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[患有与脊髓损伤相关的考芬-洛里综合征的男孩]

[Boy with Coffin-Lowry syndrome associated with spinal cord injuries].

作者信息

Kawana Yuki, Okamura Kenta, Kurahashi Kiyoyasu

机构信息

Department of Anesthesia, Yokohama City University Medical Center, Yokohama 232-0024.

出版信息

Masui. 2014 Feb;63(2):203-5.

Abstract

A 12-year-old male patient with Coffin-Lowry syndrome was scheduled for posterior cervical decompression and fusion for cervical spinal injuries. The patient had features of Coffin-Lowry syndrome including mental retardation, prominent forehead, a short nose with a wide tip, a wide mouth with full lips, short stature, microcephaly, and kyphoscoliosis. We anticipated major troubles related to anesthesia such as difficult ventilation and intubation, communication difficulty during induction and extubation, and difficulty in using a naso-pharyngeal airway. In addition, we had to stabilize neck alignments during intubation because cervical vertebrae were unstable and spinal cord has already been injured. Therefore, we scheduled slow induction with sevoflurane maintaining spontaneous respiration. As we found the full mouth opening of the patient after the induction, we inserted an intubating laryngeal mask, through which ventilation was successfully maintained. A tracheal tube was inserted through the intubating laryngeal mask. When the surgery was completed, we extubated using a tube introducer in the trachea. As we found that the patient's airway was open, we removed the introducer. In conclusion, with a thorough planning of the anesthetic management, we successfully managed anesthesia for cervical spinal surgery in a patient with Coffin-Lowry syndrome.

摘要

一名患有科芬-洛里综合征的12岁男性患者因颈椎损伤计划接受颈椎后路减压融合术。该患者具有科芬-洛里综合征的特征,包括智力发育迟缓、前额突出、鼻尖宽的短鼻、嘴唇丰满的宽嘴、身材矮小、小头畸形和脊柱侧弯。我们预计麻醉会出现重大问题,如通气和插管困难、诱导和拔管期间的沟通困难以及使用鼻咽气道困难。此外,由于颈椎不稳定且脊髓已经受损,我们在插管期间必须稳定颈部 alignment 。因此,我们计划用七氟醚进行慢诱导并维持自主呼吸。诱导后我们发现患者口腔完全张开,便插入了一个插管喉罩,通过它成功维持了通气。通过插管喉罩插入了气管导管。手术完成后,我们在气管内使用导管导入器进行拔管。当我们发现患者气道通畅时,取出了导入器。总之,通过对麻醉管理进行全面规划,我们成功地为一名患有科芬-洛里综合征的患者实施了颈椎手术的麻醉。

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