牙本質形成不良的早期診斷、重建與十年的追蹤－病例報告

牙本質形成不良是一種體染色體顯性遺傳的疾病。主要的臨床特徵有牙齒變色及牙釉質喪失導致牙本質暴露，此情況常造成嚴重齲齒、牙齒磨耗及斷裂。在放射線影像上則能觀察到球莖狀的牙冠、牙骨質－牙釉質交界顯著收窄、偏短的牙根，以及鈣化、阻塞的根管系統。因為以上的結構變異，此類病人在美觀及功能的恢復上都帶給牙醫師很大的挑戰。因此本篇病例報告提出一個中等程度牙本質形成不良（依過去分類屬第二型牙本質形成不良）的案例，從3歲1個月到13歲5個月（總共十年）於不同齒列時期的治療過程：於乳牙齒列透過樹脂乳牙套及不鏽鋼牙套，重建咬合垂直距離並恢復美觀和咀嚼功能；於混合齒列裝戴局部矯正器，以重獲不足的萌牙空間，但因年輕恆牙出現根尖病灶後被拔除而中斷；最後於恆牙齒列進行埋伏齒拔除及全口矯正與定期的追蹤。本篇也將針對牙本質形成不良之早期診斷，後續跨科治療的準則和方向，做進一步的介紹及討論。

關鍵字

牙本質形成不良；遺傳性疾病；結構異常；重建；兒童牙科

並列摘要

Dentinogenesis imperfecta (DI) is a hereditary dentine disorders that caused by an autosomal dominant mutation. Major clinical features include discoloration and loss of enamel with exposed dentin which is predisposed to severe caries, extreme wear and fracture. Bulbous crown, evident cervical constrictions and short roots with pulpal calcification and obliteration may show radiographically. Due to these dysmorphology of tooth structure, the treatment of DI patients represent a challenge for the dental practitioner while restoring the aesthetics and function. This case report presents a case with moderate form of DI, which is classified as DI type II before, from 3 year-1 month old to 13-year-5 month old (totally 10 years), and show the treatment plan in each dentition. The treatment course compromises an initial treatment to restore esthetic appearance and function by stainless steel crowns (SSCs) and strip crowns in primary dentition. Young permanent tooth extraction which caused early orthodontic treatment of space regaining failed unfortunately in mixed dentition. Impacted tooth extraction and orthodontic treatment due to space deficiency in permanent dentition. Last but not least, routine follow up is arranged imperatively. In the discussion, this case may guide dentists with respect to the need for early diagnosis and adequate multidiscipline dental approach.

並列關鍵字

Dentinogenesis imperfecta ； hereditary ； tooth dysmorphology ； rehabilitation ； pediatric dentistry

國際替代計量

牙本質形成不良的早期診斷、重建與十年的追蹤－病例報告

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