Abstract
Background and Aims
Renal transplantation is considered the most effective and less costly modality of renal replacement therapy in patients with end stage renal disease. The disparity between kidney allografts and recipients has led to a global effort to increase the pool of kidney donors. Accordingly, fibromuscular dysplasia (FMD) is no longer considered an absolute contraindication for kidney donation. The incidence of FMD is about 2.3%-5.8% in potential kidney donors. There are few cases in the literature where renal artery stenosis in allografts with known pre-transplantation FMD became worse after transplantation, indicating the importance of a proper follow up in the recipients.
This is a case of a living kidney donor with no history of hypertension, proteinuria or elevated serum creatinine, whose intra-arterial digital subtraction angiography revealed FMD lesions in the left renal artery.
Method
Case report
Results
A 54-year-old Caucasian female with medical history of hypothyroidism took the decision to offer her kidney to her 37-year-old son who was diagnosed with end-stage renal disease five years ago secondary to diabetes mellitus type I. She had no history for diabetes, hypertension and renal disease. Her vital signs on admission were heart rate of 78 beats/min and blood pressure of 130/70 mmHg. Urinalysis, biochemical profile and serological evaluations were all within normal ranges. Blood urea was 36 mg/dL and serum creatinine was 0.6 mg/dL (eGFR 97ml/min/1.73m2). The abdominal ultrasound and renogram with Tc-99m DTPA showed no remarkable findings. On intra-arterial digital subtraction angiography an abnormal succession of dilatations and multifocal stenoses of the left renal artery, characteristic of medial FMD, was found. The right renal artery was normal.
Apart from a dysfunctional permanent left femoral catheter, the patient had no other vascular access for hemodialysis because of Superior Vena Cava syndrome, so he needed urgent transplantation.
Taking all of these into consideration, the patient was offered renal transplantation as the best option. A left open donor nephrectomy was performed; the renal artery was divided distal to the stenotic dysplastic area. The allograft was placed at the right iliac fossa of the recipient with arterial and venous anastomosis to the extrarenal iliac vessels. Post-operatively, the recipient had a delayed graft function lasted 13 days. On renal artery Doppler in the allograft we found increased resistance index (RI) that gradually normalized without any intervention. An immunosuppressive regiment of tacrolimus, mycophenolate and prednisone was administered according to our center protocol. At discharge serum creatinine was 1.7 mg/dL (eGFR: 50ml/min/1.73m2).
At the year follow-up, the donor was normotensive and had near normal renal function (Cr:1.3mg/dL, eGFR: 70ml/min/1.73m2). The recipient has a well-controlled blood pressure receiving two antihypertensive drugs and maintains a satisfactory renal function.
Conclusion
Few cases with FMD in renal allografts from living and deceased donors have been described. In a review of 4 studies the authors concluded that the outcome of transplantation with allografts from living donors with medial FMD was satisfactory and these allografts could be used to increase the donor pool. Furthermore, it is strongly recommended to have a thorough pre-transplantation check of the donor as well as a close monitoring of both the donor and recipient after transplantation. This case shows that allografts harvested from carefully selected donors with renal arterial FMD can be successfully used, particularly in urgent conditions. Detailed pre-tranplantation imaging of donor’s renal arteries, selection of the appropriate screening method, as well as close monitoring of both donor and recipient for early interventions after transplantation is of paramount importance.
Case report of fibrous dysplasia in a pediatric patient: importance of adequate treatment to avoid malignization
