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

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.