先天性血管环12例诊断与治疗分析

摘要

Objective To study the clinical features, diagnosis and treatment of congenital vascular ring. Methods The clinical data(age, gender, age of onset, clinical manliestations, laboratory examinations ), imaging examinations (X-ray, echocardiography and multi-detector CT scan ), pulmonary function, bronchoscopy, diagnostic methods, treatment, surgery according to the pathologic types and follow-up of children identified as congenital vascular rings at Beijing Anzhen Hospital from July 2008 to December 2011 were retrospectively reviewed. Results Twelve cases diagnosed as congenital vascular rings were included into the study, including 3 males and 9 females, aged from 1 month to 21 years ( 7 cases less than 1 year of age ). Two cases were correctly diagnosed at the first hospital visit and 9 cases were misdiagnosed. Eleven cases had a history of recurrent cough and wheezing, 2 cases had difficulty in feeding, 3 cases had cyanosis, 10 cases had heart murmurs and 9 cases had growth retardation. The results of echocardiography were double aorta arch in 4 cases, right aortic arch with left ductus arteriosus in 1 case, pulmonary artery sling in 7 cases. Ten cases were combined with other heart malformations. Ten cases were given multi-detector CT airway reconstruction and showed tracheobronchial stenosis in 9 cases ( 5 cases with trachea, 3 cases with right principle bronchus, 1 case with left principle bronchus ), esophagus stenosis in 2 cases. Two cases were given bronchoscopy to confirm the stenosis of trachea in 1 case, right principle bronchus in 1 case. Seven patients underwent surgery according to the pathologic types including 2 cases of cutting off the small arch, 1 case of cutting off the left patent ductus arteriosus and 4 cases of reimplanting of the left pulmonary artery. One of them died. The respiratory symptoms and growth in 6 cases gradually were improved in 3 to 6 months after operation. Conclusions The children with recurrent respiratory symptoms such as chronic cough and wheeze should be examined for the possible presence of congenital vascular rings. Echocardiography as a noninvasive technique combined with multi-detector CT can clearly show the anatomy of vascular rings. Early surgical management to remove the tracheal stenosis is vital for survival.%目的 探讨先天性血管环的临床特征、诊断与治疗.方法 回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院诊断为先天性血管环患儿的临床特征、影像学检查、诊断和治疗.结果 12例先天性血管环患儿进入分析,男3例,女9例.就诊年龄1个月至21岁,<1岁7例.反复咳喘11例,喂养困难2例,青紫3例,心脏杂音10例.12例超声心动图示主动脉双弓4例、右位主动脉弓伴左位动脉导管未闭1例、肺动脉吊带7例,其中10例伴其他心脏畸形.10例64排CT气道重建示主气管狭窄5例、左主支气管狭窄1例,右侧气管狭窄3例,食管狭窄2例;2例纤维支气管镜示主气管狭窄1例,右主支气管狭窄1例.7例行手术治疗,根据血管环的病理类型选择术式,包括2例切断双主动脉弓中的次弓、1例切断右位主动脉弓伴随的左位动脉导管、4例左肺动脉移植术.1例术后死亡,6例术后3～6个月呼吸道症状逐渐好转,生长发育改善.结论 儿童中反复出现原因不明的咳喘等呼吸道症状者,应考虑到先天性血管环的可能,超声心动图联合多排CT可明确诊断,及早手术解除气管狭窄是改善预后的关键.