Primary Liver Transplantation vs. Transplant after Kasai Portoenterostomy for Infants with Biliary Atresia

Abstract

Introduction: Primary liver transplants (pLT) in patients with biliary atresia (BA) are infrequent, since most babies with BA undergo a prior Kasai portoenterostomy (KPE). This study compared transplant outcomes in children with BA with or without a prior KPE. We hypothesized that pLT have less morbidity and better outcomes compared to those done after a failed KPE. Methods: A retrospective review of patients with BA transplanted at our institution was performed. Patients were included if they received a pLT or if they were transplanted less than 2 years from KPE. Outcomes were compared between those groups. Comparisons were also made based on era (early: 1997–2008 vs. modern: 2009–2020). p < 0.05 was considered significant. Results: Patients who received a pLT were older at diagnosis (141.5 ± 46.0 vs. KPE 67.1 ± 25.5 days, p < 0.001). The time between diagnosis and listing for transplant was shorter in the pLT group (44.5 ± 44.7 vs. KPE 140.8 ± 102.8 days, p < 0.001). In the modern era, the calculated PELD score for the pLT was significantly higher (23 ± 8 vs. KPE 16 ± 8, p = 0.022). Two waitlist deaths occurred in the KPE group (none in pLT, p = 0.14). Both the duration of transplant surgery and transfusion requirements were similar in both groups. There was a significant improvement in graft survival in transplants after KPE between eras (early era 84.3% vs. modern era 97.8%, p = 0.025). The 1-year patient and graft survival after pLT was 100%. Conclusions: Patient and graft survival after pLT are comparable to transplants after a failed KPE but pLT avoids a prior intervention. There was no significant difference in pre- or peri-transplant morbidity between groups other than wait list mortality. A multicenter collaboration with more patients may help demonstrate the potential benefits of pLT in patients predicted to have early failure of KPE.

Predicting Cirrhosis and Poor Outcomes of Bile Drainage Surgery for Biliary Atresia

“…Instead, a number of cohort studies comparing salvage LTx after bile drainage surgery with primary LTx found no significant difference in patient survival or graft survival but did find a significant increase in early postoperative morbidity, such as infection and reoperation 12,13 . Lemoine et al 7 reported waitlist mortality in patients with early failure after a portoenterostomy, and a recent study by Yoeli et al 14 analyzing 3438 patients with BA in a transplantation database in the United States, which included data on waitlist mortality, concluded that bile drainage surgery should be attempted first because it may enable LTx to be postponed beyond the first year of life, thereby improving survival without jeopardizing subsequent LTx outcomes. However, the latter study did not analyze postoperative morbidity and defined patients with LTx as those without a history of abdominal surgery albeit there were some issues in the study with possible miscategorization of the patients 14 .…”

“…However, the latter study did not analyze postoperative morbidity and defined patients with LTx as those without a history of abdominal surgery albeit there were some issues in the study with possible miscategorization of the patients 14 . The previously cited monocentric study by Lemoine et al 7 noted that 5 of 14 patients with BA receiving primary LTx had previously received exploratory abdominal surgery. It is clear that certain patients with BA will not benefit from bile drainage surgery, and that primary LTx would benefit greatly if such patients could be accurately identified.…”

“…Because the success rate of bile drainage surgery remains uncertain and innovations in transplant techniques and immunosuppressive therapy have improved the survival rate to ~90%, 4 LTx is beginning to be considered as an alternative, primary, therapeutic option to bile drainage surgery 5 . However, given the scarcity of organs for transplantation, especially in infants, and the lifelong requirement for immunosuppression attendant upon transplantation, efforts have been made to choose patients most likely to benefit from primary LTx 6,7 . Unfortunately, at present no clear selection criteria are available 5 …”

“…5 However, given the scarcity of organs for transplantation, especially in infants, and the lifelong requirement for immunosuppression attendant upon transplantation, efforts have been made to choose patients most likely to benefit from primary LTx. 6,7 Unfortunately, at present no clear selection criteria are available. 5 One of the potential predictors of bile drainage surgery outcomes in patients with BA is histologically diagnosed liver fibrosis.…”

Are the Post-transplant Outcomes of Kasai's Early Failure and Late Failure Comparable to the Primary Liver Transplantation?

“…There is heterogeneity in the reports of biliary complications; some studies reported no difference in the incidence of biliary complications between groups Two distinct populations can be seen in the patients who underwent prior Kasai group. However, most studies have not distinguished between these patients when performing analysis, which might have contributed to the heterogeneity of the outcomes reported across the studies [1][2][3][4][5][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28]. Patients with K-EF tend to display similar characteristics to pLT at the time of transplantation, such as lower body weight and higher PELD score, in contrast to the K-LF group [1;5-7].…”