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Description

The mega aorta syndrome is a rare disorder with diffuse enlargement of the entire aorta.


Features - all of the following:

(1) dilatation of the all or most of the thoracic aorta

(2) dilatation of all or most of the abdominal aorta

(3) variable dilatation of major branch arteries

 

Causes:

(1) diffuse large vessel vasculitis (Takayasu, Giant Cell)

(2) myxoid degeneration of the aortic media

(3) other connective tissue disorders including Marfan's syndrome

 

Complications may include:

(1) obstructive pneumonia (due to compression of the main bronchi)

(2) neurological symptoms due to reduced perfusion through the carotid and/or vertebral arteries

(3) aortoiliac obstruction

(4) aortic rupture

(5) dissection

(6) aortic valve insufficiency

 

Management usually involves replacement of the entire aorta in 2 or more operative stages.


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