Hagl S, Jakob H, Sebening C, van Bodegom P, Schmidt K, Zilow E, Fleischer F, Ulmer H
Department of Cardiac Surgery, University Hospital, Heidelberg, Germany.
Ann Thorac Surg. 1997 Nov;64(5):1412-20; discussion 1421. doi: 10.1016/S0003-4975(97)00994-6.
Symptomatic obstruction of long-segment tracheal or bronchial portions either related to congenital instability or secondary to vascular compression are rare malformations, which remain difficult to manage. A method of external tracheal or bronchial stabilization is described.
From July 1992 to April 1995, 7 children (age range, 4 months to 4 years; mean age, 19 months) and 1 adult (age, 46 years) were operated on for severe respiratory insufficiency. In 4 cases of congenital tracheal instability, 2 children had associated type IIIb esophageal atresia. Both children with esophageal atresia had previous operations (two and three times, respectively): 1 child had aortopexy and division of a patent ductus arteriosus and another child had distal tracheal resection elsewhere, both without relief of malacia. All children were intubated and ventilated since birth for 11 to 15 months. Secondary tracheobronchomalacia due to vascular compression was seen in 4 patients caused by double aortic arch (n = 2) and persisting ligamentum arteriosum after previous ligation of a patent ductus arteriosus (n = 2), with 1 child ventilated thereafter for 5 months. Operation was performed with the aid of extracorporeal circulation in all patients but 1, and consisted of transection of vascular rings and persistent ligamentum Botalli (n = 5), closure of multiple ventricular septal defects (n = 1) and extensive mobilization of the tracheobronchial tree as well as the great arteries. External stabilization of the severely dysplastic distal trachea (n = 6) or left main bronchus (n = 2) was achieved by suspending the malacic segment within an oversized and longitudinally opened ring-reinforced polytetrafluoroethylene prosthesis. Multiple plegeted sutures were placed extramucosally to the dysplastic tracheal wall and the dyskinetic pars membranacea, as well as to the polytetrafluoroethylene prosthesis in a radial orientation. Guided by simultaneous video-assisted bronchoscopy, reexpansion of the collapsed segments was achieved by gentle traction on the sutures while tying.
Stenosis-free tracheobronchial reexpansion was achieved in all patients, as seen on repeated bronchoscopies during hospitalization and thereafter. All patients were extubated within 1 to 12 days after the operation. There was one late death, unrelated to the procedure, in a 31-month-old child 20 months after the operation. All other patients are free of stridor and in excellent clinical condition 21 to 54 months (mean, 38 months) thereafter.
The presented method of bronchoscopically guided external tracheobronchial suspension within a ring-reinforced polytetrafluoroethylene prosthesis immediately relieves severe malacia of the trachea or main bronchi in infants as well as adults without necessitating resection. Midterm preliminary data suggest that growth potential of the affected segment exists within the oversized polytetrafluoroethylene prosthesis.
与先天性气管支气管软化或血管压迫继发的长段气管或支气管部分的症状性梗阻是罕见的畸形,治疗仍然困难。本文描述了一种气管或支气管外固定方法。
1992年7月至1995年4月,7例儿童(年龄范围4个月至4岁;平均年龄19个月)和1例成人(46岁)因严重呼吸功能不全接受手术。4例先天性气管软化患者中,2例儿童合并Ⅲb型食管闭锁。这2例食管闭锁患儿均曾接受过手术(分别为2次和3次):1例患儿接受了主动脉固定术和动脉导管未闭结扎术,另1例患儿在其他地方接受了远端气管切除术,但均未缓解软化症状。所有患儿自出生后即行气管插管和机械通气,时间为11至15个月。4例患者因双主动脉弓(n = 2)和动脉导管未闭结扎术后持续存在动脉韧带(n = 2)导致血管压迫继发气管支气管软化,其中1例患儿术后机械通气5个月。除1例患者外,所有患者均在体外循环辅助下进行手术,手术包括切断血管环和动脉韧带(n = 5)、闭合多个室间隔缺损(n = 1)以及广泛游离气管支气管树和大动脉。通过将软化段悬吊于尺寸过大且纵向开口的环形增强聚四氟乙烯假体中来实现严重发育不良的远端气管(n = 6)或左主支气管(n = 2)的外固定。在发育不良的气管壁、运动障碍的膜部以及聚四氟乙烯假体上以放射状方向放置多根带垫片缝线。在同步视频辅助支气管镜引导下,在打结时通过轻柔牵拉缝线使塌陷段重新扩张。
住院期间及之后的多次支气管镜检查显示,所有患者均实现了无狭窄的气管支气管扩张。所有患者术后1至12天内拔除气管插管。1例31个月大的患儿在术后20个月出现1例与手术无关的晚期死亡。此后,所有其他患者均无喘鸣,临床状况良好,随访时间为21至54个月(平均38个月)。
本文介绍的在环形增强聚四氟乙烯假体中进行支气管镜引导下气管支气管外悬吊的方法可立即缓解婴儿及成人气管或主支气管的严重软化,无需进行切除。中期初步数据表明,在尺寸过大的聚四氟乙烯假体中,受累节段具有生长潜力。