Department of Pediatrics, Section of Pulmonary and Sleep Medicine, University of Colorado Denver (Primary Research Site), Aurora, Colorado, USA.
Division of Pediatric Pulmonology, Department of Pediatrics, University of Rochester, Rochester, New York, USA.
Pediatr Pulmonol. 2022 Feb;57(2):529-537. doi: 10.1002/ppul.25746. Epub 2021 Nov 8.
Plastic bronchitis (PB) is a condition characterized by the formation of thick airway casts leading to acute and often life-threatening airway obstruction. PB occurs mainly in pediatric patients with congenital heart disease (CHO) who have undergone staged surgical palliation (Glenn, Fontan), but can also occur after chemical inhalation, H1N1, severe COVID-19, sickle cell disease, severe asthma, and other diseases. Mortality risk from PB can be up to 40%-60%, and no treatment guideline exist. The objectives herein are to develop a standardized evaluation, classification, and treatment guideline for PB patients presenting with tracheobronchial casts, based on our experience with PB at the Children's Hospital of Colorado in Denver.
We describe 11 patients with CHO-associated PB (post-Fontan [n = 9], pre-Fontan [n = 2]) who presented with their initial episodes. We utilized histopathological analysis of tracheobronchial casts to guide treatment in these patients, utilizing our hospital-wide guideline document and classification system.
We found that 100% of post-Fontan PB patients had fibrinous airway casts, while pre-Fontan PB casts were fibrinous only in one of two patients (50%). Utilizing histopathology as a guide to therapy, PB patients with fibrin airway casts were treated with airway-delivered fibrinolytics and anticoagulants, as well as aggressive airway clearance and other supportive care measures. These therapies resulted in successful cast resolution and improved survival in post-Fontan PB patients.
We have shown an improved outcome in PB patients whose treatment plan was based on Denver's PB classification schema and standardized treatment guideline based on tracheobronchial cast histopathology.
塑型性支气管炎（PB）是一种以形成厚气道铸型导致急性、常危及生命的气道阻塞为特征的疾病。PB 主要发生在接受分期手术姑息治疗（Glenn、Fontan）的先天性心脏病（CHO）儿科患者中，但也可发生于化学吸入、H1N1、严重 COVID-19、镰状细胞病、严重哮喘和其他疾病之后。PB 的死亡率可达 40%-60%，且目前尚无治疗指南。本研究旨在根据丹佛科罗拉多儿童医院的 PB 经验，制定一个针对出现气管支气管铸型的 PB 患者的标准化评估、分类和治疗指南。
我们描述了 11 例 CHO 相关 PB 患者（Fontan 后[ n =9]，Fontan 前[ n =2]），他们首次出现症状。我们利用气管支气管铸型的组织病理学分析来指导这些患者的治疗，使用我们医院的指南文件和分类系统。
我们发现，100%的 Fontan 后 PB 患者存在纤维蛋白性气道铸型，而 Fontan 前 PB 患者的气道铸型中只有 2 例（50%）为纤维蛋白性。利用组织病理学作为治疗的指导，纤维蛋白性气道铸型的 PB 患者接受了气道给予的纤维蛋白溶解剂和抗凝剂，以及积极的气道清除和其他支持性护理措施。这些治疗方法导致了铸型的成功溶解，并改善了 Fontan 后 PB 患者的生存。
我们发现，基于丹佛 PB 分类方案和基于气管支气管铸型组织病理学的标准化治疗指南的治疗计划，PB 患者的治疗效果得到了改善。
