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Dysphagia lusoria – Causes, Symptoms & Treatment

Dysphagia lusoria is a rare swallowing disorder caused by a congenital vascular anomaly that compresses the esophagus from the outside, leading to difficulty swallowing.

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Things worth knowing about "Dysphagia lusoria"

Dysphagia lusoria is a rare swallowing disorder caused by a congenital vascular anomaly that compresses the esophagus from the outside, leading to difficulty swallowing.

What is Dysphagia lusoria?

Dysphagia lusoria is a rare type of swallowing disorder (dysphagia) caused by a congenital vascular malformation. The name derives from the Latin phrase lusus naturae, meaning a whim of nature. In the majority of cases, the condition is caused by an aberrant right subclavian artery – also called the arteria lusoria – which arises as the last branch of the aortic arch and travels behind the esophagus, compressing it from the outside instead of following its normal anatomical course.

Causes and Development

The condition is congenital and results from abnormal development of the aortic arch during embryogenesis. The most common underlying cause is an aberrant right subclavian artery, which occurs in approximately 0.5–2% of the general population. Other, less common vascular anomalies that can cause dysphagia lusoria include:

Double aortic arch, which forms a vascular ring encircling both the esophagus and trachea
Right-sided aortic arch with an aberrant left subclavian artery
Anomalies of the carotid arteries or other major vessels

Not all individuals with these anomalies experience symptoms – many remain asymptomatic throughout their lives. Symptoms often appear in adulthood, when the aberrant vessel becomes less compliant and may enlarge over time, increasing pressure on the esophagus.

Symptoms

The hallmark symptom is dysphagia – difficulty swallowing, particularly with solid foods. Common complaints include:

A sensation of pressure or a foreign body behind the sternum during eating
Difficulty swallowing solid foods, less commonly liquids
Regurgitation of undigested food
Pain during swallowing (odynophagia)
Unintentional weight loss due to eating difficulties
Occasional respiratory symptoms if the trachea is also compressed

Diagnosis

Diagnosis is typically confirmed through imaging studies. The most important diagnostic tools include:

Upper gastrointestinal endoscopy (EGD): May reveal external pulsatile compression of the esophageal wall
Barium swallow study: Demonstrates a characteristic oblique posterior indentation of the esophagus
CT angiography of the chest: Considered the gold standard for visualizing the aberrant vessel and confirming the diagnosis
MR angiography: A radiation-free alternative, particularly useful in younger patients
Esophageal manometry: Measures pressure within the esophagus and helps exclude other causes of dysphagia

Treatment

Treatment depends on the severity of symptoms. In asymptomatic or mildly symptomatic individuals, no intervention may be required.

Conservative Management

For mild symptoms, dietary modifications can provide relief – such as eating soft or pureed foods and chewing thoroughly to reduce the mechanical burden on the esophagus.

Surgical Treatment

In patients with significant symptoms, surgical correction is recommended. Available approaches include:

Transposition of the aberrant subclavian artery: The vessel is detached and re-implanted in an anatomically correct position – this is the preferred surgical approach.
Endovascular repair: In selected patients, a minimally invasive stent-graft procedure may be considered.
For a double aortic arch: Division of the smaller arch to release the vascular ring compressing the esophagus and trachea.

Surgery generally leads to significant symptom relief. The long-term prognosis after successful intervention is excellent.

References

Janssen M, Baggen MGA, Veen HF et al. - Dysphagia lusoria: clinical aspects, manometric findings, diagnosis, and therapy. In: American Journal of Gastroenterology, 2000.
Backer CL, Hillman ND, Mavroudis C - Resection of Kommerell diverticulum and left subclavian artery transfer for relief of dysphagia lusoria. In: European Journal of Cardio-Thoracic Surgery, 2001.
Levitt B, Richter JE - Dysphagia lusoria: a comprehensive review. In: Diseases of the Esophagus, 2007.

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