Dental Considerations for the Treatment of Children with Marfan Syndrome : A Case Report

馬凡氏症兒童的牙科治療與考量：病例報告

葉懿萱(Yi-Hsuan Yeh) ； 劉正芬(Jeng-Fen Liu)

《臺灣兒童牙醫學雜誌》 20卷2期 (2020/06) Pp. 59-65

https://doi.org/10.6319/TJPD.202006_20(2).0002

摘要

Marfan syndrome (MFS) is an autosomal dominant disorder primarily affecting the connective tissues of the musculoskeletal, cardiovascular, ocular and orofacial systems. The incidence is estimated at approximately one case per 5,000 to 10,000 individuals with no predilection for gender or ethnic groups. The pathogenesis of Marfan syndrome has not been fully understood, yet it is commonly believed that a mutation in the fibrillin-1 gene (FBN1) located in chromosome 15 (15q21) may be linked to the syndrome. The oral manifestations of Marfan syndrome, though considered as minor features for the diagnosis, can be identified through routine intraoral examination. Constriction of the maxilla, high and deep palate with concomitant dental crowding and posterior cross bite are the common orofacial defects - patients are usually dolicocephalic with Angle's class II skeletal malocclusion. The inherent factors of patients with Marfan syndrome should be taken into consideration for treatment planning, particularly the associated risk of infective endocarditis due to the prevalent cardiovascular problems. In this regard, paediatric dentists play an important role in the interdisciplinary treatment of Marfan syndrome children. Hence, the aim of this case report is to present the dental considerations and treatment in children with Marfan syndrome.

關鍵字

Marfan syndrome ； Oral manifestations ； Dental management ； Infective endocarditis

並列摘要

馬凡氏症候群（蜘蛛人症）為一種體染色體顯性遺傳性結締組織疾病。發病率依國外統計為1/5,000至1/10,000並且不具有人種或性別上的差異。目前致病原因尚未徹底被了解，不過大部分已知的患者是由於位在第15對染色體上的fibrillin-1(FBN1)基因發生突變所造成。由於FBN1為結締組織成分組成，其缺損會使得多種器官系統受到影響，特別是骨骼、心血管、眼睛。本疾病的口腔顏面特徵雖屬次要診斷依據，仍可以從例行口內檢查看出端倪：狹小的上頷骨、高而窄的腭弓伴隨齒列擁擠和後牙錯咬都是常見的表徵。由於此類患者經常伴有心臟方面的問題，牙科治療上需特別注意感染性心內膜炎的風險。本文將呈現一例三歲的馬凡氏症病童的牙科治療及治療計畫和照護上需要考量的因素。

並列關鍵字

馬凡氏症 ； 口腔表徵 ； 牙科治療 ； 感染性心內膜炎