From the Guidelines
Dysphagia lusoria, also known as aberrant right subclavian artery (ARSA), is a rare condition where an abnormal right subclavian artery compresses the esophagus, causing difficulty swallowing, and is often associated with an artery that arises as the last branch of the aortic arch and passes behind the oesophagus through the mediastinum, as described in the 2024 ESC guidelines for the management of peripheral arterial and aortic diseases 1. This vascular anomaly can lead to symptoms such as dysphagia, respiratory symptoms, or recurrent laryngeal nerve palsy, although many patients with this anatomical variation remain asymptomatic. Some key points to consider about dysphagia lusoria include:
- The aberrant right subclavian artery is the most common variant, and it often passes behind the oesophagus through the mediastinum, potentially causing symptoms 1.
- Kommerell’s diverticulum, a remnant of the fourth dorsal aortic arch, is found in 20%–60% of those with an aberrant subclavian artery, and surgical intervention is advised for a diverticulum orifice >30 mm or combined diverticulum and adjacent descending aorta diameter >50 mm, or both 1.
- Diagnosis typically involves imaging studies such as barium swallow studies, CT angiography, or MRI to confirm the vascular anomaly.
- Treatment depends on symptom severity, with mild cases managed conservatively and severe cases requiring surgical intervention such as arterial transposition or bypass procedures. The condition is congenital, resulting from abnormal regression of the embryonic right fourth aortic arch, and while rare, should be considered in patients with unexplained dysphagia, particularly when other common causes have been ruled out, as highlighted in the 2024 ESC guidelines 1.
From the Research
Definition and Causes of Dysphagia Lusoria
- Dysphagia lusoria, also known as aberrant right subclavian artery (ARSA), is a rare embryologic defect of the aortic arch vasculature characterized by an aberrant retro-esophageal course of the right subclavian artery (RSA), comprising a vascular sling 2.
- This condition may manifest clinically with symptoms of dysphagia or reflux due to esophageal compression by the aberrant artery 3, 4.
- The term "dysphagia lusoria" was coined by David Bayford in 1761, describing a case of long-term dysphagia that eventually led to death 3.
Clinical Presentation and Diagnosis
- Most cases of dysphagia are due to an aberrant right subclavian artery running posterior to and causing esophageal compression, but only 20-40% of aberrant arteries actually lead to trachea-esophageal symptoms, including dysphagia 2, 3.
- The majority of patients with an aberrant right subclavian artery are asymptomatic 3.
- Diagnosis can be made using multidetector computed tomography (MDCT) angiography, which can demonstrate the vascular abnormality and esophageal compression 5.
- Barium contrast examination of the esophagus can also show a characteristic diagonal impression at the level of the fourth thoracic vertebra 6.
Treatment and Management
- Treatment for dysphagia lusoria varies depending on the severity of the symptoms, with dietary modifications recommended for patients with mild to moderate symptoms and vascular reconstruction necessary for patients with severe symptoms 3, 6.
- Surgical correction may be considered for patients with intractable symptoms, even if coexisting esophageal abnormalities are present 6.
- Vascular decompression procedure surgery is often used to rectify the aberrant vascular morphology, but the presence of marked aneurysmal dilation of the aberrant RSA and/or the presence of a bicarotid trunk would favor vascular repair in the clinical decision-making due to the elevated risk of rupture and more severe esophageal compression in these specific situations 2.