quiz The most likely vascular ring in young infant

[Admin]

First one with correct answer with justification win.

A 2 month old has stridor and occasional apnea during feeding. Diagnostic evaluation, including chest x-ray and CT angiogram shows a vascular ring. What is the most likely vascular ring in this young infant with respiratory symptoms?

A Double aortic arch

B Left aortic arch with an aberrant right subclavian artery

C Pulmonary artery sling

D Right aortic arch with an aberrant left subclavian artery

E Right aortic arch with mirror imaging branching

[Admin]

The correct answer
A Double aortic arch

Vascular rings are rare congenital anomalies of aortic arch-derived structures that encircle the trachea and esophagus to a varying degree and may cause respiratory symptoms and/or feeding difficulties in newborns, infants, and children. During evaluation of these symptoms the diagnosis of vascular ring may be suspected by an abnormal chest x-ray (showing a right aortic arch or indistinct aortic arch), esophagram (obtained to evaluate feeding difficulty and showing extrinsic compression from the abnormally located vessel), or bronchoscopy (showing pulsatile, extrinsic compression of the trachea). The diagnosis of vascular ring is confirmed and the precise anatomy is confirmed with cross sectional imaging and 3 D reconstructions of either MRI or CT. Echocardiograms are also usually part of the preoperative evaluation because of the significant risk of associated congenital heart disease.

Double aortic arch is the most common type of vascular ring that causes symptoms in newborns and infants and accounts for 50-60% of operative cases in most series. The typical double aortic arch consists of a dominant right arch and a smaller left arch that can occasionally be atretic. The ductus arteriosus is typically on the left. The surgical approach for this common anomaly is through the left chest with ligation and division of the ductus and the small left aortic arch near the insertion at the descending aorta.

Right aortic arch with aberrant left subclavian artery, often originating from a diverticulum of Kommerell, is the second most common form of vascular ring that causes symptoms in infants and accounts for 30-35% of cases. Operative repair is through the left chest and consists of ligation and division of the ligamentum arteriosum with or without resection of the Kommerell diverticulum.

A left aortic arch with aberrant (retroesophageal) right subclavian artery is the most common incomplete vascular ring, being present in 0.5% of the population but it rarely causes symptoms in infants because the ductus arteriosus is usually on the left and therefore there is not a complete vascular ring around the esophagus and trachea. This anomaly may result in a slight posterior extrinsic compression of the esophagus and can cause symptoms later in life when it is known as “dysphagia lusoria”.

Pulmonary artery sling is a much less common vascular ring. It occurs when the left pulmonary artery arises from the right pulmonary artery and then passes over the right mainstem bronchus and between the trachea and esophagus. This anomaly is often associated with intrinsic tracheal stenosis secondary to complete cartilaginous tracheal rings. This anomaly is repaired via median sternotomy and cardiopulmonary bypass. Tracheal stenosis is usually corrected at the same time by either segmental resection and reanastomosis or slide tracheoplasty.

Right aortic arch with mirror image branching is another vascular ring anomaly that is not a true vascular ring in that mirror image innominate, left common carotid, and left subclavian artery and most importantly the ligamentum arteriosum are all in front of the trachea and esophagus while the right subclavian is the last branch of the aortic arch and is posterior.

[Sharon]

A

[Abusnaina mohammed]

A Double aortic arch
D Right aortic arch with an aberrant left subclavian artery


The two most common anatomic variants of true vascular rings, occurring in nearly equal frequency, are persistent double aortic arch and right aortic arch with anomalous origin of the left subclavian artery

[Admin]

The correct answer
A Double aortic arch

Vascular rings are rare congenital anomalies of aortic arch-derived structures that encircle the trachea and esophagus to a varying degree and may cause respiratory symptoms and/or feeding difficulties in newborns, infants, and children. During evaluation of these symptoms the diagnosis of vascular ring may be suspected by an abnormal chest x-ray (showing a right aortic arch or indistinct aortic arch), esophagram (obtained to evaluate feeding difficulty and showing extrinsic compression from the abnormally located vessel), or bronchoscopy (showing pulsatile, extrinsic compression of the trachea). The diagnosis of vascular ring is confirmed and the precise anatomy is confirmed with cross sectional imaging and 3 D reconstructions of either MRI or CT. Echocardiograms are also usually part of the preoperative evaluation because of the significant risk of associated congenital heart disease.

Double aortic arch is the most common type of vascular ring that causes symptoms in newborns and infants and accounts for 50-60% of operative cases in most series. The typical double aortic arch consists of a dominant right arch and a smaller left arch that can occasionally be atretic. The ductus arteriosus is typically on the left. The surgical approach for this common anomaly is through the left chest with ligation and division of the ductus and the small left aortic arch near the insertion at the descending aorta.

Right aortic arch with aberrant left subclavian artery, often originating from a diverticulum of Kommerell, is the second most common form of vascular ring that causes symptoms in infants and accounts for 30-35% of cases. Operative repair is through the left chest and consists of ligation and division of the ligamentum arteriosum with or without resection of the Kommerell diverticulum.

A left aortic arch with aberrant (retroesophageal) right subclavian artery is the most common incomplete vascular ring, being present in 0.5% of the population but it rarely causes symptoms in infants because the ductus arteriosus is usually on the left and therefore there is not a complete vascular ring around the esophagus and trachea. This anomaly may result in a slight posterior extrinsic compression of the esophagus and can cause symptoms later in life when it is known as “dysphagia lusoria”.

Pulmonary artery sling is a much less common vascular ring. It occurs when the left pulmonary artery arises from the right pulmonary artery and then passes over the right mainstem bronchus and between the trachea and esophagus. This anomaly is often associated with intrinsic tracheal stenosis secondary to complete cartilaginous tracheal rings. This anomaly is repaired via median sternotomy and cardiopulmonary bypass. Tracheal stenosis is usually corrected at the same time by either segmental resection and reanastomosis or slide tracheoplasty.

Right aortic arch with mirror image branching is another vascular ring anomaly that is not a true vascular ring in that mirror image innominate, left common carotid, and left subclavian artery and most importantly the ligamentum arteriosum are all in front of the trachea and esophagus while the right subclavian is the last branch of the aortic arch and is posterior.