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[贝克威思-维德曼综合征患者的麻醉管理]

[Anesthetic management in a patient with Beckwith-Wiedemann syndrome].

作者信息

Fujita A, Okutani R, Fukuda T, Fu K, Okamoto T

机构信息

Department of Anesthesiology, Hyogo College of Medicine, Nishinomiya.

出版信息

Masui. 1994 Sep;43(9):1389-91.

PMID:7967041
Abstract

Beckwith-Wiedemann syndrome has three major symptoms, exomphalos, macroglossia and giantism. We report the anesthetic management of a 2-year-old male, weighing 16.1 kg and with a height of 94.6 cm, associated with bilateral retention testis. The anesthesia was maintained with oxygen, nitrous oxide, and sevoflurane. The surgery was completed safely without adverse events such as difficulty in tracheal intubation or refractory hypoglycemia. The anesthetic management of this syndrome requires: an assessment of difficulty in tracheal intubation and treatment, an understanding of the capacity of the patient's glucose metabolism, and assessment of complicated deformities. In particular, it is necessary to carry out a preoperative examination of the cardiovascular system.

摘要

贝克威思-维德曼综合征有三大主要症状,即脐膨出、巨舌症和巨人症。我们报告了一名2岁男性患者的麻醉管理情况,该患者体重16.1千克,身高94.6厘米,伴有双侧隐睾。麻醉维持采用氧气、氧化亚氮和七氟醚。手术安全完成,未出现气管插管困难或难治性低血糖等不良事件。该综合征的麻醉管理需要:评估气管插管困难情况并进行处理,了解患者葡萄糖代谢能力,评估复杂畸形情况。尤其有必要对心血管系统进行术前检查。

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