121例小儿血管环的外科治疗结果分析

Outcomes of surgical treatment for 121 children with vascular rings

目的探讨完全性血管环和部分性血管环的诊断、外科治疗方法及治疗结果。方法收集2010年1月至2018年6月在广州市妇女儿童医疗中心经手术治疗的小儿完全性血管环或部分性血管环121例患儿的相关资料。其中,男81例,女40例;年龄范围为16 d至8岁。术前症状有咳嗽、喘鸣、反复呼吸系统感染、呼吸困难、吞咽困难,12例术前行机械通气。术前均行胸部X线片、心电图等常规检查,9例行食管吞钡造影,4例行气管和支气管造影,34例在术前或术中行纤维支气管镜检查,所有患儿均行心脏彩色多普勒超声和胸部CT检查。收集患儿的临床表现、术前胸部X线片、心脏彩色多普勒超声、心脏CT、纤维支气管镜、食管造影等检查结果;收集麻醉、手术处理方法,手术结果(手术病死率和手术并发症发生率)。对存活病例随访,调查存活状况和手术并发症情况,并将术后生存状况输入SPSS 16.0统计软件,用Kaplan-meier法做出术后生存曲线,评估近、中期生存状况。结果完全性血管环35例,部分性血管环86例;完全性血管环患儿中的血管环种类包括双主动脉弓22例,右主动脉弓右降主动脉合并左侧动脉导管未闭/动脉导管韧带13例;部分性血管环患儿中的血管环种类包括肺动脉吊带83例,左主动脉弓合并迷走右锁骨下动脉3例。22例双主动脉弓患儿中,左主动脉弓优势3例,右主动脉弓优势17例,双主动脉弓平衡2例;合并气管狭窄2例,手术行弱势主动脉弓切断缝合,1例气管狭窄呼吸困难症状明显,行狭窄气管切除并气管端-端吻合术;20例手术后当天拔除气管插管,1例术中大出血患儿于术后第二天拔除气管插管,另1例气管狭窄切除气管端-端吻合患儿于术后第三天拔除气管插管。13例右主动脉弓右降主动脉合并左侧动脉导管未闭/动脉导管韧带患儿中,迷走左锁骨下动脉11例,镜像分支模式2例;8例有Kommerell憩室,其中6例Kommerell憩室直径为左锁骨下动脉直径1.5倍及以上的行Kommerell憩室切除并左锁骨下动脉移植于左侧颈总动脉;该13例患儿于术后当天拔除气管插管。83例肺动脉吊带患儿中,合并气管狭窄24例,合并室间隔缺损(ventricular septal defect,VSD)4例,合并房间隔缺损(atrial septal defect,ASD)10例,合并完全性房室隔缺损(complete atrioventricular septal defect,CAVSD)1例,该83例均行左肺动脉移植于主肺动脉,气管狭窄症状明显的13例行气管成形术,手术后机械通气时间为(70.08±31.04)h,范围为40~130 h,其余70例患儿于手术当天拔除气管插管;合并的心内畸形行同期矫治,左主动脉弓合并迷走右锁骨下动脉3例,分别合并主动脉弓离断(interruption of aortic arch,IAA)1例,VSD 1例,法洛四联症(tetralogy of fallot,TOF)1例;该3例均行迷走右锁骨下动脉移植于右侧颈总动脉,于术后当天拔除气管插管。手术死亡2例,1例因术后并发纵隔感染,另1例因气管吻合口漏家长放弃治疗。手术并发症包括大出血1例、喉返神经损伤2例、左肺动脉狭窄4例。所有患儿术后食管受压症状均消失,除2例有轻度呼吸道狭窄症状外,余患儿的呼吸道症状均消失。随访时间范围为3个月至6.5年,死亡1例为气管狭窄切除端-端吻合术后吻合口反复发生肉芽肿。完全性血管环患儿术后1、3、5年生存率均为97%;部分性血管环患儿1、3、5年生存率均为98%。术后并发症包括4例气管成形术后仍然间断存在气管狭窄的症状,但症状较术前明显减轻,6例PA-Sling术后存在左肺动脉轻-中度狭窄。结论小儿血管环外科手术治疗的近-中期效果良好;气管狭窄是血管环常见合并畸形,也是影响治疗效果的重要原因。

Objective To explore the diagnosis and surgical treatment of complete vascular ring or semi-vascular ring in children and analyze the surgical outcomes.Methods From January 2010 to June 2018, retrospective analysis was performed for medical records for 121 children with complete vascular ring or semi-vascular ring undergoing operations. Their clinical symptoms, preoperative examinations of chest radiograph, echocardiography, cardiac computed tomography (CT), fiberoptic bronchoscopy and esophagography, anesthetic modes, surgical approaches, postoperative mortality & morbility and complications were recorded during the follow-ups of surviving cases and postoperative survival status. Raw data were analyzed with SPSS16.0. Kaplan-Meier method was utilized for plotting the postoperative survival curve and immediate/middle-term survival status evaluated.Results Vascular ring was complete (n=35) and partial (n=86). For complete vascular ring, there were double aortic arch (n=22) and right arch & right descending aorta & left ductus arteriosus/ductus arteriosus ligament (n=13). For semi-vascular ring, there were pulmonary artery sling (n=83) and left aortic arch & aberrant right subclavian artery (n=3). For double aortic arch, weak arch was resected and sutured. Two cases were complicated with tracheal stenosis and 1 symptomatic child underwent tracheal stenosis resection and end-to-end anastomosis. For right arch & right descending aorta & left ductus arteriosus/ductus arteriosus ligament, there was Kommerell diverticulum (n=8). There were aberrant left subclavian (n=11) and mirror image branching (n=2). Ductus arteriosus/ductus arteriosus ligament was resected. Kommerell diverticulum was resected and left subclavian artery transplanted into left common carotid artery (n=6) owing to the diameter of Kommerell diverticulum were 1.5 times larger than that of left subclavian artery. For 83 cases of pulmonary artery sling, the complications included tracheal stenosis (n=24), ventricular septal defect (VSD, n=4), atrial septal defect (ASD, n=10) and complete atrioventricular septal defect (CAVSD, n=1). Left pulmonary artery transplanted into major pulmonary artery and 13 symptomatic children of tracheal stenosis underwent sliding tracheoplasty. The complications of cardiac malformations were simultaneously corrected. For 3 children of left aortic arch and aberrant right subclavian artery, there were aortic arch dissection (IAA, n=1), VSD (n=1) and tetralogy of Fallot (TOF, n=1). Aberrant right subclavian artery was transplanted into right common carotid artery and the complications of cardiac malformations were simultaneously corrected. Two died during tracheoplasty. One case was complicated with postoperative mediastinal infection and another child was abandoned by parents due to tracheal anastomotic fistula. Surgical complications included massive hemorrhage (double-aortic arch correction & median thoracotomy, n=1), laryngeal nerve injury (complete vascular ring surgery, n=2) and left pulmonary artery stenosis (PA-Sling surgery, n=4). For double aortic arch, extubation was completed via day operation (n=20), with intraoperative hemorrhoea & next day (n=1) and Day 3 postoperatively after tracheal stenosis resection & end-to-end anastomosis (n=1). Thirteen children of right arch and right descending aorta and left ductus arteriosus/ductus arteriosus ligament were extubated in day operation. And 13/83 children of pulmonary sling underwent sliding tracheoplasty due to severe tracheal stenosis. The average mechanical ventilation time was (70.08±31.04)(40-130) hours. The other 70 were extubated in day operation. Three cases of aberrant right subclavian artery complicated with cardiac malformations were extubated in day operation. The sympoms of esophageal compression were all eliminated. Two children had mild sympotoms of airway stenosis. The follow-up period was from 3 to 78 months. One child died from recurrent granuloma at anastomotic site after end-to-end anastomosis during resection of tracheal stenosis (double aortic arch). The 1/3/5-year survival rate of complete vascular ring was 97%, 97% and 97% and 1/3/5-year survival rate of semi-vascular ring 98%, 98% and 98% respectively. Postoperative complications included intermittent tracheal stenosis after tracheoplasty (n=4). However, severity decreased significantly after surgery and mild-to-moderate left pulmonary artery stenosis persisted after PA-Sling surgery (n=6).Conclusions The immediate/middle-term postoperative outcomes are excellent for children with complete vascular ring or semi-vascular ring. Vascular ring is frequently complicated with tracheal stenosis and it may impair the surgical outcomes of vascular ring.