RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164071776
T1 Gsell-Erdheim Syndrome
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accesspediatrics.mhmedical.com/content.aspx?aid=1164071776
RD 2023/09/30
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.