Does time taken to achieve jaundice-clearance influence survival of the native liver in post-Kasai biliary atresia?

Background We reviewed the time taken for post-portoenterostomy(PE)biliary atresia(BA)patients to obtain jaundice-clearance(total bilirubin≤1.2 mg/dL;JC)post-PE to determine if JC time(JCT)is prognostic for survival of the native liver(SNL).Methods The subjects were 66 BA patients treated with PE at our institute between 1989,the year when liver transplan-tation(LTx)became available in Japan,and 2014.JCT was used to create three groups(≤30 days:n=14;31-60 days:n=31;≥61 days:n=21).Medical records were reviewed retrospectively to evaluate:age at onset of symptoms,duration of symptoms pre-PE,age and weight at PE,serum liver function tests,incidence of cholangitis,and micro-bile duct size at PE.Results Age at onset of symptoms,age and weight at PE,duration of symptoms pre-PE,and micro-bile duct size were similar for all patients in all three groups.JCT and SNL appeared to correlate because preoperative total bilirubin(7.1,9.6,10.2 mg/dL;P<0.05)was significantly lower in the JCT≤30 days group(P<0.05)while there was a significant decrease in SNL(P<0.03)and a significant increase in LTx(P<0.01)in the JCT≥61 days group.All LTx subjects who achieved JC were found to have developed cholangitis within 3 months of PE.Conclusion During the follow-up of post-PE subjects,longer JCT and cholangitis occurrence within 3 months of PE would appear to be negative prognostic factors for SNL while preoperative total bilirubin would appear to be a positive prognostic factor for SNL.

Laparoscopic Kasai portoenterostomy can be a standard surgical procedure for treatment of biliary atresia

BACKGROUND Biliary atresia(BA)is a rare pediatric disease.AIM To compare the outcomes of laparoscopic portoenterostomy(Lap-PE)with those of laparotomy(Open-PE)at a single institution.METHODS The surgical outcomes of PE were retrospectively analyzed for patients with a non-correctable type of BA from 2003 to 2020.RESULTS Throughout the assessment period,119 patients received PE for BA treatment,including 66 Open-PE and 53 Lap-PE cases.Although the operation duration was longer(medians:for Open-PE,242 min;for Lap-PE,341 min;P<0.001),blood loss was considerably less(medians:for Open-PE,52 mL;for Lap-PE,24 mL;P<0.001)in the Lap-PE group than in the Open-PE group.The postoperative recovery of the Lap-PE group was more favorable;specifically,both times to resume oral intake and drain removal were significantly shorter in the Lap-PE group.Complete resolution of jaundice was observed in 45 Open-PE cases and 42 Lap-PE cases,with no statistically significant difference(P=0.176).Native liver survival rates were>80%for both groups for the first half year post surgery,followed by a gradual decrease with time;there were no statistically significant differences in the native liver survival rates for any durations assessed.CONCLUSION Lap-PE could be a standard therapy for BA.

Management of biliary atresia:To transplant or not to transplant

Kasai procedure(KP)and liver transplantation(LT)represent the only therapeutic options for patients with biliary atresia(BA),the most common indication for LT in the pediatric population.However,KP represents by no means a radical option but rather a bridging one,as nearly all patients will finally require a liver graft.More and more experts in the field of transplant surgery propose that maybe it is time for a paradigm change in BA treatment and abandon KP as transplantation seems inevitable.Inadequacy of organs yet makes this option currently not feasible,so it seems useful to find ways to maximize the efficacy of KP.In previous decades,multiple studies tried to identify these factors which opt for better results,but in general,outcomes of KP have not improved to the level that was anticipated.This review provides the framework of conditions which favor native liver survival after KP and the ones which optimize a positive LT outcome.Strategies of transition of care at the right time are also presented,as transplantation plays a key role in the surgical treatment of BA.Future studies and further organization in the transplant field will allow for greater organ availability and better outcomes to be achieved for BA patients.

Post-hepatoportoenterostomy Acoustic Radiation Force Impulse Elastography to Predict Two-year Outcome of Biliary Atresia

Background and Aims: Early identification of prognostic factors to predict transplant/death outcome of biliary atresia (BA) is challenging. We aimed to investigate the longitudinal changes and predictive value of dynamic changes in acoustic radiation force impulse elastography with shear wave speed (SWS) quantification and other parameters within three months after hepatoportoenterostomy (HPE) for 2-year BA outcomes. Methods: Seventy-four patients who underwent HPE between July 2016 and June 2019 were prospectively enrolled. Outcomes were classified into native liver survival and transplant/death groups. Acoustic radiation force im-pulse elastography was performed sequentially at 3 months intervals post-HPE. Cox regression analysis was used to de-termine the superior SWS values and other predictors of liver transplantation or death. Results: Among patients 2 years of age, 36 survived with a native liver, nine died, and 29 underwent liver transplantation. The trend in SWS levels in the transplant/death group was significantly different from that in the native liver survival group. ΔSWS at 1-3 months post-HPE and total bilirubin at 1 month post-HPE were se-lected as superior predictors of liver transplantation or death using multivariate Cox regression models: hazard ratio (HR)=1.927;95% confidence interval (CI): 1.475-2.661;p<0.001 and HR=1.010;95% CI: 1.003-1.017;p=0.007, respectively. The combination of the selected ΔSWS and total bilirubin had good predictive power, with an area under the receiver operating characteristics curve of 0.89, specificity 94.44% and sensitivity 73.68%. Conclusions: Our results suggest that early postoperative bilirubin levels and SWS changes were reliable predictors of 2-year BA outcomes.