肺动脉吊带合并气管狭窄畸形矫治不干预气管的预后分析

发布时间：2018-05-27 00:08

本文选题：肺动脉吊带 + 气管狭窄　；参考：《南方医科大学》2017年硕士论文

【摘要】：背景:肺动脉吊带(Pulmonayartrysling,PAS)是一种罕见的先天性心血管畸形。解剖特征为异常走形的左肺动脉压迫造成气管狭窄。气管干预,指各种形式的气管狭窄矫治手术,包括外科修补成形或内科支架置入术。目前对于PAS合并气管狭窄的患儿是否在行左肺动脉移植术的同时进行气管干预依然存在很大争议。早期一部分学者认为应同时行左肺动脉矫治和气管成形术,因左肺动脉压迫所致的气管软骨环可能导致长段型气管狭窄;此外,完全性气管环导致的气管狭窄将导致术后气管狭窄长期存在,严重影响PAS患儿的预后,需要进行气管干预。由于气管手术难度大,并发症多,死亡率高。一部分学者开始尝试、并主张单纯行左肺动脉移植术,从而避免气管并发症,提高患儿预后,因为对异常起源的左肺动脉进行移位或再植可减轻或解除对气管的压迫,明显改善患儿的呼吸道症状。但目前哪些患儿能避免气管干预,无预测指标。目的分析PAS合并气管狭窄畸形矫治不干预气管的预后,寻找术前判断避免气管干预的预测指标,探讨提高该病治疗效果的方法。方法采用描述性流行病学的方法进行研究。把2011年10月-2015年11月间北京八一儿童医院17例PAS合并气管狭窄患儿被纳入研究,术前所有患儿应用纤维支气管镜检查完全气管环。临床结果和气管狭窄严重程度(气管内径和长度)被纳入评估。PAS合并气管狭窄或其他心脏畸形应用心脏彩色多普勒超声和计算机化断层显像诊断。CT结果审阅和狭窄气管内径和气管长度在某心脏中心由同一位医师审阅。收集所有纳入患儿的临床资料,包括性别、年龄、临床表现,CT影像学检查、纤维支气管镜、彩色多普勒超声心动图结果,手术方式、药物利用、术后治疗策略以及预后资料。结果共收集到17例病例。全组17例患儿初次手术均仅行肺动脉移植术未行气管干预,手术均顺利完成,术后均采用早期拔管、呼吸机辅助通气策略。其中,12例早期顺利撤离呼吸机,痊愈出院;另外5例因多次撤机失败,围术期行气管干预手术,均死亡。死亡5例中,3例行气管支架植入术(1例死于坏死性小肠结肠炎、2例死于感染及多脏器功能衰竭),2例行气管成形术(均死于感染及气管漏气)。所有存活患儿出院后随访呼吸道症状均有所减轻甚至消失。气管狭窄指数[(气管狭窄段直径/气管狭窄段长度)X100%]有无气管干预的2组患儿间差异有统计学意义,气管未干预组(10.14±1.58)%,气管干预组(5.72±1.17)%(P0.001)。对于气管狭窄指数高于(10.14±1.58)%的PAS患儿,采取单纯肺动脉移植术可获得满意的预后。对于气管狭窄指数低于(5.72±1.17)%的PAS患儿,Ⅰ期单纯行肺动脉移植术预后不良。结论对于绝大多数合并中度以上气管狭窄的PAS患儿,采取单纯肺动脉移植、早期拔除气管插管无创CPAP辅助过渡以及加强肺部护理的治疗策略可获得满意的预后,即不行气管成形术可行。气管狭窄指数可作为判断此类患儿是否可行单纯肺动脉移植重要参考指标。
[Abstract]:Background: Pulmonayartrysling (PAS) is a rare congenital cardiovascular malformation. Anatomical features are abnormal left pulmonary artery compression resulting in tracheal stenosis. Trachea intervention refers to various forms of tracheal stenosis correction, including surgical repair or internal stent placement. At present, PAS combined with tracheal stenosis There is still a lot of controversy whether or not the children are undergoing left pulmonary artery transplantation at the same time. Early some scholars believe that left pulmonary artery correction and tracheoplasty should be performed simultaneously. The tracheal ring caused by left pulmonary artery compression may lead to long segment tracheal stenosis, and the tracheal stenosis resulting from the complete tracheal ring will lead to the tracheal stenosis. Trachea stenosis in the long term, which seriously affects the prognosis of children with PAS, requires trachea intervention. Due to the difficulty of trachea operation, many complications and high mortality. Some scholars have begun to try and advocate simple left pulmonary artery transplantation to avoid tracheal complications and improve the prognosis of the children because of the abnormal origin of left pulmonary artery. Transposition or replanting can relieve or relieve the pressure of the trachea and obviously improve the respiratory symptoms of the children. But at present, which children can avoid the trachea intervention and have no prediction index. Objective to analyze the prognosis of the PAS combined with tracheal stenosis correction and not to intervene the trachea, to find the prediction index to avoid the air tube intervention before the operation, and to discuss the improvement of the treatment of the disease. Methods. Methods a descriptive epidemiological method was used to study 17 children with tracheal stenosis in Beijing August 1 children's Hospital, Beijing, November, October 2011. All children before the operation were examined by fiberoptic bronchoscopy for complete tracheal ring. Clinical results and severity of tracheal stenosis (endotracheal diameter and length). Included in the assessment of.PAS combined with tracheal stenosis or other cardiac malformation using color Doppler ultrasound and computerized tomography for the diagnosis of.CT results and the stenosis of endotracheal diameter and length of the trachea in a heart center by the same physician. All the clinical data included in the children, including sex, age, clinical manifestation, CT shadow, were collected. The results of examination, fiberoptic bronchoscopy, color Doppler echocardiography, operation mode, drug use, postoperative treatment strategy and prognosis data. Results 17 cases were collected. All 17 cases of the first operation were only performed pulmonary artery transplantation without tracheal intervention, the operation was completed successfully. Early extubation and ventilator assisted operation were used after the operation. Among them, 12 cases were successfully evacuated from the ventilator early and recovered from the hospital; the other 5 cases were failed because of multiple withdrawal. Trachea intervention was performed in the perioperative period. Among the 5 cases of death, 3 cases were treated with tracheal stenting (1 died of necrotizing enterocolitis, 2 died of infection and multiple organ failure), and 2 cases died of tracheal plasty (both died of sensation. The respiratory tract symptoms of all surviving children were relieved and even disappeared after discharge. Tracheal stenosis index [(tracheal stenosis diameter / tracheal stenosis length) of the 2 groups of children with X100%] without tracheal intervention were statistically significant, trachea pregroup (10.14 + 1.58)%, and trachea intervention group (5.72 + 1.17)% (P0.001). In children with PAS with a higher tracheal stenosis index (10.14 + 1.58)%, simple pulmonary artery transplantation can obtain satisfactory prognosis. For children with tracheal stenosis index less than (5.72 + 1.17)% of PAS, primary pulmonary artery transplantation has poor prognosis. Conclusion for the majority of children with moderate or above tracheal stenosis, simple pulmonary artery is adopted. Transplantation, the early extraction of non invasive CPAP assisted endotracheal intubation and strengthening the treatment of lung care can obtain a satisfactory prognosis, that is, no tracheoplasty is feasible. Tracheal stenosis index can be used as an important reference index for judging whether this kind of children is feasible for simple pulmonary artery transplantation.
【学位授予单位】：南方医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R726.2

【参考文献】