Vascular rings in the current era: is this a new disease requiring a new approach?

Abstract

Historically, vascular rings, formed by a right or double aortic arch, have been diagnosed in childhood following symptoms or signs of tracheo-oesophageal compression. Many of these features might be attributed to other non-surgical diagnoses, such as asthma, postviral symptoms or simply, sensory feeding behaviours. This has often led to families being falsely reassured, resulting in delayed referral to specialist services for assessment and definitive surgical treatment. Despite surgery, it is common for these postnatally diagnosed patients to have persistent symptoms.In the modern era, most vascular rings are diagnosed prenatally, having been identified on routine ultrasound screening in mid-pregnancy. This has resulted in an exponential increase in the diagnosis of the right aortic arch and double aortic arch, leading to a new form of these conditions for health practitioners to manage-a diagnosis prior to possible clinical presentation. Early symptoms of a vascular ring may be subtle, atypical or not evident despite having a significant degree of tracheal compression, and thus, there is a window for early intervention before symptoms affect a child's health. Understanding which patients with vascular rings can be managed conservatively and which require surgery to improve outcomes and reduce longer-term morbidity and physical limitations is crucial. The recent literature on the diagnosis, assessment, management and outcomes of isolated vascular rings is reviewed, and our institutional pathway to manage these patients is discussed.