Weber Angelika, Donner Birgit, Perez Marie-Hélène, Di Bernardo Stefano, Trachsel Daniel, Sandu Kishore, Sekarski Nicole
Pediatric Cardiology Unit, Department of Pediatrics and Pediatric Surgery, University Hospital Lausanne, Lausanne, Switzerland.
Division of Pediatric Cardiology, University Children's Hospital Basel, Basel, Switzerland.
Front Pediatr. 2017 Apr 10;5:67. doi: 10.3389/fped.2017.00067. eCollection 2017.
Pulmonary artery sling (PAS) is a rare congenital condition in which the left pulmonary artery (LPA) arises from the right pulmonary artery, and then passes between the trachea and the esophagus to reach the left lung, thereby forming a sling around the airway. It is often associated with intrinsic tracheal stenosis due to complete cartilaginous rings. Therapeutic management nowadays consists of one-stage reimplantation of the LPA and tracheoplasty with cardiopulmonary bypass support. Here, we present a 7-week-old boy with PAS and long-segment tracheal stenosis (LSTS) who underwent surgical intervention consisting of reimplantation of the LPA and slide tracheoplasty. Multiple respiratory and cardiovascular complications marked the postoperative course. They consisted of recurrent failed attempts in weaning off mechanical ventilation due to bronchomalacia, left vocal cord paralysis, development of granulation tissue at the anastomosis and restenosis of the trachea, and the main stem bronchi requiring balloon dilatation. The patient also developed bilateral pulmonary artery thrombosis and stenosis of the LPA. After a prolonged hospitalization, the patient is doing well without any respiratory symptoms and has a good result on follow-up bronchoscopy 1 year after the initial surgery. The stenosis of the LPA responded well to percutaneous balloon dilatation 12 months after the primary surgery. The case illustrates that even though surgical techniques are improving and are in general associated with a low morbidity and mortality, management of PAS and tracheal stenosis can still be challenging. However, good long-term outcome can be achieved if the initial postoperative phase is overcome.
肺动脉吊带（PAS）是一种罕见的先天性疾病，其中左肺动脉（LPA）起源于右肺动脉，然后在气管和食管之间穿过到达左肺，从而在气道周围形成一个吊带。它常与由于完整软骨环导致的先天性气管狭窄相关。如今的治疗方法包括在体外循环支持下一期进行LPA再植和气管成形术。在此，我们报告一名7周大患有PAS和长段气管狭窄（LSTS）的男童，他接受了包括LPA再植和滑动气管成形术的手术干预。术后过程出现了多种呼吸和心血管并发症。这些并发症包括由于支气管软化多次尝试脱机失败、左声带麻痹、吻合口肉芽组织形成和气管再狭窄，以及主支气管需要球囊扩张。患者还出现了双侧肺动脉血栓形成和LPA狭窄。经过长时间住院，患者恢复良好，无任何呼吸症状，在初次手术后1年的随访支气管镜检查结果良好。初次手术后12个月，LPA狭窄对经皮球囊扩张反应良好。该病例表明，尽管手术技术在不断改进且总体上发病率和死亡率较低，但PAS和气管狭窄的治疗仍然具有挑战性。然而，如果度过术后初期，可取得良好的长期效果。
