儿童先天性气管支气管畸形176例临床分析

Clinical analysis of 176 children with congenital tracheobronchial malformation

目的探讨儿童先天性气管支气管畸形的临床特征。方法回顾性分析2016年1月至10月重庆医科大学附属儿童医院收治住院的经支气管镜检查诊断为先天性气管支气管畸形的176例患儿资料,并电话随访出院3年内有无喘息以及手术、死亡情况。结果1.176例患儿中,年龄<3岁156例,占88.64%。2.176例患儿中先天性气管支气管狭窄54例,先天性气管支气管软化42例,支气管开口异常26例,呼吸道憩室3例,气管食管瘘1例,支气管桥1例,混合型(2种及2种以上畸形)49例。3.临床特点以反复或持续喘息(86例)、慢性咳嗽(61例)、反复呼吸道感染(34例)最为常见,其他表现包括吸气性喉鸣(14例)、肺不张(29例)、局限性肺气肿(6例)、气管插管后拔管困难(6例)。4.共139例患儿接受胸部CT检查和气道重建技术,仅44例CT作出了正确诊断。5.患儿均接受住院期间内科常规治疗、支气管镜检查治疗,其中17例行手术治疗。170例患儿好转出院,6例治疗效果欠佳。随访3年,共47例接受电话随访,随访1年、2年、3年喘息比例分别为59.57%、51.06%、38.30%;手术治疗4例,死亡2例。结论儿童先天性气管支气管畸形常见于婴幼儿,临床表现不典型,疑诊者应尽早进行胸部CT和气道重建术检查,不能明确诊断时可及时完善支气管镜检查;多数患儿预后可,少数死亡,部分患儿仍有反复或持续喘息,部分危重儿需行气道支架置入或外科手术干预。

Objective To investigate the clinical characteristics of congenital tracheobronchial malformation in children.Methods Retrospective analysis was performed on the data of 176 children with congenital tracheobronchial malformation who were diagnosed by bronchoscopy at Children′s Hospital of Chongqing Medical University from January 2016 to October 2016,and were followed up by telephone for the presence of dyspnea,operation and death within 3 years after discharge.Results(1)Among the 176 cases,there were 156 cases less than 3 years old,accounting for 88.64%.(2)Among the 176 cases,there were 54 cases with congenital tracheobronchial stenosis,42 cases with congenital tracheomalacia,26 cases with abnormal bronchial openings,3 cases with airway diverticulum,1 case with tracheoesophageal fistula,1 case with bridging bronchus,and 49 cases with mixed type(2 or more malformations).(3)The most common clinical manifestations included recurrent or persistent dyspnea(86 cases),chronic cough(61 cases),and recurrent respiratory infection(34 cases)with the most frequent occurrence.Other manifestations included laryngeal stridor(14 cases),pulmonary atelectasis(29 cases),localized emphysema(6 cases),and difficulty extubation after endotracheal intubation(6 cases).(4)Among the 139 patients who underwent chest CT examination and airway reconstruction,only 44 cases were diagnosed correctly.(5)All 176 patients received routine medical treatment and bronchoscopy during hospitalization,among which 17 cases received surgical treatment.One hundred and seventy cases were improved in their condition and discharged,and 6 cases did not respond well.A total of 47 patients were followed up by telephone for 3 years.The proportion of children with dyspnea after 1 year,2 years and 3 years were 59.57%,51.06%and 38.30%,respectively.Among the follow-up patients,4 patients received surgical treatment and 2 patients died.Conclusions Congenital tracheobronchial malformation in children often occurs in infants,and their clinical manifestations are not typical.Therefore,suspected patients should receive chest CT and airway reconstruction as early as possible,and bronchoscopy can be employed in time if the diagnosis is unclear.The majority of children can obtain a favorable prognosis,with few death cases.Partial patients would present with a risk of recurrent or persistent dyspnea,and some critically ill children shall receive the airway stent implantation or surgical intervention.