ESPN 50th Annual Meeting

ESPN 2017


 
BILATERAL NEPHRECTOMIES FOLLOWED BY LIVING RELATED DONOR RENAL TRANSPLANTATION IN A 2 YEAR OLD CHILD WITH INTRA-RENAL ANEURYSMAL FIBROMUSCULAR DYSPLASIA
JI SOO KIM (below 35) 1 KJELL TULLUS 1 ANDREW LUNN 2 NEIL J SEBIRE 1 PANKAJ CHANDAK 1 IMRAN MUSHTAQ 1 COLIN FORMAN 3 NICOS KESSARIS 1 NIZAM MAMODE 1 STEPHEN MARKS 1

1- GREAT ORMOND STREET HOSPITAL
2- NOTTINGHAM UNIVERSITY HOSPITALS NHS TRUST
3- ROYAL FREE LONDON NHS FOUNDATION TRUST
 
Introduction:

To report the successful outcome of a staged surgical approach with bilateral native nephrectomies, haemodialysis and living related donor (LRD) renal transplantation in an infant with bilateral intra-renal aneurysmal disease secondary to fibromuscular dysplasia (FMD) to avert the life-threatening risk of aneurysmal rupture.

Material and methods:

Retrospective 12 month post-transplant follow up data of a 2 year old boy with FMD who presented at 7 months of age with macroscopic haematuria (after aneurysmal bleeding) and renovascular hypertension (presenting systolic blood pressure (SBP) of 250mmHg at 7 months) requiring five anti-hypertensive agents with normal renal function. Multi-disciplinary team discussions led to a planned surgical approach in view of intra-renal aneurysm size (1.5cm) and the life-threatening risk of rupture. He underwent bilateral native nephrectomies with histology confirming FMD, followed by haemodialysis with reduction to one anti-hypertensive agent. Three months later weighing 12kg, he proceeded to LRD renal transplant from his mother (mismatch 1,1,0) with anastomosis of the renal artery and vein onto his aorta and inferior vena cava respectively. He underwent excisions of inferior mesenteric artery and right internal iliac artery aneurysms without complications.

Results:

12 months post-transplant; he is on triple immunosuppression, normotensive with SBP of 104mmHg and off all anti-hypertensive agents. He has had no biopsy-proven acute rejection nor significant infectious complications and has had no proteinuria nor donor specific antibodies. His renal allograft function remained stable (eGFR of 74mls/min/1.73m2). A CT angiography at 12 months post-transplantation has shown no further aneurysmal changes but noted changes after ligation of the right internal iliac artery.

Conclusions:

This paediatric renal transplant highlights the complexities that clinicians must consider in rare variants with anerysmal disease as part of the spectrum of FMD and is the first paediatric report of a bilateral nephrectomy for this indication.