本文選題：肺動脈吊帶 + 氣管狹窄��； 參考：《南方醫(yī)科大學(xué)》2017年碩士論文

【摘要】：背景:肺動脈吊帶(Pulmonayartrysling,PAS)是一種罕見的先天性心血管畸形。解剖特征為異常走形的左肺動脈壓迫造成氣管狹窄。氣管干預(yù),指各種形式的氣管狹窄矯治手術(shù),包括外科修補成形或內(nèi)科支架置入術(shù)。目前對于PAS合并氣管狹窄的患兒是否在行左肺動脈移植術(shù)的同時進行氣管干預(yù)依然存在很大爭議。早期一部分學(xué)者認為應(yīng)同時行左肺動脈矯治和氣管成形術(shù),因左肺動脈壓迫所致的氣管軟骨環(huán)可能導(dǎo)致長段型氣管狹窄;此外,完全性氣管環(huán)導(dǎo)致的氣管狹窄將導(dǎo)致術(shù)后氣管狹窄長期存在,嚴重影響PAS患兒的預(yù)后,需要進行氣管干預(yù)。由于氣管手術(shù)難度大,并發(fā)癥多,死亡率高。一部分學(xué)者開始嘗試、并主張單純行左肺動脈移植術(shù),從而避免氣管并發(fā)癥,提高患兒預(yù)后,因為對異常起源的左肺動脈進行移位或再植可減輕或解除對氣管的壓迫,明顯改善患兒的呼吸道癥狀。但目前哪些患兒能避免氣管干預(yù),無預(yù)測指標。目的分析PAS合并氣管狹窄畸形矯治不干預(yù)氣管的預(yù)后,尋找術(shù)前判斷避免氣管干預(yù)的預(yù)測指標,探討提高該病治療效果的方法。方法采用描述性流行病學(xué)的方法進行研究。把2011年10月-2015年11月間北京八一兒童醫(yī)院17例PAS合并氣管狹窄患兒被納入研究,術(shù)前所有患兒應(yīng)用纖維支氣管鏡檢查完全氣管環(huán)。臨床結(jié)果和氣管狹窄嚴重程度(氣管內(nèi)徑和長度)被納入評估。PAS合并氣管狹窄或其他心臟畸形應(yīng)用心臟彩色多普勒超聲和計算機化斷層顯像診斷。CT結(jié)果審閱和狹窄氣管內(nèi)徑和氣管長度在某心臟中心由同一位醫(yī)師審閱。收集所有納入患兒的臨床資料,包括性別、年齡、臨床表現(xiàn),CT影像學(xué)檢查、纖維支氣管鏡、彩色多普勒超聲心動圖結(jié)果,手術(shù)方式、藥物利用、術(shù)后治療策略以及預(yù)后資料。結(jié)果共收集到17例病例。全組17例患兒初次手術(shù)均僅行肺動脈移植術(shù)未行氣管干預(yù),手術(shù)均順利完成,術(shù)后均采用早期拔管、呼吸機輔助通氣策略。其中,12例早期順利撤離呼吸機,痊愈出院;另外5例因多次撤機失敗,圍術(shù)期行氣管干預(yù)手術(shù),均死亡。死亡5例中,3例行氣管支架植入術(shù)(1例死于壞死性小腸結(jié)腸炎、2例死于感染及多臟器功能衰竭),2例行氣管成形術(shù)(均死于感染及氣管漏氣)。所有存活患兒出院后隨訪呼吸道癥狀均有所減輕甚至消失。氣管狹窄指數(shù)[(氣管狹窄段直徑/氣管狹窄段長度)X100%]有無氣管干預(yù)的2組患兒間差異有統(tǒng)計學(xué)意義,氣管未干預(yù)組(10.14±1.58)%,氣管干預(yù)組(5.72±1.17)%(P0.001)。對于氣管狹窄指數(shù)高于(10.14±1.58)%的PAS患兒,采取單純肺動脈移植術(shù)可獲得滿意的預(yù)后。對于氣管狹窄指數(shù)低于(5.72±1.17)%的PAS患兒,Ⅰ期單純行肺動脈移植術(shù)預(yù)后不良。結(jié)論對于絕大多數(shù)合并中度以上氣管狹窄的PAS患兒,采取單純肺動脈移植、早期拔除氣管插管無創(chuàng)CPAP輔助過渡以及加強肺部護理的治療策略可獲得滿意的預(yù)后,即不行氣管成形術(shù)可行。氣管狹窄指數(shù)可作為判斷此類患兒是否可行單純肺動脈移植重要參考指標。
[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.
【學(xué)位授予單位】：南方醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R726.2

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