Table 4.
Pathology | Mutation | Age | Management | Complications |
---|---|---|---|---|
AVF/ symptomatic | MIN | 28 | Elective Endovascular repair | Alive at age 34 |
AVF/ rupture | MIN | 28 | Emergent open repair | Intraoperative death |
Dissection | MIN | 55 | Elective prophylactic iliofemoral bypass | Alive at age 59 |
Dissection | MIN | 43 | Elective prophylactic iliofemoral bypass | Alive at age 48 |
Aneurysm/dissection | HI | 27 | Elective prophylactic aortobiiliac repair | Alive at age 26 (case in figure 6) |
Dissection/ rupture | MIN | 42 | Emergent open repair | Wound infection, alive at age 50 |
Aneurysm/ rupture | MIN | 22 | Emergent open repair | Intraoperative death |
AVF: Arteriovenous fistula. MIN: mutations that lead to minimal normal type III collagen production. HI: mutations that lead to 50% normal type III collagen production.