Impact of the development of a liver transplant program on the treatment of biliary atresia in an institution in Japan.

Kasai operation for biliary atresia (BA) and living-related liver transplantation (LRLT) for failed Kasai cases have been performed by the same team in our hospital. Outcome of the treatment for BA in the pretransplantation and posttransplantation era in our institution were compared. As the "pre" group, 35 children first underwent the Kasai operation between 1982 and 1989. The "post" group consisted of 25 patients who underwent Kasai after introduction of the LRLT program in our hospital (1990). As for the reconstruction procedure after portal dissection during Kasai, jejunal interposition hepatic portoduodenostomy for the pre group was intentionally changed to Roux-en-Y procedure for the post group to decrease the risk of duodenal leakage after LRLT. Patients who needed revision of the Kasai decreased from 57% for the pre group to 28% for the post group. There was only one re-revision in the post group compared with seven in the pre group. In the pre group, 10 patients (28.6%) died at age 6 to 16 (mean, 8.5) months before the transplantation program was initiated. In the pre group, three patients could receive a cadaveric liver transplantation in other countries, and seven had LRLT in our hospital. Ten patients (40%) in the post group had LRLT at 7 to 22 (mean, 9.3) months old, and no deaths occurred in this group. When the patients who had liver dysfunction were excluded, the percentage of the patients who could be expected to survive and accomplish growth without undergoing liver transplantation was similar for the two groups, (31.4% v 36%). The change in the reconstruction procedure and the tendency for fewer revisions of Kasai procedure were effects of the start of the liver transplant program. As a general strategy for BA, Kasai operation should be the initial procedure, followed by liver transplantation for failed Kasai. In the late-diagnosed patients, primary LRLT can be considered if the liver has already been cirrhotic. LRLT in the infantile period is a significant component of this strategy for helping all patients to survive.