From the Departments of Anesthesiology and Perioperative Medicine.
Radiology.
Anesth Analg. 2023 Nov 1;137(5):1075-1083. doi: 10.1213/ANE.0000000000006513. Epub 2023 May 9.
Recently, tracheal narrowing has been recognized as a significant comorbid condition in patients with Morquio A, also known as mucopolysaccharidosis IVA. We studied a large cohort of patients with Morquio A to describe the extent of their tracheal narrowing and its relationship to airway management during anesthesia care.
This is an observational study, collecting data retrospectively, of a cohort of patients with Morquio A. Ninety-two patients with Morquio A syndrome were enrolled, among whom 44 patients had their airway evaluated by computed tomography angiography and had undergone an anesthetic within a year of the evaluation. Our hypothesis was that the tracheal narrowing as evaluated by computed tomography angiography increases with age in patients with Morquio A. The primary aim of the study was to examine the degree of tracheal narrowing in patients with Morquio A and describe the difficulties encountered during airway management, thus increasing awareness of both the tracheal narrowing and airway management difficulties in this patient population. In addition, the degree of tracheal narrowing was evaluated for its association with age or spirometry parameters using Spearman's rank correlation. Analysis of variance followed by the Bonferroni test was used to further examine the age-based differences in tracheal narrowing for the 3 age groups: 1 to 10 years, 11 to 20 years, and >21 years.
Patient age showed a positive correlation with tracheal narrowing ( rs= 0.415; 95% confidence interval [95% CI], 0.138-0.691; P = .005) with older patients having greater narrowing of the trachea. Among spirometry parameters, FEF25%-75% showed an inverse correlation with tracheal narrowing as follows: FEF25%-75% versus tracheal narrowing: ( rs = -0.467; 95% CI, -0.877 to -0.057; P = .007). During anesthetic care, significant airway management difficulties were encountered, including cancelation of surgical procedures, awake intubation using flexible bronchoscope, and failed video laryngoscopy attempts.
Clinically significant tracheal narrowing was present in patients with Morquio A, and the degree of such narrowing likely contributed to the difficulty with airway management during their anesthetic care. Tracheal narrowing worsens with age, but the progression appears to slow down after 20 years of age. In addition to tracheal narrowing, spirometry values of FEF25%-75% may be helpful in the overall evaluation of the airway in patients with Morquio A.
最近，气管狭窄已被认为是黏多糖贮积症 IVA （MPS IVA，又名 Morquio A）患者的一种重要合并症。我们研究了一组 Morquio A 患者，以描述其气管狭窄的程度及其与麻醉期间气道管理的关系。
这是一项观察性研究，通过回顾性收集数据，对一组 Morquio A 患者进行研究。共纳入 92 例 Morquio A 综合征患者，其中 44 例患者在评估后一年内通过计算机断层扫描血管造影术（computed tomography angiography，CTA）评估气道，并接受了麻醉。我们的假设是，Morquio A 患者的 CTA 评估的气管狭窄程度会随年龄增长而增加。本研究的主要目的是检查 Morquio A 患者的气管狭窄程度，并描述气道管理过程中遇到的困难，从而提高对该患者群体气管狭窄和气道管理困难的认识。此外，还使用 Spearman 秩相关分析评估了气管狭窄程度与年龄或肺活量计参数之间的关系。采用方差分析（analysis of variance，ANOVA），随后进行 Bonferroni 检验，进一步检查 3 个年龄组（1-10 岁、11-20 岁和>21 岁）的气管狭窄程度的年龄差异。
患者年龄与气管狭窄呈正相关（ rs=0.415；95%置信区间 [95%CI]，0.138-0.691；P=.005），年龄较大的患者气管狭窄程度更严重。在肺活量计参数中，FEF25%-75%与气管狭窄呈负相关：FEF25%-75%与气管狭窄：（ rs=-0.467；95%CI，-0.877 至-0.057；P=.007）。在麻醉期间，气道管理出现显著困难，包括手术取消、使用纤维支气管镜清醒插管和视频喉镜尝试失败。
Morquio A 患者存在明显的临床意义上的气管狭窄，且狭窄程度可能导致麻醉期间气道管理困难。气管狭窄随年龄增长而恶化，但 20 岁后恶化速度似乎放缓。除了气管狭窄外，FEF25%-75%的肺活量计值可能有助于对 Morquio A 患者气道进行全面评估。
