Vascular rings: a rare cause of common respiratory symptoms

Upper airway symptoms or dysphagia may be caused by vascular anomalies, for ming a ring around the trachea, oesophagus or both. To analyse the clinical presentation, use of various diagnostic techniques, treatment and follow-u p we carried out a retrospective study of 38 children who had been diagnose d with a vascular ring between 1981 and 1996. We found 74% of the vascular rings to be symptomatic, with inspiratory strider and wheezing as the main complaints. The delay between the onset of symptoms and diagnosis of a vasc ular ring in patients without associated anomalies ranged from 1 to 84 mo. Associated anomalies were found in 53% of cases and 80% of these anomalies consisted of associated cardiovascular malformations. Oesophagography prove d to be a valuable diagnostic technique when a vascular ring was suspected. Echocardiography appeared to be of little value for the diagnosis of a vas cular ring, but was essential to exclude associated cardiovascular malforma tions. Although angiography has always been considered to be the gold stand ard in the determination of the exact anatomy of vascular rings, increasing evidence is available that CT scan or MRI may replace this role. Mortality was related to co-existent tracheal deformities in 5/6 cases. Of the remai ning, preoperatively symptomatic patients, relief of symptoms was achieved immediately after surgery in 43% and within 4y after surgery in 57%. Prolon ged and recurrent respiratory complaints or dysphagia in infancy or childho od should alert the paediatrician to the possibility of a vascular ring.