Division of Plastic, Reconstructive, and Oral Surgery, Children's Hospital of Philadelphia, PA.
J Craniofac Surg. 2024;35(1):18-22. doi: 10.1097/SCS.0000000000009700. Epub 2023 Aug 30.
Patients with Treacher Collins syndrome (TCS) and attendant airway dysmorphology may be predisposed to airway complications in the perioperative period. However, limited data correlates severity of mandibular hypoplasia and airway status. This study aims to improve risk stratification for perioperative airway insufficiency in TCS by using a previously proposed mandibular severity index.
Patient demographics, perioperative airway status, difficulty of intubation, and Cormack Lehane grade were collected and compared using a TCS mandibular hypoplasia severity grading scale in patients with TCS treated between 2000 and 2022.
Twenty-six patients underwent 222 procedures with institutional mandibular severity gradings as follows: 23% Grade I, 31% Grade II, 39% Grade III, 8% Grade IV. Our severity index was associated with intubation difficulty ( P <0.001) and difficult airway status ( P <0.001), with 72% of difficult airways found in grade III and grade IV patients. Mandibular retrusion and ramal hypoplasia subscores were positively correlated with measures of airway severity ( P <0.001), whereas the gonial angle was negatively correlated ( P <0.001). Age was negatively correlated with difficult visualization for endotracheal intubation ( P =0.02) but had no association with difficult airway status ( P =0.2).
This study found a positive correlation between severity of maxillomandibular dysmorphology and perioperative airway difficulty in TCS patients. Our findings suggest that severely affected patients require heightened vigilance throughout life, as difficult airways may not completely resolve with aging. Given the risk of morbidity and mortality associated with airway complications, proper identification and preparation for challenging airways is critical for TCS patients.
Treacher Collins 综合征（TCS）患者和伴随的气道畸形可能在围手术期易发生气道并发症。然而，有限的数据与下颌骨发育不全的严重程度和气道状况相关。本研究旨在通过使用先前提出的下颌骨严重程度指数来改善 TCS 围手术期气道不足的风险分层。
收集 2000 年至 2022 年期间接受治疗的 TCS 患者的患者人口统计学、围手术期气道状况、插管难度和 Cormack Lehane 分级，并使用 TCS 下颌骨发育不全严重程度分级量表进行比较。
26 名患者接受了 222 次手术，机构性下颌骨严重程度分级如下：23%为 I 级，31%为 II 级，39%为 III 级，8%为 IV 级。我们的严重程度指数与插管困难（P<0.001）和困难气道状态（P<0.001）相关，72%的困难气道发生在 III 级和 IV 级患者中。下颌骨后缩和支骨发育不全的亚评分与气道严重程度的指标呈正相关（P<0.001），而下颌角与气道严重程度呈负相关（P<0.001）。年龄与气管内插管的可视性困难呈负相关（P=0.02），但与困难气道状态无关（P=0.2）。
本研究发现 TCS 患者的上下颌骨畸形严重程度与围手术期气道困难呈正相关。我们的研究结果表明，严重受影响的患者在整个生命周期都需要高度警惕，因为随着年龄的增长，困难气道可能不会完全缓解。鉴于与气道并发症相关的发病率和死亡率风险，对于 TCS 患者，正确识别和准备具有挑战性的气道至关重要。
