When is a child untransplantable?

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When is a child untransplantable? Professor Nizam Mamode Guys and St Thomas Hospital Evelina Children s Hospital Great Ormond Street Hospital

Page 2

Median waiting time 286 days v 829 days for adults Upper 95% confidence limit Median Lower 95% confidence limit

Source: Annual Report on Kidney Transplantation 2014/15, 2016/17, NHS NHS Blood Blood and and Transplant

Implantation Children >20kg: Iliac vessels retroperitoneal approach Children <20 kg: Midline Incision with IVC and Aorta approach

Why do children have vascular anomalies? Central lines Previous surgery Hypercoaguability Congenital disease: Mid-aortic syndrome Am J Hypertens. 2015Jul;28(7):833-46.

15yr old boy Weight 55kg ESRD due to pneumococcal HUS Left LIF incision with EIV and EIA anastomosis Immediate function but slow venous outflow noted hence started on LMWH Day 15 post Tx had a non occlusive thrombus in main renal vein at the hilum 6 weeks of dalteparin, resolved, renal function fine Last egfr 44ml/min/1.73m2

Page 8 7 year old girl, congenital nephrotic syndrome Bilat nephrectomies aged 2 years LD transplant aged 3 years, failed: thrombosis Recurrent line thromboses, SVC thrombosis Grandmother wants to donate Midline incision Dense adhesions Suprarenal IVC accessible Transplanted onto aorta and IVC, with periop heparin

Options if no IVC Page 9 Mobilise liver and use retrohepatic IVC/R hepatic vein Mesenteric veins Patel AJT 2003 Collateral veins

Page 10 Portal vein Cauley Pediatr Transplant 2013

Page 11 Orthotopic approach Martinez-Urrutia Pediatr Transplant 2007 3 patients using adult kidneys

Page 12 It may be worth exploration Consider starting recipient prior to the donor May benefit from 2 experienced recipient surgeons and 1 experienced donor surgeon

Page 13 9 year old girl, 24kg Previous right nephrectomy (dysplasia) Mid-aortic syndrome 4 months previously had graft: supra-coeliac aorta to bifurcation, with L nephrectomy (thoraco-abdominal incision) 49 yr old father is donor, 000MM Midline incision Inspection of vessels Transplant onto L CIA

Page 14 17 year old girl, 34 kg CAKUT, aortic coarctation, biltateral hydronephrosis, UTIs At 2 years: graft to aortic arch (twice) Ureters re-implanted, uretero-ureterostomy, augment, re-augment and Mitrofonoff (7 years) At 16 years: nephrectomy- HD Mother is donor

Page 15 Surgery Recipient before donor Dense adhesions Split diaphragmatic crura Deceased donor iliac artery conduit to supra-coeliac aorta, tunneled under porta hepatis Renal vein to IVC Ureter to augmented bladder Conduit Tx kidney Native kidney

Issues with DD conduit Page 16 Patient type - A1,A2;B8,B44;Bw4,Bw6;Cw5,Cw7;DR8, DR11,DR52;DQ7 Kidney type - A2,A30;B13,B44;Bw4;Cw5,Cw6;DR11,D R52;DQ7 Developed rise in Cr associated with small rise in DSA to iliac conduit No DSA to kidney? Rejection of the conduit Iliac graft type A1, A3;B35,57;Bw4,Bw6;Cw4,Cw6;DR1,DR7,DR53;DQ5,DQ9

Nov 2016- PTA for stenosis March 2017 Page 17

6 year old girl 18kg Page 18 Cause of renal failure: renovascular Previous aortic graft On haemo-dialysis (line) SVC obstruction considered unlikely any further access for dialysis available Major abdominal trauma at age 4: multiple laparotomies (4) tracheal stenosis, CVA Blood group incompatible donor: mother A into O, titres 1in 8 Highly sensitised CRF 87%

Page 19 Investigations Angiogram

Venous anatomy Page 20

Page 21 Our approach Multiple MDMs at adult and children s hospital Hospital ethics team Palliative care team and counsellors 2 consultants at surgery Donor surgery started after recipient

How can we make the surgery safer for complex kidney transplantation in children? Using 3D printing of paediatric abdominal structures and adult donor kidneys

Software: Mimics Medical 18.0 (Materialise, 2015) Flexible materials: TangoPlus FullCure930, Stratasys 3D Printer: Stratasys Objet500 Connex1 printer Rigid materials: VeroWhitePlus FullCure835, Stratasys

Proof of concept 6 yr F, 18kg Native aorta (L)LDK Arterial anastomosis window Geometrical correlation between CT/MR vs Segmented design vs model 5 independent surgeons confirmed value as a preoperative planning tool (= 5)

Case 1 Liver (R)LDK Pelvis IVC/Aorta 2 yr F, 10 kg, previous laparotomies for bowel ischaemia

Surgeon: =5 for planning and anatomy correlation; =4 for kidney placement Family consenting =5 Chandak P Journal of 3D Printing in Medicine 2017 :(1) 9-12

Case 2: 12 kg child, Renal and IMA aneurysms

Case 3 14kg F, twisting branches of aorta with high divide Twisting branches of aorta

IVC IVC 1.5 cm ; CIA 0.5 cm

Considerations for transplantation Multidisciplinary discussion Radiology, Nephrology, Tx Surgery, Anaesthetist, Vascular surgeon, Liver surgeon 2 consultant surgeons Prolonged anaesthesia- PICU Inspection of vessels Use of vascular conduits Use of 3D printing

4th PAEDIATRIC KIDNEY TRANSPLANTATION SYMPOSIUM A Multi-professional Meeting Page 33 6 th December 2018 13:00-17:00 and 7 th December 2018 9:00-17:00 London, UK Key Topics Transplantation in small children Pre-emptive transplantation how to do it Combined liver and kidney transplantation Pancreas and islet cell transplantation in children Auto-transplantation as treatment for reno-vascular hypertension Paediatric transplant recipient in 21 st century Intraoperative management what really matters ABO and HLA incompatible transplantation Limits in paediatric transplantation Complex case exchange Best abstract prize presentation Guest speaker TBC RCPCH has approved this activity for CPD in accordance with the current RCPCH CPD Guidelines www.guystransplant.wordpress.com Registration Consultants 90 ( 70 before 20th November) Trainees 70 ( 50 before 20th November) Nurses/Coordinators 20 jelena.stojanovic@doctors.org.uk Guy s, Evelina, Great Ormond Street Hospitals teams: P Chandak,N Kessaris, SD Marks, J Stojanovic, G Walsh, N Ware 3D Printing: N Byrne, A Coleman, N Karunanithy, J Carmichael, Kind permission from all the patients and families Organizing committee Dr Jelena Stojanovic Mr Nicos Kessaris Professor Nizam Mamode