Renal Transplantation for Fibromuscular Dysplasia

Abstract

This is the first report that presents renal transplantation after bilateral nephrectomy as the final treatment for severe renovascular hypertension due to fibromuscular dysplasia (FMD). We describe the history of a 1-year-old girl who suffered from renovascular hypertension due to FMD. Imaging revealed multiple bilateral stenoses of the renal artery extending into the distal branches. The hypertension proved unresponsive to pharmacologic treatment and the intrarenal peripherally located stenoses rendered a conventional approach such as transluminal or surgical angioplasty not feasible. At the age of 5 years, a unilateral nephrectomy of the most affected kidney was performed, but she remained hypertensive and developed progressive cardiomyopathy and retinopathy. At the age of 6 years the remaining kidney was removed, followed by a living related renal transplantation with a kidney donated by her mother. Posttransplantation, she developed mild hypertension due to a postanastomotic stenosis, which was easily controlled with antihypertensives. Now 8 years after transplantation, she has experienced no further blood pressure related problems. Although there is a risk of recurrence of FMD after performing a living related transplantation, our report suggests that this procedure is relatively safe, provided appropriate preoperative evaluation and follow up is performed.

Living Donor Transplantation of Kidneys with Fibromuscular Dysplasia: Indications, Surgical Techniques and Long Term Results in 11 Cases

“…No signs of FMD were observed in the remnant kidney of our LDs ,as was the case for the patients of Knops [7], Serrano [12], Nahas [13], Wolters [14], Kolettis [16], Balzer [17], and Blondin [18], whilst other authors [1,4,6] each noted signs of hypertension and FMD in the remnant kidney of one LD.…”

“…However, the majority of authors (75%) reporting on donation from LDs affected by FMD [1,[3][4][5][6][7][10][11][12][13][14][15] account for a maximum of 3 cases.…”

“…FMD still represents a challenge for any decision to harvest the kidney, particularly from a LD, due to the potential risk to develop a renovascular hypertension and/or FMD either in the remnant kidney after donation [2,3,1,4] or in the recipient's graft [5][6][7]. Moreover, the problem may only arise at the operative stage since early forms of FMD give rise to no or mild radiographic evidence.…”

Kidney transplantation in infantile myofibromatosis and fibromuscular dysplasia: a case report

“…There is only one other reported case of successful KT in a patient with FMD in the medical literature, which showed good kidney function after 8 years of follow-up and blood pressure normalized with only enalapril [ 6 ]. However, that transplantation was performed in a 6-year-old patient, without a history of infantile myofibromatosis, chemotherapy or peritoneal dialysis and without anastomosis on a repaired vessel.…”

“…FMD is likely to be, at least in part, a genetic disorder [ 4 ]. It is the biggest cause of infantile renovascular hypertension, for which first treatment consists of antihypertensive medication and, in case of inefficacy, percutaneous transluminal angioplasty [ 6 ].…”