{"title":"Endodontic management of taurodontism in a patient with Morquio syndrome: Case report of a 16-year-old girl.","authors":"Mubashir Baig Mirza","doi":"10.1111/scd.13047","DOIUrl":null,"url":null,"abstract":"<p><strong>Background and aim: </strong>Mucopolysaccharidosis (MPS) type IV, referred to as Morquio A syndrome (MAS), is a rare genetic disorder characterized by an insufficient level in the storage of glycosaminoglycans within lysosomes. Diagnosis generally depends on clinical examination, skeletal radiographs, and histochemical tests. The condition is characterized by prominent skeletal deformities, limited joint mobility, significant growth impairment, abnormalities in tooth alignment, and defects in tooth enamel. The present clinical case report aims to provide details of a MAS case with peculiar dental findings, including multiple taurodonts where emergency dental treatment was rendered.</p><p><strong>Case representation: </strong>A 16-year-old girl with short stature and prominent facial characteristics reported severe pain in the right maxillary back region. Clinical examination revealed multiple areas of alveolar bone loss, decayed #36, and temporary restoration on #16. Radiographic examination indicated multiple posterior teeth with taurodontism. Past medical history was confirmative of Morquio syndrome. Treating taurodonts endodontically is challenging due to the enlarged pulp chamber, shortened roots, and constricted root canal anatomy. Motorized canal preparation and the use of bioceramic sealer with single cone obturation were done to achieve the best results.</p><p><strong>Conclusion: </strong>The present case report encountered difficulty locating and negotiating the root canals and establishing the glide path. Using a dental operating microscope and stiff hand files for glide path and short-length Niti rotary files helped achieve the desired results. Although MPS IV is not frequently seen in dental practice, persons with this syndrome can continue to have good oral and overall health if their dental and medical condition if appropriately managed.</p>","PeriodicalId":47470,"journal":{"name":"Special Care in Dentistry","volume":null,"pages":null},"PeriodicalIF":0.9000,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Special Care in Dentistry","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1111/scd.13047","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/7/22 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"DENTISTRY, ORAL SURGERY & MEDICINE","Score":null,"Total":0}
引用次数: 0
Abstract
Background and aim: Mucopolysaccharidosis (MPS) type IV, referred to as Morquio A syndrome (MAS), is a rare genetic disorder characterized by an insufficient level in the storage of glycosaminoglycans within lysosomes. Diagnosis generally depends on clinical examination, skeletal radiographs, and histochemical tests. The condition is characterized by prominent skeletal deformities, limited joint mobility, significant growth impairment, abnormalities in tooth alignment, and defects in tooth enamel. The present clinical case report aims to provide details of a MAS case with peculiar dental findings, including multiple taurodonts where emergency dental treatment was rendered.
Case representation: A 16-year-old girl with short stature and prominent facial characteristics reported severe pain in the right maxillary back region. Clinical examination revealed multiple areas of alveolar bone loss, decayed #36, and temporary restoration on #16. Radiographic examination indicated multiple posterior teeth with taurodontism. Past medical history was confirmative of Morquio syndrome. Treating taurodonts endodontically is challenging due to the enlarged pulp chamber, shortened roots, and constricted root canal anatomy. Motorized canal preparation and the use of bioceramic sealer with single cone obturation were done to achieve the best results.
Conclusion: The present case report encountered difficulty locating and negotiating the root canals and establishing the glide path. Using a dental operating microscope and stiff hand files for glide path and short-length Niti rotary files helped achieve the desired results. Although MPS IV is not frequently seen in dental practice, persons with this syndrome can continue to have good oral and overall health if their dental and medical condition if appropriately managed.
背景和目的:粘多糖病(MPS)Ⅳ型,又称莫基奥A综合征(MAS),是一种罕见的遗传性疾病,其特点是溶酶体内糖胺聚糖的储存水平不足。诊断一般取决于临床检查、骨骼X光片和组织化学检测。该病的特征是骨骼明显畸形、关节活动受限、生长明显受阻、牙齿排列异常和牙釉质缺损。本临床病例报告旨在提供一例MAS病例的详细情况,该病例具有特殊的牙科检查结果,包括多发性牙结石,并对其进行了紧急牙科治疗:一名 16 岁女孩,身材矮小,面部特征突出,报告右上颌后部剧烈疼痛。临床检查发现多处牙槽骨缺损,36 号牙龋坏,16 号牙临时修复。影像学检查显示多颗后牙患有牙槽骨发育不良。既往病史证实为莫基奥综合征。由于牙髓腔增大、牙根变短、根管解剖结构受限,对牙髓病进行根管治疗具有挑战性。为了达到最佳效果,采用了电动根管预备和生物陶瓷封闭剂单锥封闭:本病例报告在定位和协商根管以及建立滑行路径时遇到了困难。使用牙科手术显微镜、硬质手锉滑行路径和短长的 Niti 旋转锉有助于达到预期效果。虽然 MPS IV 在牙科临床中并不常见,但如果牙科和医疗条件管理得当,该综合征患者仍可保持良好的口腔和整体健康。
期刊介绍:
Special Care in Dentistry is the official journal of the Special Care Dentistry Association, the American Association of Hospital Dentists, the Academy of Dentistry for Persons with Disabilities, and the American Society for Geriatric Dentistry. It is the only journal published in North America devoted to improving oral health in people with special needs.