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Erdheim-Gsell cystic medial necrosis as a cause of giant aneurysm of the ascending aorta
  1. H Burri,
  2. S Dielbold-Berger,
  3. D Didier
  1. Haran.Burri{at}

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A previously healthy 42 year old woman was referred after the discovery of a 3/6 diastolic murmur maximal at the aortic region, with vigorous pulsation of the suprasternal notch. Echocardiography followed by magnetic resonance imaging (left) revealed a fusiform aneurysm of the ascending aorta measuring 7 cm in diameter, without evidence of dissection, extending from the aortic root up to the brachiocephalic artery, associated with severe aortic regurgitation. The patient underwent a Bentall procedure. Histological examination of the operative material showed Erdheim-Gsell cystic medial necrosis of the aorta (below).

Transverse (A) and coronal (B) spin-echo images demonstrating aneurysm of the ascending aorta (Ao). (C) Breath-hold, three dimensional, magnetic resonance, gadolinium enhanced angiography with surface rendering reconstruction confirming aneurysm of the ascending aorta ending before the brachiocephalic artery. (D) Coronal cine gradient echo image in diastole demonstrating flow void in the left ventricle corresponding to severe aortic regurgitation (arrow).

Light microscopy of operative material of the ascending aorta with Van Gieson Elastine–Halcian blue stain at × 200 magnification, showing fragmentation of elastic fibres (curved arrow) and accumulation of extracellular matrix (straight arrow).