Maxillofacial Mazabraud's syndrome: A case report & review.

Mazabraud's syndrome is characterized by myxomas of intramuscular type present in association with fibrous dysplasia. Up to this day, approximately 80 cases of Mazabraud's syndrome have been reported, although in the head and neck territory intramuscular myxoma reports in association with fibrous dysplasia of the bone are very scarce. An unusual case of Mazabraud's syndrome in a 63 years old female displaying fibrous dysplasia of the mandible and soft tissue myxoma in the edentulous alveolar ridge in the molar area is reported. After four years of follow-up, the clinical, imagenological and microscopical findings that led to the diagnosis and treatment are discussed. This report exemplifies the diagnostic and treatment challenge of this rare disease and enhances our clinical knowledge due to its long follow-up, highlighting the need of understanding better its behavior in order to establish proper guidelines for its treatment.

Uremic leontiasis ossea, a rare presentation of severe renal osteodystrophy secondary to hyperparathyroidism.

Renal osteodystrophy is a common complication of end-stage renal failure patients. It's most severe osseous complication is characterized by massive thickening of the cranial vault and facial bones, called uremic leontiasis ossea (ULO), with only few cases reported in the literature. A case of a 47-year-old female patient with ULO is presented. Physical examination showed enlargement of the jaws, which hinders proper ventilation and feeding. The computed tomography examination showed marked osseous proliferation in the jaws causing severe bony expansion and loss of normal bony architecture in the skull and the skull base. The most relevant clinical, histopathological and laboratory findings are discussed. The uremic leontiasis ossea causes significant aesthetic and functional changes. Correct diagnosis and management of the factors responsible for the development of bone lesions due to altered bone metabolism are key factors. The maxillofacial surgeon must have the proper knowledge of patient's medical condition and bone maturation status to address an adequate surgical strategy.

Temporomandibular joint ankylosis after condylar dislocation into the middle cranial fossa: A case report.

INTRODUCTION: Dislocation of the mandibular condyle into the middle cranial fossa after a trauma is a rare event. The lack of appropriate treatment can lead to ankylosis of the temporomandibular joint (TMJ). We report about a case of TMJ ankylosis following intracranial dislocation of the mandibular condyle through the roof of the articular fossa. CASE REPORT: A 9-year-old patient was referred for a severe limitation of mouth opening that began progressively one year before. A history of chin injury due to an accidental fall was found. Preoperative CT scan showed a TMJ ankylosis on the right side combined with a dislocation of the mandibular condyle into the middle cranial fossa. Treatment consisted in an intracranial resection of the mandibular condyle, partial removal of the ankylosis block and TMJ arthroplasty. DISCUSSION: Our case is the second case of TMJ ankylosis following intracranial dislocation of the mandibular condyle and treated with arthroplasty alone published in the English literature. There is no consensus regarding the pathophysiology of TMJ ankylosis and regarding the attitude towards the ankylosis block. In our case, no recurrence was noticed after a one-year follow-up. An interdisciplinary approach is needed, including maxillofacial surgeon, neurosurgeon, physiotherapist and orthodontist.

Crecimiento sagital maxilar en fisurados unilaterales operados funcionalmente / Sagittal maxillary growth in unilateral cleft lip and palate patients following functional surgery

Objetivo. Comparar el crecimiento sagital maxilar en pacientescon fisura labio-máxilo-palatina unilateral operados a los 6 meses con criteriofuncional con pacientes normales que tengan relación consanguíneadirecta con los anteriores. Diseño del estudio. Análisis arquitectural y craneofacialde Delaire en telerradiografías de perfil en ambos grupos de pacientescuyas edades fluctúan actualmente entre los 7 y los 12 años, determinandoel crecimiento sagital del maxilar a través de la medida del ángulodel pilar maxilar anterior (C1/F1), sometiendo las medidas al test T de Studentcon una significación del 99,5%. Resultados. Se determinó el valorreal y esperado para el ángulo del pilar maxilar anterior en todos los casos.Al comparar estadísticamente los resultados, no se encontraron diferenciassignificativas en los valores promedios obtenidos. Conclusiones. El crecimientosagital maxilar de los pacientes con fisura labio-máxilo-palatina unilateraloperados a los 6 meses con criterio funcional no difiere del de aquellospacientes normales (AU)

Objective. To compare the sagittal maxillary growth between unilateral cleft lip and palate patients operated underfunctional criterion at the age of 6 months and normal patientswho were blood-related. Design. Delaire’s Architectural and Structuralcraniofacial analysis in conventional lateral radiographs of all the patients with an age range of 7-12 years, determining the sagittalmaxillary growth by the anterior maxillary pillar angle (C1/F1).These measurements were analyzed using the T-test with a 99.5% significance. Results. The real and expected value of the anteriormaxillary pillar angle was determined in all cases. By comparingthe results statistically, no significant differences were found inthe mean values obtained. Conclusion. Maxillary sagittal growthin unilateral cleft lip and palate patients operated at the age of 6months under functional criterion, does not differ from the growthof normal patients (AU)