具多發性阻生贅生齒之鎖骨顱骨發育不良－病例報告

Cleidocranial Dysplasia with Multiple Impacted Supernumerary teeth-case Report

蘇瑜(Yu Su) ； 葉淑端(Shu-Tuan Yeh) ； 盧幼情(Yo-Chin Lu) ； 黃國維(Guo-Wei Huang) ； 李燕晉(Yann-Jinn Lee) ； 張家豪(Chia-Hoa Chang)

《中華牙醫學雜誌（中文版）》 25卷2期 (2006/06) Pp. 129-142

https://doi.org/10.7106/CJD.200606.0129

摘要

鎖骨顱骨發育不良(Cleidocranial dysplasia)是一種罕見的自體顯性遺傳疾病，此疾病會影響骨骼的生長發育，故患者外觀會明顯異於常人。本病例報告為一位十二歲男童，除表現出典型的鎖骨顱骨發育不良特徵：鎖骨嚴重發育不全、恥骨骨化延遲、恆齒列延後萌牙、多發性贅生齒；其牙齒與齒列發展亦甚具特殊之處：初診時口內僅萌出十六顆牙齒（包括十一顆乳齒與五顆恆齒），而顎骨內則有二十六顆阻生恆齒及二十顆贅生齒。十三歲時經手術拔除一部分阻生的贅生齒與陸齒，追蹤治療至今。病患目前十七歲，口內萌出十三顆牙齒（包括二顆乳齒、十顆恆齒與一顆贅生齒），顎骨內仍有十八顆阻生恆齒及十二顆贅生齒，目前暫以臨時性活動義齒作過渡期的空間維持與贋復治療，將待病患成年之後再予以永久性贋復重建。針對此類鎖骨顱骨發育不良合併有顎骨發育與牙齒萌發異常之病患，建議以牙科團隊合作的方式，配合恆齒發育的狀況來拔除乳齒與贅生齒，並合併早期手術介入與適當的矯正、贋復治療，以達到改善顏面外觀與咀嚼功能之最佳效果。

關鍵字

鎖骨顱骨發育不良 ； 多發性贅生齒

並列摘要

Cleidocranial dysplasia (COD) is a rare autosomal dominant disorder. Patients suffering with COD have a characteristic appearance, which is caused by disturbance in bone growth. The male patient in this case report has the classical findings of COD: markedly hypoplastic clavicles, delayed ossification of the pubic rami, and dental anomalies including delayed eruption of the permanent dentition and multiple supernumerary teeth. In particular, only 16 teeth erupted spontaneously (ii deciduous teeth and 5 permanent teeth), but there were 26 permanent teeth and 20 supernumerary teeth impacted in both jaws. Surgical extraction of some of the impacted supernumerary and permanent teeth was performed at the age of 13 years as preliminary treatment. The patient has been followed up to the present. Currently, at 17 years of age, 13 teeth have erupted in his mouth (2 deciduous teeth, 10 permanent teeth, and I supernumerary tooth), and 30 teeth remain impacted in his jaws (18 permanent teeth, and 12 supernumerary teeth), At this point, removable dentures have been fabricated as a transitional space maintainer and prosthesis. Definite prosthetic reconstruction will be carried out when the patient reaches adulthood. An interdisciplinary approach is recommended to treat patients with cleidocranial dysplasia. Following the development of the permanent teeth, selected extraction of the deciduous and supernumerary teeth, combined with early surgical involvement, and proper orthodontic and prosthodontic treatment can be carried out to achieve the best results.

並列關鍵字

cleidocranial dysplasia ； multiple supernumerary teeth