How to suspect the presence of high-risk esophageal varices and when to start endoscopic surveillance in children with biliary atresia?

Background And Aim: Portal hypertension determines the outcome of children with biliary atresia (BA) and is common even after a successful Kasai portoenterostomy (KPE). However, there are no clear-cut guidelines on the age of starting surveillance and the modality (endoscopy vs non-invasive tests [NITs]). In this cohort study, we analyzed our database to find out the utility of NITs in detecting high-risk esophageal varices in BA.

Novel Scoring Systems and Age-Based Hepatic Shear Wave Stiffness Cut-Offs for Improving Sonographic Diagnosis of Biliary Atresia.

Objectives: To make sonographic evaluation for biliary atresia (BA) more objective and reproducible using scoring systems, and evaluate hepatic shear wave elastography (SWE) as an adjunct in sonographic diagnosis of BA.

Methods: Sixty-four infants with cholestatic jaundice were enrolled between June 2016 and March 2018 in this prospective observational cohort study. Sonography and SWE was performed with SuperSonic Aixplorer system.

Difficulty in the Diagnosis of Biliary Atresia Splenic Malformation Syndrome In Utero.

The fetus of a 30-year-old pregnant Japanese woman was diagnosed with absence of inferior vena cava (IVC) and azygos continuation of interrupted IVC without cardiac anomalies at 34 weeks of gestation, and a healthy male neonate weighing 2,910 g was delivered at 37 weeks of gestation. On day 42 after birth, direct bilirubin predominant hyperbilirubinemia and high serum gamma-GTP levels were detected. Computed tomography revealed the presence of a lobulated and accessory spleen, and laparotomy demonstrated type III biliary atresia (BA), confirming the final diagnosis of BA splenic malformation (BASM) syndrome.

Senescence and senotherapies in biliary atresia and biliary cirrhosis.

Background: Premature senescence occurs in adult hepatobiliary diseases and worsens the prognosis through deleterious liver remodeling and hepatic dysfunction. Senescence might also arises in biliary atresia (BA), the first cause of pediatric liver transplantation. Since alternatives to transplantation are needed, our aim was to investigate premature senescence in BA and to assess senotherapies in a preclinical model of biliary cirrhosis.

Pediatric Liver and Transplant Surgery: Results of an International Survey and Expert Consensus Recommendations.

Background: Pediatric liver surgery is a complex and challenging procedure and can be associated with major complications, including mortality. Best practices are not established. The aims of this study were to evaluate surgeons' individual and institutional practices in pediatric liver surgery and make recommendations applicable to the management of children who require liver surgery.

Percutaneous Transhepatic Biliary Intervention in Adult Biliary Atresia Patients After Kasai Portoenterostomy.

Kasai portoenterostomy (KP) is a standard treatment for patients with biliary atresia (BA). After KP, patients with BA occasionally develop biliary complications, such as recurrent cholangitis, biliary stricture, and cystic dilatation of the intrahepatic bile duct. Percutaneous transhepatic biliary drainage is one of the treatment options for these biliary complications.

Long-term Outcome of Asymptomatic Patients With Graft Fibrosis in Protocol Biopsies After Pediatric Liver Transplantation.

Background: The histological prevalence of allograft fibrosis in asymptomatic children after liver transplantation (LT) is well documented. However, long-term graft and patient survival remain unclear. This retrospective multicenter study aims to determine the prevalence of allograft fibrosis and analyze the long-term outcome for patients transplanted in childhood.

Clinical Characteristics and Outcome of Infants with Biliary Atresia in Bahrain.

Objectives: Though the prevalence of biliary atresia (BA) in the Middle East is low, its role in causing life-threatening liver diseases is disproportionately high. This study aimed to assess the prevalence, clinical presentations, diagnosis, surgical interventions, and long-term outcomes including survival rate, and to analyze prognostic factors affecting the survival of infants with BA in Bahrain.

Methods: A retrospective cohort review of cases of infants diagnosed with BA between January 1993 and December 2021 at Salmaniya Medical Complex, Bahrain, was conducted.

Pre- and Immediate Post-Kasai Portoenterostomy Shear Wave Elastography for Predicting Hepatic Fibrosis and Native Liver Outcomes in Patients With Biliary Atresia.

Objective: To evaluate the feasibility of ultrasound shear wave elastography (SWE) for predicting hepatic fibrosis and native liver outcomes in patients with biliary atresia.

Materials And Methods: This prospective study included 33 consecutive patients with biliary atresia (median age, 8 weeks [interquartile range, 6-10 weeks]; male:female ratio, 15:18) from Severance Children's Hospital between May 2019 and February 2022. Preoperative (within 1 week from surgery) and immediate postoperative (on postoperative days [PODs] 3, 5, and 7) ultrasonographic findings were obtained and analyzed, including the SWE of the liver and spleen.

MRI Liver Imaging Integrated with Texture Analysis in Native Liver Survivor Patients with Biliary Atresia after Kasai Portoenterostomy: Correlation with Medical Outcome after Surgical Treatment.

Kasai portoenterostomy (KP) plays a crucial role in the treatment of biliary atresia (BA). The aim is to correlate MRI quantitative findings of native liver survivor BA patients after KP with a medical outcome. Thirty patients were classified as having ideal medical outcomes (Group 1; = 11) if laboratory parameter values were in the normal range and there was no evidence of chronic liver disease complications; otherwise, they were classified as having nonideal medical outcomes (Group 2; = 19).

Findings in percutaneous trans-hepatic cholecysto-cholangiography in neonates and infants presenting with conjugated hyperbilirubinemia: emphasis on differential diagnosis and cholangiographic patterns.

Background: Biliary atresia (BA) is one of the causes of conjugated hyperbilirubinemia in infants which if untreated leads to end-stage liver disease and death. Percutaneous Trans-hepatic Cholecysto-Cholangiography (PTCC) is a minimally invasive study which can be utilized in the diagnostic work-up of these patients. This study's purpose is to describe the experience with PTCC in neonates, the imaging findings encountered, and the abnormal patterns which warrant further investigation.

A novel prediction tool based on shear wave elastography, gallbladder ultrasound, and serum biomarkers for the early diagnosis of biliary atresia in infants younger than 60 days old.

Background: Early Kasai surgery before 60 days of life results in better clinical outcomes in patients with biliary atresia (BA). We aimed to develop and validate a prediction tool for the early diagnosis of BA in infants younger than 60 days old.

Methods: This prospective study recruited consecutive infants younger than 60 days old with conjugated hyperbilirubinemia who were evaluated with an ultrasound (US) scan, including B-mode US with color Doppler flow imaging (CDFI) features and liver two-dimensional shear wave elastography (2D SWE), from March 2017 to July 2021.

Feasibility and outcomes of transjugular intrahepatic portosystemic shunts in infants.

Background: Experience with transjugular intrahepatic portosystemic shunts (TIPS) in the pediatric population, especially in infants, is limited.

Objective: To evaluate the feasibility, efficacy and safety of TIPS placement in infants.

Materials And Methods: This retrospective non-comparative observational cohort study analyzed all pediatric patients  < 12 months of age treated with TIPS while waiting for liver transplant between October 2018 and April 2021.

Preliminary exploration of the efficacy of laparoscopic fluorescence cholangiography (LFC) in the diagnosis of biliary atresia compared with intraoperative cholangiography (IOC).

Background: Intraoperative cholangiography (IOC) has been the gold standard for diagnosing biliary atresia (BA). Our study attempted to diagnose BA using laparoscopic fluorescein cholangiography (LFC).

Methods: We retrospectively included 18 patients with preoperative suspected BA as the case group and 4 without extrahepatic biliary obstruction requiring laparoscopic surgery as the control group.

Dysmorphic Gallbladder Found on Prenatal Ultrasound as a Hint for Biliary Atresia.

Biliary atresia (BA) is a rare but devastating cholangiopathy. We report a case series of dysmorphic gallbladders detected during prenatal ultrasound, which were confirmed as BA after birth. We present the prenatal ultrasound findings as well as integral follow-up, with an aim to raise awareness regarding the association between BA and dysmorphic gallbladder.

Value of gallbladder length-to-width ratio for diagnosis of biliary atresia by correlation with age.

The objective is to explore the correlation between ultrasonic gallbladder length-width ratio (LTWR) and age, and the value of differential diagnosis between biliary atresia (BA) and other hepatic cholestasis. From January 2016 to June 2022, the data of 183 patients with jaundice who underwent abdominal ultrasound and surgical exploration in the Affiliated Hospital of Zunyi Medical University were analyzed retrospectively. The demographic data, liver function, and ultrasonic parameters were recorded and analyzed.

Intraoperative indocyanine green fluorescence cholangiography can rule out biliary atresia: A preliminary report.

Background: The prognosis of BA is known to be poor if definitive surgery is performed too late. Therefore, excluding BA as a diagnosis at an early stage is crucial. Conventional cholangiography requiring cannulation through the gallbladder may be unnecessarily invasive for patients, especially when ruling out BA.

The diagnostic performance of ultrasound elastography for biliary atresia: A meta-analysis.

Background: Biliary atresia (BA) is a severe inflammatory obliterative cholangiopathy of infancy that requires early diagnosis and prompt surgical intervention. In this study, we aimed to obtain comprehensive evidence on the diagnostic performance of liver stiffness measurement by ultrasound elastography in the detection of BA through a meta-analysis.

Methods: The PubMed, EMBASE, Cochrane Library, and Web of Science databases were searched for studies that investigated the diagnostic performance of ultrasound elastography in the detection of BA up to January 10, 2022.

Diagnostic Efficacy of Advanced Ultrasonography Imaging Techniques in Infants with Biliary Atresia (BA): A Systematic Review and Meta-Analysis.

The early diagnosis of biliary atresia (BA) in cholestatic infants is critical to the success of the treatment. Intraoperative cholangiography (IOC), an invasive imaging technique, is the current strategy for the diagnosis of BA. Ultrasonography has advanced over recent years and emerging techniques such as shear wave elastography (SWE) have the potential to improve BA diagnosis.