Yamamoto S, Osuga T, Okada M, Hashimoto T, Shigematsu H, Suzuki S, Fujita K, Matsumoto N, Hori T
Second Department of Oral and Maxillofacial Surgery, Meikai University School of Dentistry, Saitoma.
Masui. 1994 Nov;43(11):1748-53.
The Freeman-Sheldon syndrome is a rare congenital myopathy and dysplasia that results in deformity of the face, hands, and feet. We describe here some problems in general anesthesia from the anesthetic management point of view. An 8-year-old girl with Freeman-Sheldon syndrome underwent surgery under general anesthesia for the correction of lip deformity and microstomia. Patients with this syndrome may present anesthetic problems involving difficulties in endotracheal intubation due to microstomia, micrognathia and neck rigidity, as well as postoperative respiratory complications and problems that relate to myogenetic morphology and myofunctional abnormalities. Preoperatively, we analyzed X-ray cephalograms to evaluate the difficulty of endotracheal intubation. Fiberscopic endotracheal intubation was performed; the time required was 55 minutes. On recovery from anesthesia and after becoming fully responsive, she was extubated in the operating room without any complications. Postoperative recovery was uneventful. Patients with Freeman-Sheldon syndrome should be managed by good preanesthetic preparation, and attention should be paid to postoperative respiratory complications due to the use of muscle relaxants and to securing intravenous access.
弗里曼-谢尔顿综合征是一种罕见的先天性肌病和发育异常，可导致面部、手部和足部畸形。我们从麻醉管理的角度描述了全身麻醉中的一些问题。一名患有弗里曼-谢尔顿综合征的8岁女孩在全身麻醉下接受了手术，以矫正唇部畸形和小口畸形。患有这种综合征的患者可能会出现麻醉问题，包括由于小口畸形、小颌畸形和颈部僵硬导致的气管插管困难，以及术后呼吸并发症和与肌源性形态及肌功能异常相关的问题。术前，我们分析了X线头影测量片以评估气管插管的难度。实施了纤维支气管镜引导下气管插管；所需时间为55分钟。麻醉苏醒且完全恢复反应后，她在手术室拔管，未出现任何并发症。术后恢复顺利。对于弗里曼-谢尔顿综合征患者，应做好充分的麻醉前准备，并应注意因使用肌肉松弛剂导致的术后呼吸并发症以及确保静脉通路。
