Döhlemann C, Mantel K, Vogl T J, Nicolai T, Schneider K, Hammerer I, Apitz J, Meisner H, Joppich I
Universitätskinderklinik, München, Germany.
Eur J Pediatr. 1995 Jan;154(1):2-14. doi: 10.1007/BF01972965.
Pulmonary sling (PS) is a congenital condition in which the left pulmonary artery (LPA) arises from the right pulmonary artery (RPA), forming a sling around the trachea causing tracheal compression. The incidence is not so rare as initially thought. Symptoms of severe airway obstruction often begin in the newborn or young infant. Echo-colour-Doppler may reveal the PS but emphysema can mask the typical findings. Deviation of fluid-filled lungs may be detected prenatally. Chest radiographs show unusual air distribution, deviation of heart and mediastinum and altered tracheobronchial angles. Bronchography and bronchoscopy demonstrate the high incidence of associated tracheal anomalies such as cartilagenous rings and long tracheal stenosis. Anterior oesophageal indentation is not always seen in the oesophogram. Magnetic resonance imaging (MRI) and computed tomography (CT) reveal the PS, but cautious interpretation is necessary because of different levels of the anomalous LPA. PS and associated cardiovascular malformations can be clearly detected by angiography. Associated extrathoracic anomalies are common. Early diagnosis and therapy of PS is mandatory and consists of reimplantation of the LPA into the pulmonary trunk and division of the ligamentum arteriosum. The postoperative course may be cumbersome necessitating bronchological interventions. Tracheal resection may be necessary but restenosis is frequent. A one-stage repair has been proposed in such cases and was successfully done in a few reported cases. Relief of respiratory obstruction is often complete when there are no associated tracheobronchial anomalies. Late postoperative course is favourable but respiratory obstructive attacks may occur with decreasing incidence over time and tracheal growth.
肺吊带(PS)是一种先天性疾病,其中左肺动脉(LPA)起源于右肺动脉(RPA),形成围绕气管的吊带,导致气管受压。其发病率并不像最初认为的那么罕见。严重气道阻塞的症状通常始于新生儿或幼儿。彩色多普勒超声可能显示肺吊带,但肺气肿可能掩盖典型表现。产前可能检测到充满液体的肺的移位。胸部X线片显示异常的空气分布、心脏和纵隔移位以及气管支气管角度改变。支气管造影和支气管镜检查显示相关气管异常的发生率很高,如软骨环和长段气管狭窄。食管造影中并不总是能看到食管前部受压。磁共振成像(MRI)和计算机断层扫描(CT)可显示肺吊带,但由于异常LPA的位置不同,需要谨慎解读。血管造影可清晰检测肺吊带及相关心血管畸形。相关的胸外异常很常见。肺吊带的早期诊断和治疗是必要的,包括将左肺动脉重新植入肺动脉干并切断动脉导管韧带。术后病程可能很复杂,需要进行支气管干预。可能需要进行气管切除,但再狭窄很常见。对于此类病例已提出一期修复,在一些报道的病例中已成功完成。当没有相关气管支气管异常时,呼吸阻塞通常可完全缓解。术后晚期病程良好,但随着时间推移和气管生长,呼吸阻塞发作可能会发生,且发生率逐渐降低。