Vascular rings: an important cause of persistent respiratory symptoms in infants and children.

ABSTRACT

OBJECTIVE: To study the pattern of symptomatic vascular rings in infants and children with particular reference to clinical features, investigations and management. SETTING: Tertiary Care Paediatric Cardiology Division at the Royal Hospital, Muscat, Sultanate of Oman. DESIGN: Retrospective study. METHODS: Data on all patients aged less than 13 years diagnosed to have symptomatic vascular rings at the Royal Hospital, Muscat, Oman from 1992 to 2001 were retrieved from hospital records. RESULTS: Sixteen patients, 8 males and 8 females, aged 15 days to 36 months were identified, and included 12 with double aortic arch and 4 with right aortic arch, aberrant left subclavian artery and ligamentum arteriosum. Associated intracardiac defects were not found in any. Noisy breathing (7/16), stridor (6/16), respiratory distress (5/16) and recurrent respiratory infections (5/16) were the common modes of presentation. The duration from onset of symptoms to diagnosis ranged from 3 weeks to 24 months. Besides suggestive clinical features, the diagnosis was suspected from the chest radiograph (presence of right aortic arch) in 7 patients and from 2-Dimensional echocardiography and Doppler studies in 5 patients. Barium swallow studies, however, documented the abnormal esophageal indentation in all the 16 patients. Confirmatory imaging techniques included digital subtraction angiography in 7/16, cardiac catheterization and angiography in 5/16, and CT and MRI of the chest in 2 each. All 16 patients underwent successful surgical repair, and 13/16 became asymptomatic. 3 patients continued to suffer from occasional respiratory infections. CONCLUSIONS: A high index of clinical suspicion coupled with the use of barium oesophagraphy enabled early diagnosis in patients with symptomatic vascular rings, and surgical repair could be performed successfully in all patients.