努南氏症候群兒童之牙科治療－病例報告

Dental Treatment of a Child with Noonan Syndrome-A Case Report

鄧伊婷(Yi-Ting Deng) ； 王姻麟(Yin-Lin Wang)

《臺灣兒童牙醫學雜誌》 10卷3期 (2010/12) Pp. 112-118

https://doi.org/10.6319/TJPD.2010.10(3).4

摘要

努南氏症候群是一種體染色體顯性遺傳疾病，有特殊的臉部表徵如眼距較寬、眼瞼下垂；身材短小、蹼狀頸、漏斗胸等多樣先天性異常，且多伴有先天性心臟病、凝血功能異常等系統性疾病、認知及語言發展遲緩異常等。口腔表徵包含高拱狹窄的上顎弓、延遲萌牙、咬合不正等。本病例為一位四歲三個月診斷為努南氏症候群男童，兩個月前經由心導管手術修補心室中隔破損，因牙齦出現膿腫四至五天才由父親帶來台大醫院兒童牙科求診。檢查後發現其上顎狹窄高拱且伴隨乳臼齒錯咬、數顆乳牙嚴重齲齒，且其語言表達及認知及發育較同年齡小孩相較遲緩。經診斷及評估後，在門診行為管理及給予預防性抗生素之下完成所有牙科治療含兒童活動義齒，目前定期回診中。兒童牙科醫師除了口腔疾病治療、加強口腔衛生教育外，更應該積極參與特殊疾病兒童整體照護工作；本文並探討相關疾病臨床表徵及治療計畫考量。

關鍵字

努南氏症候群 ； 口腔表徵 ； 全口重建

並列摘要

Noonan syndrome (NS) is an autosomal dominant genetically heterogeneous disorder frequently characterized by facial dysmorphia, short stature, congenital heart defect, skeletal malformations, bleeding disorder and other abnormalities. Oral manifestations include a narrow & high arch palate, moderate retrognathia, delay eruption and malocclusion. We present a 4-year 3-month-old boy of NS. The boy was brought by his father to our dental clinic for the treatment of dental abscess. Intraoral examination of the patient revealed posterior crossbite associated with a high, narrow palate, and severe dental caries. All dental managements including kiddy dentures were done with antibiotic prophylaxis in out-patient settings. The dental management of NS patients, particularly who have a heart defect and or bleeding disorder, should involve a comprehensive care among dental practitioner, pediatrician, cardiologist and hematologist. Multidisciplinary treatment is the key to successful management for child with NS and the pediatric dentists play an important role in the health team.

並列關鍵字

Noonan syndrome ； oral aspects ； dental rehabilitation