Nanegrungsunk D, Jericho B G
Department of Anaesthesiology University of Illinois College of Medicine at Chicago IL USA.
Anaesth Rep. 2020 Jul 23;8(2):e12054. doi: 10.1002/anr3.12054. eCollection 2020 Jul-Dec.
Ectodermal dysplasias are a rare group of heritable disorders involving the ectodermal derivatives with only a few published reports involving its anaesthetic management. We present the case of a 16-year-old boy with ectodermal dysplasia presented for elective oral surgery under general anaesthesia with a surgeon preference for nasotracheal intubation to provide adequate surgical exposure. The patient had successful nasal flexible bronchoscopic intubation despite challenging tracheal intubation conditions. We summarise previous case reports and discuss anaesthetic considerations in ectodermal dysplasia , including reduced mucus production in the respiratory tract, laryngeal incompetence, potential for difficult tracheal intubation and hyperthermia from reduced or absent sweat glands.
外胚层发育不良是一组罕见的遗传性疾病，涉及外胚层衍生物，仅有少数关于其麻醉管理的报道。我们报告一例16岁患有外胚层发育不良的男孩，因择期口腔手术接受全身麻醉，外科医生倾向于鼻气管插管以提供足够的手术视野。尽管气管插管条件具有挑战性，但患者成功进行了鼻软性支气管镜插管。我们总结了既往病例报告，并讨论了外胚层发育不良的麻醉注意事项，包括呼吸道黏液分泌减少、喉功能不全、气管插管困难的可能性以及汗腺减少或缺失导致的体温过高。
