Long-term results of pediatric liver transplantation in a combined pediatric and adult transplant program.

BACKGROUND: Liver transplantation is now routine therapy for a variety of childhood liver diseases; however, there are no detailed reports of long-term results from a Canadian centre. We reviewed data from the first 16 years of a pediatric liver transplantation program to determine survival, complications and long-term outcomes.
METHODS: The outcomes to December 2000 for all children (age less than 18 years) who received a liver transplant at the London Health Sciences Centre between April 1984 and December 1999 were reviewed. The recipients were grouped according to the period in which they received the transplant (period 1, April 1984 to July 1988; period 2, August 1988 to December 1993; or period 3, January 1994 to December 1999). Data were obtained from medical charts; in-person interviews, questionnaires or telephone contact with patients and their families; contact with referring physicians; and school records. Outcome measures included patient survival, retransplantation, complications and long-term outcomes (specifically steroid withdrawal and growth and development).
RESULTS: A total of 116 children (29 in period 1, 46 in period 2 and 41 in period 3) (median age 5.6 years at the time of the procedure) received a total of 140 liver grafts (32 in period 1, 57 in period 2 and 51 in period 3). Of the 116 patients, 23 (20%) were less than 1 year old at the time of transplantation. Biliary atresia was the most common indication for liver transplantation (57 [49%] of the 116 patients). The number of patients surviving to 1 year after transplantation was 20 (69%) of the 29 patients from period 1, 40 (87%) of the 46 patients from period 2 and 38 (93%) of the 41 patients from period 3. The percentage of patients receiving reduced size grafts from adult donors, including live donors, increased from 2/32 (6%) in period 1 to 22/51 (43%) in period 3. Retransplantation was required for 9 (31%) of the 29 patients from period 1, 6 (13%) of the 46 patients from period 2 and 7 (17%) of the 41 patients from period 3. Among these patients, 1-year survival was 33% (3/9) for period 1, 83% (5/6) for period 2 and 100% (7/7) for period 3. Eighteen of the 22 deaths occurred within 4 months after surgery. Only 3 (3%) of the 116 patients experienced post-transplant lymphoproliferative disease. Steroids were discontinued (usually within 2 years after surgery) for the following proportions of surviving transplant recipients: 17 (89%) of the 19 patients from period 1, 29 (78%) of the 37 patients from period 2 and 21 (55%) of the 38 patients from period 3. Most of the surviving patients had normal growth (82/94 or 87%) and development (73/94 or 78%), and these outcomes were consistent across the 3 periods of study.
INTERPRETATION: Survival and long-term outcomes continue to improve for most children who receive liver transplants. These improvements may be due to improved surgical technique, perioperative care and, to a lesser extent, immunosuppressive therapy.