Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Paediatr Anaesth. 2021 Dec;31(12):1316-1324. doi: 10.1111/pan.14310. Epub 2021 Oct 21.
Crouzon and Pfeiffer syndromes are rare genetic disorders characterized by craniosynostosis, exorbitism, and maxillary hypoplasia. Patients with these syndromes frequently require general anesthesia for various diagnostic and surgical procedures and may present a challenge to anesthetists with regard to airway management.
The primary aim of this study was to determine the incidence, timing, and management of perioperative upper airway obstruction in infants and children with Crouzon and Pfeiffer syndromes. The secondary aim was to determine the degree of difficulty in performing endotracheal intubation.
A retrospective review of 812 anesthetic encounters in 67 patients was conducted. The following were recorded: timing and management of episodes of perioperative upper airway obstruction, from induction of anesthesia to discharge from recovery, degree of difficulty with laryngoscopy using the Cormack-Lehane grading system and number of intubation attempts required, patient demographics, respiratory comorbidity, surgical procedure, and anesthetic airway management techniques.
Upper airway obstruction at induction of anesthesia was very common, with an incidence of 31% (167/542 anesthetic encounters affecting 54 patients). In a quarter of these incidents, bag-valve-mask ventilation was challenging, but a laryngeal mask airway was almost always effective. Upper airway obstruction on emergence from anesthesia was less common, with an incidence of 2.7% (14/515 anesthetic encounters affecting 10 patients). Contributing factors included patient comorbidity (obstructive sleep apnea, nasal stenosis) and the nature of surgery (craniofacial or airway procedures). Intubation was rarely difficult in this cohort, with 85% of laryngoscopies rated Cormack-Lehane grade 1 or 2 (n = 373), and 89% of intubations successful on the first attempt (n = 306).
Upper airway obstruction at induction of anesthesia is common in patients with Crouzon and Pfeiffer syndrome. These patients are likely to present some difficulties with perioperative airway management, especially bag-valve-mask ventilation, but rarely endotracheal intubation.
Crouzon 综合征和 Pfeiffer 综合征是两种罕见的遗传性疾病，其特征为颅缝早闭、眶距增宽和上颌骨发育不全。这些综合征患者常因各种诊断和手术程序而需要全身麻醉，这给麻醉师的气道管理带来了挑战。
本研究的主要目的是确定 Crouzon 综合征和 Pfeiffer 综合征患者围手术期上呼吸道梗阻的发生率、时间和处理方法。次要目的是确定行气管内插管的难度程度。
对 67 例患者的 812 次麻醉进行回顾性分析。记录内容包括：从麻醉诱导到恢复期间围手术期上呼吸道梗阻的发生时间和处理方法；使用 Cormack-Lehane 分级系统评估喉镜检查的难度程度和所需的插管次数；患者的人口统计学资料、呼吸合并症、手术程序和麻醉气道管理技术。
麻醉诱导时上呼吸道梗阻非常常见，发生率为 31%（542 次麻醉中有 167 次，涉及 54 例患者）。在这些病例中，有四分之一的患者行球囊面罩通气有困难，但喉罩气道几乎总是有效。麻醉苏醒时上呼吸道梗阻较少见，发生率为 2.7%（515 次麻醉中有 14 次，涉及 10 例患者）。其相关因素包括患者的合并症（阻塞性睡眠呼吸暂停、鼻狭窄）和手术性质（颅面或气道手术）。在本队列中，插管很少困难，85%的喉镜检查分级为 Cormack-Lehane 1 级或 2 级（n=373），89%的插管在首次尝试时成功（n=306）。
Crouzon 综合征和 Pfeiffer 综合征患者麻醉诱导时上呼吸道梗阻常见。这些患者在围手术期气道管理方面可能会遇到一些困难，尤其是在进行球囊面罩通气时，但行气管内插管很少困难。
