title: Aneurysms due to intimomedial mucoid degeneration.
abstract: OBJECTIVE
  Review management of patients presenting with aneurysms due to intimomedial mucoid degeneration (IMMD).
  
  
  METHODS
  Retrospective analysis.
  
  
  METHODS
  Metropolitan Vascular Service, Departments of Haematology and Pathology, University of Natal.
  
  
  METHODS
  Case records reviewed over an 11 year period. Twenty-two patients were submitted for surgery. All had histological confirmation of disease. All patients were black African (Negro) descent and nineteen were female. Average age was 52 years.
  
  
  RESULTS
  Fifteen patients had involvement of descending aorta and seven patients had extra-aortic involvement. Hypertension was noted in half of the patients. Seven patients presented acutely with rupture (4) and ischaemic lower limbs due to dissection (3). Total absence of clot within aneurysm lumen was a distinctive finding on ultrasound, CT-scan and angiography. Treatment followed standard vascular surgical principles. Three patients died following emergency aortic replacement. Three patients developed severe intraoperative bleeding problems following elective aortic aneurysm resection due to primary fibrinolysis. All had normal preoperative coagulation profiles.
  
  
  CONCLUSIONS
  IMMD is a rare disease affecting the intima and media of vessel wall involving aortic and extra aortic vessels, predominantly in younger African females with hypertension. Lack of clot within aneurysm lumen should alert surgeon to predisposition toward bleeding diathesis. Meticulous suture technique is essential as aortic wall is friable. Medium to long term results are satisfactory.
authors.affiliation: Metropolitan Vascular Service, Medical School, University of Natal, Durban, South Africa.
authors.forename: A T
authors.initials: AT
authors.lastname: Abdool-Carrim
authors.forename: J V
authors.initials: JV
authors.lastname: Robbs
authors.forename: A M
authors.initials: AM
authors.lastname: Kadwa
authors.forename: G
authors.initials: G
authors.lastname: Kenoyer
authors.forename: K
authors.initials: K
authors.lastname: Cooper
mesh_terms: D000328:Adult; D000368:Aged; D000783:Aneurysm / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D044383:Black People; D001807:Blood Vessel Prosthesis; D005260:Female; D005500:Follow-Up Studies; D006025:Glycosaminoglycans / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013019:South Africa; D017539:Tunica Intima / Q000378:metabolism / Q000473:pathology*; D017540:Tunica Media / Q000378:metabolism / Q000473:pathology*
chemical_list: D006025:Glycosaminoglycans