PDF(Pubmed)
Abstract：
We present the case of a 41-year-old man with Rapp-Hodgkin syndrome who underwent nasal septum deviation surgery under general anaesthesia. This syndrome is rare, with approximately 70 cases reported worldwide. It is one of a group of ectodermal dysplasia syndromes and results from the aberrant development of ectoderm during fetal development. Some of the clinical features may affect anaesthetic management. The most important considerations are potentially difficult airway management, the need for meticulous temperature control, and the importance of skin protection. This case was uneventful, but as there are few case reports on the management of patients with ectodermal dysplasia syndromes undergoing anaesthesia this report contributes useful knowledge. The pathogenesis and clinical features of Rapp-Hodgkin syndrome and the anaesthetic management for this patient are described.
摘要：
我们介绍了一名41岁的Rapp-Hodgkin综合征患者，他在全身麻醉下接受了鼻中隔偏曲手术。这种综合症很罕见,全球报告了大约70例病例。它是一组外胚层发育不良综合征之一，是胎儿发育过程中外胚层异常发育的结果。一些临床特征可能会影响麻醉管理。最重要的考虑因素是潜在的困难气道管理，需要细致的温度控制，以及保护皮肤的重要性.这个案子很顺利,但由于很少有关于外胚层发育不良综合征患者接受麻醉治疗的病例报告,本报告提供了有用的知识.描述了Rapp-Hodgkin综合征的发病机理和临床特征以及该患者的麻醉管理。
