婴儿先天性大血管疾病合并气道狭窄的研究

Congenital macrovascular disease with airway stenosis in children

目的探讨婴儿先天性大血管疾病合并气道狭窄的围手术期评估方法及治疗方案。方法回顾性分析2005年10月至2009年6月广东省人民医院收治的经纤支镜及胸部螺旋计算机断层扫描检查确诊为气道狭窄的大血管疾病患儿的一般临床资料及临床记录,纤支镜、心脏超声及胸部螺旋计算机断层扫描检查结果。结果研究期内收治<1岁的婴儿大血管疾病236例,确诊为气道狭窄的34例大血管疾病患儿纳入研究,气道狭窄的发生率在同期收治的大血管疾病婴儿中为14.4%(34/236),其中男30例,女4例,中位年龄2.38个月(6 d～11个月)。新生儿10例,<3个月21例,中位体质量4.47 kg(2.18～8.70 kg);主动脉梗阻性疾病24例,其中主动脉缩窄18例,主动脉弓离断6例;血管环10例,都在确诊前行纤支镜检查,镜检发现有严重的气管、支气管狭窄、畸形,中下段气管狭窄合并左、右主支气管狭窄,完整气管环形成,镜下发现狭窄部位有明显血管搏动,提示血管环畸形,9例术前行心血管计算机断层扫描、1例经手术证实为血管环,其中7例为先天性气管、支气管发育不良;24例主动脉病变均在心血管矫治术前行螺旋计算机断层扫描检查,术前发现气管狭窄者仅5例,其中1例术前纤支镜显示气道狭窄,余19例均在术中或术后经纤支镜发现气道外压性狭窄。纤支镜检查24例主动脉病变中22例有左主支气管外压性狭窄,2例伴先天性气管狭窄,2例右主支气管狭窄,1例右下支气管狭窄。34例患儿中行心血管外科手术治疗32例,4例需行主动脉悬吊固定术缓解气道狭窄,2例放弃治疗。婴儿监护病房的中位停留时间34 d(5～158 d);痊愈或好转出院的25例,占73.5%(25/34);死亡7例,占20.6%(7/34)。结论先天性大血管疾病合并气道狭窄发生率高,治疗较单纯先天性心脏病更加困难,婴儿监护病房停留时间长,病死率高。部分患儿需手术干预缓解气道狭窄,纤支镜结合胸部多层螺旋计算机断层扫描检查对这类患儿围手术期的气道病变及其与周围组织的关系能得到更多的信息,指导制定完善的手术方案。

Objectives To evaluated the perioperative assessment and therapies of congenital macrovascular disease with tracheal stenosis in infants. Methods This research was a retrospective analysis. The general clinical data, clinical records, bronchoscopy, echocardiography and chest spiral CT findings of children patients, who were diagnosed as congenital macrovascular disease with airway stenosis by bronchoscopy and chest spiral CT, from October 2005 to June 2009 in Guangdong General Hospital were analyzed. Results Two hundred and thirty six babies less than 1 year old who suffered from large vessel disease were treated during the research period, and 34 cases of them combined with tracheal stenosis were included in the study. The incidence of airway stenosis in the infant patients who suffered from macrevascular disease and treated in our hospital was 14.4% （34/236） in the same period, of which 30 males and 4 females, median age 2.38 months （6 d-ll months）, 10 cases of newborns, 21 cases of less than 3 months, weight 4.47 kg （2.18-8.70 kg）, 10 cases of vascular ring and 24 cases of aortic obstruction （including 18 cases of coarctation of the aorta and 6 aortic arch）. Ten cases of vascular ring were performed bronchoscopy before the diagnosis, finding that there were severe tracheal-bronchial stenosis, deformity, middle and lower tracheal stenosis combined with left and right main bronchial stenosis, complete tracheal rings formed. Significant vascular pulsatility in the narrowing can be found by bronchoscopy, suggesting that vascular ring malformations. Nine cases performed cardiovascular CT before the operation, and 1 patient was confirmed vascular ring in the operation, 7 cases of them were diagnosed as congenital tracheal and bronchial dysplasia. Twenty four cases of aortic lesions were performed cardiovascular spiral CT examination before the operative treatment. Preoperative examination found tracheal stenosis only in 5 cases, including 1 case whose preoperative bronchoscopy showed tracheal stenosis, and the remaining 19 cases were found airway stenosis caused by external pressure during the operation or postoperative bronchoscopy by bronchofiberscope. Bronchofiberscope result indicated that 22 of 24 aortic lesion cases were combined with left main bronchial stenosis caused by external pressure, and 2 patients were with congenital tracheal stenosis, 2 patients combined with right main bronchial stenosis, 1 patient combined with right lower lobe bronchial stenosis. Thirty two cases of 34 were performed surgical treatment, and 4 patients needed aortopexy performed to alleviate airway stenosis. There were 2 cases who gave up treatment and discharged. The median of infant care unit stay was 34 days （5-158 days）. Twenty five cases were cured or improved and discharged, accounting for 73.5% （25/34）, and 7 cases died which account for 20.6% （7/34）. Conclusions The incidence of congenital macrovascular disease combine with airway stenosis is high, and its treatment is more difficult than that of a simple congenital heart disease. Also, its ICU stay is longer and mortality is high. Some patients require surgical intervention to relieve airway stenosis. Bronchoscopy with spiral chest CT examination in these patients during the perioperative period can get more information about the airway lesions and their relationship with the surrounding tissue to guide us to make a perfect surgical program.