What is dysphagia lusoria?

Dysphagia Lusoria: Definition and Clinical Significance

Dysphagia lusoria is a rare condition characterized by difficulty swallowing due to esophageal compression from an aberrant subclavian artery, most commonly an aberrant right subclavian artery (ARSA) that passes behind the esophagus. 1

Anatomical Basis

• Dysphagia lusoria results from an embryologic anomaly in the development of the aortic arch system, specifically abnormal development of the right fourth aortic arch during embryonic stages 2
• The most common variant is an aberrant right subclavian artery (ARSA), which arises as the last branch of the aortic arch (after the left subclavian artery) and typically passes behind the esophagus through the mediastinum 1
• Less commonly, it can involve an aberrant left subclavian artery, which is typically associated with congenital heart defects such as a right aortic arch 1
• The term "lusoria" derives from Latin, meaning "freak" or "jest of nature," reflecting its unusual anatomical presentation 3

Clinical Presentation

• While often asymptomatic, approximately 30-40% of cases develop tracheoesophageal symptoms 3
• Primary symptoms include:
  • Dysphagia, particularly to solid foods 1, 2
  • Respiratory symptoms or dyspnea 2
  • Recurrent laryngeal nerve palsy in some cases 1
  • Chest pain 3
• Symptoms typically manifest later in life due to:
  • Development of arteriosclerosis causing arterial rigidity 3
  • Decreased esophageal compliance with aging 3
  • Age-related esophageal dysmotility 4

Associated Conditions

• Kommerell's diverticulum is found in 20-60% of patients with an aberrant subclavian artery 1
  • This represents a remnant of the fourth dorsal aortic arch due to incomplete regression 1
  • Can contribute to symptom severity by forming a vascular ring behind the esophagus 3
• Complications can include:
  • Complete esophageal obstruction (rare) 5
  • Food impaction 6
  • Recurrent aspiration 2

Diagnosis

• Barium esophagogram is key to initial diagnosis, showing extrinsic compression of the esophagus 3
• Definitive diagnosis requires:
  • Computed tomography (CT) angiography 3
  • Magnetic resonance imaging (MRI) 3
  • Upper gastrointestinal endoscopy may show extrinsic compression 2

Management

• Treatment approach depends on symptom severity:
  • Mild cases: Conservative management with dietary modifications (soft diet) 4
  • Severe or unresponsive cases: Surgical intervention 3
• Surgical intervention is specifically advised when:
  • Kommerell's diverticulum orifice exceeds 30 mm 1
  • Combined diverticulum and adjacent descending aorta diameter exceeds 50 mm 1
  • Symptoms are persistent despite conservative management 2
• Surgical approaches include open, endovascular, or hybrid techniques based on anatomy, comorbidities, and surgical expertise 1

Clinical Significance

• Though rare (estimated prevalence of 0.5%), dysphagia lusoria should be considered in the differential diagnosis of patients with unexplained dysphagia, particularly when symptoms are positional or related to solid foods 3
• It represents an important example of how vascular anomalies can cause gastrointestinal symptoms 2
• Recognition is particularly important in patients with late-onset dysphagia without other clear causes 4