This site is targeted at medical and radiology professionals, contains user contributed content, and material that may be confusing to a lay audience. Use of this site implies acceptance of our Terms of Use.

Midaortic syndrome

Dr Jeremy Jones and Dr Omar Bashir et al.

Midaortic syndrome (MAS) is an uncommon entity affecting children and young adults. It is characterised by progressive narrowing of abdominal aorta and its major branches¹.

Clinical presentation

The onset of symptoms is usually during young adulthood or childhood².

hypertension (most common)
intermittent claudication
renal failure

Pathology

The exact cause is unknown though it is probably due to an insult during intrauterine life. Whilst intimal and subintimal fibrosis and fragmentation of the elastic media are seen, arteritis and atherosclerosis are characteristically absent in this condition.

Radiographic features

DSA - Angiography

Whilst involvement of any portion of the abdominal aorta may been seen, the interrenal segment including the origins of the renal arteries is invariably narrowed. Typically enlarged splanchnic collaterals (IMA, arc of Riolan, marginal artery of Drummond) are noted. The aortic bifurcation and iliac arteries are spared³.