Chao Yen-Chun, Peng Chun-Chin, Lee Kuo-Sheng, Lin Shan-Miao, Chen Ming-Ren
Department of Pediatrics, Mackay Children's Hospital, Taipei, Taiwan.
Department of Otorhinolaryngolaryngology, Mackay Memorial Hospital, Taipei, Taiwan.
Int J Pediatr Otorhinolaryngol. 2016 Apr;83:1-6. doi: 10.1016/j.ijporl.2016.01.024. Epub 2016 Jan 27.
Congenital tracheobronchial stenosis (CTBS) is a rare disorder characterized by the presence of focal or diffuse complete tracheal or bronchial cartilage rings resulting in a fixed lumen narrowing. The aim of this study was to expose the association of various cardiovascular (CV) anomalies with various types of CTBS.
A retrospective review of 58 patients who had bronchoscopically proven CTBS between 1997 and 2011 was conducted. Totally, 48 cases who had received echocardiography, computed tomography and other examinations including cardiac catheterization were enrolled. There were 33 boys and 15 girls, and the median age of diagnosis was 4 months (0-44 months). The image findings of associated CV anomalies were reviewed and analyzed, along with the clinical manifestations and patients' outcome.
There were 14 generalized hypoplasia (29.2%), 10 funnel type (20.8%), 15 segmental type (31.3%), and 9 isolated bronchial stenosis cases (18.7%). Among 48 cases of CTBS, 32 patients (66.7%) had various CV anomalies including atrial septal defect (n=9), pulmonary artery sling (n=7), ventricular septal defect (n=7), patent ductus arteriosus (n=6), tetralogy of Fallot (n=3), peripheral pulmonary stenosis (n=3), etc. No significant difference existed in the incidence and types of comorbid CV anomalies among 4 types of CTBS, except that funnel type had significantly higher comorbidity of pulmonary artery sling (50%, p<0.01).
Patients with CTBS had high incidence of various associated CV anomalies. Isolated bronchial stenosis had similar high comorbidity of CV anomalies as did the 3 classical categories of congenital tracheal stenosis. The higher comorbidity of pulmonary artery sling in patients with funnel CTBS might be related to its gradual caudal narrowing of the trachea.
先天性气管支气管狭窄（CTBS）是一种罕见疾病，其特征为存在局灶性或弥漫性完整气管或支气管软骨环，导致管腔固定性狭窄。本研究旨在揭示各种心血管（CV）异常与各类CTBS之间的关联。
对1997年至2011年间经支气管镜证实为CTBS的58例患者进行回顾性研究。共纳入48例接受过超声心动图、计算机断层扫描及包括心导管检查在内的其他检查的患者。其中男33例，女15例，诊断时的中位年龄为4个月（0 - 44个月）。回顾并分析相关CV异常的影像表现、临床表现及患者预后。
有14例为广泛性发育不全（29.2%），10例为漏斗型（20.8%），15例为节段型（31.3%），9例为孤立性支气管狭窄病例（18.7%）。在48例CTBS患者中，32例（66.7%）存在各种CV异常，包括房间隔缺损（n = 9）、肺动脉吊带（n = 7）、室间隔缺损（n = 7）、动脉导管未闭（n = 6）、法洛四联症（n = 3）、外周肺动脉狭窄（n = 3）等。4种类型的CTBS中，合并CV异常的发生率及类型无显著差异，但漏斗型肺动脉吊带的合并症显著更高（50%，p < 0.01）。
CTBS患者各种相关CV异常的发生率较高。孤立性支气管狭窄与先天性气管狭窄的3种经典类型一样，CV异常的合并症发生率也较高。漏斗型CTBS患者肺动脉吊带合并症较高可能与其气管逐渐向尾端狭窄有关。
