Vascular rings and slings: A challenging diagnostic and therapeutic rare disease entity

Background

Vascular rings (VRs) are classified as true rings or partial rings. The complete VRs include double aortic arch (DAA) and right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum. The incomplete (VRs) include innominate artery compression and pulmonary artery sling.

Methods

We retrospectively analyzed the data from 90 patients with VRs and pulmonary artery slings diagnosed at Mansoura University Children’s Hospital between 2004 to 2016. The initial diagnostic clue might be a right sided aortic arch on a plain x-ray chest. Lateral views were evaluated for retro tracheal opacity, anterior tracheal bowing, and posterior indentation. In our series, bronchoscopy was used for patients with respiratory tract symptoms. Bronchoscopy was performed in 21 patients (23.3%), with finding of external tracheal wall pulsatile compression. Echocardiography was used initially in most cases as the first diagnostic tool followed by Multislice CT scan (MSCT).

Results

The age of operation was significantly earlier in patients with complete VRs versus incomplete vascular rings. By far the commonest variant was the DAA which constituted in our series 57.8%. The second common type was RAA with aberrant left subclavian and left ligamentum which constituted 30%. The third most common type was pulmonary artery sling constituting 6.7%. In our series, Echocardiography was done in 78 cases (85.6%). It was useful not only in initially diagnosing VR anomaly but also it detected associated cardiac anomalies in 4 cases. MSCT was an excellent noninvasive diagnostic modality that was used in 75 cases (83.3%). We operated 90 cases; 79 via left thoracotomy, 10 cases via median sternotomy and one case via right thoracotomy. Among the 90 cases that were operated, there was a single mortality, no cases were complicated by recurrent laryngeal nerve injury and five cases had postoperative chylothorax.

Conclusions

In our series, Echocardiography and MSCT were essential for proper diagnosis and planing for surgery. Left thoracotomy or median sternotomy provided good exposure in the operated cases. Surgery was done with low mortality and morbidity.