- 期刊
裘馨氏肌肉失養症患者在全身麻醉牙科治療中發生橫紋肌溶解：病例報告
Rhabdomyolysis Occurred in Duchenne Muscular Dystrophy Patient During General Anesthesia for Dental Care: Case Report
摘要
裘馨氏肌肉失養症（Duchenne muscular dystrophy, DMD）是一種性聯遺傳隱性肌肉萎縮症，起因為X染色體短臂Xp21位置上的DMD基因發生異常而導致無法正常生成肌肉失養蛋白（dystrophin），使得肌纖維膜上的dystrophin缺失進而造成肌細胞退化萎縮。DMD患者的口腔及顏面特徵可能會出現巨舌症、開咬、錯咬、上頜和下頜牙弓過寬、第二小臼齒延遲萌發和發育不全、肌肉功能異常。本案例報告為三歲七個月男童因全口瀰漫性齲齒被安排於全身麻醉下進行全口治療，而術後恢復過程中出現高血鉀、橫紋肌溶解、心搏停止等症狀，於後續追蹤中經肌肉切片檢驗確診為DMD之罕病案例。DMD患者在牙科考量上除了需注意吞嚥及咀嚼功能的弱化、較高機率咬合不正，也應更加強口腔衛生維護。此外若牙科治療需在全身麻醉下進行，麻醉用藥評估需更加謹慎小心。
並列摘要
Duchenne muscular dystrophy (DMD) is a X-linked recessive muscular dystrophy, which is caused by the abnormality of the DMD gene of Xp21 on the short arm of the X chromosome. It results in the inability to produce dystrophin normally. The deficiency of dystrophin on the sarcolemma causes the degeneration and atrophy of muscle cells. Orofacial manifestations of DMD may include macroglossia, open bite, wide maxillary and mandibular dental arches, delayed tooth emergence and possibly agenesis of the second premolars, and abnormal muscle function. This case report is a three-year and seven-month-old boy who was arranged for full-mouth rehabilitation under general anesthesia due to multiple dental caries. During the postoperative recovery period, symptoms such as hyperkalemia, rhabdomyolysis and cardiac arrest occurred, and he was diagnosed as a rare case of DMD by muscle biopsy later. To the dental considerations, DMD patients should not only pay attention to the weakening of swallowing and chewing functions, but also have a higher probability of malocclusion, and should also strengthen oral hygiene maintenance. In addition, if dental treatment needs to be performed under general anesthesia, the evaluation of anesthesia medication needs to be more cautious.