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目的探讨肺动脉吊带合并气管狭窄的诊断及治疗方法。方法选取2012年1月至2016年1月收治的肺动脉吊带合并气管狭窄患儿23例,依据气管狭窄程度分为轻度狭窄组(n=13)和重度狭窄组(n=10)。回顾性分析患儿临床特征、手术方式的选择及治疗效果。结果轻度狭窄组9例术前无明显呼吸道症状,重度狭窄组均有症状。轻度狭窄组仅行左肺动脉移植。重度狭窄组中完全型气管环、静息下喘鸣患儿行左肺动脉移植及气管重建术,其他患儿均不处理气管。重度狭窄组1例气管软化术中置入气管支架。轻度狭窄组患儿术后恢复顺利。重度狭窄组1例早期完全型气管环合并气管软化术中未处理气管,术后脱机困难,死于多脏器功能衰竭。3例同时行气管重建恢复顺利。放支架患儿术后气管内分泌物较多,需反复纤支镜吸痰,1个月后突然死于气道大出血。2例脱呼吸机后吸气困难严重,需无创机械通气支持。术后随访5~53个月,轻度狭窄组均无明显呼吸道症状,重度狭窄组生存患儿活动后仍有气喘2例。呼吸道症状在术后5~11个月缓解。结论肺动脉吊带合并气管狭窄临床表现无特异性,提高警觉性可以避免误诊。术前气管镜检查有助于准确评估气管狭窄的部位、类型及程度。气管狭窄的程度、类型及术前呼吸道的症状,是指导手术方式选择的重要依据。
Objective To investigate the diagnosis and treatment of pulmonary artery stenosis with tracheal stenosis. Methods Twenty-three children with pulmonary artery stenosis and tracheal stenosis admitted from January 2012 to January 2016 were divided into mild stenosis group (n = 13) and severe stenosis group (n = 10) according to the degree of tracheal stenosis. Retrospective analysis of children with clinical features, the choice of surgical approach and treatment. Results Nine patients in mild stenosis group had no obvious respiratory symptoms before operation, and severe stenosis group had symptoms. Mild stenosis group left pulmonary artery transplantation only. Severe stenosis group, complete tracheal ring, left wheezing under rest in patients with left pulmonary artery and tracheal reconstruction, the other children do not deal with the trachea. Severe stenosis group, 1 case of tracheal tube placement tracheal stent. Children with mild stenosis recovered successfully. Severe stenosis group, 1 case of early complete tracheal ring tracheal tube tracheal unprocessed untreated trachea, postoperative off-line difficulties, died of multiple organ failure. 3 cases of simultaneous tracheal reconstruction recovered smoothly. Placed stents in children with more tracheal secretions, need repeated suction bronchoscopy, 1 month after sudden death from airway hemorrhage. 2 cases of inhalation after breathing machine difficult, requiring noninvasive mechanical ventilation support. All the patients were followed up for 5 to 53 months. There were no obvious respiratory symptoms in mild stenosis group. There were still 2 cases of asthma in children surviving severe stenosis group. Respiratory symptoms were relieved 5 to 11 months after surgery. Conclusions The clinical manifestations of pulmonary artery stenosis complicated with tracheal stenosis are nonspecific. Arousing alertness can avoid misdiagnosis. Preoperative bronchoscopy helps to accurately assess the site, type and extent of tracheal stenosis. The degree and types of tracheal stenosis and the symptoms of preoperative airway are the important basis to guide the choice of surgical methods.