Vascular Rings: Presentation, Imaging Strategies, Treatment, and Outcome

Abstract

This study aimed to evaluate the presenting symptoms, the effectiveness of imaging methods, and the surgical treatment of vascular rings. Data for 44 patients (32 enrolled prospectively, 12 reviewed retrospectively) over a 10-year period in a tertiary referral center were analyzed. These patients comprised 25 patients with a left aortic arch and an aberrant right subclavian artery, 13 patients with a right aortic arch and a left subclavian artery originating from Kommerell’s diverticulum, 1 patient with a right aortic arch and an aberrant left subclavian artery, 3 patients with a double aortic arch, and 2 patients with a pulmonary sling. Respiratory symptoms were found in 25 patients and dysphagia in 6 patients. Atypical symptoms such as reflex apnea, cyanosis, syncope episodes, and exercise-induced wheezing were noted in five patients. Associated congenital heart defects were detected in 41% of the patients. The diagnostic yield was 95.23% for barium esophagography, 54.54% for echocardiography, and 66.66% for computed tomography. The anatomy could be correctly identified by magnetic resonance imaging (MRI) in 97.43% and by angiography in 90.5% of the patients. Of the 30 patients who underwent surgery, 80% were completely relieved of symptoms during a mean follow-up period of 25 ± 33.5 months. Vascular rings should not be overlooked in infants with atypical symptoms. The authors’ diagnostic procedure of choice is MRI because it is superior to angiography for delineating the relationship between abnormal vascular structures, trachea, and esophagus.

Vascular ring: Unmasked

“…Atypical symptoms such as reflex apnoea, cyanosis, syncopal episodes and exercise‐induced wheeze can also occur. Often the symptoms have been present from birth but the diagnosis not made until later, with a mean age of 3 years at diagnosis …”

“…X‐rays should also be scrutinised for tracheal deviation (which may not be apparent because of the generous thymic shadow typical in this age group) and the presence of tracheal constriction, which can manifest as narrowing of the tracheal air column and lung hyperinflation . Barium swallow is an excellent first‐line investigation, with demonstration of oesophageal indentation having a diagnostic yield of 95% . However, it may not demonstrate airway compression nor delineate the aberrant anatomy .…”

“…Barium swallow is an excellent first‐line investigation, with demonstration of oesophageal indentation having a diagnostic yield of 95% . However, it may not demonstrate airway compression nor delineate the aberrant anatomy . Kir et al .…”

A Pictorial Essay on Radiography of Swallowing and Esophageal Disorders

“…A posterior vascular indentation may or may not be a vascular ring, but in the presence of a right aortic arch, a vascular ring should be suspected. A focal nodular indentation on ap lateral the anterior portion of the esophagus at the level of the carina seen on lateral esophagram is highly suspected for a pulmonary artery sling (29)(30)(31)(32). Left-sided aortic arch with aberrant right subclavian artery is often found incidentally, but can rarely cause dysphagia.…”

Assessment of Suspected Vascular Rings and Slings and/or Airway Pathologies Using Magnetic Resonance Imaging Rather Than Computed Tomography

“…As a result AP might cause respiratory tract infections or respiratory distress [1]. Symptoms may vary and might be present from infancy, but many affected patients are misdiagnosed with bronchial asthma refractory to therapy for a long time until CCVM and corresponding AP is found later in life [2].…”