Beckwith-Wiedemann综合征(BWS)是最常见的先天性过度生长障碍,发病率约为10,000例活产婴儿中的1例。这种情况的特征是侧向过度生长,腹壁缺损,巨舌,和恶性肿瘤的易感性。历史上,患有BWS的儿童被认为有困难的气道;然而,支持这一点的大部分证据都是轶事,来自病例报告。我们的研究旨在确定BWS患者中困难气道的患病率。我们假设大多数患有BWS的患者不会有困难的气道。
我们回顾性审查了在我们机构的BWS登记处登记的患者的电子病历。纳入2012年1月至2019年7月期间麻醉的分子诊断为BWS的患者进行分析。主要结果是存在困难的气道,定义为困难的面罩通风,困难的插管,或者两者兼而有之。我们将困难的插管定义为需要3次或更多次气管插管尝试,以及需要先进的气道技术(非直接喉镜检查)进行气管插管或在直接喉镜检查期间Cormack和Lehane等级≥3。次要目标是确定插管困难和面罩通气困难的预测因素。以及不良气道事件的发生率。使用广义线性混合效应模型来解释每位患者的多个麻醉事件。
在注册登记的201名BWS患者中,60%(n=122)有一种或多种记录的麻醉药,总共310种麻醉药。22种麻醉药中存在预先存在的气道。困难气道的患病率为5.3%(95%置信区间[CI],3.0-9.3;288个案例中的18个)。困难插管的患病率为5.2%(95%CI,2.9-9.4;226中的12)。面罩通气困难的患病率为2.9%(95%CI,1.4-6.2;277中的12),并且未尝试在42种麻醉药中进行面罩通气。年龄<1岁,巨舌,较低的重量,内分泌合并症,塑料/颅面外科,减舌手术,在没有预先存在气道的情况下,阻塞性睡眠呼吸暂停与困难的气道相关。约83.8%(95%CI,77.6-88.5)的病例通过一次尝试插管。低氧血症是最常见的不良事件。
BWS患儿气管插管困难和面罩通气困难的患病率分别为5.2%和2.9%,分别。我们确定了与气道困难相关的因素,其中包括年龄<1岁,巨舌,内分泌异常,塑料/颅面外科,减舌手术,和阻塞性睡眠呼吸暂停。临床医生应该预测有这些因素的患者的气道困难。
Beckwith-Wiedemann syndrome (BWS) is the most common congenital overgrowth disorder with an incidence of approximately 1 in 10,000 live births. The condition is characterized by lateralized overgrowth, abdominal wall defects, macroglossia, and predisposition to malignancy. Historically, children with BWS have been presumed to have difficult airways; however, most of the evidence to support this has been anecdotal and derived from case reports. Our
study aimed to determine the prevalence of difficult airway in patients with BWS. We hypothesized that most patients with BWS would not have difficult airways.
We retrospectively reviewed the electronic medical records of patients enrolled in our institution\'s BWS registry. Patients with a molecular diagnosis of BWS who were anesthetized between January 2012 and July 2019 were included for analysis. The primary outcome was the presence of difficult airway, defined as difficult facemask ventilation, difficult intubation, or both. We defined difficult intubation as the need for 3 or more tracheal intubation attempts and the need for advanced airway techniques (nondirect laryngoscopy) to perform tracheal intubation or a Cormack and Lehane grade ≥3 during direct laryngoscopy. Secondary objectives were to define predictors of difficult intubation and difficult facemask ventilation, and the prevalence of adverse airway events. Generalized linear mixed-effect models were used to account for multiple anesthesia events per patient.
Of 201 BWS patients enrolled in the registry, 60% (n = 122) had one or more documented anesthetics, for a total of 310 anesthetics. A preexisting airway was present in 22 anesthetics. The prevalence of difficult airway was 5.3% (95% confidence interval [CI], 3.0-9.3; 18 of 288) of the cases. The prevalence of difficult intubation was 5.2% (95% CI, 2.9-9.4; 12 of 226). The prevalence of difficult facemask ventilation was 2.9% (95% CI, 1.4-6.2; 12 of 277), and facemask ventilation was not attempted in 42 anesthetics. Age <1 year, macroglossia, lower weight, endocrine comorbidities, plastics/craniofacial surgery, tongue reduction surgery, and obstructive sleep apnea were associated with difficult airways in cases without a preexisting airway. About 83.8% (95% CI, 77.6-88.5) of the cases were intubated with a single attempt. Hypoxemia was the most common adverse event.
The prevalence of difficult tracheal intubation and difficult facemask ventilation in children with BWS was 5.2% and 2.9%, respectively. We identified factors associated with difficult airway, which included age <1 year, macroglossia, endocrine abnormalities, plastics/craniofacial surgery, tongue reduction surgery, and obstructive sleep apnea. Clinicians should anticipate difficult airways in patients with these factors.