貓哭症患者的牙科臨床考量－病例報告

Cri-du-chat Syndrome: Dental Considerations and Report of Case

李嘉諭(Chia-Yu Li) ； 謝春怡(Chun-Yi Hsieh) ； 施文宇(Wen-Yu Shih)

《臺灣兒童牙醫學雜誌》 20卷2期 (2020/06) Pp. 85-90

https://doi.org/10.6319/TJPD.202006_20(2).0006

摘要

貓哭症是一種染色體異常的先天性疾病，因患者會有高頻且單調的貓哭聲而得名。由第五對染色體短臂的部分或全部缺損所引起，疾病盛行率約1:15,000至1:50,000。貓哭症患者常有一些先天異常如智能障礙、發展遲緩、小頭、顏顎面異常、呼吸道變形、先天性心臟病、神經系統疾病與腎病。有些症狀會影響牙科治療計畫的擬定與執行，像是小顎畸形、牙齒前突、咬合不正、高顎弓、前牙開咬、口腔衛生差、牙釉質生成不全、牙周炎以及牙齒延遲萌發。這位十三歲患者有典型症狀；此次因牙齒疼痛與動搖前來求診，後續安排在全身麻醉下完成治療。除了定期回診追蹤外，加上嚴謹細緻的預防策略，才能幫助患者與家屬維持其口腔健康，避免後續再齲齒與齒源性感染的發生。

關鍵字

貓哭症 ； 齲齒 ； 牙周炎

並列摘要

Cri-du-chat syndrome(CdCS) is a genetic disease in which affected infants are characterized by a high-pitched monochromatic cat-like crying. The syndrome results from a total or partial deletion on the short arm of chromosome 5. The estimated frequency is between 1:15,000 and 1:50,000 in live-born infants. Medical problems commonly associated with CdCS include the intellectual disabilities, delayed physical development, microcephaly, distinct facial dysmorphism, abnormalities of airway, neurological and renal abnormalities, and cardiac malformations. This case presented a 13-year old girl with CdCS referred to our hospital for her painful teeth. Intellectual disability and distinctive craniofacial features were present. Due to the invasive dental treatment and behavior problem, comprehensive dental treatment under general anesthesia was performed. Cri-du-chat syndrome may influence our management in many aspects due to its micrognathia, dental biprotrusion, dental malocclusions, high but rarely cleft palate, anterior open bite, poor oral hygiene, enamel hypoplasia, periodontitis, and retardation of eruption. In addition to regular dental recall and fluoride application, meticulous preventive strategy is more important to reduce the risk of progressive lesions and subsequent odontogenic infection.

並列關鍵字

Cri-du-chat syndrome ； Dental caries ； Periodontitis