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Vascular ring as one of rare anomalies of the aortic arch

Joanna Kwiatkowska, Janina Aleszewicz-Baranowska, Robert Sabiniewicz, Barbara Bielińska, Jan Ereciński

CaseRepClinPractRev 2002; 3(3):170-175

ID: 474512

Background: Vascular ring (VR) is a term referring to a group of congenital anomalies of the aortic arch and/or its ramifications, which may cause various degree of compression of the trachea and esophagus. The aim of the
study was to analyze the clinical presentation and imaging results in children with diagnosed VR in comparison with intraoperative and post mortem findings.
Case Report: In 1990–1998, 13 children with diagnosed VR were hospitalized in our center. The patients’ age at the time of hospitalization ranged from 2 to 13 months (mean 7.8 months). The clinical picture included dyspnea
and recurrent respiratory tract infections observed since the moment of birth in all cases. Two infants presented with dysphagia. In none of the cases congenital intracardiac defects were diagnosed. VR was diagnosed
on the basis of AP and L chest X-ray with barium meal contrast demonstrating esophageal impression. In 10 cases the diagnosis was confirmed by angiography. Surgical correction of the anomaly was performed in 10 of
the patients. One child died on the 3rd postoperative day. In another one the symptoms of tracheostenosis persist. The condition of the remaining 8 patients is very good. One child died at the age of 12 months in the course
of extensive bilateral pneumonia. 2 other children qualified for the surgery are waiting for the operation. In 8 patients, double aortic arch was diagnosed, in 2 cases right-sided aortic arch with persistent innominate artery,
in 2 left-sided aortic arch with anomaly of the right subclavian artery, an in 1 case esophageal impression was caused by the arterial ligament.
Conclusions: Vascular rings should be taken into consideration in differential diagnostics of recurrent respiratory tract infections. Chest X-ray with esophageal contrast is very useful in differential diagnostics while angiography allows precise assessment of the anatomical variant of the anomaly. The anomaly is treated surgically with good results.

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