de Santana Sarmento Dmitry José, de Carvalho Sérgio Henrique Gonçalves, Melo Saulo Leonardo Sousa, Fonseca Fátima Roneiva Alves, Diniz Denise Nóbrega, Bento Patrícia Meira, Mesquita Germana de Queiroz Tavares Borges, de Melo Daniela Pita
Department of Dentistry, Paraíba State University, Araruna, Paraíba, Brazil.
Department of Dentistry, Paraíba State University, Araruna, Paraíba, Brazil.
Oral Surg Oral Med Oral Pathol Oral Radiol. 2015 Dec;120(6):e240-6. doi: 10.1016/j.oooo.2015.08.009. Epub 2015 Aug 22.
To investigate the radiographic findings of mucopolysaccharidoses (MPS) on panoramic radiographs in a series of 16 patients.
This retrospective cross-sectional study included panoramic radiographs of 16 patients with cytogenetically diagnosed MPS. Two blinded, previously calibrated observers evaluate the presence or absence of the following features: type of dentition; stage of dental age in comparison with chronologic age; delayed exfoliation of deciduous teeth; supernumerary teeth; teeth impaction; enlarged, cyst-like dental crypt; hypercementosis; taurodontism; generalized enamel hypoplasia; enlargement of the bone marrow spaces; thinning of cortical bone; unusual morphology of condyles; and flattening of the condylar head.
The final sample was composed of 8 MPS IV, 7 MPS VI, and 1 MPS I cases. All patients presented unusual morphology of condyles. Cyst-like dental crypt (75%), taurodontism, and teeth impaction (68.75% each) were also overall common findings. Generalized enamel hypoplasia was exclusively identified in patients with MPS IV (75% of those patients).
Radiographic features of oral and maxillofacial manifestations in patients with MPS frequently encountered by dentists may help recognize the disorder. However, because of sample size limitations, it was not possible to infer any statistical relationship between the radiographic features and the types of MPS.
研究16例黏多糖贮积症（MPS）患者全景X线片的影像学表现。
这项回顾性横断面研究纳入了16例经细胞遗传学诊断为MPS患者的全景X线片。两名经过预校准且不知情的观察者评估以下特征的有无：牙列类型；与实际年龄相比的牙齿发育阶段；乳牙迟脱；多生牙；牙齿阻生；增大的囊肿样牙囊；牙骨质增生；牛牙样牙；广泛性釉质发育不全；骨髓腔增大；皮质骨变薄；髁突形态异常；以及髁突头部扁平。
最终样本包括8例MPS IV、7例MPS VI和1例MPS I病例。所有患者均表现出髁突形态异常。囊肿样牙囊（75%）、牛牙样牙和牙齿阻生（各68.75%）也是总体常见表现。广泛性釉质发育不全仅在MPS IV患者中发现（占这些患者的75%）。
牙医经常遇到的MPS患者口腔颌面部表现的影像学特征可能有助于识别该疾病。然而，由于样本量有限，无法推断影像学特征与MPS类型之间的任何统计关系。
