Medical Student SYB #1

Cristy Gustas Nov 8, 2007

What is wrong with this image??

Non contrast CT of Chest

Vascular Ring with Right Aortic Arch

    Several types of vascular rings exist in which the aortic arch is right sided. The defining feature of all vascular rings is encirclement of the trachea and esophagus by connected segments of the aortic arch and its branches. Early in the course of embryonic morphogenesis, 6 pairs of pharyngeal arch arteries develop in conjunction with the branchial pouches. The arches develop with left-right symmetry, and supply the primitive brachiocephalic structures, running from the aortic sac to the paired dorsal aortas.

Vascular Ring with Right Aortic Arch

  As normal development proceeds, a patterned regression and persistence of the various arches and right-sided dorsal aorta occur, ultimately resulting in the mature configuration of the thoracic aorta and its branches. The incidence of right aortic arch and vascular rings in general is not known, although vascular rings comprise an estimated 1% of cardiovascular malformations managed surgically.

Vascular Ring with Right Aortic Arch

  Vascular ring with a right aortic arch is associated with a band 22q11 deletion in approximately 20% of patients. Band 22q11 deletion is responsible for DiGeorge, velocardiofacial, and conotruncal anomaly face syndromes, which are often referred to as CATCH-22 syndrome .

Occasionally, patients with right aortic arch may have anomalies consistent with either limb) or CHARGE associations. VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, (posterior coloboma, heart defect, choanal atresia, retardation, genital, ear)

Vascular Ring with Right Aortic Arch

   Compression of the trachea can cause upper airway obstruction and stridor that doesn’t improve with bronchodilator therapy.

Patients may also experience swallowing problems related to esophageal compression.

Differential

aortic arch.

: asthma, bronchiolitis, congenital stridor, tracheomalacia, laryngomalacia, croup, pulmonary artery sling, vascular ring/double

reference

 1. McElhinney, Doff et al. Vascular Ring, Right Aortic

Arch.

www.emedicine.com

Nov 5, 2007.