Orofacial Manifestations in a Child with Achondroplasia: A Case Report

軟骨發育不全之口腔顏面部徵狀之病例報告

彭彥慈(Yan-Cih Peng) ； 王姻麟(Yin-Lin Wang) ； 張曉華(Hsiao-Hua Chang) ； 黃桂芬(Guay-Fen Huang) ； 郭敏光(Ming-Kuang Guo)

《臺灣兒童牙醫學雜誌》 13卷2期 (2013/12) Pp. 45-50

https://doi.org/10.6319/TJPD.2013.13(2).5

摘要

軟骨發育不全是比例失衡肢幹短小的侏儒症中最常見的一種病症，主要是由於基因突變，造成軟骨細胞發育的缺陷，尤見於長骨。此篇病例報告是描述一位六歲九個月軟骨發育不全的男童，具有典型的身體系統性及口腔顏面部的特徵，包含四肢短小、身材矮小，且具第三類骨骼型不正咬合、大頭、前額突出、塌鼻、上顎骨發育不全等凹型側臉型及雙側後齒錯咬。在數次約診以適當的行為引導下，蛀牙接受根管治療及不鏽鋼牙套復形。術後建議家長要注重男童之口腔衛生之實施、注意飲食習慣及定期口腔健康檢查。也建議在乳齒列完成換牙後，考慮齒顎矯正。

關鍵字

軟骨發育不全 ； 口腔顏面徵狀 ； 牙科治療

並列摘要

Achondroplasia is known as the most common type of short-limb dwarfism. It's caused by genetic mutation leading to defects in chondrocytes maturation. This case is a 6-year 9-month old boy with achondroplasia who presented with typical systemic and craniofacial features, including short stature, macrocephaly, class III malocclusion skeletal pattern with depressed nasal bridge, midface hypoplasia, and bilateral posterior crossbite. During dental treatments, with apporpriate behavior management, carious teeth had received pulpal therapy and SSC restorations. The dental treatment went smoothly. Oral hygiene instructioln and practice, dietary conselling and periodic examination have been emphasized and advised. Orthodontic treatment to correct bilateral posterior crossbite and crowding will be considered at early permanent dentitional stage.

並列關鍵字

Achondroplasia ； Orofacial manifestations ； Dental treatment