of the clinical presentation and study of FD/MAS. It is essential to increase the index of diagnostic suspicion and adherence to international recommendations.


Subject(s)
Humans , Female , Child, Preschool , Child , Adolescent , Adult , Young Adult , Puberty, Precocious/etiology , Puberty, Precocious/genetics , Fibrous Dysplasia of Bone/diagnostic imaging , Fibrous Dysplasia, Polyostotic/genetics , Fibrous Dysplasia, Polyostotic/diagnostic imaging , Chile/epidemiology , Cafe-au-Lait Spots/genetics
2.
Rev Med Chil ; 150(10): 1275-1282, 2022 Oct.
Article in Spanish | MEDLINE | ID: mdl-37358085

ABSTRACT

BACKGROUND: Fibrous Dysplasia/McCune-Albright Syndrome (FD/MAS) is characterized by a spectrum of manifestations that may include fibrous dysplasia of bone and multiple endocrinopathies. AIM: To describe the clinical spectrum, the study and follow-up of patients with FD/MAS cared at our institution. MATERIAL AND METHODS: Review of medical records of 12 pediatric and adult patients (11 women) who met the clinical and genetic diagnostic criteria for FD/ MAS. RESULTS: The patients' mean age at diagnosis was 4.9 ± 5.5 years. The most common initial clinical manifestation was peripheral precocious puberty (PPP) in 67% of patients and 75% had café-au-lait spots. Fibrous dysplasia was present in 75% of patients and the mean age at diagnosis was 7.9 ± 4.7 years. Ten patients had a bone scintigraphy, with an age at the first examination that varied between 2 and 38 years of age. The most frequent location of dysplasia was craniofacial and appendicular. No patient had a recorded history of cholestasis, hepatitis, or pancreatitis. In four patients, a genetic study was performed that was positive for the pathogenic variant of guanine nucleotide binding protein, alpha stimulating (GNAS). CONCLUSIONS: These patients demonstrate the variable nature of the clinical presentation and study of FD/MAS. It is essential to increase the index of diagnostic suspicion and adherence to international recommendations.


Subject(s)
Fibrous Dysplasia of Bone , Fibrous Dysplasia, Polyostotic , Puberty, Precocious , Adult , Humans , Child , Female , Child, Preschool , Adolescent , Young Adult , Fibrous Dysplasia, Polyostotic/diagnostic imaging , Fibrous Dysplasia, Polyostotic/genetics , Chile/epidemiology , Fibrous Dysplasia of Bone/diagnostic imaging , Puberty, Precocious/etiology , Puberty, Precocious/genetics , Cafe-au-Lait Spots/genetics
3.
Acta Radiol ; 61(11): 1541-1544, 2020 Nov.
Article in English | MEDLINE | ID: mdl-32075412

ABSTRACT

BACKGROUND: Fibrocartilaginous coalition of the third tarsometatarsal joint has been indicated as an extremely rare form of tarsal coalition in the radiological literature, and most articles concerned with tarsal coalition do not mention involvement of this joint. Only two reports written in the English language that approach this subject were found, an orthopedic report and an anthropological report. PURPOSE: To evaluate the prevalence of this finding and discuss and illustrate the radiological characteristics of this coalition. MATERIAL AND METHODS: A retrospective analysis of 614 computed tomography or magnetic resonance imaging scans of the ankle and/or foot, acquired at a health service within a period of three months, was performed to assess the prevalence of this coalition. RESULTS: Of the examinations characterized as valid for analysis for the purposes of the study, 17 cases compatible with fibrocartilaginous coalition of the third tarsometatarsal joint were found, thus indicating an involvement of approximately 2.97% of the examined feet. CONCLUSION: Our radiological findings are typical, and the prevalence found in this study was statistically significant, being similar to that described in the anthropological report (3.2%-6.8%).


Subject(s)
Fibrous Dysplasia of Bone/diagnostic imaging , Fibrous Dysplasia of Bone/epidemiology , Magnetic Resonance Imaging/methods , Tarsal Coalition/diagnostic imaging , Tarsal Coalition/epidemiology , Tomography, X-Ray Computed/methods , Adolescent , Adult , Cross-Sectional Studies , Female , Foot Joints/diagnostic imaging , Humans , Male , Middle Aged , Prevalence , Retrospective Studies , Tarsal Bones/diagnostic imaging , Young Adult
4.
Int. j. odontostomatol. (Print) ; 13(2): 198-202, jun. 2019. graf
Article in English | LILACS | ID: biblio-1002306

ABSTRACT

ABSTRACT: Florid osseous dysplasia (FOD) is an extensive form of osseous dysplasia where normal trabecular bone is substituted by fibrous connective tissue and amorphous mineralized tissue. Usually, the lesions are mainly asymptomatic and the patients should be followed with clinical and imaging examination, requiring no intervention. Nevertheless, due to the poor vascularization of the lesion and to local trauma, secondary infections and osteomyelitis may occur. Patients may present with pain, mucosal ulceration, lesion exposure in the oral cavity, fistula and swelling. In such cases, the correct diagnosis and management of the lesion is decisive to reestablish patient's health and quality of life. The aim of this article is to present a case of complicating secondary chronic osteomyelitis treated successfully with conservative intervention. A 68-year-old black female patient reported a "swelling of the gums" that was present for eleven years, with episodes of periodic pain and swelling. On physical examination, a papule with suppuration in the alveolar mucosa in the right side of the mandible was observed. Panoramic radiography and CBCT showed a mixed lesion surrounded by sclerotic bone. The patient was diagnosed with chronic osteomyelitis in association with FOD and treated with antibiotic therapy together with surgical curettage. The incidence, etiophatology, differential diagnosis, treatment and prevention of secondary osteomyelitis associated with FOD are discussed in the light of literature. This information might assist the dentists while choosing the best treatment options for similar cases.


RESUMEN: La displasia ósea florida (DOF) es una forma de displasia ósea donde el hueso trabecular normal es sustituido por tejido conectivo fibroso y tejido mineralizado amorfo. En general, las lesiones son de origen asintomático y los pacientes deben ser seguidos con el examen clínico. Sin embargo, debido a la pobre vascularización de la lesión y al trauma local, pueden producirse lesiones secundarias y osteomielitis. Los pacientes pueden presentarse con dolor, ulceración mucosa, lesión de exposición en la cavidad oral, fístula y edema. En estos casos, el correcto diagnóstico y tratamiento de la lesión es decisivo para reestabilizar la salud y la calidad de la vida. El objetivo de este artículo consistió en presentar un caso de complicación secundaria crónica de osteomielitis tratados con tratamiento conservador. El paciente, negro, de 68 años de edad, consulta por "hinchazón de las encías" que se presentó durante once años, con episodios de dolor. En el examen intraoral, se observó una pápula con supuración en la mucosa alveolar en el lado derecho de la mandíbula. La radiografía panorámica y CBCT mostraron una lesión mixta rodeada de hueso esclerótico. El paciente fue diagnosticado con osteomielitis crónica en asociación con DOF y fue tratado con tratamiento antibiótico junto con curetaje quirúrgico. La incidencia, la etiopatología, el diagnóstico diferencial, el tratamiento y la prevención de la osteomielitis secundaria asociada con DOF se discuten a la luz de la literatura. Esta información puede ayudar a los dentistas a elegir las mejores opciones de tratamiento para casos similares.


Subject(s)
Humans , Aged , Osteomyelitis/therapy , Osteomyelitis/diagnostic imaging , Mandibular Diseases/therapy , Conservative Treatment/methods , Radiography, Panoramic , Mandibular Diseases/diagnostic imaging , Fibrous Dysplasia of Bone/therapy , Fibrous Dysplasia of Bone/diagnostic imaging
5.
RFO UPF ; 24(1): 132-140, 29/03/2019. ilus
Article in Portuguese | LILACS, BBO - Dentistry | ID: biblio-1049257

ABSTRACT

Objetivo: relatar dois casos de displasia cemento-óssea florida, descrevendo a evolução clínico-radiográfica ao longo de 5 anos em um paciente assintomático e a abordagem cirúrgica em um caso sintomático. Relato de caso 1: paciente, melanoderma, sexo feminino, 56 anos de idade, encaminhada ao Serviço de Cirurgia Bucomaxilofacial da Universidade Federal da Bahia, por cirurgião-dentista clínico que notou alterações imagiológicas em radiografia panorâmica de rotina. Apesar da importante extensão da lesão em maxila e mandíbula, não havia qualquer sintoma ou sinal clínico de infecção. A paciente foi acompanhada durante 5 anos, com exames de imagem bianuais e medidas clínicas profiláticas. Relato de caso 2: paciente, melanoderma, sexo feminino, 57 anos, apresentou-se ao ambulatório de cirurgia queixando-se de atraso em cicatrização após remoção de um dente. A radiografia panorâmica e a tomografia computadorizada, em conjunto com dados clínicos, permitiram o diagnóstico de displasia cemento-óssea florida com infecção secundária. A paciente foi abordada por meio de osteotomia em região do defeito em mandíbula. A análise microscópica do espécime obtido confirmou a alteração displásica cementoide. Os sinais e sintomas regrediram e a paciente segue em acompanhamento. Considerações finais: a displasia cemento-óssea florida, portanto, é uma doença pouco frequente, cujas manifestações podem demandar diferentes abordagens. É importante o domínio clínico do cirurgião-dentista, uma vez que o diagnóstico equivocado pode guiar a escolhas terapêuticas com resultados insatisfatórios. (AU)


Objective: the objective of this article is to report two cases of florid cemento-osseous dysplasia describing the clinical-radiographic evolution over 5 years in an asymptomatic patient and the surgical approach in a symptomatic one. Case report 1: patient, melanoderma, female, 56 years old, referred to the service by a clinical Dentist who noticed imaging alterations in a routine panoramic radiography. Despite the important extension of the maxillary and mandibular lesion, there was no clinical sign or symptom of infection. The patient has been followed for 5 years with biannual imaging exams and prophylactic clinical measures. Case report 2: patient, melanoderma, female, 57 years old, presented to the surgery outpatient complaining of delay in healing after removal of a tooth. Panoramic X-ray and Computed Tomography together with clinical data allowed the diagnosis of florid cemento-osseous dysplasia with secondary infection. The patient was approached through osteotomy in the region of the mandible defect. The microscopic analysis of the specimen confirmed the dysplastic cementenoid alteration. Signs and symptoms regressed and the patient is in follow up. Final considerations: florida cementoosseous dysplasia is an infrequent disease, whose manifestations may require different approaches. The clinical domain of the Dentist is important, since misdiagnosis can lead to therapeutic choices with unsatisfactory results. (AU)


Subject(s)
Humans , Female , Middle Aged , Osteomyelitis/therapy , Fibrous Dysplasia of Bone/therapy , Osteomyelitis/diagnostic imaging , Radiography, Panoramic , Treatment Outcome , Cone-Beam Computed Tomography , Fibrous Dysplasia of Bone/diagnostic imaging , Maxillary Osteotomy/methods
6.
Dental Press J Orthod ; 23(3): 26-34, 2018.
Article in English | MEDLINE | ID: mdl-30088561

ABSTRACT

Florid cemento-osseous dysplasia is a sclerosing disease that affects the mandible, especially the alveolar process, and that is, in most cases, bilateral; however, in some cases it affects up to three or even four quadrants. During the disease, normal bone is replaced with a thinly formed, irregularly distributed tissue peppered with radiolucent areas of soft tissue. Newly formed bone does not seem to invade periodontal space, but, in several images, it is confused with the roots, without, however, compromising pulp vitality or tooth position in the dental arch. There is no replacement resorption, not even when the images suggest dentoalveolar ankylosis. Orthodontists should make an accurate diagnosis when planning treatments, as this disease, when fully established, is one of the extremely rare situations in which orthodontic treatment is contraindicated. This contraindication is due to: (a) procedures such as the installment of mini-implants and mini-plaques, surgical maneuvers to apply traction to unerupted teeth and extractions should be avoided to prevent contamination of the affected bone with bacteria from the oral microbiota; and (b) tooth movement in the areas affected is practically impossible because of bone disorganization in the alveolar process, characterized by high bone density and the resulting cotton-wool appearance. Densely mineralized and disorganized bone is unable to remodel or develop in an organized way in the periodontal ligaments and the alveolar process. Organized bone remodeling is a fundamental phenomenon for tooth movement.


Subject(s)
Bone Remodeling , Fibrous Dysplasia of Bone/physiopathology , Osteomyelitis/physiopathology , Tooth Movement Techniques/adverse effects , Adult , Contraindications, Procedure , Dental Implantation/adverse effects , Female , Fibrous Dysplasia of Bone/diagnostic imaging , Fibrous Dysplasia of Bone/pathology , Humans , Middle Aged , Osteomyelitis/diagnostic imaging , Osteomyelitis/pathology , Tooth Extraction/adverse effects
7.
J Oral Maxillofac Surg ; 76(11): 2348-2359, 2018 11.
Article in English | MEDLINE | ID: mdl-29859157

ABSTRACT

PURPOSE: The aims of this study were to describe the clinical and radiologic features of 383 fibro-osseous lesions (FOLs) from an oral pathology reference service in Brazil and to compare the findings with previous studies. The hypothesis of the study was that the most common type of FOL would differ from other investigations. MATERIALS AND METHODS: We conducted a descriptive and retrospective study with review of the records of the clinical and biopsy services (1990 to 2015). All records of the patients included showed a definitive diagnosis of FOL. The primary outcome variable was the type of FOL, and the predictor variables were gender, age, ethnicity, location, and clinical and radiologic characteristics. Descriptive analyses and χ2 tests were performed. The P value was set at .05. RESULTS: From the 27,998 records available, 383 showed FOLs, with 187 (48.8%) being cemento-osseous dysplasias (CODs), 103 (26.9%) being fibrous dysplasias (FDs), and 93 (24.3%) being ossifying fibromas (OFs). The mean age of the patients was 38.5 ± 17.5 years. CODs presented a predilection for female gender (n = 314, 82.0%), African descent (n = 134, 71.6%), and the mandible (n = 248, 64.6%). The most common radiologic feature was a mixed radiolucent-radiopaque image (n = 149, 51.7%). FDs and OFs were commonly diagnosed during the first and second decades of life (P < .001), whereas CODs were more frequently diagnosed beyond the third decade (P < .001). Secondary osteomyelitis was more significantly observed in CODs (P < .001) than in FDs and OFs. Swelling was more frequently reported for FDs and OFs than for CODs (P < .001). CONCLUSIONS: CODs were the most frequently observed FOLs in this Brazilian population. Female patients, patients of African descent, and patients with mandibular localization were most commonly affected by these conditions. The most common type of FOL differed from that in similar case reports or series from various geographic locations. It is believed that the data source (clinical and/or biopsy services) can directly influence the outcome.


Subject(s)
Cementoma/pathology , Fibroma, Ossifying/pathology , Fibrous Dysplasia of Bone/pathology , Jaw Diseases/pathology , Jaw Neoplasms/pathology , Adolescent , Adult , Brazil , Cementoma/diagnostic imaging , Child , Diagnosis, Differential , Female , Fibroma, Ossifying/diagnostic imaging , Fibrous Dysplasia of Bone/diagnostic imaging , Humans , Jaw Diseases/diagnostic imaging , Jaw Neoplasms/diagnostic imaging , Male , Middle Aged , Radiography, Panoramic , Retrospective Studies
8.
Dental press j. orthod. (Impr.) ; 23(3): 26-34, May-June 2018. graf
Article in English | LILACS | ID: biblio-953023

ABSTRACT

ABSTRACT Florid cemento-osseous dysplasia is a sclerosing disease that affects the mandible, especially the alveolar process, and that is, in most cases, bilateral; however, in some cases it affects up to three or even four quadrants. During the disease, normal bone is replaced with a thinly formed, irregularly distributed tissue peppered with radiolucent areas of soft tissue. Newly formed bone does not seem to invade periodontal space, but, in several images, it is confused with the roots, without, however, compromising pulp vitality or tooth position in the dental arch. There is no replacement resorption, not even when the images suggest dentoalveolar ankylosis. Orthodontists should make an accurate diagnosis when planning treatments, as this disease, when fully established, is one of the extremely rare situations in which orthodontic treatment is contraindicated. This contraindication is due to: (a) procedures such as the installment of mini-implants and mini-plaques, surgical maneuvers to apply traction to unerupted teeth and extractions should be avoided to prevent contamination of the affected bone with bacteria from the oral microbiota; and (b) tooth movement in the areas affected is practically impossible because of bone disorganization in the alveolar process, characterized by high bone density and the resulting cotton-wool appearance. Densely mineralized and disorganized bone is unable to remodel or develop in an organized way in the periodontal ligaments and the alveolar process. Organized bone remodeling is a fundamental phenomenon for tooth movement.


RESUMO A displasia cemento-óssea florida é uma doença óssea esclerosante exclusiva dos maxilares, relacionada ao osso do processo alveolar e, na maioria dos casos, envolvendo bilateralmente a mandíbula; mas há casos em que envolve três ou até os quatro quadrantes. Nesse processo, troca-se o osso normal por um tecido densamente formado, irregularmente distribuído e salpicado por áreas radiolúcidas com tecido mole. O osso neoformado parece não invadir o espaço periodontal, mas, em muitas imagens, confunde-se com as raízes, sem comprometer a vitalidade pulpar e a posição dentária na arcada. Não há reabsorção dentária por substituição, mesmo quando as imagens sugerem anquilose alveolodentária. Um diagnóstico preciso por parte do ortodontista deve ser feito em seus planejamentos, visto que essa doença, quando se encontra plenamente instalada, representa uma das raríssimas situações em que o tratamento ortodôntico está contraindicado. Nesses casos, o tratamento ortodôntico está contraindicado porque: a) alguns procedimentos, como a aplicação de mini-implantes e miniplacas, manobras cirúrgicas para tracionamento de dentes não irrompidos e exodontias, devem ser evitados, para se impedir a entrada de bactérias da microbiota bucal no osso comprometido; e b) a possibilidade de movimentação dos dentes nas áreas comprometidas praticamente inexiste, pela desorganização óssea no processo alveolar, caracterizada por elevada densidade óssea, que gera as imagens tipo flocos de algodão. O osso densamente mineralizado e desorganizado não é capaz de se remodelar e desenvolver organizadamente, nos ligamentos periodontais e no osso do processo alveolar. A remodelação óssea organizada é fundamental como um dos fenômenos necessários para o deslocamento dos dentes.


Subject(s)
Humans , Female , Adult , Middle Aged , Osteomyelitis/physiopathology , Tooth Movement Techniques/adverse effects , Bone Remodeling , Fibrous Dysplasia of Bone/physiopathology , Osteomyelitis/pathology , Osteomyelitis/diagnostic imaging , Tooth Extraction/adverse effects , Dental Implantation/adverse effects , Fibrous Dysplasia of Bone/pathology , Fibrous Dysplasia of Bone/diagnostic imaging , Contraindications, Procedure
9.
Actual. osteol ; 13(3): 233-242, Sept - DIc. 2017. ilus, tab
Article in Spanish | LILACS | ID: biblio-1117496

ABSTRACT

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)


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Young Adult , Fibrous Dysplasia of Bone/etiology , Fibrous Dysplasia of Bone/drug therapy , Osteogenesis/genetics , Osteomalacia/complications , Congenital Abnormalities , Vitamin D/therapeutic use , Osteosarcoma/etiology , Calcium/therapeutic use , Hypophosphatemia/blood , Bone Cysts, Aneurysmal/etiology , Diagnosis, Differential , Diphosphonates/administration & dosage , Diphosphonates/adverse effects , Fractures, Bone/pathology , Mesenchymal Stem Cells/pathology , Pain Management , Fibrous Dysplasia, Monostotic/etiology , Fibrous Dysplasia of Bone/genetics , Fibrous Dysplasia of Bone/blood , Fibrous Dysplasia of Bone/diagnostic imaging , Fibrous Dysplasia, Polyostotic/etiology , Fibrous Dysplasia, Polyostotic/diagnostic imaging , Craniofacial Fibrous Dysplasia/etiology , Mutation/genetics
10.
An Bras Dermatol ; 92(2): 249-252, 2017.
Article in English | MEDLINE | ID: mdl-28538890

ABSTRACT

We report a 35-year-old mulatto female patient with neurofibromatosis Type 1 who presented with facial asymmetry. The patient had two lesions: florid cemento-osseous dysplasia associated with peripheral giant cell granuloma. She was referred for surgical treatment of the peripheral giant cell granuloma and the florid cemento-osseous dysplasia was treated conservatively by a multidisciplinary team. So far, no changes have been observed in the patient's clinical status. We observed no recurrence of peripheral giant cell granuloma. To the best of our knowledge, the present case is the first report of a patient with neurofibromatosis Type 1 associated with a giant cell lesion and florid cemento-osseous dysplasia.


Subject(s)
Facial Asymmetry/etiology , Fibrous Dysplasia of Bone/complications , Granuloma, Giant Cell/complications , Neurofibromatosis 1/etiology , Osteomyelitis/complications , Adult , Female , Fibrous Dysplasia of Bone/diagnostic imaging , Fibrous Dysplasia of Bone/pathology , Granuloma, Giant Cell/diagnostic imaging , Granuloma, Giant Cell/pathology , Humans , Neurofibromatosis 1/diagnostic imaging , Neurofibromatosis 1/pathology , Osteomyelitis/diagnostic imaging , Osteomyelitis/pathology
11.
An. bras. dermatol ; An. bras. dermatol;92(2): 249-252, Mar.-Apr. 2017. graf
Article in English | LILACS | ID: biblio-838046

ABSTRACT

Abstract: We report a 35-year-old mulatto female patient with neurofibromatosis Type 1 who presented with facial asymmetry. The patient had two lesions: florid cemento-osseous dysplasia associated with peripheral giant cell granuloma. She was referred for surgical treatment of the peripheral giant cell granuloma and the florid cemento-osseous dysplasia was treated conservatively by a multidisciplinary team. So far, no changes have been observed in the patient's clinical status. We observed no recurrence of peripheral giant cell granuloma. To the best of our knowledge, the present case is the first report of a patient with neurofibromatosis Type 1 associated with a giant cell lesion and florid cemento-osseous dysplasia.


Subject(s)
Humans , Female , Adult , Osteomyelitis/complications , Granuloma, Giant Cell/complications , Neurofibromatosis 1/etiology , Facial Asymmetry/etiology , Fibrous Dysplasia of Bone/complications , Osteomyelitis/pathology , Osteomyelitis/diagnostic imaging , Granuloma, Giant Cell/pathology , Granuloma, Giant Cell/diagnostic imaging , Neurofibromatosis 1/pathology , Neurofibromatosis 1/diagnostic imaging , Fibrous Dysplasia of Bone/pathology , Fibrous Dysplasia of Bone/diagnostic imaging
12.
Article in English | MEDLINE | ID: mdl-27422424

ABSTRACT

OBJECTIVE: The aim of this study was to correlate the clinical, demographic, and radiographic aspects of 82 cases of florid osseous dysplasia (FOD) from four countries assessed by five reviewers. STUDY DESIGN: Retrospective data of patients diagnosed with FOD were obtained from archives of five institutes and analyzed according to clinical and radiographic criteria. RESULTS: A total of 82 cases of FOD were included in the study. In the majority (77.5%) of FOD cases, black women were affected. The disease mainly affected the mandible (100%), and the stage of the lesion was directly correlated with the age (P < .05). The rates of convergence showed confluence of assessment in the mandible (posterior), and the highest rate of divergence between the reviewers was in assessing involvement in the maxilla. There were no differences among the characteristics of the lesion provided by the five institutions, except for the variable proportion of black patients affected by the disease. CONCLUSIONS: FOD mostly affects the mandible of middle-aged black women, and its stage is correlated with patient age. Although panoramic radiography is used for diagnosis, the divergence in assessment of the maxilla might indicate that this method is not the most suitable to evaluate this area.


Subject(s)
Fibrous Dysplasia of Bone/diagnostic imaging , Fibrous Dysplasia of Bone/ethnology , Fibrous Dysplasia of Bone/pathology , Osteomyelitis/diagnostic imaging , Osteomyelitis/ethnology , Osteomyelitis/pathology , Adult , Age Factors , Aged , Diagnosis, Differential , Female , Humans , Internationality , Male , Middle Aged , Radiography, Panoramic , Retrospective Studies
13.
Clin Nucl Med ; 41(9): 718-21, 2016 Sep.
Article in English | MEDLINE | ID: mdl-27405032

ABSTRACT

Juvenile psammomatoid ossifying fibroma is a rare bone-forming tumor seen in craniofacial bones, which affects mainly young patients. We report scintigraphic and SPECT/CT findings of 2 patients diagnosed with this disease. One patient presented with suspicion of fibrous dysplasia and the other with suspected malignancy in the setting of a rapidly growing mass. Both cases highlight the importance of recognizing this type of tumor in young patients with abnormal uptake in paranasal bones and sinuses within the range of potential differential diagnoses including sarcomas, fibrous dysplasia, and odontogenic tumors.


Subject(s)
Bone Neoplasms/diagnostic imaging , Fibroma, Ossifying/diagnostic imaging , Maxillary Neoplasms/diagnostic imaging , Paranasal Sinuses/diagnostic imaging , Single Photon Emission Computed Tomography Computed Tomography , Adolescent , Child , Diagnosis, Differential , Female , Fibrous Dysplasia of Bone/diagnostic imaging , Humans , Maxilla/diagnostic imaging , Nasal Bone/diagnostic imaging
14.
J Digit Imaging ; 29(1): 134-40, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26307180

ABSTRACT

Fibrous dysplasia (FD) is a developmental anomaly in which the normal medullary space of the affected bone is replaced by fibro-osseous tissue. This condition is typically encountered in adolescents and young adults. It affects the maxillofacial region and it can often cause severe deformity and asymmetry. Therefore, accurate diagnosis is critical to determine the appropriate treatment of each case. In this sense, computed tomography (CT) is a relevant resource among the imaging techniques for correct diagnosis of this condition. Thus, in this paper, we propose to analyze fibrous dysplasia through its texture pattern. To accomplish this task, we propose to use lacunarity analysis, a multiscale method for describing patterns of spatial dispersion. Results indicated lower lacunarity values for fibrous dysplasia in comparison to normal bone samples, an indication that their texture images are more homogeneous, and a high separability between the classes when using principal component analysis (PCA) and decision trees for statistical analysis.


Subject(s)
Fibrous Dysplasia of Bone/diagnostic imaging , Image Processing, Computer-Assisted/methods , Maxillofacial Abnormalities/diagnostic imaging , Radiographic Image Interpretation, Computer-Assisted/methods , Tomography, Spiral Computed/methods , Facial Bones/diagnostic imaging , Female , Humans , Male , Maxilla/diagnostic imaging , Principal Component Analysis , Reproducibility of Results
15.
J Endod ; 41(11): 1923-6, 2015 Nov.
Article in English | MEDLINE | ID: mdl-26410152

ABSTRACT

Florid cemento-osseous dysplasia (FCOD) belongs to the group of fibro-osseous lesions in which normal bone is replaced by fibrous connective tissue and calcified cementum tissue of the avascular type. Among the various types of fibro-osseous lesions, FCOD is one of the most commonly encountered diseases in clinical practice and may involve 3 or 4 of the quadrants. FCOD is located in the periapical regions of teeth, and the lesions are predominantly radiolucent (osteolytic phase), become mixed over time (cementoblast phase), and ultimately become radiopaque (osteogenic phase) with a thin radiolucent peripheral halo. The characteristics of FCOD in the initial stages are similar to those of periapical lesions of inflammatory origin, which may lead to misdiagnosis. A 38-year-old woman sought dental care because of complaints of pain on the right side of her face. A clinical examination revealed no marked alterations; a panoramic radiograph was therefore requested and revealed the presence of radiolucent lesions associated with the periapical regions of some of the lower teeth. Thus, the professional referred the patient for endodontic treatment of the associated teeth with the justification that the lesions were of endodontic origin. However, the endodontist found that the teeth responded positively to a sensitivity test. The initial diagnosis could have resulted in unnecessary root canal treatment, but after careful clinical, radiographic, and tomographic assessments by different professionals, FCOD was diagnosed, conservatively treated, and regularly monitored. It is important that dentists have a basic knowledge of the various injuries that affect the jaw bones to prevent errors in diagnosis and treatment and to promote oral health.


Subject(s)
Diagnostic Errors , Fibrous Dysplasia of Bone/diagnosis , Fibrous Dysplasia of Bone/pathology , Osteomyelitis/diagnosis , Osteomyelitis/pathology , Periapical Periodontitis/diagnosis , Periapical Periodontitis/pathology , Adult , Diagnosis, Differential , Female , Fibrous Dysplasia of Bone/diagnostic imaging , Humans , Osteomyelitis/diagnostic imaging , Periapical Periodontitis/diagnostic imaging , Radiography, Panoramic
17.
Rev. otorrinolaringol. cir. cabeza cuello ; 72(3): 278-281, dic. 2012. ilus
Article in Spanish | LILACS | ID: lil-676834

ABSTRACT

La cefalea es uno de los motivos de consulta más frecuentes en medicina y se clasifica como primaria en cefaleas tensionales o migrañas y secundaria a tumores, infecciones u otras causas. Dentro del grupo de cefaleas o algias faciales secundarias se plantea la existencia del cuadro clínico de cefalea rinogénica generada por puntos de contacto de la mucosa de la cavidad nasal. En este artículo se presenta un caso de una paciente que posterior al fracaso del tratamiento farmacológico para migraña, consultó por algia facial persistente. Al realizar un examen clínico y con tomografía computarizada de las cavidades perinasales, se diagnosticó una cefalea rinogénica por contacto de mucosa secundaria a una displasia fibrosa del cornete medio. La resección quirúrgica del punto de contacto a través de una turbinectomía media realizada por cirugía endoscópica nasal abolió totalmente el dolor facial. Este caso recalca la utilidad y necesidad del estudio de las cavidades perinasales en aquellos pacientes que presentan cefalea o algia facial.


Headache is one of the most frequent symptoms in medicine that can be classified as primary, like tensional headache and migraine or secondary as in tumors, infections, or other causes. Rhinogenic headache is proposed as a clinical entity secondary to mucosal contact points within the nasal cavity. In this article we present a case of a patient that after a pharmacological treatment failure for migraine, consulted for sustained facial pain. After clinical examination and a paranasal sinus computed tomography, a rhinogenic headache secondary to middle turbinate fibrous dysplasia was diagnosed. Endoscopic surgical extirpation of contact mucosal points by a medial turbinectomy produced complete abolition of facial pain. This case illustrates the need and utility of studying paranasal sinus in those patients with headache or facial pain.


Subject(s)
Humans , Female , Adult , Turbinates/pathology , Fibrous Dysplasia of Bone/surgery , Fibrous Dysplasia of Bone/complications , Headache/etiology , Turbinates/surgery , Facial Pain/etiology , Tomography, X-Ray Computed , Fibrous Dysplasia of Bone/diagnostic imaging
18.
Article in English | MEDLINE | ID: mdl-22986246

ABSTRACT

OBJECTIVE: The aim of this multicenter study was to perform a histomorphometric analysis of peritrabecular clefting in fibrous dysplasia (FD) in an attempt to obtain data that could be useful for distinguishing between FD and ossifying fibroma (OF). STUDY DESIGN: A clinicopathologic analysis was performed in 68 patients diagnosed with FD and 37 patients diagnosed with OF. Histologic sections were scanned using an Aperio ScanScope CS. A histomorphometric analysis was performed with the aid of an image analyzer (UTHSCSA Image Tool 3.0 version) on 37 randomly selected samples of FD, and the results were compared with the 37 OF specimens. RESULTS: The presence of peritrabecular clefting was observed in 32 (86.5%) cases of FD, whereas no case of OF presented peritrabecular clefting. CONCLUSIONS: Peritrabecular clefting may be a hallmark of the lesions in patients with FD, and it may be a valuable microscopic feature for distinguishing it from OF.


Subject(s)
Fibroma, Ossifying/pathology , Fibrous Dysplasia of Bone/pathology , Jaw Neoplasms/pathology , Adult , Cone-Beam Computed Tomography , Diagnosis, Differential , Female , Fibroma, Ossifying/diagnostic imaging , Fibrous Dysplasia of Bone/diagnostic imaging , Humans , Jaw Neoplasms/diagnostic imaging , Male , Radiography, Panoramic , Retrospective Studies
19.
Gen Dent ; 57(3): e21-4, 2009.
Article in English | MEDLINE | ID: mdl-21466999

ABSTRACT

This article describes the case of a 34-year-old woman whose prosthodontist had referred her for root canal treatment of tooth No. 10, due to an initial diagnosis of apical periodontitis. Although periapical radiolucencies were present, teeth No. 10 and 11 responded positively to pulp vitality tests. A series of periapical radiographs revealed circumscribed periapical radiolucencies on teeth No. 21 and 28, while teeth No. 23, 24, and 27 were associated with mixed radiolucent/radiopaque periapical lesions. All teeth responded to cold stimulus. Based on the clinical and radiographic findings, a diagnosis of periapical cemento-osseous dysplasia was suggested. A re-evaluation 12 months later confirmed this diagnosis. Tooth No. 10 was restored and the patient was scheduled to return for clinical and radiographic follow-up after one year and three years.


Subject(s)
Cementoma/pathology , Fibrous Dysplasia of Bone/pathology , Periapical Diseases/pathology , Periapical Periodontitis/pathology , Adult , Alveolar Process/diagnostic imaging , Alveolar Process/pathology , Cementoma/diagnostic imaging , Diagnosis, Differential , Female , Fibrous Dysplasia of Bone/diagnostic imaging , Humans , Mandible , Maxilla , Osteolysis/diagnostic imaging , Osteolysis/pathology , Periapical Diseases/diagnostic imaging , Periapical Periodontitis/diagnostic imaging , Radiography
20.
Braz Dent J ; 20(4): 347-50, 2009.
Article in English | MEDLINE | ID: mdl-20069261

ABSTRACT

Florid cemento-osseous dysplasia (FCOD) has been described as a condition that characteristically affects the jaws of middle-aged black women. It usually exhibits as multiple radiopaque cemetum-like masses distributed throughout the jaws. Radiographically, FCOD appears as dense, lobulated masses, often symmetrically located in various regions of the jaws. Computed tomography, because of its ability to give axial, sagittal, and frontal views, is useful in the evaluation of these lesions. This paper presents the case of a patient who was diagnosed with FCOD on the basis of clinical and radiographic findings.


Subject(s)
Alveolar Process/pathology , Cementoma/pathology , Mandibular Neoplasms/pathology , Maxillary Neoplasms/pathology , Adult , Alveolar Bone Loss/etiology , Alveolar Bone Loss/surgery , Alveolar Process/diagnostic imaging , Cementoma/complications , Cementoma/diagnostic imaging , Dental Cementum/pathology , Female , Fibrous Dysplasia of Bone/diagnostic imaging , Fibrous Dysplasia of Bone/pathology , Humans , Mandibular Neoplasms/diagnostic imaging , Maxillary Neoplasms/complications , Maxillary Neoplasms/diagnostic imaging , Radiography , Tooth Extraction , Treatment Outcome , White People
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