Vascular rings
Key points
Affects up to 3% of the population
Complete vascular rings account for 95% of cases, while incomplete rings make up 5%
Complete Vascular Rings
Double aortic arch, often leading to posterior distal tracheal wall collapse
Right aortic arch with left ductus arteriosus
Right aortic arch with retroesophageal aberrant left subclavian artery (may have a
"Diverticulum of Kommerell" at the orgin of the left subclavian artery)
Dominant right arch present in 70-90% of double aortic arch cases
Incomplete Vascular Rings
Innominate artery compression syndrome causing right anterior tracheal wall collapse
Pulmonary artery sling, the most fatal of the rings, causing right anterior wall compression
Left aortic arch with aberrant right subclavian artery, often leading to posterior distal wall collapse
Anatomy
Aortic arch is divided into three segments:
Proximal arch (between right innominate and left common carotid arteries)
Distal arch (between left common carotid and left subclavian arteries)
Aortic isthmus (between left subclavian artery and patent ductus arteriosus)
Most common anomaly is a right aortic arch with mirror-image branching, where the descending thoracic aorta is on the right side of the chest, opposite of the typical left-sided arch
Associations
22q11 deletion syndrome
Tetralogy of Fallot
Truncus arteriosus
History and Examination
Typically presents between 0-3 years of age with stridor, dysphagia, and failure to thrive
Tracheal compression is more common than oesophageal compression
May also present in early adulthood
Radiology
Chest X-ray shows narrowed trachea in 91% of cases
Contrast imaging reveals posterior indentation of the oesophagus
Operation
Type of operation and approach varies depending on the abnormality
Open and thoracoscopic approaches are described
For several abnormalities - division of the ligamentum arteriosum is all that is needed
For a double aortic arch, the smallest side should be divided
A Diverticulum of Kommerell may need resection
Outcome
Symptoms of tracheomalacia may persist for months following surgery
References
Holcomb and Ashcraft’s Pediatric Surgery, 7th edition, 2020, Chapter 21 Management of Laryngotracheal obstruction in children
Rothstein, David H, and Dan Ostlie. "Vascular Rings." Pediatric Surgery NaT, American Pediatric Surgical Association, 2020. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829073/all/Vascular_Rings.
Rothstein, David H, and Dan Ostlie. "Vascular Ring Repair." Pediatric Surgery NaT, American Pediatric Surgical Association, 2020. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829206/all/Vascular_Ring_Repair.
Jha P, Gaillard F, Niknejad M, et al. Kommerell diverticulum. Reference article, Radiopaedia.org (Accessed on 25 Aug 2024) https://doi.org/10.53347/rID-9018