title: Dentomaxillofacial manifestations of mucopolysaccharidosis VI: clinical and imaging findings from two cases, with an emphasis on the temporomandibular joint.
abstract: OBJECTIVE
  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).
  
  
  METHODS
  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.
authors.affiliation: Department of Dentistry for Patients with Special Needs, School of Dentistry, Federal University of Pará - UFPA, Belém, PA, Brazil.
authors.forename: Rosely Maria dos Santos
authors.initials: RM
authors.lastname: Cavaleiro
authors.forename: Maria das G Rodrigues
authors.initials: Md
authors.lastname: Pinheiro
authors.forename: Lucas R
authors.initials: LR
authors.lastname: Pinheiro
authors.forename: Fabrício Mesquita
authors.initials: FM
authors.lastname: Tuji
authors.forename: Patrícia do Socorro Queiróz
authors.initials: Pdo S
authors.lastname: Feio
authors.forename: Isabel Cristina Neves
authors.initials: IC
authors.lastname: de Souza
authors.forename: Raimunda Helena Ferreira
authors.initials: RH
authors.lastname: Feio
authors.forename: Sâmia Cordovil
authors.initials: SC
authors.lastname: de Almeida
authors.forename: Ida Vanessa Doederlein
authors.initials: IV
authors.lastname: Schwartz
authors.forename: Roberto
authors.initials: R
authors.lastname: Giugliani
authors.forename: João J V
authors.initials: JJ
authors.lastname: Pinheiro
authors.forename: Luiz Carlos
authors.initials: LC
authors.lastname: Santana-da-Silva
mesh_terms: D000293:Adolescent; D018488:Bone Demineralization, Pathologic / Q000175:diagnosis; D001851:Bone Diseases, Metabolic / Q000175:diagnosis; D001853:Bone Marrow / Q000473:pathology; D054893:Cone-Beam Computed Tomography / Q000379:methods; D003795:Dental Sac / Q000473:pathology; D003803:Dentigerous Cyst / Q000175:diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007571:Jaw Diseases / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008336:Mandibular Diseases / Q000175:diagnosis; D008443:Maxillary Sinus / Q000002:abnormalities; D009085:Mucopolysaccharidosis IV / Q000175:diagnosis*; D009296:Nasal Cavity / Q000002:abnormalities; D010026:Osteosclerosis / Q000175:diagnosis; D010375:Pedigree; D011862:Radiography, Panoramic / Q000379:methods; D012391:Root Resorption / Q000175:diagnosis; D013705:Temporomandibular Joint Disorders / Q000175:diagnosis*; D014076:Tooth Diseases / Q000175:diagnosis*; D014095:Tooth, Impacted / Q000175:diagnosis