Dentomaxillofacial manifestations of mucopolysaccharidosis VI: clinical and imaging findings from two cases, with an emphasis on the temporomandibular joint.

OBJECTIVES: Using a clinical survey, panoramic, cone-beam computed tomography (CBCT), and magnetic resonance (MR) imaging, this study was conducted to ascertain primary maxillofacial abnormalities in patients with mucopolysaccharidosis VI (MPS VI). STUDY DESIGN: Two patients previously diagnosed with MPS VI underwent clinical and imaging surveys (panoramic radiographs, CBCT, and MR imaging). RESULTS: Jaw involvement was present in all patients. The most prevalent findings were enlarged marrow spaces, osteopenia, dentigerous cyst-like follicles, effacement of the jaw structures, and osteosclerosis. This is the first study to describe temporomandibular joint (TMJ) involvement for MPS VI. CONCLUSIONS: CBCT and MR imaging were needed to observe features that were not clear in conventional radiographs. Both patients reported symptoms in the TMJ and demonstrated involvement during their examinations. A multicenter study is necessary to better document maxillofacial involvement in MPS VI.

Clinical and imagiological findings of central giant cell lesion and cherubism.

Cone beam computed tomography (CBCT) is the best examination for bone lesions of the maxilla, allowing the dentist to evaluate precisely the behavior and components of the lesion and their relationship to the surrounding structures. Central giant cell lesion and cherubism are histologically very similar lesions. Therefore clinical and radiological examinations are fundamentally important for the diagnosis. The aim of this paper is to report two cases diagnosed as central giant cell lesions and cherubism using CBCT. This imaging modality was very important for the diagnosis of the lesions presented in the current study. It also allowed observing precisely the limits of the lesions, the components, the behavior and the exact relationship to adjacent structures.

Clinical and imagiological findings of central giant cell lesion and cherubism

Cone beam computed tomography (CBCT) is the best examination for bone lesions of the maxilla, allowing the dentist to evaluate precisely the behavior and components of the lesion and their relationship to the surrounding structures. Central giant cell lesion and cherubism are histologically very similar lesions. Therefore clinical and radiological examinations are fundamentally important for the diagnosis. The aim of this paper is to report two cases diagnosed as central giant cell lesions and cherubism using CBCT. This imaging modality was very important for the diagnosis of the lesions presented in the current study. It also allowed observing precisely the limits of the lesions, the components, the behavior and the exact relationship to adjacent structures.

A tomografia computadorizada de feixe cônico (TCFC) é o melhor exame para lesões ósseas da maxila, permitindo que o dentista possa avaliar com mais confiabilidade o comportamento, os componentes da lesão, e sua relação com estruturas adjacentes. A Lesão central de células gigantes e o querubismo são patologias muito semelhantes histologicamente, portanto, exames clínicos e radiológicos são de fundamental importância para o diagnóstico. O objetivo deste trabalho é relatar dois casos diagnosticados usando TCFC, um de lesões centrais de células gigantes e um de querubismo. Esta modalidade de imagem foi muito importante para o diagnóstico das patologias apresentadas neste estudo. Também permitiu observar com mais confiabilidade os limites das lesões, os componentes, o comportamento e a relação exata com as estruturas adjacentes.