François Katrien, Panzer Joseph, De Groote Katya, Vandekerckhove Kristof, De Wolf Daniel, De Wilde Hans, Marchau Fabienne, De Caluwe Wim, Benatar Abraham, Bové Thierry
Department of Congenital Cardiac Surgery, Cardiac Center, University Hospital Ghent, Ghent, Belgium.
Department Pediatric Cardiology, Cardiac Center, University Hospital Ghent, Ghent, Belgium.
Eur J Pediatr. 2017 Mar;176(3):371-377. doi: 10.1007/s00431-017-2850-y. Epub 2017 Jan 13.
Persistent respiratory or feeding problems in children may be associated with a congenital vascular ring. Surgical management is fairly standardized, but long-term outcomes are not well described. This study aims to investigate clinical presentation, surgical treatment, and risk factors for early mortality and late outcome. Our database revealed 62 surgically treated vascular ring patients between 1993 and 2014. Double aortic arch was the most common diagnosis (53%). Median age at operation was 1 year. Symptoms were mainly respiratory (89%) and feeding problems (32%). Median extubation time and hospital stay were 4 h and 5 days. Mean follow-up was 7.8 ± 5.8 years. Early mortality was 8% and was related to anatomical diagnosis, concomitant anomalies, and a need for preoperative intubation. Freedom from residual symptoms at 1 and 6 months was 63 and 82%, respectively. Freedom from inhalation therapy at the last follow-up was 82% and was influenced by a type of vascular ring and preoperative ventilation. Dysphagia symptoms always disappeared.
Surgical relief of tracheoesophageal compression is commonly effective in vascular ring anomalies. Respiratory symptoms necessitating chronic inhalation therapy only persist in a minority of children. Patients with double aortic arch are at increased risk to remain symptomatic, particularly with infectious exacerbations.
儿童持续存在的呼吸或喂养问题可能与先天性血管环有关。手术治疗相当标准化,但长期预后情况描述得并不充分。本研究旨在调查临床表现、手术治疗以及早期死亡和远期预后的危险因素。我们的数据库显示,1993年至2014年间有62例接受手术治疗的血管环患者。双主动脉弓是最常见的诊断(53%)。手术时的中位年龄为1岁。症状主要为呼吸问题(89%)和喂养问题(32%)。拔管时间和住院时间的中位数分别为4小时和5天。平均随访时间为7.8±5.8年。早期死亡率为8%,与解剖诊断、合并畸形以及术前需要插管有关。1个月和6个月时无残留症状的比例分别为63%和82%。最后一次随访时无需吸入治疗的比例为82%,受血管环类型和术前通气的影响。吞咽困难症状总是会消失。
手术解除气管食管压迫在血管环畸形中通常是有效的。仅少数儿童会持续存在需要长期吸入治疗的呼吸症状。双主动脉弓患者仍有症状的风险增加,尤其是在感染加重时。