4. A 64-year-old lady has finished the workup as a kidney donor to her husband. No proteinuria and has excellent kidney function for her age (EDTA GFR is 78 ml/min). No history of hypertension, DM or any history that contraindicates donation. Her cardiac ECHO is satisfactory. Her CT angiogram is shown below. She came to see you to discuss the results of these tests.
- Please describe the CT finding
- How would you proceed further?
Thank you, All
Please read Ibrahim et al paper. The decision is not that easy. I will accept the donor if the disease is mild, localised for a donor above 50, not hypertensive with excellent kidney function.
Yes, after informed consent and careful follow-up after donation.
Evaluation of other vascular beds are necessary, too.
Thank you, All
Will you advise this donor to go ahead and donate?
What are the criteria that should be fulfilled to accept a kidney from an FMD donor?
There is a reward for the best answer
if we can do safe reconstructive surgery for renal artery which is affected so we can use this for donation in order to extend donor pool as donation from FMD of renal artery is contraindicated for donation.
Yuto Matsushita, Daisuke Motoyama, Toshiki Ito, Takayuki Sugiyama, Atsushi Otsuka, Masaki Sano, Kazunori Inuzuka, Hideaki Miyake. Kidney transplantation from a living donor with renal artery fibromuscular dysplasia: A case report on arterial grafting of the donor renal artery. IJU Case Rep. 2020 Sep; 3(5): 201–203.
Thank you
Will you advise this donor to go ahead and donate?
Yes, however, I will counsel her in detail regarding the diagnosis of FMD and the slightly higher risk of developing hypertension or proteinuria (1).
What are the criteria that should be fulfilled to accept a kidney from an FMD donor?
Donors with FMD can be accepted for kidney donation provided that they are meticulously investigated for the presence of other vascular tree lesions or any other relative contraindications like HTN.
If the patient had several potential kidney donors, then the one with FMD should be considered the last option (1). In addition, kidney donors with FMD should be evaluated at least annually for early detection and management of HTN or impairment of kidney functions (1).
References:
1) Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545.
Thank you
Donor age, measured GFR of 78/ml seems to correlate with creatinine less than 0.9 mg/dl (78 mmol/L). Although FMD causes, renovascular HT (not present !?) is considered unsuitable for transplantation except when unilateral and grafted, in our donor we need to make sure about HT by documenting measurements and retinal examination. Supposing no real HT is present, we may accept this donor as a special situation with close monitoring.
After discussion, I would prefer the right kidney (as it seems smaller?!). It will be eligible to have the differential GFR.
I would not accept I the donor was young in his/her 30s or 40s because of the unprdictable scenario of HT at that time.
If you refuse a similar donor 30 or 40 years ,what if this lady lives for another 10 years.
Thank you, Prof. Dawlet Belal; Here, the clinic is silent, and the findings are incidental. No HT till the moment. What I meant in my comment is being a young female, especially of childbearing age, one could not predict the clinical scenario. we can not risk the donor primarily. But case by case, as in our scenario, no previous symptoms, no HT no end-organ injury. additionally, the age is over 60, the patient is keen . after evaluation by CT angiography routinely done already before transplantation, we may proceed in case of no luminal narrowing.
If there is no other donor available, this donor can be taken up due to:
1) Age: 64 years
2) No history of hypertension
3) No proteinuria with GFR 78 ml/min
4) No history of DM, normal ECHO and no other contraindication for organ donation
The CT angiogram shows bilateral FMD. Studies have shown that even donors with bilateral FMD have results similar to non-FMD donors (1,2).
The points to consider while accepting a donor with FMD include (1):
1) No other suitable living kidney donor.
2) Mild FMD disease.
3) No or mild hypertension.
4) Few or no ‘classical’ cardiovascular risk factors (smoking, obesity, dyslipidemia, etc.).
5) Unilateral or predominantly unilateral renal FMD lesions (in this case, the unaffected or less affected artery/kidney should be left in place).
6) No or mild FMD lesions in other arterial beds.
7) No dissection or aneurysm of renal or extra-renal arterial beds (head-to-pelvis CT or MR-angiography required).
8) No pregnancy considered after donation.
9) Written informed consent from both donor and recipient.
10) Agreement from both donor and recipient to be included in a prospective long-term registry.
11) Life-long annual follow-up for detection of hypertension and renal dysfunction
References:
1) Chrysochou C, Devresse A, Kanaan N, Persu A. Kidney donors with fibromuscular dysplasia, is it time to open the doors? Nephrol Dial Transplant. 2021 Jul 23;36(8):1365-1368. doi: 10.1093/ndt/gfab181. PMID: 33999202.
2) Indudhara R, Kenney, Bueschen AJ, Burns JR. Live donor nephrectomy in patients with fibromuscular dysplasia of the renal arteries. J Urol. 1999 Sep;162(3 Pt 1):678-81. doi: 10.1097/00005392-199909010-00010. PMID: 10458340.
Well done what is your decision.
If there is no other donor available, this donor can be taken up due to:
1) Age: 64 years
2) No history of hypertension
3) No proteinuria with GFR 78 ml/min
4) No history of DM, normal ECHO and no other contraindication for organ donation
The CT angiogram shows bilateral FMD. Studies have shown that even donors with bilateral FMD have results similar to non-FMD donors.
I will accept the donor if the disease is not present at any other site, and is mild
Before donation vascular anatomy of the donor kidney should be evaluated using CTA or MRA , the decision to donate kidney with abnormal arteries is discussed in a case by case and is not considered absolute contraindication
FMD is not uncommon and can be seen in 2.0–6.6% of donors during evaluation, unilateral is more common than bilateral and is commonly affecting Rt more that Lt kidney.
FMD may be focal or diffuse, renal arteries involvement in 75%, carotid arteries involvement in 74% and vertebral arteries involvement in 33% of cases. So patient with FMD are at increased risk of severe HTN, cerebrovascular stroke or TIA and evaluation of patients with FMD should include CT angiography from head to pelvis once after diagnosis
FMD are classified according to angiographic appearance into focal (single focal stenosis) or multifocal (string of beads appearance) and since there is growing evidence that focal and multifocal FMD are distinct entities (multifocal FMD is not caused by progression of focal FMD) so focal FMD donors will not progress to multifocal disease (1,2).
No clear recommendations regarding eligibility for donation in patient with FMD. But because renal artery FMD may be associated with hypertension and renal dysfunction in both donor and recipient so it is considered by KIDIGO guidelines a contraindication to transplantation
Previous studies reported variable results regarding hypertension development following donation in patient with FMD, some reported increase and other reported no increase in the incidence of HTN post-donation but these studies were small and has limited follow up period
Another retrospective study evaluating 113 kidney donors with FMD regarding the development of HTN, CVD and CKD and compared them to 452 matched donors without FMD found no increase in the risk of death, HTN, CVD, proteinuria or reduction of GFR in donors with FMD compared to those without after 15.5 ± 8.9 years of donation
So … Patients with FMD can be included in donation as there is no increase in the risk of HTN, CVD and CKD after donation if selected well
Criteria that should be fulfilled to accept a kidney from an FMD donor
So … kidney donation in patients with FMD is considered contraindicated in severe, diffuse and symptomatic disease, in case of accepting the donor, it should be considered if there are no alternatives and the risk should be discussed with the donor especially female in child bearing period
References
1- Lüscher TF , Lie JT , Stanson AW et al. Arterial fibromuscular dysplasia. Mayo Clin Proc 987; 62: 931–952
2- Schreiber MJ , Pohl MA , Novick AC. The natural history of atherosclerotic and fibrous renal artery disease. Urol Clin North Am 1984; 11: 383–392
3- McKenzie GA , Oderich GS , Kawashima A et al. Renal artery fibromuscular dysplasia in 2,640 renal donor subjects: a CT angiography analysis. J Vasc Int Radiol 2013; 24: 1477–1480
Really excellent, very comprehensive
But again the difficult question will you accept this donor
First, I ill asses the extent of the disease including visceral vascular bed using CT angiography of the whole body, if the patient has a localized disease I will accept this donor kidney (most probably the right) since she is not symptomatic and the lesion is not severe, provided that there is no alternative for the recipient
https://academic.oup.com/ndt/article/17/1/153/1838177#:~:text=JOURNAL%20ARTICLE,Karl%E2%80%90Heinz%20Dietl
yes, I will accept this donor if there is no other donors
it can be safely donate as :
old age with no Dm, no HTN , no proteinuria, normal ECHO
so I will accept this donor if the disease not present at any other sites
the most likely way to accept the donor is the possibility of performing reconstructive surgery using a vessel from the recipient to fix the renal artery.
The criteria to accept donors with FMD is :
– No other suitable living donor.
– Mild FMD disease.
– No or mild HTN.
– Few or no classical cardiovascular risk factors ( smoking, obesity, dyslipidemia…etc)
– Unilateral or predominant unilateral FMD disease – the unaffected or less affected should be left in the donor).
– No FMD, aneurysms or dissection in other arterial beds screened by head to pelvis CT angiography or MRA.
– No pregnancy considered after donation.
– Written consent from the donor and recipient should be taken after discussing the risk and outcomes.
– Agreement from both to be included in a prospective long term registry.
FMD is contraindicated for kidney transplant but if donor old age with normal blood pressure and renal function and medial type of FMD and shortage of donor pool so will accept for donation.
Reference: Yuto Matsushita, Daisuke Motoyama, et al: :Kidney transplantation from a living donor with renal artery fibromuscular dysplasia: A case report on arterial grafting of the donor renal artery; IJU Case Rep. 2020 Sep; 3(5): 201–203.
Criteria for donation:
Will you advise this donor to go ahead and donate?
I would advise donation in this case and would inform the donor that kidney donors with FMD appear to do well, do not appear to incur increased risks of hypertension, proteinuria, CVD or reduced eGFR
At the same time, the donor needs to at least screen annually for early hypertension detection and signs of kidney function decline
What are the criteria that should be fulfilled to accept a kidney from an FMD donor?
Chrysochou C, Devresse A, Kanaan N, Persu A. Kidney donors with fibromuscular dysplasia, is it time to open the doors?. Nephrology Dialysis Transplantation. 2021 Aug;36(8):1365-8.
Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrology Dialysis Transplantation. 2021 Aug;36(8):1538-45.
The CT angiogram findings have bilateral string of bead appearance classical of Fibromuscular dysplasia….
She is a 64 year old donor with no other comorbidities with normal blood pressure and no proteinuria…her FMD is mild and not causing episodes of high BP and pulmonary edema…The disease seems asymptomatic
As per the KDIGO guidelines donors with athersclerotic or FMD should not donate…
But on a individualized case based approach can be considered based on few retrospective study
The RELIVE study compared the long term outcomes of 113 kidneys from donors with FMD with age matched controls.. They reported excellent outcomes for donors with FMD with no worsening of renal functions or proteinuria or hypertension…They concluded that patients with FMD with no other vascular bed involvement may donate kidneys…
Based on the above literature,, I will counsel the donor about a small risk of worsening of hypertension and proteinuria later in life..If she is the only available donor, she can be taken up as no other end organ damage is visible at this moment after informed consent
Renal artery fibromuscular dysplasia (FMD) is generally considered a contraindication of kidney transplantation since FMD occasionally induces hypertension or renal insufficiency.
Pt has unilateral FMD
We can accept the FMD pt for donation if;Localized ,focal ,silent(no HTN),unilateral,no >50% involvement
FMD. Proper counseling both couple. Shared decision with the couple whether to proceed for kidney transplant.
Please describe the CT finding
Beaded appearance of both renal arteries –classical picture of fibromuscular dysplasia.
How would you proceed further?
KDIGO guidelines recommended not to accept FMD patients for donation ,however this is not graded recommendation as literature review on this topic is scarce .Such patients with FMD can be considered for donation only after taking informed consent from both donor and recipient and explaining all risks if no other suitable donor available and have unilateral mild FMD disease ,have no cardiovascular risk factors ,and hypertension and no aneurysm in extra renal arterial bed and pregnancy not planned in the future. Literature review revealed that few studies stated similar long-term outcomes in FMD donors to non-FMD donors in terms of renal and cardiovascular outcomes.
Regarding the above case of 64 year female with FMD and no comorbid condition and with no proteinuria and having excellent kidney function should undergo head-to-pelvis vascular scanning to rule out beaded appearance in other arterial beds before considering for donation. Also age to be considered as this age place the patient in extended criteria donor.
At our center, we do not take FMD patients for donation.
REFERENCE:
Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545
bilateral fibromuscular dysplasia FMD
Presence of incidentally detected fibromuscular dysplasia (FMD) is a challenging and controversial issue for selection of living kidney donors (LKD). Whether donors with FMD differ regarding blood pressure and kidney function after donation, and whether allograft outcomes over the long term are adversely affected are not known. Current guidelines do not provide recommendations regarding the use of kidneys from donors with FMD and there is a paucity of data on the outcomes of these donors.
Many studies found that Kidney donors with FMD appear to do well, do not appear to incur increased risks of hypertension, proteinuria, CVD or reduced eGFR, and perhaps carefully selected candidates with FMD can safely donate as long as involvement of other vascular beds is ruled out. Allograft outcomes for recipients of FMD kidneys are similar to those of all other living donors. All this results support living kidney donation from incidentally detected FMD.
Suarez MGonzalez, Amer H, Jaffer I, Vrtiska T, Rule A, Prieto M, Textor S. Fibromuscular Dysplasia in Living Kidney Donors: Characteristics and Recipient Outcomes. [abstract]. Am J Transplant. 2017; 17 (suppl 3). https://atcmeetingabstracts.com/abstract/fibromuscular-dysplasia-in-living-kidney-donors-characteristics-and-recipient-outcomes/. Accessed December 29, 2022.
Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545. doi: 10.1093/ndt/gfab039. PMID: 33566102.
1.CT showed fibromuscular dysplasia of the renal arteries. 2.I will proceed to transplantation after informed consent.
Although renal artery FMD is usually regarded as a contraindication of kidney transplantation considering the potential risks of postoperative hypertension and renal insufficiency in both the donor and recipient, there have been reports of successful cases. Kolettis et al. conducted a retrospective review of a departmental database of renal transplantations, and identified 36 donors with FMD. After transplantation, recipients from these donors achieved functional outcomes similar to those from non‐FMD donors.
criteria for acceptance
No extrarenal involvement
Normal blood pressure
Medial type FMD
If the affected renal artery segments could be precisely reconstructed.
Matsushita Y, Motoyama D, Ito T, Sugiyama T, Otsuka A, Sano M, Inuzuka K, Miyake H. Kidney transplantation from a living donor with renal artery fibromuscular dysplasia: A case report on arterial grafting of the donor renal artery. IJU Case Rep. 2020 Jul 4;3(5):201-203.
the CT shows beading of both renal arteries indicating fibromuscular dysplasia
this is a contraindication for transplantation due to risk of hypertension and renal failure in both the donor and recipient.
taking into consideration, these factors are explained to donor and recipient, and unilateral FMD is mild and the donor is elderly and the worldwide shortage of kidney donors we may proceed after informed consent from the donor and recipient
The tourtousity of renal vessels on CT suggests fibromuscular dysplasia. (1)
I will accept this donation in light of(1,2):
References:
Please describe the CT finding
Bilateral nodular renal arteries (string of beads) and bilateral normal sized kidneys are suggestive of fibro-muscular dysplasia (FMD).
How would you proceed further?
I would accept the donor.
According to KDIGO guidelines: donors with atherosclerotic renal artery disease or FMD involving the orifices with both renal arteries should not donate a kidney.
According to RELIVE study: donors with FMD, compared to donors without FMD, have the same long-term renal and cardiovascular outcomes.
FMD can be divided into focal and multi-focal according to extension of distribution.
Whole body from head to toe vascular scanning is recommended to find out the extension of FMD.
Acceptable donors with FMD should fulfill the following criteria:
1- They are the only suitable living donor.
2- Focal FMD.
3- Unilateral renal FMD lesions.
4- No or mild FMD in other arterial beds without any dissection or aneurysm in renal or extra-renal arteries.
5- No or mild hypertension.
6- No or few cardiovascular risk factors (smoking, obesity, dyslipidaemia).
7- No pregnancy is planned after donation.
8- Written informed consent from donor and recipient.
CT angiogram showed bilateral string of beads of renal arteries which indicate the diagnosis of FMD .
The criteria that must be present to accept the donor are :
So to decide whether to accept this offer or no , the answer will be yes I will accept her after make sure that all her vascular tree are normal with no severe involvement by FMD to any vascular bed.
The CT angiography shows diffuse beading of both renal arteries picture suggestive of Bilateral fibro-muscular dysplasia (FMD).
Donor has excellent kidney function according to age ,no HTN, no proteinuria.
She can be accepted as a donor if no other available donors
after counselling her and the recipient about possible development of hypertension.
In potential kidney transplant donors, the incidence of renal artery FMD has been reported to be 2.0–6.6%.
Renal artery fibromuscular dysplasia is generally considered a contraindication of kidney transplantation, since fibromuscular dysplasia occasionally induces hypertension or renal insufficiency in the recipient and/or donor.
If reconstruction of the diseased artery could be safely performed, transplantation using a kidney from a donor with renal artery fibromuscular dysplasia may be considered.
Kolettis et al. conducted a retrospective review of a departmental database of renal transplantations, and identified 36 donors with FMD. After transplantation, recipients from these donors achieved functional outcomes similar to those from non‐FMD donors under adequate preoperative assessment including arteriography.
Based on these findings, they concluded that selected patients with renal artery FMD who showed normal blood pressure and medial‐type FMD could be donors for renal transplantation.
Pfeiffer et al. also found that even kidneys from donors with severe renal artery FMD could be successfully transplanted, if the affected renal artery segments could be precisely reconstructed.
long‐term careful follow‐up for both the donor and recipient after renal transplantation is highly recommended due to possibility of developing postoperative FMD
Yuto Matsushita, Daisuke Motoyama,Kidney transplantation from a living donor with renal artery fibromuscular dysplasia: A case report on arterial grafting of the donor renal artery.IJU Case Rep. 2020 Sep; 3(5): 201–203.
CT scan shows bilateral strings of beads which indicates fibromuscular dysplasia.
These donors will be accepted after written and signed informed consent, if:
She is the only donor, with mild disease without hypertension or other cardiovascular risk factors. She has no plan for further pregnancy.
Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545.
64 old lady, neither hypertensive nor diabetic. Normal cardiac function.
CT angio shows bilateral FMD
This donor can be considered after good counselling with her recipient as well and written consent. In view of her age and If there is no alternative donor, no or minor CV risk such as smokering, obesity, and hyperlipidemia. No aneurysm or dissection in other arteries in CT or MRI angio for body arteries (cervical, chest, abd. pelvis).
CT angiography shows bilateral sting on beads appearance in both renal arteries indicate bilateral fibromascular dysplasia (FMD) of renal arteries.
This potential donor has got no contraindication of donation except this finding. FMD sometimes induce hypertension and renal insufficiency. So, it is typically a contraindication of kidney donation. Transplants are highly risky. Though, there have been some reported success as well.
Donations from individuals with FMD can be accepted if:
-There is no other available kidney donor.
-Mild disease.
-No hypertension
-Unilateral renal FMD lesions
-Involvement of no other major vessels
-Consent from both the donor and the recipient regarding future risks.
CTA showed bilateral multifocal beading of the renal arteries.
kidney donor with FMD should be carefully selected as long as the involvement of other vascular involvement without orifices involvement,however, orifices for FMD are some uncertain as FMD is a multifocal disease so this recommendation is not grated because of lack of evidence in the literature.
so donation is less likely to proceed unless FMD was undetected prior to surgery,the degree of FMD is deemed mild or as last resort.
FMD discusses case by case, especially this below criteria fulfill:
1-no other suitable living donor.
2- mild FMD.
3-mild or no HTN.
4-no CVS risk factors.
5-unilateral FMD,
6- no or mild FMD lesson in other arterial beds.
7-no dissection or aneurysm.
8-no plan for pregnancy after donation
9- consent and counselling.
so for this donor has no HTN no proteinuria no pregnancy in the future, and no CVS risk factors I will counsel and go ahead if she and the recipient agree.
references
Lentine KL , Kasiske BL , Levey AS et al. KDIGO clinical practice guideline on the evaluation and care of living kidney donors. Transplantation 2017; 101: S1–S109Google ScholarPubMedWorldCat
2Mandelbrot DA , Reese PP , Garg N et al. KDOQI US commentary on the 2017 KDIGO clinical practice guideline on the evaluation and care of living kidney donors. Am J Kidney Dis 2020; 75: 299–316Google ScholarCrossrefPubMedWorldCat
These images showed features of FMD with no HTN and proteinuria
this donor can be accepted for donation in the absence of the availability of another donor. This donor should be counselled about the possibility of having HTN in the future
Females with FMD should be counselled about pregnancy as she is after donation cannot get pregnant.
The points to consider while accepting a donor with FMD include (1):
1) No other suitable living kidney donor.
2) Mild FMD disease.
3) No or mild hypertension.
4) Few or no ‘classical’ cardiovascular risk factors (smoking, obesity, dyslipidemia, etc.).
5) Unilateral or predominantly unilateral renal FMD lesions (in this case, the unaffected or less affected artery/kidney should be left in place).
6) No or mild FMD lesions in other arterial beds.
7) No dissection or aneurysm of renal or extra-renal arterial beds (head-to-pelvis CT or MR-angiography required).
8) No pregnancy is considered after donation.
9) Written informed consent from both donor and recipient.
10) Agreement from both donor and recipient to be included in a prospective long-term registry.
11) Life-long annual follow-up for detection of hypertension and renal dysfunction
references
uptodate
Accepting donor with FMD should be individualized, the donor with FMD may be accepted if there is no other suitable donor, FMD is mild, the donor has no HTN and no other cardiovascular risk factors as smoking or obesity.
Head to pelvis CT is needed to exclude presence of FMD lesions in other arterial beds and to exclude presence of dissection or aneurysmal changes of renal or extrarenal arterial beds.
If the FMD lesion is predominantly unilateral, the less affected kidney will be left in place.
A written informed consent is required from both the donor and the recipient.
Chrysochou C, Devresse A, Kanaan N, Persu A. Kidney donors with fibromuscular dysplasia, is it time to open the doors?. Nephrology Dialysis Transplantation. 2021 Aug;36(8):1365-8.
i will accept the donation, because, currently this is unpredictable she may have hypertension in future. also the main artery is affected which may complicate and may develop hypertension in recipient.
CT angiography shows sting on beads appearance in both renal arteries indicative of bilateral fibromascular dysplasia. However, the potential donor has normal renal function, normal blood pressure and no proteinuria.No associated risk factors.
Since fibromuscular dysplasia can sometimes induce hypertension or renal insufficiency in the recipient and/or donor, it is typically regarded a contraindication of kidney transplantation donation. Transplants are risky, however there have been some reported successes.
Donations from individuals with FMD can only be received if:
There is no other viable kidney donor.
Mild FMD disease.
No hypertension or mild hypertension.
No classic risk factors for cardiovascular disease
Unilateral or primarily unilateral renal FMD lesions
In other artery beds, FMD lesions are absent or moderate.
No arterial dissection or aneurysm of the renal or extrarenal beds
After donation, pregnancy is not a consideration.
Written consent from both the donor and the recipient.
Donor and recipient consent to participation in a future long-term registry.
This renogram shows bilateral Renal artery fibromuscular dysplasia. FMD is generally considered a contraindication of kidney transplantation donation since fibromuscular dysplasia occasionally induces hypertension or renal insufficiency in the recipient and/or donor. However, there have been reports of successful transplanted cases.
Moreover, Adrogue HE et al. in 113 kidney donors with FMD discovered during donor evaluation found, No donor with FMD developed an eGFR <30 mL/min/1.73 m2 or end-stage kidney disease and do not appear to incur increased risks of hypertension, proteinuria, or CVD.
References
1- Matsushita Y, Motoyama D, Ito T, Sugiyama T, Otsuka A, Sano M, Inuzuka K, Miyake H. Kidney transplantation from a living donor with renal artery fibromuscular dysplasia: A case report on arterial grafting of the donor renal artery. IJU Case Rep. 2020 Jul 4;3(5):201-203. doi: 10.1002/iju5.12188. PMID: 32914075; PMCID: PMC7469844.
2- Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545. doi: 10.1093/ndt/gfab039. PMID: 33566102.
kidney donors with FMD appear to do well, do not appear to increased risks of hypertension, proteinuria, CVD or reduced eGFR, and perhaps carefully selected candidates with FMD can safely donate as long as involvement of other vascular beds is ruled out.
CT scan showing bilateral beaded appearance of renal arteries suggestive of FMD.
As per guidelines FMD is a contraindication to take these patients as donors, interestingly this patient is completely asymptomatic and blood pressure is also normal which usually is present in 67% of patients .
she can be considered for donation only if there is no other donor
Detailed history should be taken for headache
claudication, TIA , smoking , dyslipidemia etc
patient should undergo whole body angiogram specially to look for carotids.
High risk consent
Its a very difficult decision and must be taken with full caution
Irregularities (beading) of both renal arteries, consistent with fibromuscular dysplasia.
Both KDIGO [1] and KDOQI guidelines state that ‘a donor candidate with atherosclerotic renal artery disease or fibromuscular dysplasia involving the orifices of both renal arteries should not donate a kidney. However, the meaning of ‘orifices’ for FMD is somewhat uncertain as multifocal FMD mostly affects mid-to-distal segments of renal arteries. Furthermore, this recommendation is ‘not graded’ because of the lack of robust evidence in the literature. Consequently, nephrologists and transplant surgeons are often faced with a dilemma when counselling potential kidney donors in whom FMD has been found incidentally on pre-surgery screening tests or perioperatively.
practices across the world vary, with donation less likely to proceed unless FMD was undetected before surgery, the degree of FMD is deemed mild or as a last resort.
For me, I will decline donation as there is no strong evidence to accept such cases.
Donations from people with FMD can be accepted only when:
Reference:
Chrysochou C, Devresse A, Kanaan N, Persu A. Kidney donors with fibromuscular dysplasia, is it time to open the doors? Nephrol Dial Transplant (2021) 36: 1365–1368.
CT angiogram shows beaded like appearance of right renal artery more than left artery consisting of fibromuscular dysplasia.
FMD is non atherosclerotic disease diagnostic by CT angiogram and catheter based angiography to finding location and morphology of renal artery.
treatment by medical to control blood pressure or angioplasty by catheter or surgery
would you proceed further?
Generally fibromuscular dysplasia is contraindicated for transplant. However due to shortage of kidney donor i will accept for donation if the donor old age and unilateral and normal blood pressure and medial type FMD.
If we accept for donation should counselling regarding long term fallow up for hypertension and renal insufficiency for both recipient and donor.
Problem list:
Age 64-year-old female potential donor to her husband.
Excellent renal function (EDTA GFR is 78 ml/min) and no proteinuria.
No history of hypertension, DM.
Cardiac evaluation OK.
Renal CT angiogram – fibromascular dysplasia.
Please describe the CT finding
Sting on beads appearance in both renal arteries indicates fibromascular dysplasia.
How would you proceed further?
The donor is 65 years of age incidentally found FMD with no hypertension, indicates mild disease. However the age put her on extended criteria for donation.
By KDIGO guidelines patients with atherosclerotic renal artery disease or FMD involving both renal arteries should not be considered for donation.
A study by Adrogue et al.showed no significant increased risk of hypertension among donors in a 15 years follow up period, but there was a significant increased risk of proteinuria this was no more significant after a propensity score matching and none of the donors with FMD developed ESRD, and the incidences of cardiovascular diseases were comparable in FMD and normal cohort.
Indudhara et al.., found none of 19 patients with FMD who underwent nephrectomy developed hypertension, proteinuria or deterioration of renal function after 4.5 years follow-up.
In these studies the exact definition and the method used to diagnose FMD and it extension was not defined. They did not specify the type of FMD – multifocal or focal, presence of related aneurysms or dissection.
It is recommended by International FMD Consensus to do systematic head-to-pelvis vascular scanning in such cases, so I would order it before consider her as a donor.
This case needs a multidisciplinary team decision including FMD specialist!
The criteria to accept donors with FMD is :
– No other suitable living donor.
– Mild FMD disease.
– No or mild HTN.
– Few or no classical cardiovascular risk factors ( smoking, obesity, dyslipidemia…etc)
– Unilateral or predominant unilateral FMD disease – the unaffected or less affected should be left in the donor).
– No FMD, aneurysms or dissection in other arterial beds screened by head to pelvis CT angiography or MRA.
– No pregnancy considered after donation.
– Written consent from the donor and recipient should be taken after discussing the risk and outcomes.
– Agreement from both to be included in a prospective long term registry.
To me and according to our local facilities I would not accept such a case for donation.
References:
[1] Chrysochou C, Devresse A, Kanaan N, Persu A. Kidney donors with fibromuscular dysplasia, is it time to open the doors? Nephrol Dial Transplant. 2021 Jul 23;36(8):1365-1368. doi: 10.1093/ndt/gfab181. PMID: 33999202.
[2] Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545. doi: 10.1093/ndt/gfab039. PMID: 33566102.
CT findings :
Showed Beaded appearance of both renal arteries >> mostly FMD
According to medical history:
old age > 60 yrs
No history of hypertension
Not DM
No proteinuria and has excellent kidney function
Cardiac ECHO is satisfactory
Can be considered as potential donor after the following:
1-There is No other potential donors
2-No other systematic affections of FMD of other arteries (Carotids ,coronaries and Iliac arteries ) >> Vascular mapping is highly recommended ( Olin et al. and Plouin et al. , systematic head-to-pelvis vascular scanning as recommended in the International FMD Consensus )
3-Counselling the donor for the risk of FMD of affection other renal arteries
4-Mild FMD , preferred unilateral ones , unifocal better than diffuse or multifocal pattern
5- No or mild hypertension
6- No dissection or aneurysm of renal or extra-renal arterial beds CT or MR-angiography required)
7-written consents from both the donor and the recipient
8-silent (asymptomatic ) FMD
So I will accept the donor after these previous considerations
1- Based on this case selected patients with renal artery FMD who showed normal blood pressure and medial‐type FMD could be donors for renal transplantation
2-Some FMD kidney donors can safely donate since they have a similar long-term incidence of mortality, CVD, and hypertension as well as a lower eGFR to the general donor population.
3-The study of Adrogue et al. shows that donors with FMD have similar long-term renal and cardiovascular outcomes compared with donors without FMD.
REFERENCES :
Lentine KL , Kasiske BL , Levey AS et al. KDIGO clinical practice guideline on the evaluation and care of living kidney donors. Transplantation 2017; 101: S1–S109
Adrogue HE , Evans A , Murad DN et al. Long term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant 2021; 36: 1538–1545
Gonzalez Suarez M , Amer H , Jaffer I et al. Classification of fibromuscular dysplasia in kidney donors based on severity and extent of lesion. ATC Abstracts [Internet]. Available from: https://atcmeetingabstracts.com/abstract/classification-of-fibromuscular-dysplasia-in-kidney-donors-based-on-severity-and-extent-of-lesion/ (16 March 2021, date last accessed)
Please describe the CT finding
CTA showed bilateral multifocal beadings of the renal arteries, consistent with FMD.
How would you proceed further?
1. Kidney donors with FMD appear to do well, do not appear to incur increased risks of hypertension, proteinuria, CVD or reduced eGFR, and perhaps carefully selected candidates with FMD can safely donate as long as involvement of other vascular beds is ruled out1.
2. Both KDIGO and KDOQI guidelines state that ‘a donor candidate with atherosclerotic renal artery disease or fibromuscular dysplasia (FMD) involving the orifices of both renal arteries should not donate’ a kidney2. This recommendation is ‘not graded’ because of the lack of robust evidence in the literature.
3. Practices across the world vary, with donation less likely to proceed unless:
· FMD was undetected prior to surgery.
· The degree of FMD is deemed mild or as a last resort.
4. Tentative arguments supporting kidney donation in a subject with FMD2
· No other suitable living kidney donor.
· Mild FMD disease.
· No or mild hypertension.
· Few or no ‘classical’ cardiovascular risk factors (smoking, obesity, dyslipidemia, etc.).
· Unilateral or predominantly unilateral renal FMD lesions (in this case, the unaffected or less affected artery/kidney should be left in place).
· No or mild FMD lesions in other arterial beds.
· No dissection or aneurysm of renal or extra-renal arterial beds (head-to-pelvis CT or MR-angiography required).
· No pregnancy considered after donation.
· Written informed consent from both donor and recipient.
· Agreement from both donor and recipient to be included in a prospective long-term registry.
I will go ahead and this donation as long as:
· The donor is at old age with satisfactory cardiac and renal functions.
· No HTN.
· No proteinuria.
· Mild FMD.
Reference
1. Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545. doi: 10.1093/ndt/gfab039. PMID: 33566102.
2. Constantina Chrysochou, Arnaud Devresse, Nada Kanaan, Alexandre Persu, Kidney donors with fibromuscular dysplasia, is it time to open the doors?, Nephrology Dialysis Transplantation, Volume 36, Issue 8, August 2021, Pages 1365–1368, https://doi.org/10.1093/ndt/gfab181
-CT angiogram finding shows string of beads of the renal arteries bilaterally suggestive of Fibromuscular dysplasia
– Kidney transplantation from donors with FMD is generally contraindicated as it can lead to hypertension and renal insufficiency in the recipient and the donor as well.
But to face the shortage of available grafts a safe strategy as reconstruction of the artery can be applied according to the degree of FMD .
This current donor doesnot have hypertension neither renal impairment nor proteinuria
Studies conducted on small number of donors with variable degrees of FMD declared similar and comparable outcomes of donors without FMD regarding hypertension, reduction of GFR and proteinuria over 4 years follow up and more than 10 years follow up
So this donor can be accepted after detailed work up involving MDT and vascular surgeon expertise due to the surgical challenges
Also regular follow up will be needed for both donor and recipient
Reference
– Matsushita Y, Motoyama D, Ito T, Sugiyama T, Otsuka A, Sano M, Inuzuka K, Miyake H. Kidney transplantation from a living donor with renal artery fibromuscular dysplasia: A case report on arterial grafting of the donor renal artery. IJU Case Rep. 2020 Jul 4;3(5):201-203
-Horacio E Adrogue, Andrew Evans, Dina N Murad, Hana Nguyen, Sean A Hebert, Duc T Nguyen, Edward A Graviss, Hassan N Ibrahim, Long-term outcomes of kidney donors with fibromuscular dysplasia, Nephrology Dialysis Transplantation, Volume 36, Issue 8, August 2021, Pages 1538–1545
The CT scan shows the renal arteries have multiple beaded appearances that alternating stenosis and dilatations casing string of beads appearance. It can affect the vessels in two ways either multifocal or focal. The typical angiographic features include vascular loops, fusiform vascular ectasias, and a string of beads and a less typical feature consists of arterial dissection, aneurysm, and subarachnoid hemorrhage. So from the mentioned findings of the CT scan, the patient has fibromuscular dysplasia (FMD).
The donor has to be monitored for blood pressure and if not then it will be a positive for possible donation but based on KDIGO guidelines donors with FMD and other forms of atherosclerotic renal artery disease should not be a donor due to the stenosis of both renal arteries.
In this case, based on the angiogram, there are multiple beads that put her at a high risk of being a potential donor with post-surgery complications like elevated blood pressure.
Due to the multiple beads of stenosis and dilatation, there can be problems during the surgery as it relates to the recipient. Also, it has been confirmed that there can be complications like hypertension or renal insufficiency.
There have been studies that have demonstrated that reconstructing the renal artery with the recipient artery can have successful outcomes for the donor’s kidney. A study conducted with a potential patient donor with FMD had successful transplantation and maintain a serum creatinine level of less than 2 mg/dl for over 3 years. Still, the recipient has a reconstruction of the diseased artery.
So with reconstructive renal artery surgery, a donor with FMD can donate a kidney but will need proper follow-ups.
References:
Matsushita, Y., et al. IJU Case report (2020). Kidney transplantation from living donor with renal artery fibromuscular dysplasia: A case report on arterial grafting of the donor renal artery. doi: 10.1002/iju5.12188
Fibromuscular dysplasia (FMD) is a rare blood vessel disorder in which some of the strong, flexible cells of arteries are replaced with cells that are more fibrous. Fibrous cells are less strong and also less flexible. This change in composition of the arteries leads to their becoming stiffer and more prone to damage.
In this specific case, there is no functional impairment, after all, the potential donor is not hypertensive, does not present a decrease in renal function, and does not present proteinuria, being an accidental finding in a patient without target organ lesions.
I would proceed with the transplant, but after an extensive discussion with the donor about the risks involved in having only one kidney and the post-operative care.
Keep BMI below 30, proper diet, exercise, and avoid metabolic disease.
Additional investigation is needed to confirm that the disease is localized without systemic involvement.
this case of FMD (beaded string renal arteries). FMD is a non-atherosclerotic, non-inflammatory vascular disease leading to arterial stenosis, commonly affecting multiple arteries, (Renal 75-80%), (cervical 75%), and others.
data obtained from long-term outcomes from RELIVE data; 113 donors with FMD, compared with 452 matched donors without FMD. Showed that donors with FMD did not have a significantly increased risk of HTN, (22.2% vs 19.8%), with no more proteinuria, CKD, or ESKD, compared with controls
so, based on this weak evidence we have obtained from a retrospective observational study we should counsel potential donors very carefully before accepting her
and good pre and post-operative assessments should be done regarding
References;
Hyo-Sin K, Soo Jin Na C, Ho Kyun L. Successful kidney transplantation Using a deceased donor graft with fibromuscular dysplasia. Trans Proceed. 2019;51:2842–4.
Long-term outcomes of kidney donors with fibromuscular dysplasiaHoracio E Adrogue 1, Andrew Evans 1, Dina N Murad 1, Hana Nguyen 1, Sean A Hebert 1, Duc T Nguyen 2, Edward A Graviss 2 3, Hassan N Ibrahim
Incidental fibromuscular dysplasia in potential renal donors: long-term clinical follow-upA H Cragg 1, T P Smith, B H Thompson, T P Maroney, A W Stanson, G T Shaw, D W Hunter, S T Cochran
Lentine KL , Kasiske BL , Levey AS et al. KDIGO clinical practice guideline on the evaluation and care of living kidney donors. Transplantation 2017; 101: S1–S109Google ScholarPubMedWorldCat
2Mandelbrot DA , Reese PP , Garg N et al. KDOQI US commentary on the 2017 KDIGO clinical practice guideline on the evaluation and care of living kidney donors. Am J Kidney Dis 2020; 75: 299–316
Kaufman JA, Lee MJ. Vascular and interventional radiology, the requisites. Mosby Inc. (2004) ISBN:0815143699.
_ The CT shows beaded appearance of both renal arteries suggestive of FMD, which is not uncommon finding discovered accidently in many of female donors during preparation to donate.
_I will accept the current donor, if she is the only avialble option.
_ as she is more than 60 years, asymptomatic yet, so it seems that it is mild disease , no hypertension or protinuria and has normal creatinine for age
_ but counseling about the mild increased risk of hypertension after donation with close follow up after donation for protinuria and hypertension are essential.
_Exclusion of other risk factors for hypertension in the donor as obesity, smoking or dyslipidemia should be done before donation, together with exclusion of any vascular abnormalities else where in the carotid or vertebral arteries.
CT angiography. Shows Bilateral multifocal beaded lesion suggests FMD.
How would you proceed further?
Yes.
Adrogue et al. Shows is a valuable contribution to the literature and the largest study on
the outcome of living kidney donors with FMD so far.
It provides important data regarding the favourable long-term cardiovascular and renal
safety for selected kidney donors afflicted by FMD. (1)
These data suggest that some patients with mild FMD involvement and who are clinically
asymptomatic might be eligible for kidney donation.
Tentative arguments supporting kidney donation in a subject with FMD
(to be discussed on a case-by-case basis and updated according to the evolution of knowledge) (2)
unaffected or less affected artery/kidney should be left in place).
References:
1- 3Adrogue HE , Evans DN et al. Long term outcomes of kidney donors with
fibromuscular dysplasia. Nephrol Dial Transplant 2021; 36: 1538–1545.
2- Constantina Chrysochou et AL. Kidney donors with fibromuscular dysplasia, is it time
to open the doors? Nephrology Dialysis Transplantation, Volume 36, Issue 8, August
2021, Pages 1365–1368.
Please describe the CT finding
Ct reveal a beaded appearance of both renal arteries
How would you proceed further?
· FMD of the renal arteries can be an incidental finding, with about 4.4% of asymptomatic potential kidney donors had evidence for FMD
· In the past, it was believed that FMD was a disease of young women. However, older individuals account for a large proportion of affected patients in several cohorts.
· As patients with FMD have a high prevalence of arterial aneurysm and dissection, every patient diagnosed with FMD should have one-time, head-to-pelvic cross-sectional imaging
· The preferred imaging strategy is CTA, and MRA is an alternative
· FMD involves the middle or distal segment of the arteries, and the “string of beads” appearance is unique to FMD
· duplex ultrasonography should be used for diagnosis of FMD only in high-volume centers with expertise in this technique or if CTA and MRA cannot be performed
TREATMENT OF RENAL ARTERY FIBROMUSCULAR DYSPLASIA
· Antihypertensive therapy:
– Most patients with multifocal FMD of the renal arteries and hypertension will require antihypertensive therapy, even if they undergo revascularization
– the initial drug class of choice in FMD is an ACEi or ARB, if not controlled, add thiazide or long acting DHPCCB.
· Indications for revascularization
– Those with recent-onset hypertension, particularly younger patients with focal FMD who are less likely to have underlying atherosclerotic disease
– Those with resistant hypertension despite compliance with an appropriate three-drug regimen
– Those who are unable to tolerate antihypertensive medications or who are noncompliant with their medication regimen
– Adults with bilateral FMD, or unilateral renal artery FMD to a single functioning kidney, with unexplained progressive renal insufficiency suspected to be due to renal artery stenosis
– Hypertensive children with renal artery FMD.
Please describe the CT finding
The above image is of arterial phase of CT angiogram. In both sides, 2 segmental arteries are arising from the main renal arteries and have beaded appearance in their entire course suggestive of FIBROMUSCULAR DYSPLASIA.
How would you proceed further?
Looking to above findings and looking to the age of potential donor of 64 years, I shall go ahead with donation. If the potential donor would have been of younger age with similar findings, then considering bilateral FMD, I would have avoided donation.
Please describe the CT finding
Coronal plane CTA showing bilateral beading of (string of beads) renal arteries that is characteristic of FMD.
////////////////////////////
How would you proceed further?
KDIGO & KDOQI guidelines state that ‘a donor candidate with atherosclerotic RAD or FMD involving the orifices of both renal arteries should not donate a kidney’. However, ‘orifices’ for FMD uncertain (multifocal FMD mostly affects mid-to-distal segments of renal arteries).
There is no robust evidence in the literature, & practices are variable worldwide; donation is less likely to proceed unless:
FMD was undetected before surgery
FMD was mild or as a last resort.
Adrogue et al. showed that donors with FMD have similar long-term renal & CV outcomes compared with control donors.
Gonzalez Suarez et al. ((15-year data on 38 donors with FMD (35% with bilateral disease) out of 2250 kidney donors)): the rates of HTN & proteinuria were not higher with FMD versus controls.
Cragg et al. reported no difference in the likelihood of donation resulting in progression to HTN in patients with FMD.
These results suggest that, at least, some patients with mild, silent FMD could be eligible for donation, & would increase the pool of available donors.
Suggested criteria for accepting FMD for donation include:
1. No other suitable LKD
2. Mild FMD disease
3. No or mild HTN
4. Few or no CV risk factors (smoking, obesity, dyslipidemia, etc.)
5. Unilateral or predominantly unilateral renal FMD lesions (the sound or less affected kidney should be left in place)
6. No or mild FMD lesions in other arterial beds
7. No dissection or aneurysm of renal or extra-renal arterial beds (head-to-pelvis CT or MR-angiography required)
8. No pregnancy considered after donation
9. Written informed consent from both donor & recipient
10.Agreement from both donor & recipient to be included in a prospective long-term registry.
References
1. Constantina Chrysochou, Arnaud Devresse, Nada Kanaan, and Alexandre Persu. Kidney donors with fibromuscular dysplasia, is it time to open the doors? Nephrol Dial Transplant (2021) 36: 1365–1368 doi: 10.1093/ndt/gfab181
2. L. Varennes et al. Fibromuscular dysplasia: what the radiologist should know: a pictorial review. Insights Imaging (2015) 6:295–307 DOI 10.1007/ s 13244-015-0382-4
Very good it seems you will accept this donor!
Renal artery FMD is an idiopathic disease accompanied by distorted architecture of the renal arterial wall.
Selection of living kidney donors (LKD) with renal artery (RA) fibromuscular dysplasia (FMD) is controversial, as traditional diagnostic classifications lack estimates on FMD severity.
A classification was established to assess severity and location of FMD. “Extent” was defined as the sum of identified segments involved. Suitability for donation was based on surgical and medical judgment regarding outcome of donor and recipient, in addition to conventional donor selection criteria.
Grade
Grade. Severity. Description
1. Mild. Minimal contour change, subtle webbing (<20% alteration in
. Diameter of renal artery)
2. Moderate. Beaded segment of renal artery, 20-50% alteration in the , . . Diameter without dilatation/aneurysmal changes (<20% e. . . Expansion of renal artery)
3. Severe. Extensive alteration of renal artery >50% and tortuosity . without a neurysmal changes
4. Aneurysmal >10mm dilatation of renal artery
Potential LKD with incidental FMD vary substantially with regards to severity and extent of disease.
evaluating allograft and donor outcomes based on measurable indices of FMD severity
Suarez MGonzalez, Amer H, Jaffer I, Vrtiska T, Rule A, Prieto M, Textor S. Classification of Fibromuscular Dysplasia in Kidney Donors Based on Severity and Extent of Lesion. [abstract]. Am J Transplant. 2017; 17 (suppl 3). https://atcmeetingabstracts.com/abstract/classification-of-fibromuscular-dysplasia-in-kidney-donors-based-on-severity-and-extent-of-lesion/. Accessed November 3, 2022.
Thankyou so what is your decision in the index case (although the grade of stenosis is not mentioned.
Please describe the CT finding
CT angiogram showed single main renal artery bilaterally with beaded apperances of both renal arteries. This image suggestive of bilateral fibromuscular dysplasia (FMD) in the donor
How would you proceed further?
Both KDIGO and KDOQI guidelines stated that ‘a donor candidate with atherosclerotic renal artery disease or fibromuscular dysplasia (FMD) involving the orifices of both renal arteries should not donate’ a kidney.
However,
study by Adrogue et al.showed 113 donors with FMD were compared with 452 propensity score-matched donors without FMD. Donors with FMD did not demonstrate a significantly increased risk of hypertension on follow-up (22.2% versus 19.8% in donors without FMD).
Although they had an almost twice as high rate of proteinuria compared with the whole cohort (20.6% versus 13.7%, P 0.04), this difference was no longer significant after propensity score matching and it did not translate into a higher rate of chronic kidney disease defined as an estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2 , and none of the donors with FMD developed end-stage kidney disease during follow-up
Gonzalez Suarez et al. reported 15-year data on 38 donors with FMD (35% with bilateral disease) of a total of 2250 kidney donors. While donors with FMD were more likely to display lack of nocturnal dipping on follow-up (46% versus 35%, P 0.007), higher rates of hypertension and albuminuria were not found compared with controls without FMD, despite donors with FMD being older than controls.
Cragg et al. found 26.3% of 19 FMD donors versus 26.6% of 30 FMD non-donors developed hypertension, implying no difference in the likelihood of donation resulting in progression to hypertension in patients with FMD.
these studies we have to take into consideration that given the worldwide organ shortage , these results definitely need to be taken into consideration as they suggest that at least some patients with mild, silent FMD could be eligible for living kidney donation, and thus increase the pool of available kidney grafts.
Constantina Chrysochou et al did an extensive review and came out with guide to choose LD from FMD cohort:
Tentative arguments supporting kidney donation in a subject with FMD
I would adopt these selection criteria in choosing LD form FMD donor. I would reject this donor considering bilateral FMD disease.
References
*The CT angiogram of this donor showed: bilat. single renal arteries with beading all through their courses picture suggestive of Bilateral fibro-muscular dysplasia (FMD). FMD can be found in 2-6.6% of candidate donors. Mostly, medial subtype frequency 75-80% (mid-to distal portion of renal artery affected ) with favorable results with stent fixation.
*Donors with FMD can safely donate kidneys with long-term outcomes comparable to donors without FMD .so, I will accept this proposed donor after counselling her regarding life style modification ( low salt low fat diet , stop smoking) and regarding incidence of gaining hypertension and renal dysfunction. There is increase incidence of Cerebro-vascular strokes and hypertension because FMD can affect carotid and vertebral arteries as well so , a systematic head-to-pelvis vascular scanning should be done before donation by CTA. A written consent should be taken from both donor and recipient.
*Criteria for accepting a donor having FMD : only mild disease can be accepted with best out-comes (small sized , medial sub-type lesion affecting only one renal artery. No past medical history ( not DM, nor HTN neither dyslipidemic ) , systemic vascular scan didnt show any aneurysms or any arterial dissection , no available compatible LKD, long waiting list.
References:
1.Gornik HL, Weinberg I, Wilson S, etal.,: First International Consensus on the diagnosis and management of fibro-muscular dysplasia. Vasc Med. 2019 ;24(2):164-189.
2.Nguyen H, Hebert SA, Nguyen DT, etal; Long-term outcomes of kidney donors with fibro-muscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545.
Thankyou does the age have an impact on the decision?
The CT angiogram of the index prospective donor without any co-morbidities shows single main renal arteries bilaterally with beading of both renal arteries, suggestive of bilateral fibromuscular dysplasia (FMD) in the donor (1)
Fibromuscular dysplasia (FMD) can be incidentally detected (2-6.6%) in prospective donors (2). There are no guidelines available regarding kidney donation by someone having FMD.
Donors with FMD, when compared to those without FMD, have been shown to have no difference with respect to hypertension, cardiovascular disease (MI, TIA, stroke, heart failure and revascularization), ESRD development or death (2). Donors with FMD can safely donate kidneys with long-term outcomes similar to donors without FMD (3).
A systematic head-to-pelvis vascular scanning should be done as part of pre-donation workup (1).
Considering the involvement of bilateral renal arteries, I will reject this donor.
If there is no other donor available in the family, and the donor is amenable to lifestyle modifications (weight control, regular exercise, smoking cessation), then she can be taken up as a prospective donor after detailed counselling.
Such donors should be followed-up annually for detection of hypertension and renal dysfunction.
References:
1) Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, Bruno RM, de Leeuw P, Fendrikova-Mahlay N, Froehlich J, Ganesh SK, Gray BH, Jamison C, Januszewicz A, Jeunemaitre X, Kadian-Dodov D, Kim ES, Kovacic JC, Mace P, Morganti A, Sharma A, Southerland AM, Touzé E, van der Niepen P, Wang J, Weinberg I, Wilson S, Olin JW, Plouin PF. First International Consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 2019 Apr;24(2):164-189. doi: 10.1177/1358863X18821816. Epub 2019 Jan 16. Erratum in: Vasc Med. 2019 Oct;24(5):475. Erratum in: Vasc Med. 2021 Aug;26(4):NP1. PMID: 30648921.
2) Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545. doi: 10.1093/ndt/gfab039. PMID: 33566102.
3) Chrysochou C, Devresse A, Kanaan N, Persu A. Kidney donors with fibromuscular dysplasia, is it time to open the doors? Nephrol Dial Transplant. 2021 Jul 23;36(8):1365-1368. doi: 10.1093/ndt/gfab181. PMID: 33999202.
Thank you, Amit
See my question above.
CT finding:
Donor management:
References:
Dear Dr Ban,
I like your logical approach, and comprehensive reply.
Ajay
4. A 64-year-old lady has finished the workup as a kidney donor to her husband. No proteinuria and has excellent kidney function for her age (EDTA GFR is 78 ml/min). No history of hypertension, DM or any history that contraindicates donation. Her cardiac ECHO is satisfactory. Her CT angiogram is shown below. She came to see you to discuss the results of these tests.
——————————————————————————————————————-
HISTORY
——————————————————————————————————————-
Please describe the CT finding
——————————————————————————————————————-
How would you proceed further?
Outcomes of kidney donors with FMD. The data come from the Renal and Lung Living DonorEvaluation (RELIVE):-
In summary, the study of Adrogue et al (3) shows that donors with FMD have similar long term renal and cardio-vascular outcomes compared to donors without FMD.
Tentative arguments supporting kidney donation in a subject with FMD
(to be discussed on a case-by-case basis and updated according to the evolution of knowledge):-
### Whilst waiting for additional evidence, we propose a conservative set of
criteria to take into account before considering kidney donation in a subject with FMD.
???If Reconstruction of the diseased artery could be safely performed, transplantation using a kidney from a donor with renal artery fibromuscular dysplasia may be considered.
——————————————————————————————————————-Reference
The Author(s) 2021. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserve
Dear Dr Wadi,
I like your logical approach, and comprehensive reply.
Ajay
Thanks alot for you Prof.Sharma
Describe the CT findings
The CT angiogram shows that each kidney is being supplied by one renal artery arising from the abdominal aorta. The renal artery on each side is branching into the segmental arteries. There is beading appearance of the renal artery bilaterally with areas of calcification but with normal diameter. There would be two differential diagnosis – Fibromuscular dysplasia and atherosclerosis of the renal arteries
Since there is beaded appearance with normal flow this is FMD.
FMD is found in 2-6.6% of potential donors and is considered to be a contraindication to donation as it can lead to development of HTN and renal insufficiency in both the recipient and the donor
How would you proceed further?
The donor should be counseled against donation
Her other vascular beds should be assessed especially the carotids
She should be advised for regular follow up to assess BPs and renal function
IJU Case Reports (2020) 3, 201–203
Dear Dr Bagha,
I like your logical approach and well structured reply.
Ajay
Please describe the CT finding :
How would you proceed further?
Thankyou
CT scan showed bilateral kidneys with acceptable size, with nodular appearance of renal arteries bilaterally (string of Beads) classic feature for FMD.
FMD:
– A noninflammatory, nonatherosclerotic disorder that leads to arterial stenosis, occlusion, aneurysm, dissection, and arterial tortuosity.
-These findings have been observed in nearly every arterial bed
– Commonly involved arteries are the renal and internal carotid arteries.
– Disease presentation may vary widely, depending upon the arterial segment involved and the severity of disease.
– In addition, the “string of beads” appearance is unique to FMD. Thus, atherosclerotic disease and FMD can typically be distinguished radiographically.
It can also occur in isolation or in combination with atherosclerosis.
– Individuals with FMD are usually younger and have fewer cardiovascular risk factors.
– Patients with atherosclerosis are usually older and have typical CVD factors.
– Atherosclerosis usually involves the ostial or proximal segment of the arteries, whereas FMD involves the middle or distal segment.
Both KDIGO and KDOQI guidelines state that ‘a donor candidate with atherosclerotic renal artery disease or FMD involving the orifices of both renal arteries should not donate’ a kidney.
Data from RELIVE showed that donors with FMD have similar long-term renal and cardiovascular outcomes compared with donors without FMD (slight increase in proteinuria but no increase risk of CKD
The extension of FMD (and possible presence of related aneurysms or dissections), and the type of FMD; multifocal or focal has an implications for prognosis.
International FMD Consensus recommended systematic head-to-pelvis vascular scanning.
The potential donor has acceptable eGFR, no proteinuria and no other contraindication for donation. However, he needs systematic head-to-pelvis vascular scanning.
Some patients with mild FMD involvement and who are clinically asymptomatic might be eligible for kidney donation.
The benefits and potential harms of the procedure should be carefully weighed and explained, taking into account both the donor’s and the recipient’s perspectives including the potential risk of a subsequent arterial event in other vascular beds in donors with known FMD and the surgical procedures to apply for patients with bilateral renal FMD and/or FMD including involvement of aortic and iliac vessels.
Factors supporting kidney donation in a subject with FMD
No other suitable living kidney donor.
Mild FMD disease.
No or mild hypertension.
Few or no ‘classical’ cardiovascular risk factors (smoking, obesity, dyslipidemia, etc.).
Unilateral or predominantly unilateral renal FMD lesions (in this case, the unaffected or less affected artery/kidney should be left in place).
No or mild FMD lesions in other arterial beds.
No dissection or aneurysm of renal or extra-renal arterial beds (head-to-pelvis CT or MR-angiography required).
No pregnancy considered after donation.
Written informed consent from both donor and recipient.
Agreement from both donor and recipient to be included in a prospective long-term registry.
Conclusion:
As the evidence is not yet conclusive and the potential donor has bilateral disease, it is advisable to preclude donation.
References:
Constantina Chrysochou, Arnaud Devresse, Nada Kanaan, Alexandre Persu, Kidney donors with fibromuscular dysplasia, is it time to open the doors?, Nephrology Dialysis Transplantation, Volume 36, Issue 8, August 2021, Pages 1365–1368, https://doi.org/10.1093/ndt/gfab181
Thankyou Exellent
This is the characteristic finding of Fibromuscular dysplacia (FMD) in the above study. The above potential donor had the following positive data;
Definition;
FMD is a non-atherosclerotic, non-inflammatory vascular disease leading to arterial stenosis, commonly affected multiple arteries, (Renal 75-80%), (cervical 75%), and others.
Guidelines;
Next step;
In oreder to increase demand of kidney donation and advances of renal transplantation, and improved outcome this potential donor can proceed for donation as she had some of the following tentative arguments that supporting kidney transplantation in donor with FMD;
Refferences;
Hyo-Sin K, Soo Jin Na C, Ho Kyun L. Successful kidney transplantation Using a deceased donor graft with fibromuscular dysplasia. Trans Proceed. 2019;51:2842–4.
Long-term outcomes of kidney donors with fibromuscular dysplasiaHoracio E Adrogue 1, Andrew Evans 1, Dina N Murad 1, Hana Nguyen 1, Sean A Hebert 1, Duc T Nguyen 2, Edward A Graviss 2 3, Hassan N Ibrahim
Incidental fibromuscular dysplasia in potential renal donors: long-term clinical follow-upA H Cragg 1, T P Smith, B H Thompson, T P Maroney, A W Stanson, G T Shaw, D W Hunter, S T Cochran
Lentine KL , Kasiske BL , Levey AS et al. KDIGO clinical practice guideline on the evaluation and care of living kidney donors. Transplantation 2017; 101: S1–S109Google ScholarPubMedWorldCat
2Mandelbrot DA , Reese PP , Garg N et al. KDOQI US commentary on the 2017 KDIGO clinical practice guideline on the evaluation and care of living kidney donors. Am J Kidney Dis 2020; 75: 299–316
Kaufman JA, Lee MJ. Vascular and interventional radiology, the requisites. Mosby Inc. (2004) ISBN:0815143699.
Thankyou well done
2.How to proceed;
(Horacio E. Adrogue et al.2021)
Thankyou well done for noticing other possible abnormalities which can anticipate future problems.
Please describe the CT finding
a bilateral string of beads in both renal arteries likely FMD without evidence of aneurysmal dilatation and normal size of both kidneys
How would you proceed further?
Need to rule out carotid arteries involvement or other vascular beds involvement other than renal arteries if negative and being asymptomatic > 50yeras with normal BP and no other vascular involvement, also m GFR 78ml/min still can be considered for donation with mild risk as per limited evidence (2), however, the current guideline is not recommended the donation from FMD patients but in her case being asymptomatic and the age above 60 with no hypertension or proteinuria and if she is the only available donor with the good immunological match I would consider her for donation after full discussion with both the donor and recipient.
References:
1. Olin JW. Recognizing and managing fibromuscular dysplasia. Cleve Clin J Med. 2007 Apr;74(4):273-4, 277-82.
2.Adrogue HE, Evans A, Murad DN, Nguyen H, Hebert SA, Nguyen DT, Graviss EA, Ibrahim HN. Long-term outcomes of kidney donors with fibromuscular dysplasia. Nephrol Dial Transplant. 2021 Jul 23;36(8):1538-1545.
Thankyou the difficult question to answer is :
she is ok now ,what will be the situation in another 5-10 years .!