Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome

Abstract

Marfan's syndrome is a systemic disorder of the connective tissue caused by mutations in the extracellular matrix protein fibrillin-1, with aortic dissection and aneurysm being its most life-threatening manifestations. Kidney transplantation for end-stage renal disease (ESRD) in patients with Marfan's syndrome has not been reported in the literature, and the rate of the incidence of dissection or aneurysm in the iliac artery is unknown. Here, we present a patient with Marfan's syndrome with ESRD due to severe renal ischemia caused by massive bleeding from thoracoabdominal aortic dissection leading to transplant surgery of a living kidney procured from the patient's mother. After kidney transplantation, the renal function normalized without vascular complications, and stable graft function along with negative results for both microhematuria and proteinuria continued for two years. Also, vascular complication such as aneurysm or dissection of the iliac artery was not observed using ultrasonography during the follow-up period. ESRD patients with Marfan's syndrome might be suitable for kidney transplantation, but long-term and careful observations are needed.

Figure 1

Three-dimensional computed tomography scan demonstrating no dissection or aneurysm in the iliac and external iliac arteries. (a) Anterior to posterior imaging. (b) Right-to left-sided imaging. R-IIA: right internal iliac artery; R-EIA: right external iliac artery.

Figure 2

(a) The left renal vein of donor kidney anastomosed to the right external iliac artery end-to-side with a continuous suture. (b) The renal artery anastomosed to the right iliac artery by end-to-side, continuous and interrupted sutures. (c) All anastomoses completed right before the declamping of vessels.(d) There was no bleeding at the anastomoses, and blood flow to the renal artery was stable.