Beckwith-Wiedemann综合征患者的麻醉管理:病例汇报与文献复习

Anesthesia management of Beckwith-Wiedemann syndrome:a case report and review of literatures

目的探讨Beckwith-Wiedemann综合征(BWS)患者的麻醉管理。方法本文报道了1例行腹股沟疝和脐膨出修补术的Beckwith-Wiedemann综合征患儿的麻醉管理,并对相关文献进行复习。结果经充分术前评估,收集患儿病史并排除困难气道、低血糖等风险后,经静脉诱导后顺利置入气管导管并在静吸复合全麻下完成手术;术后清醒拔管,无低氧血症和低血糖等不良事件发生。结论巨舌所致困难气道、低血糖等是BWS患者麻醉管理所面临的主要问题。麻醉医师应充分评估病情从而制定适当的麻醉管理方案。

Objective To investigate the anesthesia management of Beckwith-Wiedemann syndrome.Methods The anesthesia management of a child with Beckwith-Wiedemann syndrome was retro-spectively analyzed,and the related literatures were reviewed.Results After sufficient preoperative systemic evaluation,knowing the patient′s history and confirming the risks such as difficult airway and hypoglycaemia,an intravenous induction and intubation were succeed and the operation was successfully completed under combined intravenous and inhalation anesthesia.The patient recovered well after operation.Conclusion Difficult airway caused by macroglossia and hypoglycaemia are the main problems in BWS anesthesia management.Anesthesiologists should fully understand these problems and make appro-priate preoperative evaluation of BWS patients so as to formulate appropriate anesthesia management plan.