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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.
ABSTRACT
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)
Subject(s)
Humans , Male , Female , Bone and Bones , Plastic Surgery Procedures , Facial Asymmetry/congenital , Fibrous Dysplasia, Monostotic/surgery , Craniofacial Fibrous Dysplasia/surgery , Mutation , Pathology , Wounds and Injuries , Diagnosis , Esthetics , GeneticsABSTRACT
Introduction: Fibrous dysplasia (FD) of bone is an enigma with no proper guideline. Treatment currently consists of curettageand bone grafting in an attempt to eradicate the lesion and to prevent progressive deformity. No definite criteria have beenestablished to identify patients at high risk of presenting pathological fractures.Purpose: The purpose of the study was to explore the effect of combination bisphosphonate therapy in diminishing pain,preventing fractures, lowering N-telopeptide values, and leading to partial resolution of FD lesions.Materials and Methods: At Medical College, Kolkata, 10 patients with monostotic FD in lower extremities were treated between2014 and 2018 and included in the study. All patients underwent full skeletal survey followed by core needle biopsy with the helpof magnetic resonance imaging and C-arm guidance. After confirmation, closed intramedullary nail without reaming was usedin all cases. Bone grafting was not performed. Zoledronic acid was given intravenously at the dose of 4 mg every 6 months.Patients were allowed full weight-bearing on the affected extremities on the 2nd post-operative day.Results: Seven patients were female and three were male; their mean age was 26.9 years. The mean duration of follow-upwas 30.5 months. We get good to average results. Clinicoradiological improvement of all cases was observed.Conclusion: As a result of this study, we believe that intramedullary fixation can be performed successfully. Treatment ofmonoostotic fibrous dysplasia with adjuvant bisphosphonate therapy resulted in effective pain control and early return offunctional activity. This will avoid problems that may occur following pathological fractures.
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Introducción: La displasia fibrosa es una patología benigna, poco frecuente, que afecta los huesos, se caracteriza por remplazo gradual del tejido óseo por fibroso, que genera alteraciones óseas estéticas y funcionales; histológicamente presenta una proliferación excesiva de las células mesenquimales que forman hueso. Objetivo: Conocer los aspectos relacionados con la toma de decisiones, con relación con el tratamiento quirúrgico o la opción de controles clínicos y radiográficos, en la displasia fibrosa. Presentación del caso: Se reportan dos casos clínicos de pacientes pediátricos, que acudieron al Hospital Napoleón Franco Parejas de Cartagena-Colombia, por presentar displasia fibrosa, que generó asimetría facial unilateral, y compromete la estética del paciente y no se reportó sintomatología dolorosa; se describen características de la patología y criterios básicos, útiles en la toma de decisión terapéutica, ya sea abordaje quirúrgico radical, conservador o solo realizar control clínico y radiográfico de la lesión. Conclusiones: Los criterios clínicos como asimetría facial, deformidad estética y el compromiso funcional, son básicos en la elección quirúrgica como opción terapéutica, en la displasia fibrosa(AU)
Introduction: Fibrous dysplasia is an uncommon, benign pathology that affects the bones and is characterized by the gradual replacement of bone tissue by a fibrous one, creating aesthetic and functional bone disorders. Histologically, it presents an excessive proliferation of the mesenchymal cells that form the bone. Objective: To know the aspects related to decision making, regarding surgical treatment or the option of carrying out clinical and radiographic controls of fibrous dysplasia. Case presentation: We report two cases of pediatric patients who attended the Children´s Hospital Napoleón Franco Pareja (HINFP) in Cartagena-Colombia presenting fibrous dysplasia that produced unilateral facial asymmetry compromising the aesthetics of patients, but reporting no painful symptoms. We describe the characteristics of the pathology and the basic useful criteria in making therapeutic decisions, with either conservative radical surgical approach or only performing a clinical and radiographic control of the lesion. Conclusions: Clinical criteria such as facial asymmetry, aesthetic deformity, and functional commitment are basic aspects in the therapeutic option for patients with in fibrous dysplasia(AU)
Subject(s)
Humans , Male , Child , Adolescent , Fibrous Dysplasia of Bone/surgery , Clinical Decision-Making/methods , ColombiaABSTRACT
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.
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La displasia fibrosa (DF) es una patología benigna rara, generalmente asintomática, que afecta el tejido óseo. Debido al remplazo gradual del tejido óseo por tejido conectivo amorfo se pueden producir alteraciones óseas estéticas y funcionales. En este artículo se realiza una revisión de la literatura sobre la DF y se relata un caso clínico de una mujer de 49 años de edad que presenta deformidad facial debida al crecimiento lento y progresivo del maxilar derecho. Con esto pretendemos ofrecer una secuencia diagnóstica para establecer el diagnóstico definitivo de la DF monostótica, brindando los principios básicos para el manejo adecuado de los pacientes que sufren de esta enfermedad.
Fibrous dysplasia (FD) is a rare benign pathology, generally asymptomatic affecting the bone tissue. Due to the gradual replacement of the bone tissue by amorphous connective tissue, aesthetic and functional bone alterations can occur. A review of the literature on FD is made and we report a clinical case of a 49-year-old woman presenting facial deformity as a result of the slow and progressive growth of the right maxillar. With this paper, we intend to offer a diagnostic sequence to establish the definitive diagnosis of monostotic FD, providing the basic principles for the proper management of patients suffering from this disease.
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Paciente do sexo feminino, 41 anos, cor branca, com queixa de dor lombar há mais de três anos, sem irradiações, submetida há vários exames radiográficos sendo diagnosticada, por biópsia, doença de Paget monostótica na terceira vértebra lombar. Trata-se de uma localização incomum.
Female patient, 41 years, Caucasian, with lumbar pain for more than three years, without irradiation, submitted to various radiological exams and diagnosed by biopsy with monostotic Paget's disease of the third lumbar vertebrae, which is an uncommon location.
Subject(s)
Humans , Female , Middle Aged , Fibrous Dysplasia, Monostotic , Osteitis Deformans , SpineABSTRACT
Fibrous dysplasia is a developmental anomaly of bone formation, which is characterized by a replacement of the normal bone and marrow by fibrous tissue as well as small, woven spicules of bone. These lesions may exist in a monostotic or polyostotic form and are generally found in the 4th decade of age or younger with a slightly higher incidence in girls than boys. We report a case of monostotic fibrous dysplasia that was associated with a pathologic fracture and a kyphotic deformity in the cervical spine.
Subject(s)
Bone Marrow , Congenital Abnormalities , Fibrous Dysplasia, Monostotic , Fractures, Spontaneous , Incidence , Osteogenesis , SpineABSTRACT
Fibrous dysplasia is a developmental disease of the bone, characterized by the replacement of normal spongiosa and filling of the medullary cavity of affected bones by an abnormal fibrous tissue. Fibrous dysplasia arising in the paranasal sinus is rare and usually secondary to the extension of diseases from adjacent bones, and is rarely limited to the sinuses. Two patterns are predominant: he monostotic type, in which only one bone is affected; and the polyostotic type, in which multiple bones are affected. Recently, we experienced a case of monostotic fibrous dysplasia which was confined to the maxillary sinus and approached by the Caldwell-Luc method. Hence, we report a case of fibrous dysplasia of the maxillary sinus with a review of literature.