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Purpose: Bile lake (BL) formation following Kasai portoenterostomy (KPE) can complicate the prognosis of biliary atresia (BA). Percutaneous transhepatic biliary drainage (PTBD) performed under fluoroscopic/ultrasonographic (US) guidance is discussed for the management of BL. Methods: A retrospective review of 64 BA patients treated by KPE (open = 31, laparoscopic = 33) at a single center (2004–2023) identified 9 BL cases (9/64; 14.1%). PTBD was indicated for jaundice or cholangitis refractory to antibiotic therapy. Results: All BL were asymptomatic, diagnosed after an episode of postoperative cholangitis. KPE type was not correlated with BL incidence; 2/31 (6.5%) for open and 7/33 (21.2%) for laparoscopic; p = 0.15. Median onset was postoperative day 273 (IQR: 170–920). One case resolved with antibiotics while another case required early liver transplantation (LTx) due to advanced hepatic dysfunction unrelated to BL. All remaining cases (7/9) had PTBD at a median of 14.3 months (IQR: 7.3–34.7) post-KPE, with successful resolution in 6/7; one case required two PTBD procedures. Post-PTBD biliary peritonitis (n = 3) was resolved by abdominal lavage (laparoscopic = 2; open = 1). Conclusion: Screening for BL is advisable in all postoperative BA patients especially when cholangitis occurs. Although the risk for biliary peritonitis warrants caution, PTBD seems a viable option for managing BL formation after KPE. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a rare disease of unknown etiology which leads to cirrhosis and death if left untreated. The standard of care is an early hepatoportoenterostomy (HPE). Long-term follow-up is mandatory, during which most patients will require a liver transplant. Activin A belongs to the transforming growth factor-β (TGF-β) superfamily. TGF-β is a central regulator in chronic liver disease. We have studied the expression of activin A in liver tissue collected intraoperatively during the HPE. We included patients who underwent HPE in a single medical center. Clinical, ultrasonographic, and pathohistological data were collected. Activin A immunostaining was performed. Expression in the bile duct epithelium and hepatocytes was scored as either weakly positive, moderately positive, or strongly positive. Patients were then divided into three groups accordingly. We observed the outcome after the HPE at 3 months, 2 years, and at the end of follow-up. The study encompassed 37 patients. At 3 months after HPE, 92.3% of those with a weakly positive activin A reaction (group A) achieved good jaundice clearance, whereas only 44.4% of those with a moderately (group B) and 40% of those with a strongly positive reaction (group C) achieved good jaundice clearance (p = 0.008). Furthermore, 2 years after the HPE, 92.3% of those in group A survived with native liver (SNL), but only 33.3% of those in group B and 46.7% of those in group C had SNL (p = 0.007). At the end of follow-up, 83.3% of those in group A survived with native liver, as did 33.3% in group B and 40% in group C. Activin A is a valuable pathohistological predictor of the outcome of BA after an HPE.

Controversies in management of biliary atresia (BA) after hepatoportoenterostomy (HPE) lead to variable treatment protocols. We implemented standardized medical management after HPE, customizing the use of antibiotics and corticosteroids based on patient-specific factors. In this retrospective analysis, 20 consecutive infants underwent HPE for BA and were compared to a historical cohort. Analysis of successful biliary drainage 3 months after HPE (defined as serum total bilirubin <2 mg/dL) was the primary endpoint; survival with native liver at 2 years was the secondary endpoint. Sixteen of 20 (80%) infants had successful bile drainage, compared to 8 of 20 (40%) infants in the historical cohort (P = 0.0225). Sixteen of 20 patients in the new protocol have reached 2 years of age or required liver transplantation. Among the sixteen, 11 (68.8%) are alive with native livers versus 10 of 20 (50%) in the historical cohort (P = 0.0970). This preliminary report suggests the potential benefit of tailored use of postoperative antibiotics and corticosteroids in improving biliary drainage after HPE. III. [ABSTRACT FROM AUTHOR]

Background: Hepatopulmonary syndrome (HPS) is a complication in biliary atresia (BA) children following hepatoportoenterostomy. Liver transplantation (LT) was the definitive treatment of HPS. However, little was known about the risk factors between HPS and mortalities. We aimed to evaluate the role of HPS and the predictors of complications after LT in BA children., Methods: One hundred and twenty (54 males and 66 females) children with BA receiving LT were retrospectively enrolled. The primary outcome was postoperative biliary/vascular complication rates and the secondary outcome was post-LT mortality rates., Results: Among 120 BA children receiving LT, six (5%) children were diagnosed with HPS before LT. The overall survival rate of LT in BA children was 80% and the overall survival rate of LT in BA children with and without HPS was 17% and 83%, respectively. Vascular complications and HPS were predictors for poor overall survival rates both in univariate logistic regression analyses (hazard ratio [HR], 11.63 and 5.96; P < 0.0001 and P = 0.001, respectively) and multivariate logistic regression analyses (HR, 10.02 and 4.16; P < 0.0001 and P = 0.007, respectively). Kaplan-Meier analysis indicated the predictive role of HPS on poor overall survival rates (P < 0.0001), higher risks of biliary complications (P < 0.0001), and higher risks of jaundice (P < 0.01) post-LT., Conclusion: The present study comprising children over long-term follow-up revealed that the development of pre-LT HPS had a poor impact on overall survival rates and higher risks of biliary complications in BA children receiving LT., Competing Interests: Declaration of competing interest The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.)

Objectives: To identify and distinguish between racial and socioeconomic disparities in age at hepatology care, diagnosis, access to surgical therapy, and liver transplant-free survival in patients with biliary atresia (BA)., Methods: Single-center retrospective cohort study of 69 BA patients from 2010 to 2021. Patients were grouped into White and non-White cohorts. The socioeconomic milieu was analyzed utilizing neighborhood deprivation index, a census tract-based calculation of six socioeconomic variables. The primary outcomes of this study were timing of the first hepatology encounter, surgical treatment with hepatic portoenterostomy (HPE), and survival with native liver (SNL) at 2 years., Results: Patients were 55% male and 72% White. White patients were referred at a median of 34 days (interquartile range [IQR]: 17-65) vs. 67 days (IQR: 42-133; p = 0.001) in non-White patients. White infants were more likely to undergo HPE (42/50 patients; 84%) compared to non-White (10/19; 53%), odds ratio (OR) 4.73 (95% confidence interval: 1.46-15.31; p = 0.01). Independent of race, patients exposed to increased neighborhood-level deprivation were less likely to receive HPE (OR: 0.49, p = 0.04) and achieve SNL (OR: 0.54, p = 0.02)., Conclusions: Racial and socioeconomic disparities are independently associated with timely BA diagnosis, access to surgical treatment, and transplant-free survival. Public health approaches to improve screening for pathologic jaundice in infants of diverse racial backgrounds and to test and implement interventions for socioeconomically at-risk families are needed., (© 2024 The Authors. Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: In 2022, the Biliary Atresia and Related Diseases (BARD) community reached a consensus for the definition of suspected and confirmed cholangitis for biliary atresia (BA) patients after hepatoportoenterostomy (HPE). This study assessed the new standardized BARD definition in a retrospective, multicenter cohort study., Methods: We included BA cases managed between 2010 and 2020 at the Hannover Medical School and Geneva University Hospitals' Swiss Pediatric Liver Center. The standardized BARD cholangitis definition assesses four clinical items and four imaging/laboratory items to define cholangitis. The definition was retrospectively applied to all BA cases having presented, according to their physician, cholangitis within the first year after the HPE. The diagnosis defined by the standardized BARD definition was compared with the final clinical diagnosis made by physicians. The Spearman's correlation coefficient was used to test for correlation between diagnoses made by standardized and clinical appreciation., Results: Of 185 consecutive BA patients, 59 (32%) had at least one episode of cholangitis within the first year after HPE. The correlation between the clinician's impression and the standardized BARD definition was very strong ( r  = 0.8). Confirmed cholangitis definition coincided with the clinician's impression (2.5 [±0.7]/4 clinical items, 2.6 [±0.5]/4 imaging/laboratory items). For suspected cholangitis, the threshold for diagnosis was lower within the standardized BARD definition (1.1 [±0.3]/4 clinical items, 2.2 [±0.8]/4 laboratory/imaging items)., Conclusions: This first retrospective application of the standardized BARD cholangitis definition reveals a very strong correlation with the physician's assessment before standardization. A prospective study is needed to further refine the standardized definition for cholangitis in BA patients., Competing Interests: The authors declare no conflict of interest., (© 2024 The Authors. JPGN Reports published by Wiley Periodicals LLC on behalf of The European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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 impulse elastography was performed sequentially at 3 months intervals post-HPE. Cox regression analysis was used to determine 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 selected 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., Competing Interests: The authors have no conflict of interests related to this publication., (© 2023 Authors.)

(1) Background: Acute cholangitis during the first year after Kasai hepatoportoenterostomy (HPE) has a negative impact on patient and native liver survival. There are no consistent guidelines for the definition, treatment, and prophylaxis of cholangitis after HPE. The aim of this study was to develop definition, treatment, and prophylaxis guidelines to allow for expeditious management and for standardization in reporting. (2) Methods: the Delphi method, an extensive literature review, iterative rounds of surveys, and expert panel discussions were used to establish definition, treatment, and prophylaxis guidelines for cholangitis in the first year after HPE. (3) Results: Eight elements (pooled into two groups: clinical and laboratory/imaging) were identified to define cholangitis after HPE. The final proposed definitions for suspected and confirmed cholangitis are a combination of one element, respectively, two elements from each group; furthermore, the finding of a positive blood culture was added to the definition of confirmed cholangitis. The durations for prophylaxis and treatment of suspected and confirmed cholangitis were uniformly agreed upon by the experts. (4) Conclusions: for the first time, an international consensus was found for guidelines for definition, treatment, and prophylaxis for cholangitis during the first year after Kasai HPE. Applicability will need further prospective multicentered studies. [ABSTRACT FROM AUTHOR]