Neurofibrosarcoma of the mandible derived from neurofibromatosis

Neurofibrosarcoma is a rare malignant neoplasm of the head and neck region and accounts for 8% to 16% of all cases. Its origin is varied and may stem from cells of the peripheral nerves, develop de novo, or result from malignant transformation of preexisting neurofibromas. Because the features of neurofibrosarcomas are heterogeneous, the data retrieved during clinical examinations are of great aid for diagnosis. In this case, owing to clinical features and the fact that the patient had neurofibromatosis type 1, the hypothesis of neurofibrosarcoma was promptly established. The final diagnosis was confirmed by associating clinical, imaging, and pathological data. After the treatment, the patient has been followed up for 10 years, with no evidence of recurrence.

Neurofibrosarcoma of the mandible derived from neurofibromatosis.

Neurofibrosarcoma is a rare malignant neoplasm of the head and neck region and accounts for 8% to 16% of all cases. Its origin is varied and may stem from cells of the peripheral nerves, develop de novo, or result from malignant transformation of preexisting neurofibromas. Because the features of neurofibrosarcomas are heterogeneous, the data retrieved during clinical examinations are of great aid for diagnosis. In this case, owing to clinical features and the fact that the patient had neurofibromatosis type 1, the hypothesis of neurofibrosarcoma was promptly established. The final diagnosis was confirmed by associating clinical, imaging, and pathological data. After the treatment, the patient has been followed up for 10 years, with no evidence of recurrence.

Simple Bone Cyst Mimicking Stafne Bone Defect.

Stafne bone defects (SBDs) are asymptomatic lingual bony defects in mandible, which are usually detected as an incidental finding in plain radiographs. Similarly, simple bone cysts (SBCs) are most frequently found in posterior region of the mandible and also are asymptomatic. However, due to anatomical structures overlap in plain radiographs, there is a possibility of misdiagnosing SBD that often is diagnosed by presumptive diagnosis observed in plain radiograph. This report shows an unusual case that a SBC resembles a SBD.

Impact of Temporomandibular Joint Discectomy on Condyle Morphology: An Animal Study.

PURPOSE: Temporomandibular disorders lead to parafunctional activity that may alter bone remodeling of mandibular components. This animal study aimed to assess the impact of temporomandibular joint discectomy on condylar bone microarchitecture. MATERIALS AND METHODS: A total of 30 one-month-old Wistar rats were assessed and divided into 3 equal groups (2 test groups and 1 control group) of 10. The first test group underwent disc removal, the second test group underwent disc and condylar cartilage removal, and the 10 remaining rats were analyzed as sham-operated controls, following a split-mouth design. The rats were killed humanely 2 months after surgery, and the respective mandibles were scanned with micro-computed tomography for quantitative morphometric analysis. RESULTS: There were significant differences among the 3 groups analyzed (disc removal, disc and condylar cartilage removal, and sham-operated control) for bone volume fraction (ratio of bone volume to total volume, P = .044), structure model index (P < .001), fractal dimension (P = .024), and porosity (P = .023). In addition, operated and contralateral nonoperated sides significantly differed for all variables in at least 1 of the test groups (P < .05) but not in the control group (P > .05). CONCLUSIONS: Within the limitations of this study, our results suggest that discectomy may lead to alterations of the mandibular condylar morphology.

Mantle cell lymphoma: involvement of nodal and extranodal sites in the head and neck, with multifocal oral lesions.

Mantle cell lymphoma (MCL) is a malignant B-cell neoplasm, which comprises monomorphic and small- to medium-sized mantle zone-derived lymphoid cells. It is characterized by chromosomal translocation t(11;14)(q13;q32) and CCND1 truncation, resulting in cell cycle deregulation. It is an aggressive type of non-Hodgkin lymphoma with a propensity to present with extranodal involvement. This study shows the case of an 80-year-old Caucasian male who complained of a 2-month progressive swelling on the right side of his face. The magnetic resonance imaging exam showed multifocal involvement of the head and neck, including oral manifestations, bilateral parotid glands, palate, tongue, and floor of the mouth. An incisional biopsy of the tumor mass was performed. The morphological and immunophenotypic findings were consistent with the diagnosis of MCL. The patient died 4 months later, without any chance of undergoing a therapeutic approach. Although MCL is a rare condition, it should be subjected to a differential diagnosis when affecting the maxillofacial area. Imaging exams and both immunohistochemical and morphological analyses are needed to reach the correct diagnosis. Here, we present an unusual MCL with multifocal involvement of the head and neck.

Mantle cell lymphoma: involvement of nodal and extranodal sites in the head and neck, with multifocal oral lesions

Mantle cell lymphoma (MCL) is a malignant B-cell neoplasm, which comprises monomorphic and small- to medium-sized mantle zone-derived lymphoid cells. It is characterized by chromosomal translocation t(11;14)(q13;q32) and CCND1 truncation, resulting in cell cycle deregulation. It is an aggressive type of non-Hodgkin lymphoma with a propensity to present with extranodal involvement. This study shows the case of an 80-year-old Caucasian male who complained of a 2-month progressive swelling on the right side of his face. The magnetic resonance imaging exam showed multifocal involvement of the head and neck, including oral manifestations, bilateral parotid glands, palate, tongue, and floor of the mouth. An incisional biopsy of the tumor mass was performed. The morphological and immunophenotypic findings were consistent with the diagnosis of MCL. The patient died 4 months later, without any chance of undergoing a therapeutic approach. Although MCL is a rare condition, it should be subjected to a differential diagnosis when affecting the maxillofacial area. Imaging exams and both immunohistochemical and morphological analyses are needed to reach the correct diagnosis. Here, we present an unusual MCL with multifocal involvement of the head and neck.

Strontium Ranelate Effect on the Repair of Bone Defects and Molecular Components of the Cortical Bone of Rats.

This study was conducted to evaluate the effects of treatment with strontium ranelate (SR) on the repair of bone defects and molecular components of bones in femurs. Adult female rats (n=27) were subjected to ovariectomy (OVX) or Sham surgery. Thirty days after surgery, a defect was made in the femur and the animals were then divided into three groups: OVX, SHAM and OVX+SR. Euthanasia was performed four weeks after the bone defect surgery. Repair in bone defect was assessed by computed microtomography (µCT) and chemical composition of cortical bone was analyzed by Fourier transform infrared (FTIR) spectroscopy and energy dispersive X-ray spectroscopy (EDS). The trabecular thickness (Tb.Th) of the newly formed bone in the OVX+SR group was significantly higher than that for the OVX group. The collagen maturity in the OVX+SR group was smaller than in the other two groups. In this group, a significant increase in the amount of strontium (Sr) and a decrease in the amount of calcium (Ca) embedded to bone tissue were also observed. Systemic treatment with SR improved microarchitecture of the newly formed bone inside the defect, but decreased cross-linking of mature collagen in cortical bone.

Strontium Ranelate Effect on the Repair of Bone Defects and Molecular Components of the Cortical Bone of Rats

Abstract This study was conducted to evaluate the effects of treatment with strontium ranelate (SR) on the repair of bone defects and molecular components of bones in femurs. Adult female rats (n=27) were subjected to ovariectomy (OVX) or Sham surgery. Thirty days after surgery, a defect was made in the femur and the animals were then divided into three groups: OVX, SHAM and OVX+SR. Euthanasia was performed four weeks after the bone defect surgery. Repair in bone defect was assessed by computed microtomography (μCT) and chemical composition of cortical bone was analyzed by Fourier transform infrared (FTIR) spectroscopy and energy dispersive X-ray spectroscopy (EDS). The trabecular thickness (Tb.Th) of the newly formed bone in the OVX+SR group was significantly higher than that for the OVX group. The collagen maturity in the OVX+SR group was smaller than in the other two groups. In this group, a significant increase in the amount of strontium (Sr) and a decrease in the amount of calcium (Ca) embedded to bone tissue were also observed. Systemic treatment with SR improved microarchitecture of the newly formed bone inside the defect, but decreased cross-linking of mature collagen in cortical bone.

Resumo Este estudo foi conduzido para avaliar os efeitos do tratamento com ranelato de estrôncio (RE) na reparação de defeitos ósseos e componentes moleculares de ossos nos fêmures. Ratas adultas (n = 27) foram submetidas a ovariectomia (OVX) ou cirurgia Sham. Trinta dias após a cirurgia, um defeito foi feito no fêmur e os animais foram então divididos em três grupos: OVX, SHAM e OVX+RE. A eutanásia foi realizada quatro semanas após a cirurgia de preparo do defeito ósseo. A reparação do defeito ósseo foi avaliada por microtomografia computorizada (μCT) e a composição química do osso cortical foi analisada por espectroscopia de infravermelho de transformada de Fourier (FTIR) e espectroscopia por energia dispersiva de raios X (EDS). A espessura do osso trabecular (Tb.Th) recém formado no grupo OVX+SR foi significativamente maior que a do grupo OVX. A maturidade do colágeno no grupo OVX+SR foi menor do que nos outros dois grupos. Neste grupo, observou-se também um aumento significativo na quantidade de estrôncio (Sr) e uma diminuição na quantidade de cálcio (Ca) no tecido ósseo. O tratamento sistêmico com RE melhorou a microarquitetura do osso recém formado dentro do defeito, mas diminuiu a reticulação do colágeno maduro no osso cortical.

Fibrosarcoma ameloblástico en la mandíbula / Ameloblastic fibrosarcoma arising in the mandible

Ameloblastic fibrosarcoma is a rare odontogenic neoplasm and is considered the malignant counterpart of ameloblastic fibroma. The diagnosis is made by histopathological and immunohistochemical evaluation, since the epithelial component remains benign and the mesenchymal component becomes malignant. Until 2012, only 72 cases were published in English-literature. This article presents a case of intraoral mass at the posterior mandible of a 23 year-old female patient. Panoramic radiography showed a multilocular radiolucent lesion with ill-defined borders and tooth involvement. The mandibular canal presented loss of architecture also. The computed tomography images (bone window) showed hypodense lesion leading to expansion, tapering and irregular destruction of cortical, and tooth involvement. Incisional biopsy was performed for histopathological evaluation. The results revealed a mixed lesion with epithelial and mesenchymal cellular proliferation. At immunohistochemical analysis, the mesenchymal portion was vimentin positive and the epithelial component was positive for cytokeratin AE1-AE3. It also showed p53 intense labeling in all tumorous cells. The final diagnosis was ameloblastic fibrosarcoma. The lesion was surgically excised with clear margins. The radiographic appearance, even imperative for treatment planning, poorly contributed to final diagnosis, which was reached by histopathological and immunohistochemical evaluations. The treatment is still controversial, without a definition regarding chemotherapy and radiotherapy as coadjutant treatment(AU)

El fibrosarcoma ameloblástico es una neoplasia odontogénica poco frecuente y es considerada la contraparte maligna del fibroma ameloblástico. El diagnóstico se realiza mediante la evaluación histopatológica e inmunohistoquímica, ya que el componente epitelial sigue siendo benigno y el componente mesenquimal se convierte en maligno. Hasta 2012, solo 72 casos fueron publicados en la literatura inglesa. En este artículo se presenta un caso de masa intraoral en la mandíbula parte posterior, de una paciente de 23 años de edad. La radiografía panorámica mostró una lesión radiolúcida multilocular con bordes mal definidos y con un diente incluso en la lesión. El canal mandibular también presentaba pérdida de la arquitectura. La tomografía computarizada (TC) (ventana de hueso) presentó lesión hipodensa que provocaba una expansión que se estrechaba y destruía irregularmente la cortical, además envolvía la pieza dentaria. Se realizó biopsia incisional para evaluación histopatológica. Los resultados revelaron una lesión mixta con proliferación celular epitelial y mesenquimal. En el análisis inmunohistoquímico, la porción mesenquimal fue positivo para vimentina y el componente epitelial fue positivo para citoqueratina AE1-AE3. También mostró marcación intensa para p53 en todas las células tumorales. El diagnóstico final fue de fibrosarcoma ameloblástico. La lesión fue extirpada quirúrgicamente con márgenes de seguridad. El aspecto radiológico, aunque imprescindible para la planificación del tratamiento, poco contribuyó al diagnóstico final, que fue alcanzado por las evaluaciones histopatológicas e inmunohistoquímicas. El tratamiento sigue siendo controvertido, sin una definición respecto de la quimioterapia y la radioterapia como tratamiento coadyuvante(AU)

Reliability and Reproducibility of Manual and Automated Volumetric Measurements of Periapical Lesions.

INTRODUCTION: The present study aimed to test the reliability and reproducibility of 2 methods: manual and automated segmentation (using a threshold-based region growing algorithm) for measuring the volume of periapical lesions. METHODS: A total of 43 cone-beam computed tomographic scans (14 men and 29 women, mean age of 54.6 ± 8.5 years) were analyzed by 3 observers. Intraobserver reproducibility and interobserver reliability were assessed using the intraclass correlation coefficient. Parametric correlation between manual and automated volumetric measurements was performed. In addition, the Student t test was also used to compare the mean time required for manual and automated volumetric measurements. RESULTS: Automated segmentation showed slightly higher intraclass correlation coefficient values for all observers. A strong significant correlation was found between manual and automated volumetric measurements performed by the 3 observers. A significant difference in the mean procedure time was also found between both methods (P = .001). CONCLUSIONS: Within the limitations of this study, the present results suggest that automated segmentation with a region growing algorithm is faster and slightly more reliable to calculate the volume of periapical lesions.

Evaluation of basilar expansion and internal septa of human sphenoidal sinus using cone beam computed tomography

O objetivo deste estudo foi avaliar os tipos e as frequências de expansão basilar do seio esfenoidal e septos internos utilizando tomografi a computadorizada de feixe cônico. Imagens arquivadas de 300 indivíduos adultos de ambos os gêneros foram recuperadas. Foi realizada uma análise descritiva relacionando idade e gênero à expansão basilar do seio esfenoidal e a tipos de septos internos e frequências. As associações entre expansão basilar do seio esfenoidal, septos internos e gênero para cada grupo de idade foram avaliadas por meio do teste do qui-quadrado ou teste exato de Fisher. Entre todas as imagens avaliadas, 69% apresentaram expansão basilar do seio esfenoidal, das quais 81% foram consideradas críticas. Septos internos foram observados em 60% das imagens. Não houve relação entre presença de expansão basilar do seio esfenoidal, gênero e idade. Septos internos apresentaram-se independentes do gênero; no entanto, dentre os indivíduos com mais de 40 anos de idade, 36% tinham apenas um septo principal, 6% tinham septos acessórios, e 18% tinham ambos os tipos de septos. A tomografia computadorizada é um método preciso que deve ser considerado para a avaliação desse segmento anatômico a fi m de evitar a exposição desnecessária à radiação.

The objective of this study was to assess the types and frequencies of basilar expansion of the sphenoidal sinus and internal septa by using cone beam computed tomography. Archived images from 300 adult subjects of both genders were retrieved. A descriptive analysis relating age and gender to basilar expansion of the sphenoidal sinus and internal septa types and frequencies was performed. The associations between basilar expansion of the sphenoidal sinus, internal septa and gender for each age group were assessed using the chi-square test or Fisher’s exact test. Among all the images evaluated, 69% showed basilar expansion of the sphenoidal sinus, of which 81% were considered critical. Internal septa were observed in 60% of the images. There was no relationship between the presence of basilar expansion of the sphenoidal sinus and gender and age. Internal septa were independent of gender; however, of the subjects older than age 40, 36% had only a main septum, 6% had accessory septa, and 18% had both types of septa. Cone beam computed tomography is an accurate method that should be considered for the evaluation of this anatomic segment in order to avoid unnecessary exposure to radiation.

Correlation among the changes observed in temporomandibular joint internal derangements assessed by magnetic resonance in symptomatic patients.

PURPOSE: The objective was to assess possible correlations among the anatomy and position of the articular disc, the morphology of the articular eminence and of the condyle, and the presence of joint effusion. MATERIALS AND METHODS: Magnetic resonance images of 142 joints of symptomatic patients were assessed. The articular disc was classified as normal, elongated, or folded; displacement was classified as normal, anterior with reduction, anterior without reduction, or posterior, lateral, or medial; the condyle was classified as rounded, convex, flattened, or angulated; and the articular eminence was classified as box, sigmoid, flattened, or deformed. RESULTS: The most frequent forms of the articular disc, articular eminence, and condyle were normal, box, and flattened, respectively. Associations were confirmed between the form of the articular disc and its anterior and lateral positions; between the form of the condyle and an anterior position of the disc; and between the form of the articular eminence and the form of the disc. The form of the articular eminence was the only variant that was not associated to the presence of joint effusion. CONCLUSIONS: Magnetic resonance imaging allowed the clear observation of articular structures, without attributing a cause-and-effect relation. The prevalence of changes observed was associated with the diagnosis of internal derangement and was statistically proved.

Precisão de medições lineares em tomografias computadorizadas por feixe cônico e helicoidal, realizadas em mandíbulas humanas / Accuracy of linear bone measurements cone-beam and spiral computed tomography in human mandibles

Introdução: a obtenção de métodos de imagem capazes de reproduzir com adequada precisão as dimensões maxilomandibulares é de vital importância para o diagnóstico e o planejamento seguro de procedimentos cirúrgicos. Objetivo: verificar, in vitro, a precisão de medições lineares de um exame de tomografia computadorizada helicoidal e de dois sistemas de tomografia computadorizada por feixe cônico. Método: dez mandíbulas humanas secas foram submetidas a três diferentes exames de tomografia: CBCT i-CAT, CBCT NewTom 3G e HCT helicoidal Picker CT Twin Flash. As medições realizadas diretamente na mandíbula foram feitas com um paquímetro digital, e as tomográficas por meio do software ImplantViewer 2.604. Foram mensuradas seis regiões de cada mandíbula seca, sendo agrupadas em duas regiões de primeiro molar inferior (Sítio PMI), duas regiões de primeiro pré-molar inferior (Sítio PPMI) e em duas regiões de incisivo lateral inferior (Sítio ILI). Resultados: nos sítios ILI e PPMI, as três tomografias estudadas demonstraram precisão semelhante entre si. No sítio PMI, a CBCT i-CAT mostrou-se mais precisa que as dos demais sistemas. Conclusão: pode-se concluir que nos sítios ILI e PPMI as três TCs estudadas apresentam limites de concordância e de precisão semelhantes, e que no sítio PMI a CBCT i-CAT apresentou um limite de concordância de menor amplitude, sendo mais precisa que a dos demais exames.

Availability of imaging methods able to accurately reproducing the maxillo-mandibular dimensions is important for diagnosis and safe planning of surgical procedures. Aim: The aim of this in vitro study was to verify the accuracy of linear measurements in images obtained with a system of spiral and two systems of cone-beam computed tomography (CT). Methods: Ten dry human mandibles were subjected to three different CT scans: I-Cat® CBCT, NewTom-3G® CBCT, and Picker® SCT. Measurements in the mandible were made with a digital caliper and measurements in the images were made with the ImplantViewer® software. Six regions were measured in each dry mandible, being distributed into two regions in each of the lower first molar (LFM), lower first pre-molar (LFPM), and lower lateral incisor (LLI) sites. Results: Similar accuracy was observed among the three images at sites LLI and LFPM. Measurements obtained with the I-Cat CBCT scan at site LFM were shown to be more accurate than the those obtained with the other two CT scan systems. Conclusions: It can be concluded that the three CTs studied herein showed similar limits of agreement and precision at sites LLI and LFPM, and I-Cat CBCT showed limits of agreement with smaller amplitude and greater accuracy than other examinations performed at site LFM. Conclusion: It can be concluded that the three CTs studied herein showed similar limits of agreement and precision at sites LLI and LFPM, and ICat CBCT showed limits of agreement with smaller amplitude and greater accuracy than other examinations performed at site LFM.

Assessing joint effusion and bone changes of the head of the mandible in MR images of symptomatic patients

The aim of the present study was to investigate the relationship between degenerative bone changes of the head of the mandible and the presence of joint effusion (JE). This study was based on sagittal magnetic resonance imaging (MRI) reports of 148 temporomandibular joints (TMJs) of 74 patients complaining of pain and/or dysfunction in the TMJ area. The mandible heads were surveyed for osteoarthritis characteristics, which were classified as osteophytosis, sclerosis or erosion. The presence of JE was checked whenever high signal intensity was observed in the articular space. The results evidenced the presence of bone changes in 30% of the sample. Osteophytes and erosions were the changes most commonly observed. JE was reported in 10% of TMJs. The results from the statistical tests revealed that bone changes in the head of the mandible are associated with the presence of JE.

Assessing joint effusion and bone changes of the head of the mandible in MR images of symptomatic patients.

The aim of the present study was to investigate the relationship between degenerative bone changes of the head of the mandible and the presence of joint effusion (JE). This study was based on sagittal magnetic resonance imaging (MRI) reports of 148 temporomandibular joints (TMJs) of 74 patients complaining of pain and/or dysfunction in the TMJ area. The mandible heads were surveyed for osteoarthritis characteristics, which were classified as osteophytosis, sclerosis or erosion. The presence of JE was checked whenever high signal intensity was observed in the articular space. The results evidenced the presence of bone changes in 30% of the sample. Osteophytes and erosions were the changes most commonly observed. JE was reported in 10% of TMJs. The results from the statistical tests revealed that bone changes in the head of the mandible are associated with the presence of JE.

Assessment of osteoporotic alterations in achondroplastic patients: a case series.

Achondroplasia (ACH) is the most common form of human dwarfism and has been associated with biochemical alterations of the bone tissue, also observed in cases of osteoporosis. The present case series aimed at assessing low bone density, diagnosed with spinal bone mineral density (BMD) analysis and with panoramic radiograph measurements, in ACH patients. Spinal BMD was measured by means of dual-energy X-ray absorptiometry at the lumbar region (L1-L4). On dental panoramic radiographs of the patients, the mandibular cortical width was measured separately on the right and left sides. The Klemetti Index was also assigned as appropriate for evaluating the cortical area below the mandibular foramen. Additional parameters such as patient age, gender, body mass index, and number of teeth were also recorded. BMD results showed that 5/11 cases presented with skeletal osteopenia/osteoporosis diagnoses. Additionally, mandibular cortical erosion was detected in panoramic radiographs in 8/11 cases. The BMD and panoramic radiographic alterations found in this study suggest that the diagnosis of low bone density may have a special clinical relevance in cases of bone tissue disorders, such as achondroplasia.

Analysis of the cephalometric pattern of Brazilian achondroplastic adult subjects

OBJECTIVE: The aim of this study was to assess the position of the cranial base, maxilla, and mandible of Brazilian achondroplastic adult subjects through cephalometric measurements of the cranio-dento-facial complex, and to compare the results to normal patterns established in literature. METHODS: Fourteen achondroplastic adult subjects were evaluated based on their radiographic cephalometric measurements, which were obtained using the tracings proposed by Downs, Steinner, Bjork, Ricketts and McNamara. Statistical comparison of the means was performed with Student's t test. RESULTS: When compared to normal patterns, the cranial base presented a smaller size in both its anterior and posterior portions, the cranial base angle was acute and there was an anterior projection of the porion; the maxilla was found to be smaller in size in both the anteroposterior and transversal directions, it was inclined anteriorly with anterior vertical excess, and retropositioned in relation to the cranial base and to the mandible; the mandible presented a normal-sized ramus, a decreased body and transverse dimension, a tendency towards vertical growth and clockwise rotation, and it was slightly protruded in relation to the cranial base and maxilla. CONCLUSION: Although we observed wide individual variation in some parameters, it was possible to identify significant differences responsible for the phenotypical characteristics of achondroplastic patients.

OBJETIVO: avaliar o tamanho e o posicionamento da base do crânio, da maxila e da mandíbula de indivíduos acondroplásicos brasileiros adultos, a partir de medidas cefalométricas do complexo dentoesqueletofacial. Confrontar os dados obtidos aos padrões de normalidade estabelecidos na literatura. MÉTODOS: foram avaliados 14 indivíduos acondroplásicos adultos, utilizando algumas grandezas cefalométricas radiográficas obtidas a partir dos traçados preconizados por Downs, Steinner, Björk, Ricketts e McNamara. Para análise estatística, foi aplicado o teste t de Student na comparação de médias. RESULTADOS: em relação aos padrões de normalidade estabelecidos, a base craniana apresentou dimensões diminuídas, tanto na porção anterior quanto na posterior. O ângulo da base craniana apresentou-se agudo e houve projeção anterior do pório. A maxila apresentou dimensões menores no sentido anteroposterior e transversal, anteroinclinada, excesso vertical anterior e retroposicionada em relação à base do crânio e da mandíbula. A mandíbula apresentou ramos com dimensão normal e redução nas dimensões do corpo e transversal, tendência de crescimento vertical e rotação no sentido horário, discretamente protruída em relação à base do crânio e da maxila. CONCLUSÃO: ainda que tenha sido observada ampla variação individual em alguns dos parâmetros, foi possível identificar diferenças significativas responsáveis pela apresentação fenotípica característica dos pacientes acondroplásicos.

Manifestação da artrite psoriática na articulação temporomandibular - características imaginológicas / Manifestation of psoriatic arthritis in the temporomandibular joint - imaging features

A artrite psoriática é uma artropatia crônica de etiologia e patogênese não totalmente esclarecidas, que, embora rara, pode acometer a articulação temporomandibular. É definida como psoríase associada com artrite infla-matória, erosiva e crônica, a qual é soronegativa para o fator reumatoide. Objetivo: O objetivo do presente estudo é apresentar uma revisão de literatura sobre as características imaginológicas da artrite psoriática na articulação tempoporomandibular. Revisão da literatura: A artrite psoriática pode se apresentar por diversos padrões de envolvimento articular com graus variados de severidade, seguido um curso desde uma leve sinovite a uma artropatia erosiva progressiva. A elaboração do diagnóstico diferencial pode apresentar variados graus de dificuldade. Assim, uma história detalhada do paciente e exame físico geral são de extrema importância para elaboração do diagnóstico e devem ser considerados quando da avaliação das imagens das le-sões articulares. O uso da tomografia computadorizada no diagnóstico de artrite psoriática deve fazer parte do processo de avaliação. A ressonância magnética, o ultrassom e a cintilografia têm papel importante no diagnóstico precoce da artrite psoriática na articulação temporomandibular e no monitoramento da evolução da doença, podendo indicar, inclusive, a alteração do seu tratamento. Considerações finais: Conclui-se que a artrite psoriática envolvendo a articulação temporomandibular tem como características a dor e a limitação funcional, causando destruição progressiva dos componentes ósseos e dos teciduais articulares, clara-mente indicada em exames imaginológicos. Assim, a ressonância magnética assume papel fundamental no diagnóstico, monitoramento da evolução da doença e na orientação quanto ao seu tratamento.

Análise por meio de ressonância magnética em pacientes sintomáticos da presença de derrame articular na articulação temporomandibular em função do posicionamento do disco articular / Analysis by magnetic resonance imaging in symptomatic patients of the presence of joint effusion in temporomandibular joint related to articular disc position

A articulação temporomandibular (ATM) é considerada a articulação mais complexa do corpo humano. Assim como outras do sistema músculo-esquelético, ela pode ser acometida pelas mesmas doenças e desordens, como por exemplo, deslocamento de disco (DD), doenças articulares degenerativas (osteoartrite - OA), artrites inflamatórias e sinovites. Baseado nos laudos obtidos por meio das análises das imagens em norma sagital das ATMs de pacientes sintomáticos encaminhados para a realização de exame por ressonância magnética (RM) foi avaliada a presença de derrame articular (DA) decorrente do posicionamento do disco articular. A amostra foi constituída por 148 ATMs de 74 pacientes sintomáticos. Os resultados dos testes estatísticos revelaram que a presença de DA está associada ao posicionamento do disco articular.

Apert syndrome: clinical and radiographic features and case report / Síndrome de Apert: características clínicas e radiográficas e relato de caso

PURPOSE: Apert syndrome is a rare type I acrocephalosyndactyly syndrome characterized by craniosynostosis, severe syndactyly of the hands and feet, and dysmorphic facial features. Presents autosomal dominant inheritance assigned to mutations in the fibroblast growth factor receptors gene. The oral cavity of Apert patients includes a reduction in the size of the maxilla, tooth crowding, anterior open-bite of the maxilla, impacted teeth, delayed eruption, ectopic eruption, supernumerary teeth, and thick gingiva. The mandible usually is within normal size and shape, and simulates a pseudoprognathism. CASE DESCRIPTION: A female patient, 13 years old, with diagnosis of Apert syndrome, attended a dental radiology clinic. The clinical signs were occular anomalies, dysmorphic facial features, syndactyly and oral features observed clinically and radiographically. The patient was referred to a specialized center of clinical care for patients with special needs. CONCLUSION: Because of the multiple alterations in patients with Apert syndrome, a multidisciplinary approach, including dentists and neurosurgeons, plastic surgeons, ophthalmologists and geneticists, is essential for a successful planning and treatment.

OBJETIVO: A síndrome de Apert é um tipo raro de acrocefalossindactilia do tipo I caracterizada por cranioestenose, sindactilia severa das mãos e dos pés, e características faciais dismórficas. Apresenta herança autossômica dominante atribuída a mutações no gene referente aos receptores do fator de crescimento de fibroblastos. A cavidade oral de pacientes de Apert apresenta uma redução no tamanho da maxila, apinhamento dentário, mordida aberta anterior da mandíbula superior, dentes retidos, erupção atrasada, erupção ectópica, dentes supranumerários, gengival e espessura. A mandíbula é no tamanho e forma normais, e simula um pseudoprognatismo. Um caso de síndrome de Apert é apresentado. DESCRIÇÃO DO CASO: Paciente do sexo feminino, 13 anos de idade, com diagnóstico de síndrome de Apert, foi atendida em um serviço de radiologia odontológica. A paciente apresentava anomalias oculares, características faciais dismórficas e sindactilia, aos exames clínico e radiográfico. A paciente foi encaminhada a um centro especializado para atenção a pessoas com necessidades especiais. CONCLUSÃO: Uma abordagem multidisciplinar, incluindo cirurgiões-dentistas e neurocirurgiões, cirurgiões plásticos, oftalmologistas e geneticistas, é essencial para um planejamento bem sucedido e tratamento de casos de síndrome de Apert devido às múltiplas alterações nesses pacientes.