Ultrasound for the Diagnosis of Biliary Atresia: A Meta-Analysis

Zhou, Luyao; Shan, Quanyuan; Tian, Wei; Wang, Zhu; Liang, Jin-Yu; Xie, Xiaoyan

doi:10.2214/ajr.15.15336

Cited by 65 publications

(60 citation statements)

References 51 publications

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“…However, early diagnosis of BA remains challenging in infants with cholestasis . Imaging of the gallbladder and biliary tree under sonographic and magnetic resonance cholangiopancreatography examination are widely used to diagnose BA in cholestatic infants, but the diagnostic accuracy of these tests are unsatisfactory . Patent biliary scintigraphy can exclude a diagnosis of BA, but the absence of excretion may also be observed in infants with other cholestatic liver diseases .…”

mentioning

confidence: 99%

Transient elastography is useful in diagnosing biliary atresia and predicting prognosis after hepatoportoenterostomy

Lee

Lin

et al. 2018

Hepatology

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confidence: 99%

Transient elastography is useful in diagnosing biliary atresia and predicting prognosis after hepatoportoenterostomy

Lee

Lin

et al. 2018

Hepatology

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“…with reduced proliferation and luminal deciduation (Uemura et al, 2013;Higashiyama et al, 2017). This leads to hypoplastic and noncontractile gallbladders that are similar to the abnormal gallbladders in human BA patients (Tan Kendrick et al, 2003;Kanegawa et al, 2003;Zhou et al, 2016;Aziz et al, 2011;Hwang et al, 2018). Along with the increased PBG/PPG density in Sox17 +/− gallbladders (Fig.…”

Section: Discussionmentioning

confidence: 86%

“…In contrast, the 'perinatal' form mainly consists of 'isolated' BA, the largest group with neither clear etiology nor any appreciable defects in other tissues/organs except for the bile ducts (Kelay and Davenport, 2017). Despite these potential heterogeneous causes, one of the most reliable characters of BA is the presence of gallbladder abnormality, such as an echogenic non-identical, atrophic, noncontractile and/or irregularly shaped gallbladder without a definable luminal wall (Tan Kendrick et al, 2003;Kanegawa et al, 2003;Zhou et al, 2016;Aziz et al, 2011;Hwang et al, 2018). However, the pathological phenotypes and their causes in human BA gallbladders are unclear.…”

Section: Introductionmentioning

confidence: 99%

Gallbladder wall abnormality in biliary atresia of mouse Sox17+/− neonates and human infants

Uemura

Higashi

Pattarapanawan

et al. 2020

Disease Models &Amp; Mechanisms

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Biliary atresia (BA) is characterized by the inflammation and obstruction of the extrahepatic bile ducts (EHBDs) in newborn infants. SOX17 is a master regulator of fetal EHBD formation. In mouse Sox17 +/− BA models, SOX17 reduction causes cell-autonomous epithelial shedding together with the ectopic appearance of SOX9-positive cystic duct-like epithelia in the gallbladder walls, resulting in BA-like symptoms during the perinatal period. However, the similarities with human BA gallbladders are still unclear. In the present study, we conducted phenotypic analysis of Sox17 +/− BA neonate mice, in order to compare with the gallbladder wall phenotype of human BA infants. The most characteristic phenotype of the Sox17 +/− BA gallbladders is the ectopic appearance of SOX9-positive peribiliary glands (PBGs), so-called pseudopyloric glands (PPGs). Next, we examined SOX17/SOX9 expression profiles of human gallbladders in 13 BA infants. Among them, five BA cases showed a loss or drastic reduction of SOX17positive signals throughout the whole region of gallbladder epithelia (SOX17-low group). Even in the remaining eight gallbladders (SOX17high group), the epithelial cells near the decidual sites were frequently reduced in the SOX17-positive signal intensity. Most interestingly, the most characteristic phenotype of human BA gallbladders is the increased density of PBG/PPG-like glands in the gallbladder body, especially near the epithelial decidual site, indicating that PBG/PPG formation is a common phenotype between human BA and mouse Sox17 +/− BA gallbladders. These findings provide the first evidence of the potential contribution of SOX17 reduction and PBG/PPG formation to the early pathogenesis of human BA gallbladders. This article has an associated First Person interview with the joint first authors of the paper.

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“…BA was diagnosed in four patients (Table 2). In two of these patients, the common bile duct, the common hepatic duct, PEDIATRIC IMAGING: US-guided Percutaneous Cholecystocholangiography for Exclusion of Biliary Atresia Zhou et al (11)(12)(13), is not always reliable. First, the development of fibrosis may not be sufficiently rapid to make an echogenic band in the early stage of BA (5,11,14).…”

Section: Resultsmentioning

confidence: 99%

Percutaneous US-guided Cholecystocholangiography with Microbubbles for Assessment of Infants with US Findings Equivocal for Biliary Atresia and Gallbladder Longer than 1.5 cm: A Pilot Study

Zhou

Chen

et al. 2018

Radiology

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Purpose To evaluate the feasibility of ultrasonographically (US) guided percutaneous cholecystocholangiography (PCC) for early exclusion of biliary atresia (BA) in infants suspected of having BA with equivocal US findings or indeterminate type of BA and a gallbladder longer than 1.5 cm at US. Materials and Methods This study was approved by the ethics committee; written informed parental consent was obtained. From February 2016 to December 2016, nine infants (four boys, five girls; mean age, 60.2 days; median age, 57 days; age range, 23-117 days) with conjugated hyperbilirubinemia and gallbladder longer than 1.5 cm at US were referred for US-guided PCC after US findings were equivocal for BA (n = 7) or the type of BA was unclear (n = 2). PCC was performed with a US machine with incorporated contrast pulse sequencing, contrast-specific software, and a linear transducer by injecting diluted contrast material via an 18-gauge needle. Images from US and US-guided PCC were evaluated in consensus by two radiologists. US criteria for BA were fibrotic cord sign (>2 mm) and gallbladder length-to-width ratio greater than 5.2. BA was excluded at PCC when contrast material was visualized in the gallbladder, common hepatic ducts, and common bile duct and during passage to the duodenum. Patients in whom BA was diagnosed after PCC underwent surgery or liver biopsy as the reference standard. Nonparametric and Fisher exact tests were used. Results US-guided PCC was successful in all patients. There were no procedural-related complications. BA was excluded in five of the nine patients. The median serum direct bilirubin level in these patients slightly decreased 1 week after PCC, from 91.1 μmol/L (interquartile range [IQR], 81.6-113.8 μmol/L) to 65.3 μmol/L (IQR, 57.8-74.7 μmol/L); however, this difference was not statistically significant (P = .062). BA was diagnosed in four patients, with the diagnosis confirmed at surgery (n = 2) or liver biopsy (n = 2). BA in two patients with unclear type of BA was defined as type III without patency of the common bile duct in one patient and as type III with patency of the common bile duct in the other. Conclusion In this highly selected group of infants with indeterminate type of BA or inconclusive US findings, US-guided PCC enabled the diagnosis of BA in four infants and the exclusion of BA in five. US-guided PCC may be a safe and effective tool to exclude BA early in infants with equivocal US findings. RSNA, 2017.

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Ultrasound for the Diagnosis of Biliary Atresia: A Meta-Analysis

Abstract: The triangular cord sign and gallbladder abnormalities are the two most accurate and widely accepted ultrasound characteristics for diagnosing or excluding biliary atresia. Other ultrasound characteristics are less valuable for diagnosis or exclusion of biliary atresia.

Cited by 65 publications

References 51 publications

Transient elastography is useful in diagnosing biliary atresia and predicting prognosis after hepatoportoenterostomy

Transient elastography is useful in diagnosing biliary atresia and predicting prognosis after hepatoportoenterostomy

Gallbladder wall abnormality in biliary atresia of mouse Sox17+/− neonates and human infants

Percutaneous US-guided Cholecystocholangiography with Microbubbles for Assessment of Infants with US Findings Equivocal for Biliary Atresia and Gallbladder Longer than 1.5 cm: A Pilot Study

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