戴克-大衛道夫-梅森症候群之麻醉處理病例報告

Anesthetic Approach to the Dyke-Davidoff-Masson Syndrome — A Case Report

所謂的戴克-大衛道夫-梅森症候群（Dyke-Davidoff-Masson syndrome），係一罕見之單側大腦萎縮疾患。臨床表徵可能有面部不對稱，對側萎縮（包括軀幹和肢體）併偏癱，語言困難，智力退化，以及癲癇。由於此徵候群對多重生理系統造成影響，尤其是在氣道，潛在肌肉病變，以及癲癇等方面，此類病患之麻醉極度挑戰著麻醉醫師。然而尚未見有此類病人之麻醉臨床報告。這篇病例報中，我們報告了一名診斷有戴克-大衛道夫-梅森症候群之五歲女童被安排接受頭骨圓挫環鋸術。我們敘述了此類病患可能面臨的問題並加以處理和預防，使此次麻醉得以成功完成。

The so-called Dyke-Davidoff-Masson syndrome (DDMS) is a rare disorder of cerebral hemiatrophy. The clinical presentation may consist of facial asymmetry, contralateral atrophy (including the trunk, and the extremities) and hemiparesis, speech difficulties, mental retardation, and epilepsy. Because it involves multiple systems, especially problem of the airway, occult myopathy, and seizure disorder, anesthesia for such a patient is a challenge to any anesthesiologist. However, there are no clinical reports which concern the anesthetic management of such patients in the literature. We herein report a 5-year-old girl, a sufferer of DDMS, scheduled for burr trephination. The successful anesthetic management is brought forward with highlights of inference from the experience.