What is the best management approach for Dysphagia Lusoria (difficulty swallowing due to an aberrant right subclavian artery)?

Management of Dysphagia Lusoria

Surgical intervention is recommended for symptomatic dysphagia lusoria patients, with the preferred approach being resection of the aneurysmal segment of the aberrant right subclavian artery (diverticulum) and adjacent aorta with graft replacement. 1

Diagnostic Approach

• CT angiography (CTA) is the test of choice for diagnosing an aberrant right subclavian artery due to its excellent visualization of vascular structures and their relationship to surrounding tissues 2
• MRI/MRA is an excellent alternative when radiation exposure is a concern or when the patient has contraindications to iodinated contrast 2
• Two key measurements should be obtained using cross-sectional imaging:
  • The diverticulum orifice (measured at the aortic wall) 3
  • The combined diameter of the diverticulum and adjacent descending thoracic aorta 3

Clinical Presentation

• Dysphagia lusoria typically occurs in adults as the aberrant artery enlarges (Kommerell diverticulum) 1
• The aberrant right subclavian artery courses behind the esophagus in approximately 80% of patients, causing compression and dysphagia 1
• In most adult patients, the aorta is also abnormal and prone to aneurysm formation, dissection, and rupture 1

Management Algorithm

Indications for Intervention

• Symptomatic patients with dysphagia attributable to the aberrant vessel 1, 3
• Asymptomatic patients with:
  • Kommerell's diverticulum orifice >3.0 cm 3
  • Combined diameter of the diverticulum and adjacent descending aorta >5.0 cm 3

Surgical Options

1. Open Surgical Approach (Preferred for most patients):

   • Resection of the aneurysmal segment of the subclavian artery (diverticulum) and adjacent aorta 1
   • Replacement with a graft to maintain vascular continuity 1, 4
   • Can be performed through right anterolateral thoracotomy with direct end-to-side anastomosis to the ascending aortic arch 4
   • Alternative approach: right supraclavicular incision for simultaneous correction of dysphagia and revascularization of the right upper extremity 5

2. Hybrid Approach:

   • Right carotid to subclavian bypass combined with endovascular occlusion of the aberrant vessel 6
   • Considered safer and less invasive for appropriate candidates 6

3. Endovascular Approach:

   • Exclusion of the right subclavian artery origin and adjacent aorta using an aortic endograft 1
   • Important limitation: long-term follow-up data for this approach is not available, and compression and aneurysm growth may continue 1

Special Considerations

• Complete resection of the aberrant vessel origin is crucial, as a persistent aberrant right subclavian artery stump may cause postoperative residual dysphagia 7
• When approaching through a right supraclavicular incision, accessing the origin of the aberrant vessel can be challenging 7
• In patients with right aortic arch variants, additional evaluation for associated anomalies is recommended 8

Follow-up

• Regular imaging surveillance is recommended following intervention to monitor for recurrence or development of new aortic pathology 8
• For patients managed non-operatively, regular imaging surveillance is recommended to monitor for aneurysm development 3, 8

Pitfalls to Avoid

• Failing to recognize associated aortic abnormalities, which are common in these patients 1
• Incomplete resection of the aberrant vessel origin, which may lead to persistent symptoms 7
• Underestimating the risk of aneurysm formation, dissection, and rupture in untreated patients 1