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Background: Two-dimensional shear wave elastography (2D-SWE) has been proposed for detecting liver fibrosis in biliary atresia. Objectives: To assess the performance of 2D-SWE for detecting advanced liver fibrosis and cirrhosis in patients with biliary atresia. Materials and Methods: Five electronic databases were searched to identify studies investigating the performance of 2D-SWE for diagnosing liver fibrosis in biliary atresia in children. We constructed the summary receiver operating characteristic (SROC) curves of 2D-SWE for detecting advanced liver fibrosis and cirrhosis, and then calculated the area under the SROC curves (AUROCs). Results: Six studies with 470 patients (ages 55 days to 6.6 years) were included. The median correlation coefficient of 2D-SWE with pathological liver fibrosis stages was 0.779 (range: 0.443‒0.813). The summary AUROCs for advanced liver fibrosis and cirrhosis were 0.929 and 0.883, respectively. The summary sensitivity and specificity of 2D-SWE for advanced liver fibrosis were 88% (95% confidence interval [CI]: 80‒94%) and 85% (95% CI: 77‒91%) with I values of 0% and 45.6%, respectively, and for cirrhosis were 80% (95% CI: 72‒87%) and 82% (95% CI: 77‒86%) with I values of 12.9% and 0%, respectively. The diagnostic odds ratio (DOR) of 2D-SWE for advanced liver fibrosis and cirrhosis were 40.3 (95% CI: 18.2‒89.4) and 18.9 (95% CI: 11.2‒31.7), respectively. For preoperative detection of cirrhosis, the pooled AUROC, sensitivity, specificity, and DOR based on the four 2D-SWE studies were 0.877, 79% (95% CI: 71‒86%), 82% (95% CI: 77‒86%), and 17.58 (95% CI: 10.35‒29.85), respectively. Conclusions: Results show that 2D-SWE has potential as a non-invasive tool for detecting advanced liver fibrosis and cirrhosis in patients with biliary atresia. [ABSTRACT FROM AUTHOR]

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. The sample consisted of 10 infants with chronic liver disease. All had refractory ascites and decreased portal vein size. Their mean age ± standard deviation was 5 ± 1 months and their mean weight was 5.4 ± 1.0 kg. We calculated the pediatric end-stage liver disease score and portosystemic gradients before and after TIPS placement. We used ultrasound to check for complications and to assess the presence of ascites. We used paired-sample t-test for the mean comparison of paired variables. Results: Ten TIPS procedures were performed that were technically and hemodynamically successful except for one, in which an extrahepatic portal puncture required surgical repair. Ascites resolved in three infants and was reduced in six. The portal vein size remained stable after TIPS placement. Four infants had early stent thrombosis and two had late stent thrombosis treated with angioplasty or covered stents. Conclusion: TIPS placement in infants is a feasible, safe and effective procedure. [ABSTRACT FROM AUTHOR]

The gallbladder is not part of the standard survey of a fetus during obstetrical ultrasound. Yet, some anomalies can be detected. Most are clinically benign, but some are features of or associated with more serious complications. Biliary atresia constitutes the diagnosis with the worst prognosis. Its in utero diagnosis is difficult. Still, markers do exist and should be searched for. Knowledge of the normal appearances, variants and abnormalities of the gallbladder contributes to proper management. Any suspicion should lead to meticulous postnatal workup. Pre- or postnatal magnetic resonance imaging can provide additional information in select cases. [ABSTRACT FROM AUTHOR]

Background: Percutaneous ultrasound (US)-guided cholecystocholangiography is effective in diagnosing biliary atresia for infants with a gallbladder >1.5 cm in length on US. However, whether it is still effective for other types of gallbladders needs further clarification. Objective: To evaluate the diagnostic performance and safety of percutaneous US-guided cholecystocholangiography combined with liver biopsy in children with suspected biliary atresia and with different types of gallbladders on US. Materials and methods: Sixty-five infants were referred for percutaneous US-guided cholecystocholangiography with microbubbles and liver biopsy after an equivocal (n=39) or highly suspected (n=26) US diagnosis of biliary atresia. Two radiologists evaluated US and percutaneous US-guided cholecystocholangiography images in consensus. One pathologist independently evaluated liver specimens. We used the unpaired t-test, Mann-Whitney U test and chi-square test to analyze the data. Results: Of the 65 infants, 59 (90.8%) underwent a successful percutaneous US-guided cholecystocholangiography, with both sensitivity and specificity of 100%. All six infants for whom puncture failed had contracted gallbladders. The sensitivity and specificity of liver biopsy in the diagnosis of biliary atresia were 89.7% (26/29) and 83.3% (30/36), respectively. When percutaneous US-guided cholecystocholangiography and liver biopsy were combined, all infants gained correct diagnosis, and in 35 infants (97.2%, 35/36) biliary atresia could be excluded without intraoperative cholangiography. Twenty-two of 65 infants (33.8%) had fluid collections around the liver related to puncture. None of these complications needed treatment. Conclusion: Percutaneous US-guided cholecystocholangiography combined with liver biopsy appears safe and effective for excluding or confirming biliary atresia in cholestatic infants with a dilated gallbladder on US. [ABSTRACT FROM AUTHOR]

Biliary atresia is challenging to diagnose because many of the clinical and imaging features of this condition overlap with those of other causes of cholestasis in newborns. When jaundice persists beyond 2 weeks of age, the neonate should be evaluated for cholestasis, and biliary atresia — the most common cause of neonatal cholestasis — should be considered. It is critical to diagnose biliary atresia early because failure to treat can result in hepatic fibrosis and death in less than 1 year. In this paper, we review the current diagnostic imaging methods, differential considerations and treatment options for biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Neonatal/infantile jaundice is relatively common, and most cases resolve spontaneously. However, in the setting of unresolved neonatal cholestasis, a prompt and accurate assessment for biliary atresia is vital to prevent poor outcomes. Objective: To determine whether shear wave elastography (SWE) alone or combined with gray-scale imaging improves the diagnostic performance of US in discriminating biliary atresia from other causes of neonatal jaundice over that of gray-scale imaging alone. Materials and methods: Infants referred for cholestatic jaundice were assessed with SWE and gray-scale US. On gray-scale US, two radiology readers assessed liver heterogeneity, presence of the triangular cord sign, hepatic artery size, presence/absence of common bile duct and gallbladder, and gallbladder shape; associated interobserver correlation coefficients (ICC) were calculated. SWE speeds were performed on a Siemens S3000 using 6C2 and 9 L4 transducers with both point and two-dimensional (2-D) SWE US. Both univariable and multivariable analyses were performed, as were receiver operating characteristic curves (ROC) and statistical significance tests (chi-squared, analysis of variance, t-test and Wilcoxon rank sum) when appropriate. Results: There were 212 infants with biliary atresia and 106 without biliary atresia. The median shear wave speed (SWS) for biliary atresia cases was significantly higher (P<0.001) than for non-biliary-atresia cases for all acquisition modes. For reference, the median L9 point SWS was 2.1 m/s (interquartile range [IQR] 1.7–2.4 m/s) in infants with biliary atresia and 1.5 m/s (IQR 1.3–1.9 m/s) in infants without biliary atresia (P<0.001). All gray-scale US findings were significantly different between biliary-atresia and non-biliary-atresia cohorts (P<0.001), intraclass correlation coefficient (ICC) range 0.7–1.0. Triangular cord sign was most predictive of biliary atresia independent of other gray-scale findings or SWS — 96% specific and 88% sensitive. Multistep univariable/multivariable analysis of both gray-scale findings and SWE resulted in three groups being predictive of biliary atresia likelihood. Abnormal common bile duct/gallbladder and enlarged hepatic artery were highly predictive of biliary atresia independent of SWS (100% for girls and 95–100% for boys). Presence of both the common bile duct and the gallbladder along with a normal hepatic artery usually excluded biliary atresia independent of SWS. Other gray-scale combinations were equivocal, and including SWE improved discrimination between biliary-atresia and non-biliary-atresia cases. Conclusion: Shear wave elastography independent of gray-scale US significantly differentiated biliary-atresia from non-biliary-atresia cases. However, gray-scale findings were more predictive of biliary atresia than elastography. SWE was useful for differentiating biliary-atresia from non-biliary-atresia cases in the setting of equivocal gray-scale findings. [ABSTRACT FROM AUTHOR]

We aim to present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses to biliary cirrhosis, end-stage liver failure and death within the first years of life. Differentiating biliary atresia from other nonsurgical causes of neonatal cholestasis is difficult as there is no single method for diagnosing biliary atresia and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. In this second part, we discuss the roles of magnetic resonance (MR) cholecystopancreatography, hepatobiliary scintigraphy, percutaneous biopsy and percutaneous cholecysto-cholangiography. Among imaging techniques, ultrasound (US) signs have a high specificity, although a normal US examination does not rule out biliary atresia. Other imaging techniques with direct opacification of the biliary tree combined with percutaneous liver biopsy have roles in equivocal cases. MR cholecystopancreatography and hepatobiliary scintigraphy are not useful for the diagnosis of biliary atresia. We propose a decisional flowchart for biliary atresia diagnosis based on US signs, including elastography, percutaneous cholecysto-cholangiography or endoscopic retrograde cholangiopancreatography and liver biopsy. [ABSTRACT FROM AUTHOR]

Background: Although fibrosis is the main determinant of liver stiffness, other disease-related factors usually disregarded in studies on liver elastography, such as inflammation and cholestasis, may influence liver stiffness. Objective: To evaluate the accuracy of two-dimensional (2-D) shear wave elastography in assessing liver fibrosis in children with chronic liver disease by controlling for the confounding role of several disease- and patient-related factors. Materials and methods: Three disease groups were studied: 1) various chronic liver diseases, 2) autoimmune hepatitis and 3) biliary atresia. The METAVIR (meta-analysis of histological data in viral hepatitis) score was used for fibrosis staging and grading of necroinflammatory activity. Multiple linear regression was used to evaluate the relationship between liver stiffness measurements and disease-related factors. The diagnostic accuracy of elastography for predicting fibrosis stages was assessed by calculating the area under the receiver operating characteristic curves. Results: The various chronic liver diseases group (n=32; 7.1±4.9 [mean±standard deviation] years) showed liver stiffness of 8.9±5.0 (mean±standard deviation) kPa, the autoimmune hepatitis group (n=33; 8.1±4.4 years) of 7.1±2.7 kPa, and the biliary atresia group (n=19; 0.2±0.1 years) of 19.7±15.2 kPa. Liver stiffness measurements differed across METAVIR fibrosis categories in all disease groups. The highest values were found in biliary atresia, at fibrosis stages ≥F2 (F2: 12.4±1.6 kPa, F3: 17.8±2 kPa, F4: 41.5±12.4 kPa). Liver stiffness was strongly associated only with fibrosis (P<0.0001) in various chronic liver diseases, but with necroinflammatory activity (P<0.0001) and fibrosis (P=0.002) in autoimmune hepatitis, and with age (P<0.0001), fibrosis (P<0.0001) and cholestasis (P=0.009) in biliary atresia. Optimal cutoffs for detecting advanced fibrosis (≥F3) were 16 kPa (area under curve: 0.98; sensitivity: 87.5%; specificity: 96.7%) in biliary atresia and 8.7 kPa (area under curve: 0.98; sensitivity: 93.8%; specificity: 96.1%) in other chronic liver diseases. Conclusion: Two-dimensional shear wave elastography is reliable in assessing liver fibrosis in children with chronic liver diseases. [ABSTRACT FROM AUTHOR]

We present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses towards biliary cirrhosis, end-stage liver failure and death by age 3. Differentiation of biliary atresia from other nonsurgical causes of neonatal cholestasis is challenging because there is no single method for diagnosing biliary atresia, and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. Concerning imaging, our systematic literature review shows that ultrasonography is the main tool for pre- and neonatal diagnosis. Key prenatal features, when present, are non-visualisation of the gallbladder, cyst in the liver hilum, heterotaxy syndrome and irregular gallbladder walls. Postnatal imaging features have a very high specificity when present, but a variable sensitivity. Triangular cord sign and abnormal gallbladder have the highest sensitivity and specificity. The presence of macro- or microcyst or polysplenia syndrome is highly specific but less sensitive. The diameter of the hepatic artery and hepatic subcapsular flow are less reliable. When present in the context of acholic stools, dilated intrahepatic bile ducts rule out biliary atresia. Importantly, a normal US exam does not rule out biliary atresia. Signs of chronic hepatopathy and portal hypertension (portosystemic derivations such as patent ductus venosus, recanalised umbilical vein, splenomegaly and ascites) should be actively identified for - but are not specific for - biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia is a progressive obliterative cholangiopathy affecting both extrahepatic and intrahepatic biliary trees, resulting in fibrous obliteration of the biliary tract and subsequent development of cirrhosis. Objective: The aim of this study was to find noninvasive indices to predict the status of hepatic fibrosis in children with biliary atresia. Materials and methods: We retrospectively measured the volume of the hepatic lobes and spleen from MR images, obtained biochemical data and analyzed the relationship between the imaging and biochemical indices, and the pathological status of hepatic fibrosis in 35 children with biliary atresia. Results: A combined index was obtained by logistic regression: logit (likelihood of cirrhosis) = 0.00043 x age at MR examination + 1.67 x aspartate aminotransferase and platelet ratio index (APRI) + 0.0029 x body-surface-area-adjusted left liver lobe volume (BSA adLLV) – 6.57 (log-likelihood chi-square P<0.05, pseudo-R2=0.59). The area under the receiver operator characteristic curve of age at MR examination, APRI, BSA adLLV and the combined index for prediction of cirrhosis were 0.91, 0.86, 0.83 and 0.94, respectively. The optimal cut-off value (sensitivity and specificity) of age at MR examination, APRI, BSA adLLV and combined index were 132 (86% and 92%), 1.3 (91% and 85%), 855.5 (96% and 62%) and 0.689 (91% and 92%). The accuracy of age at MR examination, APRI, BSA adLLV and combined index were 89%, 89%, 83% and 91%, respectively. Conclusion: A combined noninvasive index of age, aspartate aminotransferase and platelet ratio index, and the body-surface-area-adjusted left liver lobe volume measured from MR images is a potential marker of liver cirrhosis in children with biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Doppler US is the primary screening for post-liver transplant vascular complications, but indeterminate findings require further imaging.Objective: To evaluate whether contrast-enhanced US improves diagnostic assessment of vascular complications suspected by Doppler US.Materials and Methods: We retrospectively reviewed Doppler US and contrast-enhanced US studies obtained in the first week following liver transplant. Doppler US was performed twice daily for the first 5 postoperative days, and CEUS in the first postoperative day and when vascular complications were suspected. We correlated Doppler US and CEUS with surgical findings, and clinical and imaging follow-up. We evaluated Doppler US and CEUS quality in demonstrating the main hepatic artery (HA) at the porta hepatis as follows: Grade 0 = not seen, Grade 1 = only segments, Grade 2 = entire main HA, and Grade 3 = entire main HA to the intrahepatic branching. We used a Wilcoxon signed rank test to test the difference between Doppler US and CEUS methods.Results: Twenty-nine children (15 girls, 14 boys) were identified, with median age 2.2 years (range 0.5-17.6 years). The most common transplant indication was biliary atresia (n=13). There was significantly (P<0.0001) improved main HA visualization with CEUS. In five children, CEUS was performed to evaluate suspected vascular complications; CEUS confirmed normal vascularity in two. CEUS demonstrated portal vein thrombosis (n=2) and main HA thrombosis (n=1), confirmed at surgery. In one child the main HA thrombosis was missed; marked HA narrowing was seen retrospectively on CEUS.Conclusion: Immediately following liver transplantation, CEUS improves main HA visualization and diagnostic assessment of vascular complications. [ABSTRACT FROM AUTHOR]

Background: Transjugular intrahepatic portosystemic shunt (TIPS) placement has been extensively studied in adults. The experience with TIPS placement in pediatric patients, however, is limited.Objective: The purpose of this study was to report technical success and clinical outcomes in pediatric patients undergoing TIPS placement.Materials and Methods: Twenty-one children - 12 (57%) boys and 9 (43%) girls, mean age 12.1 years (range, 2-17 years) - underwent TIPS placement from January 1997 to January 2017. Etiologies of hepatic dysfunction included biliary atresia (n=5; 24%), cryptogenic cirrhosis (n=4; 19%), portal or hepatic vein thrombosis (n=4, 14%), autosomal-recessive polycystic kidney disease (n=3; 14%), primary sclerosing cholangitis (n=2; 10%) and others (n=3, 14%). Indications for TIPS placement included variceal hemorrhage (n=20; 95%) and refractory ascites (n=1; 5%). Technical success, manometry findings, stent type, hemodynamic success, complications, liver enzymes, and clinical outcomes were recorded.Results: TIPS placement was technically successful in 20 of 21 (95%) children, with no immediate complications. Mean pre- and post-TIPS portosystemic gradient was 18.5±10.7 mmHg and 7.1±3.9 mmHg, respectively. Twenty-two total stents were successfully placed in 20 children. Stents used included: Viatorr (n=9; 41%), Wallstent (n=7; 32%), Express (n=5; 23%), and iCAST (n=1; 5%). All children had resolution of variceal bleeding or ascites. TIPS revision was required in 9 (45%) children, with a mean of 2.2 revisions. Hepatic encephalopathy developed in 10 children (48%), at a mean of 223.7 days following TIPS placement. During the study, 6 (29%) children underwent liver transplantation.Conclusion: TIPS placement in pediatric patients has high technical success with excellent resolution of variceal hemorrhage and ascites. TIPS revision was required in nearly half of the cohort, with hepatic encephalopathy common after shunt placement. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia commonly leads to liver fibrosis and cirrhotic complications, including esophageal varices.Objective: To evaluate liver and spleen stiffness measurements using acoustic radiation force impulse (ARFI) imaging for diagnosing grade of liver fibrosis and predicting the presence of esophageal varices in patients treated for biliary atresia.Materials and Methods: ARFI imaging of the spleen and native liver was performed in 28 patients with biliary atresia. We studied the relation between ARFI imaging values and liver histology findings (n=22), upper gastrointestinal endoscopy findings (n=16) and several noninvasive test results. Diagnostic accuracy was assessed using receiver operating characteristic curve analyses.Results: Liver stiffness measurements exhibited a significant difference among the different grades of liver fibrosis (P=0.009), and showed higher values in patients with high-risk esophageal varices than in the other patients (P=0.04). The areas under the receiver operating characteristic curves of liver stiffness measurements for liver fibrosis grades  ≥ F2, ≥F3 and = F4 were 0.83, 0.93 and 0.94, respectively. Patients with high-risk esophageal varices were preferentially diagnosed by the combined liver and spleen stiffness measurements (area under the curve, 0.92).Conclusion: Liver and spleen stiffness measurements using ARFI imaging are potential noninvasive markers for liver fibrosis and esophageal varices in patients treated for biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Children with biliary atresia are prone to developing progressive hepatic fibrosis and biliary cirrhosis following the Kasai operation. The only treatment is liver transplantation.Objective: To assess liver fibrosis by acoustic radiation force impulse elastography (ARFI) in children who had Kasai operation, with the goal of identifying an ARFI value cut-off for children requiring liver transplantation.Materials and Methods: Of the 32 post-Kasai children included, 19 were transplanted or listed for transplantation (group A), while 13 were not on the list during their follow-up (group B). We recorded biopsies, blood samples and ARFI values over time, including at Kasai operation and at transplantation. We estimated an association between groups and continuous variables using generalized estimating equations, and we compared categorical variables using the Fisher exact test.Results: Portal hypertension signs were similar in both groups, whereas ARFI values were higher in group A (mean±standard deviation=3.3±1.2 m/s) than in group B (2.0±0.7 m/s; P=.0003). Eighteen of 19 (94.7%) children in group A and 6/13 (46.2%) children in group B presented with two consecutive ARFI values ≥2 m/s (sensitivity=7%, specificity=53.8%; P=0.003).Conclusion: We found that children who were transplanted had two consecutive ARFI values ≥2 m/s during follow-up. ARFI for evaluation of post-Kasai liver fibrosis may assist the long-term assessment of biliary atresia and may even guide treatment decisions. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia is a rapidly progressive liver disease necessitating prompt diagnosis and surgical intervention, so it must be promptly distinguished from other neonatal/infantile liver diseases. Objective: To determine whether US shear wave elastography (SWE) can differentiate biliary atresia from other neonatal/infantile liver diseases based on liver hardness. Materials and methods: Eleven children younger than 1 year who had suspected liver disease underwent anatomically and temporally-related hepatic shear wave elastography and clinically indicated percutaneous core needle biopsy. Shear wave elastography was performed immediately prior to liver biopsy at the targeted biopsy site using an Acuson S3000 US system/9L4 transducer (Siemens Medical Solutions USA, Malvern, PA). Shear wave elastography was performed using Virtual Touch Quantification (VTQ) and Virtual Touch IQ (VTIQ) modes, and six shear wave speed measurements were acquired from each subject for each mode. Children were placed in two groups based on histology, biliary atresia ( n = 6) vs. non-biliary atresia (other neonatal/infantile liver diseases) ( n = 5), and mean shear wave speed measurements were compared using the unpaired student's t-test (two-tailed). A P-value <0.05 was considered significant. Results: Using the VTQ mode, mean liver shear wave speed was 2.08 ± 0.17 m/s for the biliary atresia group and 1.28 ± 0.13 m/s for the non-biliary atresia group ( P < 0.0001). Using the VTIQ mode, mean liver shear wave speed was 3.14 ± 0.73 m/s for the biliary atresia group and 1.61 ± 0.23 m/s for the non-biliary atresia group ( P = 0.003). Ishak liver fibrosis scores ranged from 3 to 6 for the biliary atresia group and from 0 to 1 for the non-biliary atresia group. Conclusion: Liver shear wave speed is abnormally increased in neonates and infants with biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Magnetic resonance cholangiopancreatography (MRCP) could aid in the diagnosis of biliary atresia, a hepatic pathology with thin, irregular or interrupted biliary ducts. There is little published evidence of MRCP appearances in normal neonates and young infants. Objective: To assess the use of MR cholangiopancreatography in visualizing the biliary tree in neonates and infants younger than 3 months with no hepatobiliary disorder, and to assess this visibility in relationship to the child's age, weight, and sedation and fasting states. Materials and methods: Between December 2008 and October 2010 our department performed MRI of the brain, orbits and face on 16 full-term neonates and infants. Each child was younger than 3 months (90 days) and without any hepatobiliary disorders. The children were scanned with a respiratory-gated 0.54 × 0.51 × 0.4-mm 3-D MRCP sequence. We used a reading grid to assess subjectively the visibility of the extrahepatic bile ducts along with extrahepatic bile duct confluence. The visibility of the extrahepatic bile duct confluence was assessed against age, weight, and sedation and fasting states. Results: The extrahepatic bile duct confluence was seen in 10 children out of 16 (62.5%). In the neonate sub-group (corrected age younger than 30 days), the MRCP was technically workable and the extrahepatic bile duct confluence was seen in four cases out of eight (50%). This visualization was up to 75% in the subgroup older than 30 days. However, statistically there was no significant difference in visibility of the extrahepatic bile duct confluence in relationship to age, weight or MRCP performance conditions (feeding, fasting or sedation). Conclusion: The complete normal biliary system (extrahepatic bile duct confluence included) is not consistently visualized in infants younger than 3 months old on non-enhanced MRCP. Thus the use of MRCP to exclude a diagnosis of biliary atresia is compromised at optimal time of surgery. [ABSTRACT FROM AUTHOR]

Background: Hepatobiliary scintigraphy is highly sensitive for diagnosing biliary atresia; however, its specificity has varied in the literature from 35% to 97%. Objective: The purpose of this study was to re-evaluate the accuracy of phenobarbital-enhanced hepatobiliary scintigraphy in differentiating biliary atresia from other causes of neonatal cholestasis. Materials and methods: We retrospectively reviewed all hepatobiliary scans of infants with cholestasis at our institution from December 1990 to May 2011. Per our routine protocol the scans were obtained after pretreatment with phenobarbital (5 mg/kg/day for 5 days) to achieve a serum level of ≥15 mcg/ml. Normal hepatic uptake with no biliary excretion by 24 h was considered consistent with biliary atresia. Results: One hundred eighty-six infants with 210 hepatobiliary scans composed the study group. Forty-three (23%) infants had the final diagnosis of biliary atresia. Hepatobiliary scintigraphy was 100% sensitive, 93% specific and 94.6% accurate in diagnosing biliary atresia. Of the 186, 39/111 (35.1%) term and 2/68 (2.9%) preterm infants had biliary atresia; two of seven children with unknown gestational age also had biliary atresia. Other diagnoses included neonatal hepatitis, total parenteral nutrition cholestasis, Alagille syndrome, cystic fibrosis, choledochal cyst, hypothyroidism, alpha-1 antitrypsin deficiency and persistent cholestasis of unknown etiology. Conclusion: Phenobarbital-enhanced hepatobiliary scintigraphy is highly accurate in differentiating biliary atresia from other causes of neonatal cholestasis. Biliary atresia is rare in premature infants. [ABSTRACT FROM AUTHOR]

A Correction to this paper has been published: https://doi.org/10.1007/s00247-020-04936-2 [ABSTRACT FROM AUTHOR]

Hepatobiliary scintigraphy is an important diagnostic modality for work-up of neonatal cholestasis. Therefore, our objective was to evaluate the literature regarding the accuracy of hepatobiliary scintigraphy in differentiating biliary atresia from non-biliary atresia causes of cholestasis (collectively called neonatal hepatitis). Our search included Medline, SCOPUS and Google Scholar. Only studies using Tc-99 m-labeled immunodiacetic acid (IDA) derivatives were included. Overall, 81 studies were included in the meta-analysis. Pooled sensitivity and specificity were 98.7% (range 98.1-99.2%) and 70.4% (range 68.5-72.2%), respectively. Factors that increased specificity included the use of radiotracers with high hepatic extraction, administration of hepatic-inducing drugs (such as phenobarbital), use of a calculated dose/kg and administration of a booster dose in cases of non-excretion of the tracer in the bowel. SPECT imaging and duodenal fluid sampling also had high specificity; however, they need further validation because of the low number of studies. Semiquantitative imaging methods do not seem to have any incremental value. We conclude that hepatobiliary scintigraphy using IDA derivatives can be very useful for diagnostic work-up of neonatal cholestasis. To improve the specificity, several measures can be followed regarding type and dose of the radiotracer and imaging protocols. Non-imaging methods seem to be promising and warrant further validation. [ABSTRACT FROM AUTHOR]

Background: Historically, HIDA is the initial diagnostic test in the evaluation of biliary atresia (BA). Non-excreting HIDA scans can yield false-positive results leading to negative laparotomy. Objective: Cholestatic infants must be evaluated promptly to exclude biliary atresia (BA) and other treatable hepatic conditions. Intraoperative cholangiogram (IOC) is the gold standard for diagnosing BA, but requires surgical intervention. Percutaneous transhepatic cholecysto-cholangiography (PTCC) and liver biopsy are less invasive and have been described in small case series. We hypothesized that PTCC and liver biopsy effectively exclude BA, thus avoiding unnecessary IOC. Materials and methods: Retrospective review of cholestatic infants who underwent PTCC, biopsy or cholescintigraphy at a tertiary children's hospital from August 1998 to January 2009. Group differences were evaluated and the receiver operator curve and safety of PTCC determined. Results: One-hundred twenty-eight cholestatic infants were reviewed. Forty-six (36%) underwent PTCC. Forty-one out of 46 (89%) had simultaneous PTCC and liver biopsy. PTCC was completed successfully in 19/23 (83%) children despite a small or absent GB on initial US. Negative laparotomy rate was 1/6 (17%) for simultaneous PTCC/liver biopsy. Complications occurred in 4/46 including bleeding ( n = 2), fever with elevated transaminases ( n = 1) and oxygen desaturations ( n = 1). Conclusion: PTCC, particularly when performed in combination with simultaneous liver biopsy, effectively excludes BA in cholestatic infants with acceptable morbidity. PTCC can frequently be performed when a contracted gallbladder is seen on initial US exam. Negative laparotomy rate is lowest when PTCC is coupled with simultaneous liver biopsy. [ABSTRACT FROM AUTHOR]

Background: Ultrasound (US) is used to identify causes of neonatal cholestasis. We describe a potential sonographic pitfall, the 'pseudo gallbladder,' in biliary atresia (BA). Objective: To describe the Pseudo Gallbladder sign (PsGB sign). Materials and methods: Sonograms/clinical records of 20 confirmed BA infants and 20 non-BA cases were reviewed retrospectively. For the BA group, preoperative sonography and surgical and pathological findings were examined. For the non-BA group, sonographic features and pathological findings were examined. The PsGB sign is defined as a fluid-filled structure, located in the expected region of the gallbladder, measuring ≤ 15 mm in length but without a well-defined or normal-appearing gallbladder wall. Results: A recognizable gallbladder and normal gallbladder wall were present in all non-BA infants. However, none of the BA infants had a sonographically normal gallbladder. Seventy-three percent of BA patients had a PsGB, and in 27% no gallbladder or gallbladder-like structure was detected. Conclusion: A gallbladder-like structure in BA is common and can be misinterpreted as a normal gallbladder, delaying diagnosis and therapy. Recognition of this imaging pitfall, described here as the pseudo gallbladder sign, will help avoid this error. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia (BA) is a progressive, obliterative cholangiopathy that occurs in neonates with hepatic portoenterostomy the treatment of choice, but early surgery is important for optimum outcomes. MRI, including MR cholangiopancreatography (MRCP) may be a diagnostically useful alternative to US, but the heavily T2-weighted sequences used include not only bile duct signals, but also other heterogeneously high signal intensities from surrounding structures. Objective: To evaluate the effects of gadolinium when used to decrease background signal intensity on T2-weighted MR cholangiopancreatography (MRCP) in infants and to evaluate the qualitative improvement of the depiction of the common bile duct (CBD) for evaluating neonatal cholestasis. Materials and methods: Our Institutional Review Board approved this prospective study. MRCP was performed with gadopentetate dimeglumine injection using a 1.5-T scanner. Pre- and postcontrast MRCP images were compared. Forty-nine infants (male:female = 21:28; age 0-12 months, mean 2.3) were included. The final diagnoses were biliary atresia (BA) in 28 cases and non-BA in 21. Quantitative analysis was conducted using region-of-interest measurements of mean signal intensities of the liver, pancreatic head and gallbladder (if defined). Qualitative analysis was performed by four radiologists who subjectively scored image confidence in the presence of CBD on a 4-point scale (0 for definitely absent, 1 for probably absent, 2 for probably present, and 3 for definitely present). Results: The signal-to-noise ratios were significantly decreased in the liver and pancreatic head after contrast medium enhancement (mean 5.7→4.0 in liver and mean 44.9→12.7 in the pancreatic head; P < 0.0001), and this finding was constant in both the BA and the non-BA group. The mean confidence score in the presence of CBD decreased in the BA group (0.9→0.5; P < 0.0001), but did not change significantly in the non-BA group (2.0→2.1; P = 0.459) after contrast medium enhancement. Both intra- and interobserver agreement was higher after contrast medium enhancement ( P = 0.046). Conclusion: Gadopentetate dimeglumine-enhanced MRCP increased the diagnostic confidence of absence of the CBD in cholestatic infants with increased intra- and interobserver agreement. [ABSTRACT FROM AUTHOR]

Background: The degree of hepatic fibrosis in biliary atresia (BA) correlates with the prognosis of the disease and thus, early diagnosis of liver fibrosis is clinically important. Liver biopsy is the gold standard for the evaluation of liver fibrosis, but it is an invasive procedure requiring sedation in children. Therefore, it is desirable to identify a noninvasive method for diagnosis and follow-up of hepatic fibrosis. Objective: The purpose of this study is to evaluate the possibility of quantifying liver fibrosis in infants by T2 relaxation time measurements. Materials and methods: The institutional review board approved this prospective study and parental informed consent was obtained. During MR cholangiopancreatography using a 1.5-T MR scanner in infants with neonatal cholestasis, T2 relaxation time of the liver was calculated with the mean signal intensities measured on images obtained using spin-echo sequences (TR/TE, 2,000/20, 40, 60, 80, 100, 120, 140, 160 ms). A normal control study was performed during spinal MRI in infants with anorectal malformation and normal liver enzyme profiles. A liver biopsy was obtained in the children with cholestasis. The correlation between histopathological fibrosis stage and T2 relaxation time was evaluated by Kendall's Tau-b test. Results: Twenty-five infants (male: female, 12:13; age range 0-11 months, mean 3.2 months), 14 with neonatal cholestasis (9 BA and 5 non-BA) and 11 normal controls were included in this study. Relaxation times (mean ± standard deviation [SD]) for the liver were 57.8 ms ± 8.8 in the normal control group ( n = 11) and 56.8 ms ± 9.6 in the BA group ( n = 9) without statistically significant differences ( P = 0.811). T2 relaxation times were not significantly different between the low stage (≤F1) and high stage (≥F2) fibrosis (mean 57.8 vs 56.8; P = 0.934). Conclusion: T2 relaxation of a normal infant liver at 1.5-T had a mean value of 57.8 ms, which is comparable with adult data (46-57 ms). However, T2 relaxation time was not different in patients with BA and did not correlate with stage of fibrosis. [ABSTRACT FROM AUTHOR]

Biliary atresia and related disorders of the biliary tree, such as choledochal cyst, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in infants and children. Pediatric biliary tract diseases include a variety of entities with a wide range of clinical presentations. Radiology plays an important role in the diagnosis and management of these pathologies. Unrecognized causes of biliary disease, like biliary atresia, can lead to liver transplantation during the first year of life. The aim of this article is to review the imaging of pediatric biliary disorders, including the implications of interventional radiology in some biliary diseases. [ABSTRACT FROM AUTHOR]

We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results. [ABSTRACT FROM AUTHOR]

We report a newborn with bilious vomiting and the rare combination of pyloric atresia, annular pancreas and ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum. Radiologic, sonographic and percutaneous transhepatic transcholecystic cholangiographic (PTTC) findings, with surgical correlation, are presented. [ABSTRACT FROM AUTHOR]

Objective. To describe the gallbladder ghost triad and evaluate its usefulness in the sonographic diagnosis of extrahepatic biliary atresia (BA). Methods. From October 1997 to February 2002, 217 fasted infants with cholestatic jaundice aged 2–12 weeks were examined sonographically . We defined the gallbladder ghost triad as gallbladder length <1.9 cm, lack of smooth/complete echogenic mucosal lining with an indistinct wall and irregular/lobular contour, and used it as a criteria for BA. Gallbladder wall thickness, triangular cord, diffuse periportal echogenicity and hepatic artery calibre were also recorded. Diagnosis of BA was confirmed surgically and histologically. Results. Thirty of 31 babies with BA demonstrated the gallbladder ghost triad. No false-positives were recorded. The 31st BA baby showed a normal gallbladder at 6 weeks, but developed the ghost triad at 8 weeks. Gallbladder wall thickening was seen in 46/186 non-BA babies, but not in BA. Triangular cord was observed in 24/31 babies. Twenty-two of 186 non-BA babies and 5/31 BA babies showed diffuse periportal echogenicity. The hepatic artery appeared more prominent in BA. All 31 babies diagnosed sonographically as BA had surgery. Three non-BA babies had "negative" laparotomies showing hypoplastic bile ducts. Conclusions. The gallbladder ghost triad is a very accurate sign of BA. Indeterminate cases require close follow-up. [ABSTRACT FROM AUTHOR]

We describe the US findings in two vomiting newborns affected by different forms of pyloric atresia, a rare congenital anomaly that includes a spectrum of lesions limited to the antro-pyloric region of the stomach and with various inheritance mechanisms and syndromic associations. [ABSTRACT FROM AUTHOR]

Background. To evaluate the accuracy and utility of the triangular cord sign and gallbladder length in diagnosing biliary atresia by sonography.¶Materials and methods. Sixty fasted infants with cholestatic jaundice aged 2–12 weeks were examined sonographically using a 5–10 MHz linear array transducer, focusing on the triangular cord sign (as described by Choi et al. [1]), the gallbladder, and ducts. The triangular cord is defined as a triangular or tubular echogenic density seen immediately cranial to the portal vein bifurcation; it represents the fibrotic remnant of the obliterated cord in biliary atresia. The findings were blinded to blood chemistry, 99 mTc-DISIDA hepatobiliary scintigraphy, and liver biopsy. Diagnosis of biliary atresia was confirmed at surgery and histology. Non-biliary atresia infants resolved medically. Comparative charges of the various investigations was made.¶Results. Twelve infants had biliary atresia, and ten demonstrated a definite triangular cord. The two false-negatives had small or nonvisualized gallbladders. No false-positives were recorded. Gallbladder length ranged from 0–1.45 cm with a mean of 0.52 cm in biliary atresia compared to a mean of 2.39 cm in nonbiliary atresia infants. 99 mTc-DISIDA hepatobiliary scintigraphy showed no excretion (false-positive) in 23 % of nonbiliary atresia cases. Scintigraphy and liver biopsy charges were 2 and 6 times that of sonography, respectively.¶Conclusion. The triangular cord sign and gallbladder length together are noninvasive, inexpensive, and very useful markers for biliary atresia. [ABSTRACT FROM AUTHOR]

Twenty-eight infants with jaundice were evaluated with ultrasound and radionuclide scans. Comparison of these studies with pathologic, surgical and clinical examinations demonstrated good correlation between ultrasonic and radionuclide studies. Ultrasound is an important preliminary study in the workup of such patients. Combined imaging provides the most information in a patient with suspected biliary atresia. [ABSTRACT FROM AUTHOR]

Four infants with prolonged cholestasis, of differing aetiologies, developed metabolic bone disease during infancy. Radiologically three distinct patterns of skeletal abnormality were found: classical vitamin D deficiency rickets; axial skeleton demineralization with rib fractures and rickets; and in one patient, long term defective remodelling of tubular bones. Variations in the age of onset of rickets may be related to the baby's gestational maturity at birth, while its severity is probably more closely linked to the interval before vitamin therapy was commenced. In such infants increased supplements of prophylactic vitamin D are recommended. [ABSTRACT FROM AUTHOR]

Accurate noninvasive biomarkers for BA have remained elusive and liver biopsy has continued to have the highest positive predictive value. The authors concluded that MMP-7 has a high discriminatory value for BA and that, as a biomarker, it should substantially simplify diagnostic algorithms for neonatal cholestasis. However, after TIPS creation, 15.8% of patients had recurrent variceal bleeding. [Extracted from the article]

Percutaneous transluminal angioplasty is increasingly used in children. We present a case of successful balloon angioplasty of an occluded surgical splenorenal shunt in a 4-year-old child who underwent liver transplantation because of biliary atresia. Percutaneous reopening of the shunt is a relatively safe procedure that may spare patients of surgical intervention. [ABSTRACT FROM AUTHOR]

We report a 3.5-month-old boy with polysplenia syndrome who demonstrated hemiazygos continuation of the inferior vena cava, extrahepatic biliary atresia, multiple splenunculi, bowel malrotation, and the rare finding of brainstem and cerebellar hypoplasia. A possible pathogenesis for cerebellar hypoplasia in this syndrome is suggested after review of the literature. The importance of seeking associated anomalies in biliary atresia, which may be possible indicators of polysplenia syndrome, is stressed since these patients need appropriate management when surgery is considered. [ABSTRACT FROM AUTHOR]

We describe an infant with intrahepatic portosystemic venous shunt (IPSVS), which was detected by MR angiography. IPSVS is rare and its cause is disputed. However, with improvements in imaging the number of reports of IPSVS identified incidentally in patients without definite symptoms is increasing. The present case is the first associated with congenital biliary atresia and the youngest reported in the literature. [ABSTRACT FROM AUTHOR]