Surgical management of anomalous left pulmonary artery causing tracheobronchial obstruction. Pulmonary artery sling.
An anomalous left pulmonary artery causing tracheobronchial obstruction is a rare malformation seen in infancy. Sixty-four cases have been described in the literature, with 17 survivors with or without surgical therapy. We reviewed the literature and the 5 cases seen at The Children's Memorial Hospital during the past 20 years. Three of these patients survived, 2 of whom have not been previously reported. The main clinical features are progressive respiratory distress, cyanosis, and apneic episodes resulting from obstruction of the lower trachea and bronchi by an anomalous left pulmonary artery. Obstructive emphysema with or without atelectasis is very common in this condition and should suggest to the clinician the possiblity of this anomaly. The important diagnostic clues are an anterior indentation of the esophagus on esophagography, narrowing of the lower end of the trachea and right bronchus on bronchography, and anomalous origin of the left pulmonary artery from the right on angiography. We prefer to approach this anomaly by a left anterolateral thoracotomy with transection and end-to-end anastomosis of the anomalous left pulmonary artery in front of the trachea. Respiratory complications due to residual tracheomalacia are common after the operation.
D001982:Bronchial Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D001995:Bronchography; D001999:Bronchoscopy; D004947:Esophagus / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008722:Methods; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D001261:Pulmonary Atelectasis / Q000209:etiology; D011656:Pulmonary Emphysema / Q000209:etiology; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology*; D014135:Tracheal Stenosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery