scholarly journals Spontaneous rupture of abdominal aorta — an unusual manifestation of an idiopathic cystic medial necrosis (Gsell-Erdheim syndrome) and literature overview

2016 ◽  
Vol 22 (1) ◽  
pp. 16-19
Author(s):  
Aleksander Lukasiewicz ◽  
Andrzej Marszalek
Keyword(s):  
Abdominal Aorta ◽  
Spontaneous Rupture ◽  
Cystic Medial Necrosis ◽  
Unusual Manifestation ◽  
Literature Overview ◽  
Medial Necrosis

Cystic medial necrosis Erdheim Gsell as a rare reason for spontaneous rupture of the ascending aorta

VASA ◽  
2011 ◽  
Vol 40 (2) ◽  
pp. 147-149 ◽  
Author(s):  
Gary ◽  
Seinost ◽  
Hafner ◽  
Gorkiewicz ◽  
Brodmann
Keyword(s):  
Sudden Death ◽  
Spontaneous Rupture ◽  
Ascending Aorta ◽  
Atheromatous Plaque ◽  
Cystic Medial Necrosis ◽  
Threatening Condition ◽  
Life Threatening ◽  
Medial Necrosis

Spontaneous rupture of the ascending aorta is a life-threatening condition requiring immediate intervention. The rupture usually leads to sudden death as a result of hemopericardium or hemothorax. The underlying histopathological condition in the cases described so far was mostly an atheromatous plaque. Some other rare underlying conditions were also described. We report a case of cystic medial necrosis Erdheim Gsell as a reason for fatal spontaneous rupture of the ascending aorta.

Cystic medial necrosis Erdheim-Gsell

VASA ◽  
2011 ◽  
Vol 40 (4) ◽  
pp. 336-337
Author(s):  
Leu ◽  
Gary ◽  
Brodmann
Keyword(s):  
Cystic Medial Necrosis ◽  
Medial Necrosis

scholarly journals Fatal aorticMyceliophthora thermophilainfection in a patient affected by cystic medial necrosis

1998 ◽  
Vol 36 (2) ◽  
pp. 113-118
Author(s):  
C. Farina ◽  
A. Gamba ◽  
R. Tambini ◽  
H. Beguin ◽  
J.L. Trouillet
Keyword(s):  
Cystic Medial Necrosis ◽  
Medial Necrosis

CYSTIC MEDIAL NECROSIS AND DISSECTING CEREBRAL ANEURYSMS

1990 ◽  
Vol 49 (3) ◽  
pp. 301
Author(s):  
D. P. Fosmire ◽  
L. Pitts ◽  
C. Jacques ◽  
C. M. Greco
Keyword(s):  
Cerebral Aneurysms ◽  
Cystic Medial Necrosis ◽  
Medial Necrosis

Genetic diseases of the aorta: aortic diseases associated with bicuspid aortic valves

ESC CardioMed ◽  
2018 ◽  
pp. 2613-2615
Author(s):  
Folkert Meijboom ◽  
Gert-Jan Sieswerda
Keyword(s):  
Aortic Wall ◽  
Structural Integrity ◽  
Genetic Diseases ◽  
Basic Research ◽  
Ascending Aorta ◽  
Flow Profile ◽  
Aortic Diseases ◽  
Cystic Medial Necrosis ◽  
Medial Necrosis ◽  
Genetically Determined

There is a genetic cause of bicuspid aortic valve. Dilatation of the aortic root and ascending aorta has long been considered genetically determined too and treated accordingly. In recent years, basic research combined with advanced cardiac imaging has made a strong case for another cause of this dilatation: an altered flow profile in the ascending aorta due to the abnormal geometry of the bicuspid valve, leading to an area of altered wall shear stress, which in turn leads to remodelling of the aortic wall, with apoptosis of vascular smooth muscle cells and disruption of media layer as a result. These histological changes, previously referred to as cystic medial necrosis, form the basis of a loss of structural integrity of the aortic wall, which makes it prone to dilatation, dissection, and rupture.

Giant Circumflex Coronary Artery Aneurysm Associated With Cystic Medial Necrosis in a Non-Marfan Patient

2007 ◽  
Vol 83 (2) ◽  
pp. 668-670 ◽  
Author(s):  
Koji Takeda ◽  
Goro Matsumiya ◽  
Motonobu Nishimura ◽  
Hajime Matsue ◽  
Yasuhiko Tomita ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Coronary Artery Aneurysm ◽  
Artery Aneurysm ◽  
Circumflex Coronary Artery ◽  
Cystic Medial Necrosis ◽  
Medial Necrosis ◽  
Marfan Patient
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