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Spontaneous rupture of abdominal aorta — an unusual manifestation of an idiopathic cystic medial necrosis (Gsell-Erdheim syndrome) and literature overview
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Abstract
In this report, a case of a male, 40-year-old patient who suffered from spontaneous rupture of undilated abdominal aorta, a manifestation of idiopathic cystic media necrosis (Gsell-Erdheim syndrome), is presented. Spontaneous ruptures of medium-to-large arteries observed in this syndrome are a consequence of elastic fibres degeneration and glucose-amino-glycans (GAG) depositions in the arterial media. Gsell-Erdheim syndrome is also observed in a large portion of spontaneous aortic dissections. Histopathological criteria for the diagnosis of this disease, as well as clinical implications for current treatment, especially endovascular procedures in the aortic dissections, are discussed.
Abstract
In this report, a case of a male, 40-year-old patient who suffered from spontaneous rupture of undilated abdominal aorta, a manifestation of idiopathic cystic media necrosis (Gsell-Erdheim syndrome), is presented. Spontaneous ruptures of medium-to-large arteries observed in this syndrome are a consequence of elastic fibres degeneration and glucose-amino-glycans (GAG) depositions in the arterial media. Gsell-Erdheim syndrome is also observed in a large portion of spontaneous aortic dissections. Histopathological criteria for the diagnosis of this disease, as well as clinical implications for current treatment, especially endovascular procedures in the aortic dissections, are discussed.
Keywords
cystic medial necrosis of aorta, complications, aortic diseases
About this articleTitle
Spontaneous rupture of abdominal aorta — an unusual manifestation of an idiopathic cystic medial necrosis (Gsell-Erdheim syndrome) and literature overview
Journal
Issue
Article type
Case report
Pages
16-19
Published online
2016-09-29
Page views
1055
Article views/downloads
1403
DOI
10.5603/AA.2016.0004
Bibliographic record
Acta Angiologica 2016;22(1):16-19.
Keywords
cystic medial necrosis of aorta
complications
aortic diseases
Authors
Aleksander Lukasiewicz
Andrzej Marszalek