Triangular Cord Research Articles

A prospective multicentre study evaluating the performance of the modified simple biliary atresia scoring system in predicting biliary atresia.

Early diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates. We evaluate the performance of the modified Simple BA Scoring System (SBASS) in diagnosing BA. We performed a prospective, cross-sectional study on infants with cholestatic jaundice (June 2021-December 2022). Modified SBASS scoring was applied and compared to the eventual diagnosis (as per intraoperative cholangiogram (IOC) and liver histopathology). The score (0-6), consists of gall bladder length < 1.6cm (+ 1), presence of triangular cord sign (+ 1), conjugated bilirubin:total bilirubin ratio > 0.7(+ 2), gamma-glutamyl transferase (GGT) ≥ 200 U/L (+ 2). 73 were included: Fifty-two (71%) had BA. In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15 (72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the modified SBASS showed sensitivity of 96.2%, specificity of 61.9% and overall accuracy of 86.3% in diagnosing BA. Area under receiver operating characteristic curve was 0.901. GGT had the highest sensitivity (94.2%), while triangular cord sign showed the highest specificity at 95.2%. The SBASS provides a bedside, non-invasive scoring system for exclusion of BA in infantile cholestatic jaundice and reduces the likelihood of negative surgical explorations.

Diagnostic Accuracy of Ultrasound in Cholestatic Infants with Biliary Atresia.

Biliary atresia (BA) is an obstructive cholangiopathy that involves the intrahepatic and extrahepatic bile ducts. Ultrasound (US) can aid in evaluation of the biliary system and be efficiently used in daily practice. However, most studies on US for diagnosing BA have been conducted in developed countries. Therefore, we have aimed to evaluate the diagnostic accuracy of US in BA in infants with cholestasis from a developing country. This retrospective study used data collected from our hospital medical records. The US findings were compared with the gold standard intraoperative or cholangiography findings. Thirty-five BA patients (19 males and 16 females) and 36 controls (20 males and 16 females) were included in the study. Most of the patients (85.7%) were ≤ 6 months old. The absence of a gallbladder demonstrated 71.42% sensitivity (Sn), 91.67% specificity (Sp), 89.29% positive predictive value (PPV), 76.74% negative predictive value (NPV), 8.57 positive likelihood ratio (LR+), and 0.31 negative likelihood ratio (LR-) for diagnosing BA. The triangular cord sign demonstrated 14.28% Sn, 100% Sp, 100% PPV, 76.74% NPV, ∞ LR+, and 0.86 LR- for diagnosing BA. The combination of gallbladder absence and a positive triangular cord sign demonstrated 82.85% Sn, 91.67% Sp, 90.63% PPV, 84.61% NPV, 9.95 LR+, and 0.19 LR- for diagnosing BA. The diagnostic accuracy of US in BA is high, indicating that it can be the imaging tool of choice in infants with cholestasis. Ultrasound is safe and can be easily used in daily practice without the risk of radiation exposure.

Porta hepatis lymph nodes on US: not only identify biliary atresia but also predict outcomes after Kasai portoenterostomy surgery

ObjectivesTo evaluate the usefulness of porta hepatis lymph nodes (PHLNs) on ultrasonography (US) scans in diagnosing biliary atresia (BA) and predicting the outcomes after Kasai portoenterostomy (KPE) surgery.MethodsA total of 668 patients from one hospital were enrolled in the study (542 non-BA and 126 BA). The independent and combined diagnostic efficacy of PHLNs, triangular cord (TC) thickness, and gallbladder morphology were assessed by drawing the receiver operating characteristic (ROC) curves and counting the area under the ROC curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). The US features, histopathological findings of PHLNs, and serum total bilirubin (TBIL) levels 3 months post-KPE were correlated.ResultsThe AUC, sensitivity, specificity, PPV, and NPV of PHLNs with hyperechogenicity and a maximum length larger than 8.4 mm were 0.898, 81.8%, 97.8%, 89.6%, and 95.8%, respectively. The combination of PHLNs, TC thickness, and gallbladder morphology achieved the best overall diagnostic efficacy among all indicators with an AUC of 0.927 and a sensitivity of 99.2%. The germinal center number and bile particle number of PHLNs were positively correlated with pathological size and US echogenicity intensity of PHLNs, respectively (r = 0.591, 0.377, p = 0.001, 0.004). The pathological size of PHLNs in BA patients was negatively correlated with jaundice clearance status 3 months after KPE surgery (r = −0.385, p = 0.047).ConclusionPHLNs with hyperechogenicity and a maximum length > 8.4 mm are useful US indicators for BA diagnosis. Additionally, the enlargement of PHLNs might play a role in predicting outcomes of KPE surgery.Critical relevance statementThe article proposed for the first time that PHLNs with hyperechogenicity and a maximum length > 8.4 mm are a useful US indicator for diagnosing BA.Key PointsPHLNs may be helpful in diagnosing BA and predicting outcomes after surgery.Enlarged hyperechoic PHLNs are a useful diagnostic indicator for BA, and play a role in predicting surgical outcomes.These findings can assist clinicians in more accurately diagnosing BA, enabling more timely treatments.Graphical

Comparison for the diagnostic performance of early diagnostic methods for biliary atresia: a systematic review and network meta-analysis.

Biliary atresia (BA), a progressive condition affecting canalicular-bile duct function/anatomy, requires prompt surgical intervention for favorable outcomes. Therefore, we conducted a network meta-analysis of common diagnostic methods to assess their performance and provide evidence-based support for clinical decision-making. We reviewed literature in PubMed, EMBASE, and Cochrane for BA diagnostics. The search included gamma-glutamyl transferase (GGT), direct/combined bilirubin, matrix metalloproteinase 7 (MMP-7), ultrasonic triangular cord sign (TCS), hepatic scintigraphy (HS), and percutaneous cholangiocholangiography/percutaneous transhepatic cholecysto-cholangiography (PCC/PTCC). QUADAS-2 assessed study quality. Heterogeneity and threshold effect were evaluated using I2 and Spearman's correlation. We combined effect estimates, constructed SROC models, and conducted a network meta-analysis based on the ANOVA model, along with meta-regression and subgroup analysis, to obtain precise diagnostic performance assessments for BA. A total of 40 studies were included in our analysis. GGT demonstrated high diagnostic accuracy for BA with a sensitivity of 81.5% (95% CI 0.792-0.836) and specificity of 72.1% (95% CI 0.693-0.748). Direct bilirubin/conjugated bilirubin showed a sensitivity of 87.6% (95% CI 0.833-0.911) but lower specificity of 59.4% (95% CI 0.549-0.638). MMP-7 exhibited a total sensitivity of 91.5% (95% CI 0.893-0.934) and a specificity of 84.3% (95% CI 0.820-0.863). TCS exhibited a sensitivity of 58.1% (95% CI 0.549-0.613) and high specificity of 92.9% (95% CI 0.911-0.944). HS had a high sensitivity of 98.4% (95% CI 0.968-0.994) and moderate specificity of 79.0% (95% CI 0.762-0.816). PCC/PTCC exhibited excellent diagnostic performance with a sensitivity of 100% (95% CI 0.900-1.000) and specificity of 87.0% (95% CI 0.767-0.939). Based on the ANOVA model, the network meta-analysis revealed that MMP-7 ranked second overall, with PCC/PTCC ranking first, both exhibiting superior diagnostic accuracy compared to other techniques. Our analysis showed no significant bias in most methodologies, but MMP-7 and hepatobiliary scintigraphy exhibited biases, with p values of 0.023 and 0.002, respectively. MMP-7 and ultrasound-guided PCC/PTCC show diagnostic potential in the early diagnosis of BA, but their clinical application is restricted due to practical limitations. Currently, the cutoff value of MMP-7 is unclear, and further evidence-based medical research is needed to firmly establish its diagnostic value. Until more evidence is available, MMP-7 is not suitable for widespread diagnostic use. Therefore, considering cost and operational simplicity, liver function tests combined with ultrasound remain the most clinically valuable non-invasive diagnostic methods for BA.

Predictive modeling for early detection of biliary atresia in infants with cholestasis: Insights from a machine learning study

Cholestasis, characterized by the obstruction of bile flow, poses a significant concern in neonates and infants. It can result in jaundice, inadequate weight gain, and liver dysfunction. However, distinguishing between biliary atresia (BA) and non-biliary atresia in these young patients presenting with cholestasis poses a formidable challenge, given the similarity in their clinical manifestations. To this end, our study endeavors to construct a screening model aimed at prognosticating outcomes in cases of BA. Within this study, we introduce a wrapper feature selection model denoted as bWFMVO-SVM-FS, which amalgamates the water flow-based multi-verse optimizer (WFMVO) and support vector machine (SVM) technology. Initially, WFMVO is benchmarked against eleven state-of-the-art algorithms, with its efficiency in searching for optimized feature subsets within the model validated on IEEE CEC 2017 and IEEE CEC 2022 benchmark functions. Subsequently, the developed bWFMVO-SVM-FS model is employed to analyze a cohort of 870 consecutively registered cases of neonates and infants with cholestasis (diagnosed as either BA or non-BA) from Xinhua Hospital and Shanghai Children's Hospital, both affiliated with Shanghai Jiao Tong University. The results underscore the remarkable predictive capacity of the model, achieving an accuracy of 92.639 % and specificity of 88.865 %. Gamma-glutamyl transferase, triangular cord sign, weight, abnormal gallbladder, and stool color emerge as highly correlated with early symptoms in BA infants. Furthermore, leveraging these five significant features enhances the interpretability of the machine learning model's performance outcomes for medical professionals, thereby facilitating more effective clinical decision-making.

Development and validation of a model for early diagnosis of biliary atresia

Background and aimsEarly diagnosis of biliary atresia (BA), particularly distinguishing it from other causes of neonatal cholestasis (NC), is challenging. This study aimed to design and validate a predictive model for BA by using the data available at the initial presentation.MethodsInfants presenting with NC were retrospectively identified from tertiary referral hospitals and constituted the model design cohort (n = 148); others were enrolled in a prospective observational study and constituted the validation cohort (n = 21). Clinical, laboratory, and abdominal ultrasonographic features associated with BA were assessed. A prediction model was developed using logistic regression and decision tree (DT) analyses.ResultsThree predictors, namely, gamma glutamyl transpeptidase (γGT) level, triangular cord sign (TC sign), and gallbladder abnormalities, were identified as factors for diagnosing BA in multivariate logistic regression, which was used to develop the DT model. The area under the receiver operating characteristic (ROC) curve (AUC) value for the model was 0.905, which was greater than those for γGT level, TC sign, or gallbladder abnormalities alone in the prediction of BA.ConclusionA simple prediction model combining liver function and abdominal ultrasonography findings can provide a moderate and early estimate of the risk of BA in patients with NC.

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

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. 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. Novel scoring systems were developed incorporating established sonographic parameters and hepatic stiffness values and analysed using SPSS software. Of the 18 patients confirmed as BA, 3 were misdiagnosed on conventional sonography (16.7%) as non-BA. Gall bladder (GB) wall irregularity and fasting GB length were the most accurate (93.8%) and most specific (97.8%) individual parameters, respectively. A significant difference was noted in the triangular cord (TC) thickness of BA and non-BA infants (p <0.001), with a high specificity of 95.6% for a 4 mm cut-off value for a positive TC sign. Comparison of hepatic SWE stiffness among age-matched groups of BA and non-BA showed significant differences (≤60d: p=0.003; >60d: p <0.001) but with a reduced accuracy (93.8%). Diagnostic accuracy of greyscale scoring system (96.9%), greyscale + elastography scoring system in ≤60 d (94.4%) and >60 d (97.8%) were better than that of conventional sonographic diagnosis (93.8%). Grey scale scoring system improves the accuracy of sonographic diagnosis of BA without any additional cost or time penalty along with making it universally reproducible. SWE has only an adjunctive role, if any, in the sonographic diagnosis of BA.

Radial nerve palsy caused by a rapidly growing intramuscular hematoma in an infant with biliary atresia: a case report

BackgroundBiliary atresia (BA) is a rare cause of persistent jaundice in infants that can result in vitamin K malabsorption and vitamin K deficiency bleeding (VKDB). We present an infant with BA who developed a rapidly growing intramuscular hematoma in her upper arm after a vaccination which caused a radial nerve palsy.Case presentationAn 82-day-old girl was referred to our hospital because of a rapidly growing left upper arm mass. She had received three doses of oral vitamin K before age 1 month. At age 66 days, she received a pneumococcal vaccination in her left upper arm. On presentation, she showed no left wrist or finger extension. Blood examination revealed direct hyperbilirubinemia, liver dysfunction, and coagulation abnormalities, indicating obstructive jaundice. Magnetic resonance imaging showed a hematoma in the left triceps brachii. Abdominal ultrasonography revealed an atrophic gallbladder and the triangular cord sign anterior to the portal vein bifurcation. BA was confirmed on cholangiography. VKDB resulting from BA in conjunction with vaccination in the left upper arm were considered the cause of the hematoma. The hematoma was considered the cause of her radial nerve palsy. Although she underwent Kasai hepatic portoenterostomy at age 82 days, the obstructive jaundice did not sufficiently improve. She then underwent living-related liver transplantation at age 8 months. The wrist drop was still present at age 1 year despite hematoma resolution.ConclusionsDelayed detection of BA and inadequate prevention of VKDB can result in permanent peripheral neuropathy.

Shear Wave Elastography: A Reliable Secondary Parameter for Diagnosing Biliary Atresia in Infants With Neonatal Cholestasis.

Objective In this study, we aimed tooptimize various grayscale, Doppler, and elastography parameters and evaluate their diagnostic performance in the preoperative diagnosis of biliary atresia (BA). Materials and methods A total of 158 infants aged <6 months with neonatal cholestasis (NC) were enrolled in the study and sonography was performedafter four hours of fasting. For comparison of elastography, 31 exclusively age-matched controls, not suffering from liver disease, were included separately. Triangular cord and gallbladder (GB) parameters were considered as primary parameters, while right hepatic artery (RHA) caliber, RHA-to-right portal vein (RPV) ratio, hepatic subcapsular flow (HSF), and shear wave elastography (SWE) were considered as secondary parameters. Diagnosis of infants with BA was confirmed on histopathology. Data were presented as mean ±standard deviation (SD) and frequency. Differences between groups were compared using the Chi-square test and the unpaired student t-test. Receiver operating characteristic (ROC) curve analysis was done for individual ultrasound/Doppler/SWE parameters to calculate the optimal cutoff value. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated for each parameter and their combinations. Results Of the primary parameters, GB contractility index (CI) and length showed the highest sensitivity and specificity respectively. A cutoff of 14 kPA was derived for SWE for the diagnosis of BA. Among secondary parameters, SWE had the best diagnostic performance, better than even the individual primary parameters. A combination of primary parameters with SWE inseries showed the highest accuracy. Conclusion Among secondary parameters, elastography can prove to be highly useful. The highest accuracy in diagnosing BA can be obtained by combining primary parameters with SWE.

Diagnostic Value of Ultrasound Parameter in Neonatal Biliary Atresia Based on Histopathological Results (Ultrasound study of triangular cord sign and gallbladder abnormality)

Objective: Biliary atresia is an obliterative progressive disease that forms fibrotic tissue in the biliary tract in the neonatal phase. In some European countries, the reported incidence of biliary atresia varies between 1/14,000 and 1/20,000 live births. Ultrasound examination is the modality used to diagnose Biliary Atresia. Ultrasound assessed the sensitivity, specificity, and accuracy of the Triangular Cord Sign and gallbladder abnormality consisting of gallbladder contractility and wall echogenicity. We evaluated the diagnostic test results of Triangular Cord Sign and gallbladder contractility and wall echogenicity with ultrasound examination based on histopathological results of neonatal biliary atresia Material and Methods:This diagnostic test was performed retrospectively between January 2019 and December 2020 with consecutive sampling techniques. A total of 61 pediatric patients clinically diagnosed with biliary atresia underwent ultrasound examination of triangular cord sign and gall bladder abnormality examined by a pediatric radiologist. Histopathological results of liver biopsy specimens confirming biliary atresia were available for all those patients. Data were analysed to assess accuracy, sensitivity, specificity, PPV, and NPV of ultrasound compared with histopathology result Result: The Triangular Cord Sign sensitivity was found to be 70%, specificity 18.2%, PPV 79.5%, NPV 11.8%, and accuracy 60.7%. There was a significant relationship between Triangular Cord signs and histopathology. Gallbladder contractility parameter sensitivity was 76. %, specificity 18.2%, PPV 80.9%, NPV 14.3%, and accuracy 65.6%. However, there was no significant relationship between the histopathology results and the gallbladder wall's echogenicity, with a sensitivity of 70%, specificity of 18.2%, PPV of 79.5%, NPV of 11.8%, and accuracy of 60.7%. Conclusion:The appearance of the triangular cord sign on ultrasonography has a significant relationship with the results of histopathology in biliary atresia

Gamma-Glutamyl Transferase Combined With Conventional Ultrasound Features in Diagnosing Biliary Atresia: A Two-Center Retrospective Analysis.

To develop and validate a biliary atresia (BA) diagnostic score based on serum gamma-glutamyl transferase (GGT) levels and conventional ultrasound features for discriminating BA in patients with jaundice from two centers. A total of 958 patients from one hospital were classified as the derivation cohort, and 725 patients from another hospital were classified as the validation cohort. The optimal GGT cutoff value for diagnosing BA was calculated in the derivation cohort and subsequently verified in the validation cohort. Gallbladder abnormalities and the triangular cord (TC) sign were evaluated in all patients. A BA diagnostic score was developed for diagnosing BA using the GGT levels, gallbladder abnormalities and the TC sign based on the data from the derivation cohort followed by external validation. Based on the optimal cutoff value 350.0 U/L, GGT yielded a sensitivity of 59.3% and specificity of 85.4% in diagnosing BA. The area under the receiver operating characteristic curve (AUC 0.724) was inferior to that of the gallbladder (AUC 0.911, P < .001) and comparable to that of the TC sign (AUC 0.771, P=.128). The combination of GGT and ultrasound diagnosis could help to reduce the misdiagnosis of 9 infants with BA. The BA diagnostic score yielded a sensitivity of 93.3% and specificity of 95.0% with the highest AUC in this study (0.941). GGT can add diagnostic value to ultrasound examination when diagnosing BA. The BA diagnostic score based on GGT, gallbladder abnormalities and the TC sign shows satisfactory discrimination abilities in BA.

The Liver Biopsy in Neonatal Cholestasis: Just a Cherry on Top?

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Describe imaging features of four cases diagnosed with cystic biliary atresia micmicking choledocal cyst on MRI

Purpose: to describle in detail the significant signs in hepatobiliary MRI, that help to give different diagnosis between biliary atresia (BA) with big cyst at hepatic hilar and choledochal cyst in small childrent and infant.Method: presenting 4 cases with clinical diagnosed of biliary atresia that based on clinical signs and US, MRI results, all 4 cases were operated, taken cholangiogram in surgery to confirm the diagnosis of cystic biliary atresia and had pathological results.Results: All 4 patients were under 3 months old, all are female with enlarged liver; gallbladder were in the normal size limit but all of them had abnormal shape: deformation, irregular wall, communicated directly with common bile duct. All 4 cases have cystic diameter over 10mm. No case of all has further biliary signal that above or under the cyst on MRI but one case has contrast material filling intraliver bile duct draining directly to cystic lesion on cholangiogram intraoperation . These are 3 cases of cystic BA type III and 1 cases of cystic BA type I. 2 cases has positive triangular cord sign (TC sign).Conclusion: Hepatobiliary MRI with its basic sequences, specially with MRCP is helpful to give difirential diagnosis of cystic BA and choledochal cyst in the young childrent.

'Triangular Cord' Sign in Biliary Atresia

담도폐쇄증은 원인은 알 수 없으나 간외 담도의 섬유화로 내강이 막히면서 간문부에 ‘섬유성 담도잔유물’의 형태로 남아있는 질환이다. ‘Triangular cord’ (이하 TC) sign은 간문부에 남아있는 섬유성 담도잔여물을 초음파 검사에서 나타내는 소견이다. 본 종설에서는 담도폐쇄증의 수술 및 초음파 소견의 비교, TC sign의 정의, 객관적인 진단기준, 진단의 정확도 및 감별 진단에 대하여 기술하고자 한다.

Diagnostic Imaging of Biliary Atresia

담도폐쇄증은 신생아 담즙 정체의 드물지만 중요한 원인이다. 적절한 치료와 예후를 위해서는 정확한 조기 진단이 중요하다. 본 종설에서는 초음파 검사, MRI, 간담도 핵의학스캔, 담관조영술 등의 영상 검사에서 담도폐쇄증의 영상 소견을 검토하였다. 지금까지 알려진 주요 영상의학적 소견들은 담낭의 비정상적인 모양과 크기, 간문맥 주변의 비후를 나타내는 ‘triangular cord’ sign, 총담관이 보이지 않고, 간동맥 혈류가 증가하는 소견 등과 함께 동반된 기형의 유무들을 보이는 것이다. 담도폐쇄증의 진단을 위해 초음파 검사, MRI, 간담도 핵의학스캔, 담관조영술 등의 여러 영상 검사들을 시행하며, 1차 영상 검사로 초음파 검사가 중요하다. 본 종설에서는 각 영상 검사들에서 지금까지 알려진 담도폐쇄증의 소견들과 함께 조기 신생아기에서 담도폐쇄증 진단의 어려움, 그리고 간 섬유화를 예측하기 위한 영상의 역할도 검토하였다. 본 종설이 담도폐쇄증 진단에 도움이 되길 바란다.

The triangular cord ratio and the presence of a cystic lesion in the triangular cord. Suggested new ultrasound findings in the early diagnosis of Biliary Atresia.

In biliary atresia (BA), the ultrasonic triangular cord (TC) sign is positive at ≥ 3mm, but sometimes there is BA even if it is ≤ 3mm. For improving the ultrasonographic diagnosis, we have established a new evaluation, adding the ratio of the anterior/posterior thickness (TC ratio) in the hyperechoic area and the presence of a cystic lesion in the triangular cord (TCC). We examined 24 cases of suspected BA who demonstrated acholic stools from 2006 to 2020. We retrospectively reviewed the timing of ultrasonographic diagnosis, the gallbladder diameter, gallbladder mucosal irregularity, the TC sign, TCC, and the TC ratio. In the BA group (n = 10) vs the Non-BA group (n = 14), the age at ultrasonography was 75 ± 41.7 vs. 81 ± 39.1days (p = 0.72), the gallbladder diameter was 12.1 ± 9.7 vs. 24.2 ± 6.96mm (p = 0.02), irregularity of gallbladder mucosa was 7 cases vs. 1 case (p < 0.01), and TC sign was 3.9 ± 1.3 vs. 2.0 ± 0.49mm (p = 0.01), respectively. TCC was observed in 8/10 cases in the BA group and none in the Non-BA group (p < 0.01). TC ratio was 3.40 ± 0.68 (BA group) and 1.59 ± 0.41 (Non-BA group) (p < 0.01). The ultrasonic TC ratio improves the diagnostic accuracy of BA. TCC is a specific finding in the BA group.

Recent advances in the use of ultrasound and related techniques in diagnosing and predicting outcomes in biliary atresia.

Biliary atresia (BA) is the leading cause of chronic liver disease and the most common indication for pediatric liver transplantation. The use of ultrasound (US) and related techniques continues to evolve to help diagnose BA as well as potentially to help predict outcomes after treatment with the Kasai portoenterostomy (KP). There are no US findings that are definitive for BA; however, signs which are consistent with BA include gallbladder abnormalities, the triangular cord sign, presence of hepatic subcapsular flow, and hilar lymphadenopathy. Elastography techniques to measure liver stiffness may also increase the diagnostic accuracy of detecting BA, particularly in older infants or without other US findings. In addition, both US and elastography are still being studied as potential methods to predict outcomes after KP such as the development of portal hypertension and the need for liver transplant. US findings in the diagnosis of BA are well characterized. Future studies will help determine the utility of elastography in diagnosing BA, as well as both US and elastography in monitoring and predicting disease outcomes after KP.

Ultrasonography is useful in differentiating between cystic biliary atresia and choledochal cyst.

With the advent of ultrasonography (US), perinatal diagnosis of biliary cystic malformation (BCM) has become more prevalent. BCM includes cystic biliary atresia (CBA) and choledochal cyst (CC). Both share similar ultrasonographic features and clinical manifestations, and yet the postnatal management is very different. This study was to assess whether CBA can be distinguished from CC by US. We retrospectively studied the clinical data of 98 BCM patients aged less than 130days between January 2013 and November 2015. The patients were divided into the CBA group and the CC group based on intraoperative cholangiography. The ultrasonographic features in each group were then analyzed. Thirty-one children had CBA (type I: 24, type III: 7) and 67 children had CC (all: type I). The cyst volume did not show a significant change in the CBA group while the cyst volume increased postnatally in the CC group. Preoperative cyst volume can be used as a sensitive indicator to distinguish choledochal cyst from cystic biliary atresia. Gallbladder size in the two groups differed significantly (P = 0.034); 23 of 31 patients in the CBA group had atrophic gallbladder, whereas none of the patients in the CC group had atrophic gallbladder (P < 0.001). The triangular cord sign was detected in ten patients in the CBA group and in none of the patients in the CC group (P < 0.001). Serial prenatal and postnatal ultrasonographic studies may help differentiate CBA from CC in the majority of BCM, facilitating prenatal counseling and postnatal management.

Ultrasound shear wave elastography: does it add value to gray-scale ultrasound imaging in differentiating biliary atresia from other causes of neonatal jaundice?

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. 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. 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 9L4 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. 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 2.1m/s (interquartile range [IQR] 1.7-2.4m/s) in infants with biliary atresia and 1.5m/s (IQR 1.3-1.9m/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. 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.

Web-based calculator for biliary atresia screening in neonates and infants with cholestasis.

BackgroundDistinguishing biliary atresia from non-biliary atresia in patients with cholestasis is challenging, as these conditions have a similar clinical presentation. We developed and externally validated a screening model for biliary atresia and devised a web-based calculator for use in clinical settings.MethodsA screening model was developed based on data from 227 cholestatic infants (82 and 145 with and without biliary atresia, respectively) and validated in 234 infants (90 and 144 with and without biliary atresia, respectively) admitted to three hospitals. Variables were selected from routine examination results using the least absolute shrinkage and selection operator method and entered into a logistic regression model to construct a biliary-atresia-risk–predicting equation. Cutoff values for risk stratification were estimated using model sensitivity, derived from the receiver-operating characteristic curves.ResultsThe final screening model included seven variables (i.e., weight at admission, clay-colored stools, γ-glutamyl transpeptidase and albumin levels at admission, abnormal gallbladder, triangular cord sign, and change in total bilirubin levels). The model generated an area under the curve of 0.94 with a sensitivity of 91.46 and specificity of 86.62 in the derivation cohort. This was confirmed in the validation cohort, as we found an area under the curve of 0.93 with a sensitivity of 93.1 and specificity of 80.15. Patients were stratified into three risk groups (low-, moderate-, and high-risk groups). Biliary atresia was excluded in the low-risk group. The high-risk group showed a higher detection rate of biliary atresia compared to the stool color screening method alone. This model was integrated into a user-friendly web-based system.ConclusionsThe screening tool had a high predictive value and may help in decision-making by physicians at tertiary and community hospitals.