• Rosely Maria dos Santos Cavaleiro, Maria das G Rodrigues Pinheiro, Lucas R Pinheiro, Fabrício Mesquita Tuji, Patrícia do Socorro Queiróz Feio, Isabel Cristina Neves de Souza, Raimunda Helena Ferreira Feio, Sâmia Cordovil de Almeida, Ida Vanessa Doederlein Schwartz, Roberto Giugliani, João J V Pinheiro, and Luiz Carlos Santana-da-Silva.
• Department of Dentistry for Patients with Special Needs, School of Dentistry, Federal University of Pará - UFPA, Belém, PA, Brazil.
• Oral Surg Oral Med Oral Pathol Oral Radiol. 2013 Aug 1; 116 (2): e141-8.

ObjectivesUsing 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 DesignTwo patients previously diagnosed with MPS VI underwent clinical and imaging surveys (panoramic radiographs, CBCT, and MR imaging).ResultsJaw 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.ConclusionsCBCT 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.Copyright © 2013 Elsevier Inc. All rights reserved.

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