Retroesophageal aortic arch: diagnostic and therapeutic implications of a rare vascular ring

Retroesophageal aortic arch (REAA) can be further divided to right or left retroesophageal aortic arch. The right REAA has right ascending and left descending aorta with retroesophageal segment, whereas the left REAA has left ascending and right descending aorta. The REAA with retroesophageal segment may cause tracheoesophageal compression by the formation of a vascular ring. A total of eight patients were identified from the database from 1996 to 1999. Among them, five were right REAA and three were left REAA. The age at diagnosis varied from 4 days to 16 years (median 8 years). The clinical findings and initial diagnostic investigations, including the chest X-ray, echocardiography and esophagogram, suspected the presence of this anomaly. The diagnosis can be confirmed by ultrafast computed tomography with or without three-dimensional reconstruction. Half of the total patients, two of right and two of left REAA were symptomatic and needed operation. The symptoms related to the retroesophageal aortic arch appeared during infancy and early childhood (six cases), and only rarely in late childhood and adults (two cases). Half of the patients were associated with congenital heart disease, but no specific patterns of congenital heart disease were identified. The sensitivity of the early diagnosis of REAA by chest roentgenogram, echocardiography, esophagogram and angiography was 37% (3/8), 0% (0/7), 100% (1/1) and 60% (3/5), respectively. The sensitivity of ultrafast CT was 100% (8) in delineating both retroesophageal segment and complete vascular ring. Operation to relieve the vascular compression was performed in two cases with right REAA and two with left REAA. Only one died after the operation, due to pulmonary arterial sling and tracheal stenosis. The surviving patients had been asymptomatic during follow up. In conclusion, REAA can be best diagnosed by ultrafast computed tomography with or without three-dimensional reconstruction. If symptomatic, surgery to relieve the compression is effective and safe.