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Pulmonary sling: morphological findings. Pre- and postoperative course
C Döhlemann1, K Mantel, T J Vogl
1Universitätskinderklinik, München, Germany.
Insights
Pulmonary sling (PS) is a congenital condition causing airway compression. Early diagnosis and surgical repair, involving LPA reimplantation, are crucial for relieving obstruction and improving outcomes.
Area of Science:
- Congenital cardiovascular anomalies
- Pediatric respiratory diseases
- Thoracic surgery
Background:
- Pulmonary sling (PS) is a rare congenital anomaly where the left pulmonary artery arises abnormally, encircling the trachea.
- It can cause severe airway obstruction in infants, often presenting with respiratory distress.
- Associated tracheal and cardiovascular anomalies are common, complicating diagnosis and management.
Purpose of the Study:
- To review the diagnostic modalities and management strategies for pulmonary sling.
- To highlight the importance of early diagnosis and surgical intervention.
- To discuss the challenges and outcomes associated with surgical repair.
Main Methods:
- Review of diagnostic imaging techniques including echocardiography, chest radiography, bronchography, bronchoscopy, MRI, CT, and angiography.
- Discussion of surgical management, primarily LPA reimplantation and division of the ligamentum arteriosum.
- Analysis of postoperative complications and long-term outcomes.
Main Results:
- Various imaging modalities aid in PS diagnosis, though interpretation requires care.
- Associated tracheal anomalies are frequent and impact surgical outcomes.
- Surgical repair offers relief of obstruction, but complications like restenosis can occur.
- Complete relief is more likely in the absence of tracheobronchial anomalies.
Conclusions:
- Pulmonary sling requires prompt diagnosis and surgical intervention for optimal outcomes.
- Management can be complex due to associated anomalies, necessitating tailored approaches.
- While surgical repair is effective, long-term follow-up is important for managing potential late complications.
Abstract:
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.