Cystic median necrosis Erdheim-Gsell

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The Erdheim-Gsell cystic median necrosis ( Erdheim-Gsell syndrome ), sometimes also called Erdheim-Gsell cystic median degeneration , is a pathological change in the large arteries (arteries), especially the aorta . It is also referred to synonymously as Erdheim-Gsell's disease , mucoid-cystic median necrosis or idiopathic cystic median necrosis . Erdheim-Gsell cystic median necrosis is characterized by a loss of smooth muscle fibers and elastic fibers in the arterial wall. It is a predisposing factor for arterial dissections , i.e. splitting of arterial vessel wall layers, with the formation of an aneurysm dissecans . About 8 to 10 percent of all aortic aneurysms are caused by Erdheim-Gsell's cystic median necrosis. The ascending aorta and the aortic arch are primarily affected .

Cause and development of the disease

The exact cause of the cystic median necrosis is usually not known, so it is referred to as idiopathic genesis. Congenital connective tissue diseases such as Marfan's syndrome , endocrinological diseases such as underactive thyroid ( hypothyroidism ) and hypercortisolism as well as long-term arterial high blood pressure are considered to be less common causes .

The disease leads to apoptosis , that is, programmed cell death of the muscle cells in the arterial wall. As things stand today, the term necrosis is therefore incorrect. Necrosis is rarely detectable. In addition to the loss of cells, there is also a loss of elastic fibers . This and the apoptosis result in gaps in the vascular wall that are filled with a mucoid substance or mucopolysaccharide , so that the term " cystic " in the name of the disease is also considered incorrect. The vascular wall is weakened by the filled gaps and can therefore tear more easily. The splitting of the vascular wall in the area of ​​the media , a vascular wall layer, leads to the formation of a dissecting aneurysm ( aneurysm dissecans ).

Medical history

The disease was named after the physicians Otto Gsell (1902–1990) and Jakob Erdheim (1874–1937). Gsell introduced the term cystic median necrosis of the aorta in 1928. In 1929 Erdheim named the three main characteristics from a histopathological point of view: non-inflammatory loss of smooth muscle cells in the vascular wall, fragmentation of the elastic fibers and an increase in the basophilic substance in these areas.

Individual evidence

  1. ↑ a b c d e U. N. Riede; M. Werner, N. Freudenberg: Basic knowledge general and special pathology , Heidelberg, Springer Verlag , 2009, ISBN 978-3540792130 , p. 207
  2. ↑ Carlos Thomas: Special Pathology. Schattauer Verlag, 1996, ISBN 978-3794517138 , p. 201.
  3. ↑ J Freyschmidt: manual diagnostic radiology: Cardiovascular System. Berlin, Springer 2007, ISBN 978-3540414209 , p. 144.
  4. ↑ a b T. M. Tadros, MD Klein, OM Shapira: Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. In: Circulation , Volume 119, Number 6, February 2009, pp. 880-890, ISSN  1524-4539 . doi : 10.1161 / CIRCULATIONAHA.108.795401 . PMID 19221231 . (Full text freely accessible).
  5. ↑ a b H. Bankl: Arbeitsbuch Pathologie: special Pathologie , Facultas, 1998, ISBN 978-3850764704 , p. 48.
  6. ↑ MG Lewis: Idiopathic Medionecrosis Causing aortic incompetence in: British medical journal. Volume 1, Number 5448, June 1965, pp. 1478, ISSN  0007-1447 . PMID 14288088 . PMC 2166656 (free full text). (Full text freely accessible).

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