Displasia fibrosa en seno etmoidal y esfenoidal: a propósito de un caso

Resumen La displasia fibrosa es una enfermedad del hueso, benigna, idiopática, de base genética y de progresión lenta, que se caracteriza por el reemplazo progresivo del hueso normal con tejido fibrótico, entremezclado con trabéculas óseas irregulares. El cráneo también es un sitio frecuente de afectación, los huesos del complejo craneofacial, incluida la mandíbula, el maxilar, la base y la bóveda craneal, son los principalmente afectados. Los huesos etmoidales, esfenoidales, frontales y temporales son afectados con poca frecuencia. En este artículo se presenta un caso de una paciente con cefalea y dolor en hemicara derecha, exoftalmos y edema periorbitario ipsilateral. Luego del examen físico, se realizó tomografía computarizada y biopsia del tumor, llegando al diagnóstico de displasia fibrosa de seno etmoidal y esfenoidal. Se maneja en forma conservadora, con seguimiento cada 6 meses para evaluar evolución. Es un caso con una localización infrecuente, y que debe ser cuidadosamente evaluado para adoptar la conducta terapéutica correcta.

Abstract Fibrous dysplasia is a slowly progressive, genetically based, benign, idiopathic bone disease characterized by progressive replacement of normal bone with fibrotic tissue, interspersed with irregular bone trabeculae. The skull is also a frequent site of involvement, the bones of the craniofacial complex, including the mandible, the maxilla, the base and the cranial vault, are mainly affected. The ethmoid, sphenoid, frontal, and temporal bones are affected at a low rate. This article presents a case of a patient with headache and pain in the right side, exophthalmos, and ipsilateral periorbital edema. After the physical examination, a computed tomography and biopsy of the tumor were performed, reaching the diagnosis of fibrous dysplasia of the ethmoid and sphenoid sinus. Conservative management is given, with follow-up every 6 months to assess evolution. It is a case with an infrequent location, and it must be carefully evaluated to take therapeutic behavior.

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)

Análise de imagem de lesões fibro-ósseas / Fibro-osseous lesions image analysis

As lesões fibro-ósseas (LFOs) caracterizam-se pela substituição do osso normal por tecido conjuntivo fibroso e material mineralizado. Displasia fibrosa, fibroma ossificante e displasia cemento-óssea (DCO) compõem este grupo, que além da semelhança histopatológica, pode sobrepor características radiográficas. A correlação de dados clínicos, radiográficos e histopatológicos é necessária para o diagnóstico definitivo. Este trabalho teve como objetivo descrever e avaliar exames odontológicos de imagem de indivíduos diagnosticados com LFOs. Foram desenvolvidos três estudos com metodologias independentes. O primeiro estudo avaliou os efeitos da displasia cemento-óssea em estruturas anatômicas adjacentes por meio de tomografia computadorizada de feixe cônico (TCFC). Foram avaliados 60 casos de DCO, com 244 áreas de lesão envolvendo 426 dentes. A maioria das lesões apresentou aproximadamente 10 mm de diâmetro. Afilamento (n=80) expansão (n=62) e perfuração (n=60) foram os efeitos comuns na cortical óssea. A lâmina dura e o espaço do ligamento periodontal foram descontínuos em todos os dentes. No segundo estudo, 50 radiografias panorâmicas de mulheres com diagnóstico de DCO foram pareadas por idade com 50 radiografias panorâmicas de mulheres sem DCO. Os grupos DCO e não-DCO foram comparados através do índice de espessura da cortical mandibular (ECM), a dimensão fractal (DF) da região de osso trabecular e cortical e o índice cortical mandibular (ICM). O índice de ECM do grupo DCO foi de 3,12 mm (2,15-4,55) e do grupo não-DCO foi 3,52 mm (1,90- 4,70) com diferença estatística entre os grupos (p=0,034). A DF do trabeculado ósseo alveolar normal foi semelhante nos dois grupos, enquanto que menores médias de DF no osso cortical foram encontradas no grupo DCO (p=0,046). A classificação C3 do ICM foi mais comum no grupo DCO (p=0,009). O terceiro foi um estudo preliminar que avaliou a DF em imagens bidimensionais e tridimensionais de TCFC de seis casos de displasia fibrosa e seis de fibroma ossificante. Cortes de reconstrução panorâmica, axial, coronal e sagital foram selecionados em quatro espessuras: 0,25; 5; 15 e 25 mm, e avaliados em 3 formatos de regiões de interesse (RI). Duas metodologias compararam quatro formatos de volumes do interior das lesões. A média de DF das imagens bidimensionais de reconstrução panorâmica e axiais, com 0,25 mm de espessura mostrou diferença significativa entre displasia fibrosa e fibroma ossificante (p<0.05), independente do formato da RI (p>0,05). A média da DF de imagens tridimensionais foi diferente entre os grupos de lesões para a maioria das comparações (p<0.05), independente da metodologia usada. Informações detalhadas sobre a lesão e o envolvimento de estruturas adjacentes podem ser visualizadas em TCFC. Métodos não invasivos como os índices radiomorfométricos e análise fractal podem ser úteis na detecção de baixa densidade mineral óssea, ou mesmo como possível ferramenta de estudo da complexidade de imagens de LFOs.

Fibro-osseous lesions (FOLs) are characterized by the replacement of normal bone with fibrous connective tissue and mineralized material. The group includes the fibrous dysplasia, ossifying fibroma and cemento-osseous dysplasia (COD). These three lesions show similar histopathological and radiographic features. Clinical, radiographic and histopathological data correlation is necessary for the definitive diagnosis. This study aimed to describe and to evaluate dental imaging exams of individuals diagnosed with FOLs. Three studies with independent methodologies were developed. The first study evaluated the effects of COD on the adjacent anatomical structures by means of cone beam computed tomography (CBCT). Sixty cases of COD were evaluated. The assessment of the 60 cases involved 244 lesion areas and 426 teeth. Most lesions presented nearly 10 millimeters (mm) of diameter. Thinning (n=80), expansion (n=62) and perforation (n=60) were common effects on the cortical bone. All 426 teeth presented discontinuation of the lamina dura and of the periodontal ligament space. In the second study, 50 panoramic radiographies of women diagnosed with COD were matched with 50 panoramic radiographies of women without COD according to the individuals' age. Comparisons between groups regarding the mandibular cortical width index (MCW), the fractal dimension (FD) of the region of the trabecular and the cortical bone and the mandibular cortical index (MCI) were carried out. The MCW of the COD group was 3.12 mm (2.15-4.55) and the MCW of the non-COD group was 3.52 mm (1.90-4.70). A significant difference between groups was observed (p=0.034). FD of the normal trabecular alveolar bone was similar in both groups. For the cortical bone, a lower mean FD was observed in the COD group (p=0.046). The MCI C3 was more common in the COD group (p=0.009). The third was a preliminary study, in which the FD was evaluated on twodimensional and three-dimensional CBCT images of six cases of fibrous dysplasia and six cases of ossifying fibroma. Panoramic reconstruction, axial, coronal and sagittal slices were selected in four thicknesses (0.25, 5, 15 and 25 mm) and evaluated in three shapes of regions of interest (ROI). Two methodologies compared four volume formats from inside the lesions. A significant difference between fibrous dysplasia and ossifying fibroma was observed for the mean FD of the twodimensional images of panoramic reconstruction and axial slices with a thickness of 0.25 mm (p<0.05), regardless of the ROI shape (p>0.05). The mean DF of threedimensional images was different between lesion groups for most comparisons (p <0.05), regardless of the methodology used. Detailed information on lesions and involvement of adjacent structures can be viewed in CBCT. Non-invasive methods, such as radiomorphometric indices and fractal analysis may be useful in the detection of low bone mineral density or as a helpful tool for the differentiation of distinct types of FOLs.

Displasia fibrosa craneofacial avanzada por remodelación ósea / Advanced craniofacial fibrous dysplasia of bone remodeling

La displasia fibrosa es un trastorno del desarrollo esquelético del mesénquima de formación ósea que se manifiesta como un defecto en la maduración y diferenciación de los osteoblastos. La displasia fibrosa representa alrededor de 2.5% de todos los tumores óseos y más del 7% de los tumores benignos. El objetivo de este artículo es presentar un caso de displasia fibrosa poliostótica en la región craneofacial, al cual se le realizaron estudios de imagen, biopsia incisional para diagnóstico definitivo y remodelación ósea para mejorar el contorno facial y la calidad de vida del paciente.

Fibrous dysplasia is an abnormal bone growth, it causes abnormal growth or swelling of bone maturation and differentiation. Fibrous dysplasia represents 2.5% of all bone tumors and more than 7% of benign tumors. The aim of this report is to present the findings of a polyostotic fibrous dysplasia case in the craniofacial skeleton. An incisional biopsy was done to make the diagnosis, after that, a bone remodeling surgery was performed to improve the facial contour and in general to improve the quality of life of the patient.

Presentación de un paciente con displasia fibrosa ósea cráneo facial / Presentation of a Patient with Bone Craniofacial Fibrous Dysplasia

Se presentó un paciente que llegó al Centro Médico de Diagnóstico de Alta Tecnología Francisco de Miranda, Venezuela, en el mes de junio de 2012 con un diagnóstico presuntivo de displasia fibrosa cráneo facial monostótica. El paciente con 55 años de edad presentaba asimetría facial por aumento de volumen del maxilar superior e inferior izquierdo y cigomático de este lado. En la exploración física se constató aumento de volumen del sexto y décimo arco costal y deformidad en la columna dorso lumbar. Se realizaron estudios tomográficos, se mostraron lesiones de displasia fibrosa a otros niveles, arcos costales, columna dorso lumbar y fémur izquierdo que demostraron que el paciente era portador de una displasia fibrosa cráneo facial poliostótica.

A patient who came to Francisco de Miranda Center, Venezuela, in June 2012 with a monostotic craniofacial fibrous dysplasia diagnosis was presented. The case was a 55- year -old patient with a facial asymmetry due to a volume increase of the upper maxillary and lower left maxillary and zygomatic of this side. In physical examination an increase volume of the sixth and tenth costal arc and deformation of dorsolumbar spinal column was found. A tomographic study was performed that revealed lesions of fibrous dysplasia in other levels, costal arcs, dorsolumbar spinal column and left femur that showed a poliostotic craniofacial fibrous dysplasia.

A Case of Fibrous Dysplasia Involving the Nasal Septum

Fibrous dysplasia is a developmental disease of bone in which the normal substance of the interior of the bone is replaced by fibro-osseous connective tissue. It exhibits varying degrees of osseous metaplasia histologically. Craniofacial fibrous dysplasia frequently affects the maxilla, frontal bone, and mandible. The nasal septum is rarely affected. There is only one reported case of fibrous dysplasia arising in the septum internationally. Hence, we report a case of fibrous dysplasia of the nasal septum with a review of literature.