Midaortic syndrome

Midaortic syndrome (MAS) is an uncommon entity affecting entity affecting children and young adults. It is characterised by 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 unknown in most cases 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 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 ((e.g. IMAinferior mesenteric artery, arc of Riolan, marginal artery artery of Drummond) are noted. The aortic bifurcation and iliac arteries are arteries are spared³.

Treatment and prognosis

Endovascular treatment is the preferred mode as it is a minimally invasive treatment option.

Balloon Balloon expandable stents/ stent grafts are usually placed to expand the lumen of the aorta.

Complex Complex endovascular / surgical options such as fenestrated grafts with re-implantation of the renal arteries will be required if the renal arteries are involved.

Differential diagnosis

Imaging differential considerations include²

• Takayasu arteritis^{ref required}
• neurofibromatosis^{ref required}