8.6 Vascular Rings
Key Features
The trachea and esophagus are completely or incompletely surrounded by vascular structures.
Compression of the trachea, the bronchi, and/or the esophagus may occur.
Most symptomatic malformations present during infancy or early childhood.
The term vascular ring refers to an aortic arch abnormality in which the trachea and esophagus are surrounded by vascular structures. It may be complete or incomplete. The greater the degree of compression the vascular ring causes, the more severe the symptoms are and the earlier they present. For symptomatic patients, treatment is generally surgical.
Embryology and Anatomy
Vascular rings arise during embryonic development from the abnormal evolution of the arterial branchial arch system. In normal embryonic vascular development, ventral and dorsal aortae are connected by six pairs of aortic arches. The first, second, and fifth arches regress, as does a portion of the right fourth arch. This leaves the usual left aortic arch. Residual segments of the third, fourth, and sixth arches develop into the mature anatomy of the mediastinal vascular structures. Inappropriate persistence or development of segments leads to congenital aortic arch anomalies.
The most common vascular ring is a double aortic arch, accounting for 50 to 60% of symptomatic vascular rings. A right aortic arch with an aberrant left subclavian artery is the second most common, accounting for 12 to 25% of cases. Other vascular anomalies include a right aortic arch with mirror image branching and left ductus arteriosus, a pulmonary artery sling, an anomalous innominate artery, and left aortic arch anomalies.
Epidemiology
At autopsy, 3% of the population has a congenital anomaly of the aortic arch system. Most are asymptomatic. Vascular rings account for less than 1% of congenital cardiovascular malformations.
Clinical
Signs and Symptoms
Symptoms depend on the location and degree of vascular compression. Wheezing, stridor, aspiration, cyanotic or apneic attacks, and dysphagia are characteristic. Feeding may exacerbate stridor. Dysphagia is worsened by solid foods. Recurrent respiratory infections such as aspiration pneumonia may also be present.
Differential Diagnosis
Asthma
Tracheomalacia
Bronchiolitis
Laryngeal stenosis
Congenital stridor
Laryngeal web
Croup
Foreign body aspiration
Laryngomalacia
Evaluation
Physical Exam
Physical findings vary, often in accordance with the patient′s history. Stridor is characteristically expiratory. It is often associated with cough, tachypnea, and rhonchi. Expiratory, high-pitched wheezes and intercostal retractions can also be appreciated. Patients may hold their neck in hyper-extension to alleviate respiratory distress. Respiratory findings typically do not improve with nebulized bronchodilator treatment and are worsened by exertion. Pulmonary infection may be the presenting symptom, especially in older children.
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