先天性血管环的外科诊断与治疗(1)
[摘要] 目的 总结先天性血管环患儿的临床资料和外科手术治疗经验。 方法 回顾性分析2011年3月～2015年10月在上海市儿童医院接受手术治疗的35例先天性血管环患儿临床资料，34例合并不同程度气管狭窄，临床表现为反复肺炎、慢性咳嗽、喘鸣、呼吸窘迫、吞咽困难和反复呕吐。22例肺动脉吊带、6例双主动脉弓和1例右位主动脉弓伴左侧动脉导管患儿采用在非体外循环下手术治疗，6例肺动脉吊带患儿采用在体外循环下手术治疗。所有患儿气管狭窄术中均未处理。术后观察呼吸道症状、消化道症状、生长发育情况和气管狭窄的变化。 结果 35例患儿均顺利完成手术，术后呼吸机辅助时间为(47.80±133.19)h，监护室时间为(116.42±190.02)h，住院时间为(19.22±13.07)d；6例患儿体外循环时间为(125.16±29.99)min；3例患儿主动脉阻断时间为(70.00±13.00)min。1例患儿术后并发气管内肉芽生成造成气道狭窄加重，脱离呼吸机困难，术后10 d行气管内支架置入手术，术后顺利脱离呼吸机。2例患儿拔出气管插管后呼吸困难，再次气管插管呼吸机辅助，经积极处理后顺利撤离呼吸机。1例患儿术后42 d仍不能脱离呼吸机，最终家长放弃治疗。全组34例患儿顺利出院，1例术后放弃治疗。术后随访1个月～4年，患儿术后呼吸道症状完全消失17例，喉喘鸣症状明显好转14例，反复呕吐消失2例。患儿生长发育改善，气管狭窄随访中。 结论 先天性血管环患儿易出现呼吸道和消化道症状，临床上出现反复肺炎、呼吸困难、喘鸣、吞咽困难和反复呕吐症状的患儿应怀疑血管环的可能，心脏超声和多排螺旋CT血管造影检查可确诊，早发现、早期手术治疗效果良好，合并心内畸形和局限性的气管狭窄并不需要同时纠治。
[关键词] 先天性；血管环；双主动脉弓；肺动脉吊带
[中图分类号] R726.2 [文献标识码] A [文章编号] 1673-7210(2016)02(b)-0144-04
Surgical diagnosis and treatment for congenital vascular ring
LI Xiaobing WANG Beini SHEN Li XIE Yewei ZHANG Rufang▲
Department of Cardiothoracic Surgery， Children's Hospital of Shanghai Children's Hospital Affiliated to Shanghai Jiaotong University， Shanghai 200062， China
[Abstract] Objective To summarize the clinical experience of surgical treatment and the clinical information of children diagnosed of congenital vascular ring. Methods The clinical information of 35 patients diagnosed of congenital vascular ring treated in Children's Hospital of Shanghai from March 2011 to October 2015 were analyzed retrospectively， among whom 34 patients suffered from different degree of tracheostenosis， clinical manifestation were repeated pneumonia， chronic cough， asthma wheezing， respiratory distress， dysphagia and repeated vomiting. 22 cases of pulmonary artery sling， 6 cases of double aortic archard and 1 case of right aortic arch with left artery catheter accepted vascular repairation without extracorporeal circulation. 6 patients of pulmonary artery sling completed vascular repairation undergoing extracorporeal circulation. The tracheostenosis weren't dealt with in the operation. Respiratory symptoms， enteron symptoms， physical development and the change of tracheostenosis were observed postoperatively. Results All 35 patients received the surgery successfully. The mean mechanical ventilation time was (47.80±133.19) h， the mean ICU observing time was (116.42±190.02) h， the mean length of stay was (19.22±13.07) d. The mean time of 6 cases of extracorporeal circulation was (125.16±29.99) min. The mean time of 3 cases of aortic clamp was (70.00±13.00) min. 1 patient suffered from endotrachealgranulation tissue formation which aggravated the tracheostenosis. The patient accepted endotracheal metallic stents placement 10 d after surgery and weaned from ventilator after the placement. 2 patients suffered from dyspnea when weaned from ventilator， they accepted intubation immediately and were able to the remove the trachea cannula successfully. 1 patient wasn't able to be weaned from ventilator 42 d after surgery and the parents gave up treatment. 34 patients were discharged successfully and 1 case gave up treatment after operation. Postoperative follow-up lasted for 1 month to 4 years. In 17 cases， respiratory symptoms subsided completely. In 14 cases， asthma wheezing subsided. In 2 cases， recurrent vomitting subsided. Pediatric physical were improved and the tracheostenosis was under follow-up visit. Conclusion Patients suffered from congenital vascular ring are easily to have respiratory and enteron symptoms. Patients with manifestation of recurrent pneumonia， dyspnea， asthma wheezing， dysphagia and recurrent vomiting should be considered to be congenital vascular ring. Echocardiography and MSCTA can confirm the diagnosis. The outcome of surgery can be good if diagnosed early. Congenital vascular ring associated with intracardiac anomalies and focal limited tracheostenosis do not need to be treated simultaneously., 百拇医药(李小兵 王蓓妮 沈立 等)
[关键词] 先天性；血管环；双主动脉弓；肺动脉吊带
[中图分类号] R726.2 [文献标识码] A [文章编号] 1673-7210(2016)02(b)-0144-04
Surgical diagnosis and treatment for congenital vascular ring
LI Xiaobing WANG Beini SHEN Li XIE Yewei ZHANG Rufang▲
Department of Cardiothoracic Surgery， Children's Hospital of Shanghai Children's Hospital Affiliated to Shanghai Jiaotong University， Shanghai 200062， China
[Abstract] Objective To summarize the clinical experience of surgical treatment and the clinical information of children diagnosed of congenital vascular ring. Methods The clinical information of 35 patients diagnosed of congenital vascular ring treated in Children's Hospital of Shanghai from March 2011 to October 2015 were analyzed retrospectively， among whom 34 patients suffered from different degree of tracheostenosis， clinical manifestation were repeated pneumonia， chronic cough， asthma wheezing， respiratory distress， dysphagia and repeated vomiting. 22 cases of pulmonary artery sling， 6 cases of double aortic archard and 1 case of right aortic arch with left artery catheter accepted vascular repairation without extracorporeal circulation. 6 patients of pulmonary artery sling completed vascular repairation undergoing extracorporeal circulation. The tracheostenosis weren't dealt with in the operation. Respiratory symptoms， enteron symptoms， physical development and the change of tracheostenosis were observed postoperatively. Results All 35 patients received the surgery successfully. The mean mechanical ventilation time was (47.80±133.19) h， the mean ICU observing time was (116.42±190.02) h， the mean length of stay was (19.22±13.07) d. The mean time of 6 cases of extracorporeal circulation was (125.16±29.99) min. The mean time of 3 cases of aortic clamp was (70.00±13.00) min. 1 patient suffered from endotrachealgranulation tissue formation which aggravated the tracheostenosis. The patient accepted endotracheal metallic stents placement 10 d after surgery and weaned from ventilator after the placement. 2 patients suffered from dyspnea when weaned from ventilator， they accepted intubation immediately and were able to the remove the trachea cannula successfully. 1 patient wasn't able to be weaned from ventilator 42 d after surgery and the parents gave up treatment. 34 patients were discharged successfully and 1 case gave up treatment after operation. Postoperative follow-up lasted for 1 month to 4 years. In 17 cases， respiratory symptoms subsided completely. In 14 cases， asthma wheezing subsided. In 2 cases， recurrent vomitting subsided. Pediatric physical were improved and the tracheostenosis was under follow-up visit. Conclusion Patients suffered from congenital vascular ring are easily to have respiratory and enteron symptoms. Patients with manifestation of recurrent pneumonia， dyspnea， asthma wheezing， dysphagia and recurrent vomiting should be considered to be congenital vascular ring. Echocardiography and MSCTA can confirm the diagnosis. The outcome of surgery can be good if diagnosed early. Congenital vascular ring associated with intracardiac anomalies and focal limited tracheostenosis do not need to be treated simultaneously., 百拇医药(李小兵 王蓓妮 沈立 等)