{"title":"Orthodontic treatment of a patient with pycnodysostosis","authors":"Peter Ngan, Dami Kim, Guoqiang Guan","doi":"10.25259/apos_56_2024","DOIUrl":null,"url":null,"abstract":"Pycnodysostosis (PYCD) is a rare lysosomal storage disease of the bone caused by a mutation in the gene that codes the enzyme cathepsin K. Patients diagnosed with PYCD demonstrate certain physical characteristics, including deformity in craniofacial structure for which patients seek orthodontic treatments to improve occlusal functions and esthetics. In addition, growth hormone therapy is indicated for these patients due to their short stature. This article is the first to report on the successful completion of the non-surgical orthodontic treatments of a patient diagnosed with PYCD. The patient was treated in two phases due to a waiting period for growth hormone therapy. However, superimposition of pre-and post-treatment radiographs showed no evidence of accelerated skeletal growth at the conclusion of the hormonal therapy. The rate of orthodontic tooth movement for Phase I with conventional appliance and Phase II treatment with clear aligners was similar with healthy individuals treated with four premolars extraction. Surgical exposure of the impacted permanent molars was contraindicated as no posterior dentition was available as anchorage for molar uprighting. Extraction of impacted molars in the mandible was performed with care due to the high risk of fracture. Orthognathic surgery treatment carries an increased risk of osteomyelitis for these patients due to dense bone with poor vascularity. Therefore, non-surgical camouflage orthodontic treatment can be the best option to achieve functional occlusion for patients diagnosed with PYCD.","PeriodicalId":42593,"journal":{"name":"APOS Trends in Orthodontics","volume":null,"pages":null},"PeriodicalIF":0.5000,"publicationDate":"2024-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"APOS Trends in Orthodontics","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.25259/apos_56_2024","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"DENTISTRY, ORAL SURGERY & MEDICINE","Score":null,"Total":0}
引用次数: 0
Abstract
Pycnodysostosis (PYCD) is a rare lysosomal storage disease of the bone caused by a mutation in the gene that codes the enzyme cathepsin K. Patients diagnosed with PYCD demonstrate certain physical characteristics, including deformity in craniofacial structure for which patients seek orthodontic treatments to improve occlusal functions and esthetics. In addition, growth hormone therapy is indicated for these patients due to their short stature. This article is the first to report on the successful completion of the non-surgical orthodontic treatments of a patient diagnosed with PYCD. The patient was treated in two phases due to a waiting period for growth hormone therapy. However, superimposition of pre-and post-treatment radiographs showed no evidence of accelerated skeletal growth at the conclusion of the hormonal therapy. The rate of orthodontic tooth movement for Phase I with conventional appliance and Phase II treatment with clear aligners was similar with healthy individuals treated with four premolars extraction. Surgical exposure of the impacted permanent molars was contraindicated as no posterior dentition was available as anchorage for molar uprighting. Extraction of impacted molars in the mandible was performed with care due to the high risk of fracture. Orthognathic surgery treatment carries an increased risk of osteomyelitis for these patients due to dense bone with poor vascularity. Therefore, non-surgical camouflage orthodontic treatment can be the best option to achieve functional occlusion for patients diagnosed with PYCD.
侏儒症(Pycnodysostosis,PYCD)是一种罕见的溶酶体贮积性骨病,由编码 cathepsin K 酶的基因突变引起。确诊为PYCD 的患者会表现出某些身体特征,包括颅面部结构畸形,患者会寻求正畸治疗以改善咬合功能和美观。此外,由于患者身材矮小,生长激素治疗也适用于这些患者。本文首次报道了一名被诊断为PYCD的患者成功完成非手术正畸治疗的案例。由于生长激素治疗需要等待一段时间,该患者的治疗分两个阶段进行。然而,治疗前后的 X 光片叠加显示,在激素治疗结束时,并没有骨骼加速生长的迹象。使用传统矫治器进行第一阶段治疗和使用透明矫治器进行第二阶段治疗的牙齿移动率与拔除四颗前臼齿的健康人相似。由于没有后牙作为臼齿直立的锚,因此禁忌手术暴露受影响的恒磨牙。由于下颌阻生臼齿骨折的风险很高,因此拔除时要小心谨慎。由于骨质致密且血管不畅,正颌手术治疗会增加这些患者患骨髓炎的风险。因此,对于被诊断为PYCD的患者来说,非手术伪装正畸治疗是实现功能性咬合的最佳选择。