Department of Otorhinolaryngology Head and Neck Surgery Inselspital, Bern University Hospital, University of Bern, 3010, Bern, Switzerland.
Division of Respiratory Medicine, Department of Paediatrics, Inselspital, University of Bern, 3010, Bern, Switzerland.
Eur J Pediatr. 2022 Jan;181(1):303-309. doi: 10.1007/s00431-021-04198-6. Epub 2021 Jul 21.
Major congenital anomalies are known to play a role in the management and prognosis of airway obstruction. Most studies assess acquired forms of airway obstruction. Data on congenital or otherwise non-acquired forms of airway obstruction is sparse. In this retrospective, single-institution cohort study, we sought to evaluate and compare the patterns of airway obstruction in children with and without major congenital anomalies, and to assess the impact of management and outcome, irrespective of aetiology. Fifty-five patients were included, 23 with and 32 without underlying major congenital anomalies. Multilevel airway obstruction (usually affecting the nasopharynx, oropharynx, and the trachea) was more common in children with congenital anomalies (91% vs. 41%, p < .001). Consequently, these children required more frequent and earlier surgical management, especially tracheostomy and adenotonsillar surgery.Conclusions: Major congenital anomalies are associated with multilevel airway obstruction and poor functional prognosis. A simple clinical definition considering impact of major congenital anomalies on development and growth may help guide management plans following endoscopic evaluation of the entire airway and flanked by multidisciplinary discussions. What is Known: • Children with major comorbidities display increased disease severity and more prevalent multilevel airway obstruction • Previous studies include both children with acquired and non-acquired forms of airway obstruction; therefore, the actual impact major comorbidities in children with non-acquired causes of airway obstruction remain unclear. What is New: • A total of 42% children in this study population had major comorbidities with and impact on growth and/or psychomotor development, with a higher prevalence of multilevel airway obstruction and worse rates of functional improvement/recovery. • Children with major comorbidities require tracheostomy more often and earlier than those without major comorbidities, and remain tracheostomy-dependent for a longer time.
先天性重大畸形已知会影响气道阻塞的管理和预后。大多数研究评估后天性气道阻塞。关于先天性或其他非后天性气道阻塞的数据很少。在这项回顾性的单机构队列研究中，我们旨在评估和比较患有和不患有主要先天性畸形的儿童的气道阻塞模式，并评估病因不明的管理和结果的影响。共纳入 55 例患者，其中 23 例存在先天性畸形，32 例不存在先天性畸形。多层气道阻塞（通常影响鼻咽、口咽和气管）在先天性畸形患儿中更为常见（91%比 41%，p<.001）。因此，这些患儿需要更频繁和更早的手术治疗，尤其是气管造口术和腺样体切除术。结论：主要先天性畸形与多层气道阻塞和不良功能预后相关。一个简单的临床定义，考虑主要先天性畸形对发育和生长的影响，可能有助于指导整个气道内镜评估后的管理计划，并辅以多学科讨论。已知情况：•患有主要合并症的儿童显示出更高的疾病严重程度和更普遍的多层气道阻塞；•以前的研究包括后天性和非后天性气道阻塞的儿童；因此，先天性气道阻塞的非后天性病因的儿童的实际影响仍不清楚。新情况：•本研究人群中有 42%的儿童患有主要合并症，对生长和/或精神运动发育有影响，多层气道阻塞的患病率更高，功能改善/恢复的比例更差。•患有主要合并症的儿童比不患有主要合并症的儿童更常需要且更早进行气管造口术，并且需要更长时间依赖气管造口术。
