ANEURYSMS DUE TO INTIMOMEDIAL MUCOID DEGENERATION

Objective: Review of management of patients presenting with aneurysms due to intimomedial mucoid degeneration (IMMD). Design: Retrospective analysis. Setting: Metropolitan Vascular Service Departments of Haemat ology and Pathology, University of Natal. Materials and methods: Case records reviewed over an 11 year period. Twenty-two patients were subm itted for surgery. All had histological confirmation of disease. All p atients were black African (Negro) descent and nineteen were female. A verage age was 52 years. Results: Fifteen patients had involvement of descending aorta and seven patients had extra-aortic involvement. Hype rtension was noted in half of the patients. Seven patients presented a cutely with rupture (4) and ischaemic lower limbs due to dissection (3 ). Total absence of clot within aneurysm lumen was a distinctive findi ng on ultrasound, CT-scan and angiography. Treatment followed standard vascular surgical principles. Three patients died following emergency aortic replacement. Three patients developed severe intraoperative bl eeding problems following elective aortic aneurysm resection due to pr imary fibrinolysis. All had normal preoperative coagulation profiles. Conclusion: IMMD is a rare disease affecting the intima and media of v essel wall involving aortic and extra aortic vessels, predominantly in younger African females with hypertension. Lack of clot within aneury sm lumen should alert surgeon to predisposition toward bleeding diathe sis. Meticulous suture technique is essential as aortic wall is friabl e. Medium to long term results are satisfactory.