婴幼儿血管环外科诊治

摘要

目的 总结婴幼儿先天性血管环外科诊治的临床经验.方法 2009年9月至2010年12月诊断并手术治疗14例血管环患儿,其中男9例,女5例；年龄2月～6岁；体重4.5～15.0 kg.所有病例术前均行X线胸片、超声心动图及增强多排螺旋CT检查确诊,行食管造影及纤维支气管镜检查各5例.病理类型包括双主动脉弓1例、肺动脉吊带7例、右位主动脉弓伴左位动脉导管/韧带6例,7例患儿合并心内畸形.全组患儿存在不同范围(12％～62％)和程度(45％～74％)的气管狭窄,5例术前存在局限性食管狭窄.12例患儿在体外循环下手术同期矫治心内畸形,2例在非体外循环下手术.结果 术后辅助呼吸7～ 308 h,中位数21 h；ICU停留16 ～314 h,中位数79.5 h.1例肺动脉吊带患儿住院死亡(7.1％).13例出院.失访1例,12例随访1～ 15个月,患儿消化道症状消失,活动耐力改善.呼吸道症状不同程度缓解,5例(41.7％,5/12例)仍然存在呼吸道症状.结论 临床上对有长期呼吸道和(或)消化道症状的患儿需考虑血管环的可能,增强CT是确定血管环诊断的最佳方法.血管环应尽早手术治疗,合并的长段气管狭窄需手术治疗,近-中期效果良好.%Objective To explore the diagnosis and surgical treatment experience of vascular ring in infants and children.Methods Fourteen cases (9 boys and 5 girls,aged 2 months to 6 years,weighted 4.5 - 15.0 kg) with vascular ring were diagnosed and treated surgically in Children's Hospital of Chongqing Medical University from Sep.2009 to Dec.2010.All children underwent X-ray,echocardiography and spiral computed tomography examination preoperatively.Bronchoscopy and barium swallow was performed in 5 cases respectively.The pathological types of vascular rings included double aortic arch in 1 ( 7.1％ ),pulmonary artery sling in 7 (50.0％),right aortic arch with left patent ductus arteriosus or persistent left ligament in 6 (42.9％).Associated cardiac anomalies were present in 7 (50.0％) patients.Tracheal stenosis of different length ( 12％ -62％ ) and severity (45％ -74％ cross-sectional luminal narrowing) was observed in the group.Barium swallow in 5cases showed localized compression of the esophagus.12 cases underwent repair of vascular ring with cardiopulmonary bypass (CPB),and the associated congenital heart defects were repaired simultaneously.2 cases of right aortic arch with left patent ductus arteriosus or persistent left ligament underwent surgery without CPB.Results The median duration of CPB in 12 cases and aortic cross-clamp time in 7 patients were 77.5 minutes ( range:55 - 186 minutes) and 36 minutes ( range:22 - 110 minutes) respectively.The median duration of postoperative ventilation and ICU stay were 21 hours (range:7 -308 hours) and 79.5 hours (range:16-314 hours) respectively.One baby with pulmonary artery sling died on the postoperative 12th day ( in-hospital mortality 7.1％ ).Of the 13 cases discharged from the hospital,1 case were lost to follow up.In the follow-up ( 1- 15 months) of 12 cases,digestive symptoms were disappeared.Development,exercise tolerance and symptoms showed obvious improvement,although 5 (41.7％) patients had residual respiratory problems.Conclusion Prolonged or recurrent aerodigestive issues in children should alert the pediatrician to the possibility of a vascular ring.Multislice spiral CT scanning is the best imaging modality.All vascular rings should be surgically corrected,and the associated long-segment severe tracheal stenosis needs.The short to midterm outcomes of surgical division are excellent.