Vascular rings and slings

Since the mid-1940s, the phrase “vascular ring” has referred to congenital vascular anomalies of the aortic arch system that encircle and compress the esophagus and trachea causing various symptoms. The concept of vascular sling was described five decades later. The diagnosis of vascular ring or pulmonary artery sling should be suspected in any infant or child presenting with symptoms of respiratory distress, noisy breathing, or dysphagia. Suspicion of the diagnosis is often generated by the plain chest x-ray. However, the diagnosis is best established by CT imaging which accurately delineates the anatomy of the vascular ring and the associated tracheal pathology. All patients diagnosed with a vascular ring should have an echocardiogram because of the incidence of associated congenital heart disease. Bronchoscopy should be performed in all cases to assess for additional tracheal pathology and to provide a clinical assessment of the degree of tracheomalacia and bronchomalacia. This chapter aims to overview the embryology, the anatomy and pathophysiology, the clinical presentation and diagnosis, and the management of the entities included in the spectrum of vascular rings and slings.