Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort

BACKGROUND Biliary atresia(BA)is the most common indication for pediatric liver transplantation,although portoenterostomy is usually performed first.However,due to the high failure rate of portoenterostomy,liver transplantation has been advocated as the primary procedure for patients with BA.It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes.AIM To investigate the effect of prior portoenterostomy in infants un-dergoing liver transplantation for BA.METHODS This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil.Patients with BA were divided into two groups:Those undergoing primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy.Continuous variables were compared using the Student’s t-test or the Kruskal-Wallis test,and categorical variables were compared using theχ2 or Fisher’s exact test,as appropriate.Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis.Patient and graft survival analyses were conducted with the Kaplan–Meier product-limit estimator,and patient subgroups were compared using the two-sided log-rank test.RESULTS Forty-two patients were included in the study(25[60%]girls),23 undergoing liver transplantation without prior portoenterostomy,and 19 undergoing liver transplantation with prior portoenterostomy.Patients with prior portoenterostomy were older(12 vs 8 months;P=0.02)at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores(13.2 vs 21.4;P=0.01).The majority of the patients(35/42,83%)underwent livingdonor liver transplantation.The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis(39 vs 11%),but this result did not reach statistical significance.Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation,graft-to-recipient weight ratio,and use of vascular grafts.Finally,the groups did not significantly differ in terms of post-transplant survival.CONCLUSION In our study,prior portoenterostomy did not significantly affect the outcomes of liver transplantation.

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.

Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis

Background:It is controversial whether adjuvant steroid treatment should be given to biliary atresia rBAl patients following a Kasai portoenterostomy(KPE).This study aimed to quantitatively and systematically evaluate the effect of adiuvant steroid therapy post-KPE in relation to maior clinical outcomes ofBA patients.

Use of shear wave elastography for the diagnosis and follow-up of biliary atresia: A meta-analysis

BACKGROUND Timely differentiation of biliary atresia(BA)from other infantile cholestatic diseases can impact patient outcomes.Additionally,non-invasive staging of fibrosis after Kasai hepatoportoenterostomy has not been widely standardized.Shear wave elastography is an ultrasound modality that detects changes in tissue stiffness.The authors propose that the utility of elastography in BA can be elucidated through meta-analysis of existing studies.AIM To assess the utility of elastography in:(1)BA diagnosis,and(2)post-Kasai fibrosis surveillance.METHODS A literature search identified articles that evaluated elastography for BA diagnosis and for post-Kasai follow-up.Twenty studies met criteria for meta-analysis:Eleven for diagnosis and nine for follow-up post-Kasai.Estimated diagnostic odds ratio(DOR),sensitivity,and specificity of elastography were calculated through a random-effects model using Meta-DiSc software.RESULTS Mean liver stiffness in BA infants at diagnosis was significantly higher than in non-BA,with overall DOR 24.61,sensitivity 83%,and specificity 79%.Post-Kasai,mean liver stiffness was significantly higher in BA patients with varices than in patients without,with DOR 16.36,sensitivity 85%,and specificity 76%.Elastography differentiated stage F4 fibrosis from F0-F3 with DOR of 70.03,sensitivity 96%,and specificity 89%.Elastography also differentiated F3-F4 fibrosis from F0-F2 with DOR of 24.68,sensitivity 85%,and specificity 81%.CONCLUSION Elastography has potential as a non-invasive modality for BA diagnosis and surveillance post-Kasai.This paper’s limitations include inter-study method heterogeneity and small sample sizes.Future,standardized,multi-center studies are recommended.

Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia

Biliary atresia(BA) is one of the major hepatobiliary abnormalities in infants and one of the causes of hepatic osteodystrophy.Bone disease may be caused by the malabsorption of calcium and magnesium by vitamin D in hepatobiliary diseases in which bile flow into the intestines is deficient or absent.Bone fracture before Kasai hepatic portoenterostomy or within one month after the procedure in an infant with BA is very rare.We herein report two infants:one infant with BA who initially presented with a bone fracture before Kasai hepatic portoenterostomy,and the other at 4 wk after Kasai hepatic portoenterostomy,and also provide a review of the literature.Moreover,we conclude that clinicians should consider BA in infants with bone fracture during early infancy.

Changing trends of surgical treatment of hilar bile duct cancer:clinical and experimental perspectives

HISTORICAL POINT OF VIEWCarcinoma of the confluence of the hepatic ductshas been thought to be a rare disease until recently.Carcinoma of the large hepatic ducts was rarelydiagnosed correctly premorterned.Because of itsdeeply seated location,resection was once thoughtto be impossible.In 1957,Altemeier reported

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.

Prognostic Factors Related to In-hospital Death in Children with Biliary Atresia:Analysis of a Nationwide Inpatient Database

Background and Aims:Patients with biliary atresia(BA)are prone to hepatic decompensation,which might eventually lead to death.This study aimed to identify the possible risk factors affecting in-hospital death in BA patients in China.Methods:We collected data from the Hospital Quality Monitoring System,a national inpatient database.All patients aged up to 2 years old with a diagnosis of BA were included.The subjects were divided to three groups,including Kasai portoenterostomy(KP),liver transplantation(LT),and no surgery.Logistic regression with Firth’s method was performed to identify potential influencing variables associated with in-hospital death.Results:During the year 2013 to 2017,there were 14,038 pediatric admissions with a diagnosis of BA.The proportion of in-hospital death in pediatric BA admissions was 1.08%.Compared with patients under six months,there was a higher risk of in-hospital death for children aged six months to 1 year and 1–2 years old.Clinical signs,including cirrhosis,variceal bleeding,and hepatic encephalopathy,were significantly associated with the risk of in-hospital death.In no surgery group,compared to those in Beijing and Shanghai,BA patients admitted in other districts had a lower risk of in-hospital death(OR=0.39,95%CI:0.21,0.70).However,in the LT group,patients admitted in other districts had a higher risk of in-hospital death(OR=9.13,95%CI:3.99,20.87).Conclusions:In-hospital survival remains unsatisfactory for pediatric BA patients with severe complications.Furthermore,more resources and training for BA treatment,especially LT,are essential for districts with poor medical care in the future.

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.