Overview
Kommerell diverticulum (also known as Kommerell's diverticulum) is a rare congenital vascular anomaly of the aortic arch in which there is a bulging outpouching (diverticulum) at the origin of an aberrant subclavian artery arising from the aortic arch or descending aorta. It is classified under congenital malformations of the great arteries (ICD-10: Q25.4). The diverticulum represents a remnant of the embryonic fourth aortic arch and is most commonly associated with an aberrant right subclavian artery (arteria lusoria) arising from a left aortic arch, or less frequently with an aberrant left subclavian artery from a right aortic arch. The anomaly primarily affects the cardiovascular system but can secondarily impact the respiratory and digestive systems through compression of adjacent mediastinal structures. Many individuals with Kommerell diverticulum remain asymptomatic throughout life, with the condition discovered incidentally on imaging studies. When symptoms occur, they are typically caused by compression of the esophagus (dysphagia lusoria — difficulty swallowing) or trachea (dyspnea, chronic cough, stridor, or recurrent respiratory infections). In some cases, the diverticulum may enlarge over time, and there is a recognized risk of aneurysmal dilation, dissection, or rupture, which can be life-threatening. Symptoms may present at any age, from infancy through adulthood, depending on the size of the diverticulum and degree of vascular ring formation. Management depends on the severity of symptoms and the size of the diverticulum. Asymptomatic patients with small diverticula may be monitored with periodic imaging surveillance. Surgical intervention is recommended for symptomatic patients or when the diverticulum is large (generally greater than 3 cm) due to the risk of rupture. Surgical options include open repair with division of the aberrant vessel and reimplantation, or increasingly, hybrid and endovascular approaches including thoracic endovascular aortic repair (TEVAR). Early diagnosis and appropriate management are important to prevent potentially fatal complications.
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Kommerell diverticulum.
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Specialists
View all specialists →No specialists are currently listed for Kommerell diverticulum.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Kommerell diverticulum.
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Common questions about Kommerell diverticulum
What is Kommerell diverticulum?
Kommerell diverticulum (also known as Kommerell's diverticulum) is a rare congenital vascular anomaly of the aortic arch in which there is a bulging outpouching (diverticulum) at the origin of an aberrant subclavian artery arising from the aortic arch or descending aorta. It is classified under congenital malformations of the great arteries (ICD-10: Q25.4). The diverticulum represents a remnant of the embryonic fourth aortic arch and is most commonly associated with an aberrant right subclavian artery (arteria lusoria) arising from a left aortic arch, or less frequently with an aberrant left s
How is Kommerell diverticulum inherited?
Kommerell diverticulum follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.