Vascular ring

Vascular ring
Synonyms
Pronounce	N/A
Specialty	Cardiology, Pediatrics
Symptoms	Stridor, dysphagia, respiratory distress
Complications	Tracheomalacia, esophageal compression
Onset	Infancy
Duration
Types	Double aortic arch, Right aortic arch with aberrant left subclavian artery
Causes	Congenital heart defect
Risks
Diagnosis	Chest X-ray, Echocardiogram, CT scan, MRI
Differential diagnosis	Asthma, Laryngomalacia, Gastroesophageal reflux disease
Prevention
Treatment	Surgical repair
Medication
Prognosis	Generally good with treatment
Frequency	Rare
Deaths

A congenital vascular anomaly affecting the trachea and esophagus

Vascular ring is a rare congenital defect in which the aorta or its branches form a complete or partial ring around the trachea and esophagus, potentially causing compression and leading to respiratory and digestive symptoms. This condition is a type of congenital heart defect and can vary in severity depending on the specific anatomy of the vascular structures involved.

Anatomy and Pathophysiology

A vascular ring occurs when the normal embryological development of the aortic arch and its branches is altered, resulting in abnormal vascular structures that encircle the trachea and esophagus. The most common types of vascular rings include:

• Double aortic arch: This is the most common type of complete vascular ring, where the aorta splits into two arches that encircle the trachea and esophagus before rejoining into a single descending aorta.
• Right aortic arch with aberrant left subclavian artery: In this variant, the aorta arches to the right instead of the left, and the left subclavian artery arises abnormally, often causing a partial ring.
• Pulmonary sling: This occurs when the left pulmonary artery arises from the right pulmonary artery and passes between the trachea and esophagus, forming a sling-like structure.

Clinical Presentation

Symptoms of a vascular ring can vary depending on the degree of compression on the trachea and esophagus. Common symptoms include:

• Respiratory distress, especially during feeding or crying
• Stridor, a high-pitched wheezing sound caused by disrupted airflow
• Dysphagia, or difficulty swallowing
• Recurrent respiratory infections
• Chronic cough

Diagnosis

The diagnosis of a vascular ring is typically made using imaging studies. These may include:

• Chest X-ray: May show tracheal narrowing or deviation.
• Barium swallow: Can reveal esophageal compression.
• Echocardiography: Useful for visualizing the heart and great vessels.
• CT scan or MRI: Provides detailed images of the vascular structures and their relationship to the trachea and esophagus.

Treatment

The primary treatment for symptomatic vascular rings is surgical intervention. The specific surgical approach depends on the type of vascular ring and the structures involved. The goal of surgery is to relieve the compression on the trachea and esophagus by dividing or repositioning the abnormal vascular structures.

Prognosis

With appropriate surgical treatment, the prognosis for individuals with a vascular ring is generally good. Most patients experience significant improvement in symptoms following surgery. However, some may have residual respiratory or swallowing difficulties that require ongoing management.

Related pages

• Congenital heart defect
• Trachea
• Esophagus
• Aorta