魯賓斯坦-泰比氏症(Rubinstein-Taybi syndrome, RTS),或稱大拇指症,是由基因異常造成的多重先天異常之症候群,新生兒盛行率約為1:100,000~125,000。主要臨床表徵有生長缺乏、特殊臉部特徵、高度拱形上腭、爪形咬頭、心智發育遲滯及拇指寬大等。另外,約三分之一的患者有先天性心臟缺陷。本篇病例報告為8歲4個月男童,於出生一個月內,經本院小兒科醫師藉臨床表徵診斷為RTS。患者因配合度不佳,未曾接受任何牙科處置,由本院小兒科醫師建議後初次至牙科門診求診,主訴希望評估治療全口多顆齲齒。臨床及放射線檢查後,嘗試於門診保護性約束下進行治療,但因行為極度不配合,與家長討論後,安排在全身麻醉下完成其餘齲齒治療,並持續追蹤口腔衛生狀況及恆牙萌發。本篇病例報告之目的在於藉由介紹患有RTS之8歲男童的牙科治療過程,進而探討對此類患者進行牙科處置時的臨床注意事項。
Rubinstein-Taybi syndrome (RTS), so-called broad thumb-hallux syndrome, is a multiple congenital anomalies-mental retardation syndrome caused by pathogenic variant of gene, for which the birth prevalence is 1:100,000 to 125,000. The main clinical characteristics include: growth deficiency, special facial features, highly arched palate, talon cusps, mental retardation and broad thumbs. Also, about one third of affective individuals have congenital heart defect. This case report revealed a 8-year-4-month old boy, who was diagnosed with RTS via clinical characteristics by pediatrician in one month after birth. He didn't accept any dental management before, owning to his uncooperative behavior. Following the advice from pediatrician, he was first brought to our dental outpatient department for evaluation and management of multiple caries. After clinical and radiographic examination, we tried dental treatment under protective stabilization at outpatient department. Because of extremely uncooperative behavior, we arranged full mouth dental treatment under general anesthesia after discussion with his families. The aim of this case report is discussing about the clinical consideration of dental management in patients with RTS through the dental treatment course of the 8-year old boy with RTS.
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