TY - CHAP M1 - Book, Section TI - Gsell-Erdheim Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Aneurysms and dissections of the aorta usually result from degenerative changes in the aortic wall. Thoracic aortic aneurysms and dissections are primarily associated with a characteristic histologic appearance known as “medial necrosis” or “Erdheim cystic medial necrosis” which is characterized by degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and accumulation of basophilic ground substance. The association between congenital bicuspid aortic valve and medial necrosis of the aorta has been suggested. Ectopia of the pigment layer of the iris onto the anterior surface of the iris has been associated with this medical condition. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164071776 ER -