Experience with living related liver transplantation in 63 children

The incentive to develop intrafamilial living related liver transplantation (LRLT) originated from the shortage of cadaveric organ supply. We report o ur experience with LRLT in 63 children during 1993-1998 in the frame of a p rotocol approved by the Ethics Committee of our Institution. During this period, 152 potential intrafamilial (mostly parental) donors we re evaluated; 44 (28,5%) were excluded because of surgical (n = 4), medical (n = 39) or psychosocial reason (n = 1). Out of 108 who matched all medica l, surgical and psychological criteria of selection, 45 did not underwent l iving donation because their child received a cadaveric graft (n = 22; LRLT was their second option) or because one of the parents who had both been s elected was chosen [by the surgical team because of more favourable anatomy (n = 8) or by mutual agreement between the two parents (n = 5)]. Sixty-thr ee living donors (36 mothers, 24 fathers, one grand mother, one aunt and on e uncle) underwent procurement of the left robe (n = 52), the left lobe ext ended to part of segment IV (n = 8) or a Left hepatectomy (n = 3) without m ortality or any serious morbidity. Their median hospital stay was 7 days (r ange: 6-12); full physical rehabilitation and normalization of liver tests were usually obtained within three weeks. Their psychological follow-up did not disclose any longstanding serious sequellae. The median age of the recipients was 13 months (range 5-189); 30 were young er than one year at the time of transplant. Their median weight was 8,1 kg (range : 4,3 to 60);36 had an actual weight under 10 kg. Fifty-two received an ABO identical and 11 received an ABO compatible transplant. The native liver diseases were similar to common data in children, with bil iary atresia being the most frequent indication (74,6%). The median weight of the graft was 260 gr (range : 138-680) with a median ratio between the g raft weight and the recipient body weight of 3,17% (range: 0,75-8,08). All grafts were implanted orthotopically with semi-microvascular reconstruction of the hepatic vein, portal vein and hepatic artery [end to end anastomosi s in 58 (2 arteries were reconstructed in 7 patients) and interposition of an iliac arterial allograft from the infrarenal aorta in 5]. Base line immunosuppression consisted of a triple drug regimen including st eroids, Azathioprine and either Cyclosporine-Sandimmun(R) (n = 9), Cyclospo rine Microemulsion formulation - Neoral(R) (n = 13) or Tacrolimus - Prograf t(R) (n = 41). Biopsy-proved acute rejection was treated with intravenous b olus of steroids; steroid-resistant acute rejection was treated by a switch from Cyclosporine to Tacrolimus or addition of Mycophenolate-Mofetil (Cell cept(R)) in Tacrolimus treated patients. Actuarial patient survival was 91,8% and 89,6% after LRLT at one and five y ears post-transplant, respectively, and 87,5% and 82,8% at one and five yea rs, respectively, in 90 patients who received a cadaveric graft during the same interval. Actuarial graft survival was 91,8% and 84,1% after LRLT at o ne and five years, respectively, and 76,4% and 73,3% at one and five years, respectively, after cadaveric transplants. Vascular thrombosis was observed in 9,5% of the patients (arterial thrombos is: 1,6%; portal thrombosis: 7,9%) without graft loss. Biliary complication s were observed in 26,9% (bile leak from cut surface in 3,1%, anastomotic s tricture in 22,2% and intrahepatic stricture in 1,5%); two patients died fr om septic shock possibly related to uncompletely relieved anastomotic stric ture; all other biliary complications were successfully treated either cons ervatively or surgically. The incidence of acute rejection was 90,9% in 22 patients with Cyclosporine -based immunosuppression;acute rejection was corticoresistant in 50%. It wa s 46,3% in 41 patients with Tacrolimus-based immunosuppression (64% with Pr ograft(R) in capsules and 18,7% with Prograft(R) in granules); no acute rej ection was corticoresistant. One patient in each group developed chronic re jection tin spite of switch to Tacrolimus in a patient initially treated wi th Cyclosporine and following full withdrawal of immunosuppression for post transplant lymphoproliferation in a patient immunosuppressed with Tacrolimu s);both patients were successfully retransplanted with a cadaveric graft. The incidence of posttransplant lymphoproliferative disorder was 14,2% and similar whatever the main immunosuppressant (13,6% in the Cyclosporine grou p and 1 1,6% in the Tacrolimus group). One of the 9 patients with PTLD died of uncontrolled disease. In conclusion, clear ethical guidelines in the frame of a protocol approved by the Institution Ethics Committee should be followed in Living related l iver transplantation. Safety for the donor should be maximized; extensive s urgical expertise with all types of liver resection and transplants includi ng split grafts is a prerequisite. Results regarding patient and graft surv ival are superior to those obtained with cadaveric transplants. Implementat ion of LRLT in expert teams is a valid way to obviate the shortage of cadav eric transplants.