Gsell-Erdheim syndrome

Alternative eponyms

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A historic term for idiopathic necrosis of the medial layer of the aorta with subsequent formation of cysts, without inflammatory changes, often leading to aortic rupture.

Description

A historic term for idiopathic necrosis of the medial layer of the aorta with subsequent formation of cysts, without inflammatory changes, often leading to aortic rupture. Assumed to be a frequent cause of aneurisma dissecans aorta - aorta dissection, a dramatic sequence of aortic rupture. Inheritance is autosomal dominant.

The first clinical observations and diagnosing was done already in 1856 by Swaine and Lathan. The first comprehensive in-depth clinical description of the disease picture was made by Gsell in 1928, while Erdheim in 1929 worked over the pathological anatomy and coined the term «medionecrosis aortae idiopathica».

Bibliography

  • O. Gsell:
    Wandnekrosen der Aorta als selbständige Erkrankung und ihre Beziehung zur Spontanruptur. [Virchow’s] Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1928, 270: 1-36.
  • J. Erdheim:
    Medionecrosis aortae idiopathica (cystica). [Virchow’s] Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1929, 273: 454-479. Medionecrosis aortae idiopathica cystica. [Virchow’s] Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1930, 276: 187-229.

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