Vascular Ring Abnormalities

Vascular rings are uncommon. They are formed by vascular and ligamentous structures that encircle the airway and esophagus, causing extrinsic compression and obstruction of these hollow structures. Rings form as a result of developmental failure of parts of the paired 4th-6th aortic arches (Bonnard et al. 2003). Almost 20% of vascular ring malformations occur in association with congenital cardiac

Fig. 2.15. Esophageal bronchus. Barium esophagram demonstrates that the right main bronchus arises from the esophagus. The right lung is completely opacified

anomalies. Some rings are incomplete and asymptomatic, and are only incidentally discovered on chest radiographs or esophagrams. Symptoms vary with the degree of constriction of the vascular ring around the airway and esophagus. It is not surprising that the double aortic arch, which encircles the airway and the esophagus, causes the most severe symptoms. A double aortic arch is formed by a larger superior right aortic arch and a more inferior smaller left aortic arch. Symptoms from vascular rings are principally respiratory. Gastrointestinal symptoms and dysphagia are less common, and are more often encountered with a left retroesophageal subclavian artery arising from a right aortic arch (Bonnard et al. 2003). A right aortic arch with mirror-image branching acts like a vascular ring if the left ductus arteriosus passes between the right descending aorta and the left pulmonary artery.

The treatment is surgical relief of the constriction. The basic principle of vascular ring surgery is to divide non-functional or non-critical components of the ring. Surgical relief of the double aortic arch entails dividing the lesser of the two arches. An atretic portion is an optimal location for division of the arch. In cases with a right aortic arch and liga-mentum arteriosum, the ductus remnant is divided, and the trachea and esophagus are released from adhesive bands (Backer et al. 2005).

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