PACHAR, J.V. et al. Aortic arch aneurysm secondary to medial degeneration: Report of a case presenting as sudden juvenile death. []. , 16, 3, pp.161-165. ISSN 1988-611X.

Thoracic aortic aneurysms are a significant cause of death in Western countries. Frequently, sudden unexpected death due to acute dissection and/or rupture of the aorta is the first manifestation of a silent disease with progressive aortic dilatation. These situations in young people, and without atherosclerotic changes of aortic wall, are suspicious for genetic disorders. However, there are other possible causes of the dilatation and subsequent rupture/dissection of the thoracic aorta, being the most frequent the idiopathic medial degeneration, also called Erdheim-Gsell syndrome or Erdheim Disease. We report a case of sudden death in an 18-year-old woman with no medical history and no marfanoid habitus, secondary to the rupture of a saccular aneurysm of the thoracic aorta. Histological examination confirmed the existence of a degeneration of the media layer of the aorta, without another abnormality. Genetic studies for collagenopathies conducted in her sister were negative. The rupture of a thoracic aortic aneurysm due to idiopathic medial degeneration was established as the cause of death.

: Thoracic aortic aneurysm; Rupture; Idiopathic medial degeneration; Sudden death; Forensic pathology.

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