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.
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.
Authors: Dardo Menditti; Luigi Laino; Marco Cicciù; Antonio Mezzogiorno; Letizia Perillo; Marco Menditti; Gabriele Cervino; Lorenzo Lo Muzio; Alfonso Baldi Journal: Int J Clin Exp Pathol Date: 2015-12-01
Authors: Cai Wen; Rong Jiang; Zhi-Qiang Zhang; Bo Lei; Yuan-Zheng Yan; Ying-Quan Zhong; Long Tang Journal: World J Clin Cases Date: 2022-04-26 Impact factor: 1.534
Authors: Damian Drążewski; Małgorzata Grzymisławska; Katarzyna Korybalska; Natasza Czepulis; Marian Grzymisławski; Janusz Witowski; Anna Surdacka Journal: Int J Environ Res Public Health Date: 2017-03-09 Impact factor: 3.390