open access

Vol 22, No 1 (2016)
Case reports
Published online: 2016-09-29

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

Aleksander Lukasiewicz, Andrzej Marszalek
DOI: 10.5603/AA.2016.0004
·
Acta Angiologica 2016;22(1):16-19.

open access

Vol 22, No 1 (2016)
Case reports
Published online: 2016-09-29

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 article
Title

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

Journal

Acta Angiologica

Issue

Vol 22, No 1 (2016)

Pages

16-19

Published online

2016-09-29

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

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