Children and young people who present with behaviour that challenges on a background of intellectual disability commonly present to paediatricians for support. This paper gives a structure to explore the possible biological, psychosocial and environmental underlying problems and suggests ways to assess and address these.
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.
A vascular ring is when vascular structures encircle the trachea and oesophagus and can lead to compression of these structures, and in the current era, these are commonly diagnosed prenatally. This pictorial piece describes the anatomical features of the three most encountered vascular rings in contemporaneous practice, namely, right aortic arch with left-sided ductal ligament and aberrant left subclavian artery; right aortic arch with left-sided ductal ligament and mirror image branching pattern and double aortic arch. A combination of fetal, paediatric and surgical images are used to display the anatomical features.
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