Role of indocyanine green-guided near-infrared fluorescence imaging in identification of the cause of neonatal cholestasis.

To evaluate the efficacy and safety of indocyanine green (ICG)-guided near-infrared fluorescence (NIRF) imaging during surgery to diagnose the cause of neonatal cholestasis (NC). Data on NC patients who underwent both NIRF with ICG and conventional laparoscopic bile duct exploration (the gold standard) at our institute from January 2022 to December 2022 were retrospectively analyzed. The patients' baseline characteristics and liver function outcomes were collected and analyzed, and the diagnostic consistency was compared between the 2 methods. In total, 16 NC patients were included in the study, comprising 8 (50%) male and 8 (50%) female patients, ranging in age from 42 to 93 days, with a median age of 54.4 ±â€…21 days. During surgery, all the patients underwent NIRF with ICG, followed by conventional laparoscopic bile duct exploration. Finally, 15 of the patients were diagnosed with biliary atresia (BA) (1 with type-I BA, and 14 with type-II BA). The other patient was diagnosed with cholestasis. The diagnostic results from fluorescence imaging with ICG were consistent with those from conventional laparoscopic bile duct exploration. ICG-guided NIRF is associated with an easy operation, less trauma, and good safety. Also, its diagnostic accuracy is similar to conventional laparoscopic bile duct exploration.

A Pilot Study for Biliary Atresia Diagnosis: Fluorescent Imaging of Indocyanine Green in Stool.

BACKGROUND: Biliary atresia is the most common cause of obstructive jaundice in infants and conventional cholangiography is the current diagnostic gold standard. Fluorescent cholangiography with indocyanine green can enhance biliary tree visualization during surgery because it is exclusively excreted into the bile ducts and eventually into the intestine. Therefore, we hypothesized that indocyanine green presence in stool could confirm bile duct patency in infants. METHODS: A prospective single center cohort study was performed on infants (age ≤ 12 months) with and without jaundice after obtaining IRB approval. Indocyanine green was administered intravenously (0.1 mg/kg). Soiled diapers collected post-injection were imaged for fluorescence. RESULTS: After indocyanine green administration, fluorescence was detected in soiled diapers for control patients (n = 4, x = 14 h22 m post-injection) and jaundiced patients without biliary atresia (n = 11, x = 13 h28 m post-injection). For biliary atresia patients (n = 7), post-injection soiled diapers before and after Kasai portoenterostomy were collected. Fluorescence was not detected in stool from 6 of 7 biliary atresia patients. As a test, indocyanine green detection in stool was 97% accurate for assessing biliary patency. CONCLUSION: Fluorescent Imaging for Indocyanine Green (FIInd Green) in stool is a fast and accurate approach to assess biliary patency non-invasively in infants. LEVEL OF EVIDENCE: Level III.

Preoperative imaging findings to predict 2-year native liver survival after the Kasai procedure in patients with biliary atresia.

OBJECTIVES: To investigate the feasibility of using preoperative imaging indices to predict 2-year native liver survival after the Kasai procedure in patients with biliary atresia (BA). MATERIALS AND METHODS: The retrospective review included 190 BA patients who underwent the Kasai procedure between 2000 and 2020, with preoperative US and/or MRI, excluding cases with less than 2-year follow-up period. Multivariable logistic regression analysis was performed to identify imaging indices to predict 2-year native liver survival. Kasai failure was defined as the need for liver transplantation or death within 2 years of the Kasai procedure. RESULTS: Of the 90 patients included, all had preoperative US, and 61 also had MRI. Kasai failure occurred in 52% (47/90). Preoperative US identified gallbladder length (OR 0.40, 95% CI 0.17-0.95, p = 0.039; cutoff 1.6 cm, AUC 67.66) and biliary cysts (OR 24.64, 95% CI 1.97-308.08, p = 0.013) as significant Kasai failure predictors, with a combined accuracy of 73% (60/82). For patients having both preoperative US and MRI, significant predictors were hepatic artery diameter (OR 6.75, 95% CI 1.31-34.88, p = 0.023; cutoff 2 mm, AUC 73.83) and biliary cysts (OR 23.89, 95% CI 1.43-398.82, p = 0.027) on US, and gallbladder length (OR 0.25, 95% CI 0.08-0.76, p = 0.014; cutoff 1.2 cm, AUC 74.72) and spleen size (OR 2.53, 95% CI 1.02-6.29, p = 0.045; cutoff 6.9 cm, AUC 73.72) on MRI, with a combined accuracy of 85% (52/61). CONCLUSION: Preoperative US and/or MRI enhance the 2-year native liver survival prediction in BA patients after the Kasai procedure. CLINICAL RELEVANCE STATEMENT: BA patients with hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and absence of biliary cysts (US/MRI) have a decreased likelihood of 2-year native liver survival. KEY POINTS: • Preoperative US and/or MRI can predict the probability of achieving 2-year native liver survival following the Kasai procedure. • Combining US and MRI improved the accuracy to 85% for predicting 2-year native liver survival in BA patients. • The hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and no biliary cysts (US/MRI) are significant predictors of Kasai failure in patients with biliary atresia.

Percutaneous Ultrasound Cholangiography With Microbubbles in Children With Biliary Diseases.

ABSTRACT: The application of intracavity contrast-enhanced ultrasound in the evaluation of biliary disease has been confirmed valuable among pediatric population. This pictorial essay aims to demonstrate the role of percutaneous ultrasound cholangiography (PUSC) with microbubbles in the diagnosis of different pediatric biliary diseases in our center. The biliary system's morphologic characteristics in PUSC mode of neonatal hepatitis, biliary atresia, choledochal cysts, and biliary complications of hepatobiliary surgery are presented.

Laparoscopic indocyanine green fluorescence imaging technique in rare type II cystic biliary atresia.

In recent years, it has been reported that indocyanine green can be used for intraoperative navigation in Kasai surgery. However, there are no reports of its application in surgery for rare type II cystic biliary atresia. We report a girl presented with jaundice and light-colored stools. Laboratory tests showed impaired liver function with elevated serum bilirubin and bile acid levels. The abdominal ultrasound and MRCP suggested a common hepatic duct cyst. A diagnosis of choledochal cyst was suspected and biliary atresia could not be excluded. Conservative treatment was unsatisfactory. Laparoscopic exploration with indocyanine green fluorescence was performed on the 38th day of her life, and intraoperative diagnosis of type II CBA was made because the common hepatic duct cyst and its downstream anatomical structures did not show fluorescence. The postoperative bilirubin and bile acid levels decreased significantly and she was discharged two weeks after surgery. This result suggests that indocyanine green can be safely used in laparoscopic surgery for type II CBA, which not only helps in the differential diagnosis of CBA and choledochal cyst, but also confirms bile flow in real time.

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. Hepatic fibrosis, according to the METAVIR score at the time of Kasai portoenterostomy and native liver outcomes during postsurgical follow-up, were compared and correlated with imaging and laboratory findings. Poor outcomes were defined as intractable cholangitis or liver transplantation. The diagnostic performance of SWE in predicting METAVIR F3-F4 and poor hepatic outcomes was analyzed using receiver operating characteristic (ROC) analyses. RESULTS: All patients were analyzed without exclusion. Perioperative advanced hepatic fibrosis (F3-F4) was associated with older age and higher preoperative direct bilirubin and SWE values in the liver and spleen. Preoperative liver SWE showed a ROC area of 0.806 and 63.6% (7/11) sensitivity and 86.4% (19/22) specificity at a cutoff of 17.5 kPa for diagnosing F3-F4. The poor outcome group included five patients with intractable cholangitis and three undergoing liver transplantation who showed high postoperative liver SWE values. Liver SWE on PODs 3-7 showed ROC areas of 0.783-0.891 for predicting poor outcomes, and a cutoff value of 10.3 kPa for SWE on POD 3 had 100% (8/8) sensitivity and 73.9% (17/23) specificity. CONCLUSION: Preoperative liver SWE can predict advanced hepatic fibrosis, and immediate postoperative liver SWE can predict poor native liver outcomes in patients with biliary atresia.

Kasai Portoenterostomy Delays: Is a HIDA Scan Worth the Wait?

BACKGROUND: Biliary Atresia (BA), an obstructive cholangiopathy, is the most common cause of end-stage liver disease and liver transplantation in children. Timely differentiation of BA from other causes of neonatal jaundice remains a challenge, yet is critical to improving outcomes. METHODS: Clinical characteristics including demographics, age at jaundice presentation, age at hepatobiliary scintigraphy, age at surgery, severity of liver fibrosis, and native-liver survival were reviewed in infants with hyperbilirubinemia and suspected BA for this single center retrospective cohort study. We investigated the accuracy of hepatobiliary scintigraphy as well as elapsed time from jaundice presentation to diagnostic intervention. RESULTS: BA was suspected in 234 infants. BA was identified in 17% of infants with hepatobiliary scintigraphy and 72% of infants who underwent operative exploration without hepatobiliary scintigraphy. Elapsed time from jaundice presentation to Kasai Portoenterostomy (KPE) for BA patients was 2.1x longer if hepatobiliary scintigraphy was obtained (p = 0.084). The mean age at KPE for this cohort was 66.8 days (n = 54), with a significantly higher mean age at KPE (75.2 days) for infants who were later listed or underwent liver transplantation (p = 0.038). Histologically, the lowest liver fibrosis scores were seen in infants undergoing KPE <30 days old and worsened significantly with increased age (p < 0.001). CONCLUSION: Hepatobiliary scintigraphy compared to operative exploration for the diagnostic evaluation of infants with suspected BA introduces significant time delays to KPE but enables avoidance of surgery in some infants. The temporal pattern of worsening cholestatic liver injury from BA with each day of increased age highlights the importance of intervening as early as possible for the best prognosis. TYPE OF STUDY: Retrospective study, Level of evidence: III.

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. METHODS: A 16-year single-center retrospective study of patients with persistent neonatal cholestasis (suspected BA) undergoing PTCC. Patient demographics, laboratory values, PTCC images, pathology and surgical reports were reviewed. RESULTS: 73 patients underwent PTCC (68% male, mean age 8.7 weeks, mean weight 4.0 Kg). The majority of studies were normal (55%). Abnormal patterns were identified in 33 cases, 79% were diagnosed with BA and 12% with Alagille syndrome. Non-opacification of the common hepatic duct with a narrowed common bile duct (42%) and isolated small gallbladder (38%) were the most common patterns in BA. CONCLUSION: PTCC is a minimally invasive study in the diagnostic work-up of infants presenting with conjugated hyperbilirubinemia (suspected BA). Further invasive investigations or surgery can be avoided when results are normal.

Prediction of Fetal Biliary Atresia Based on Second and Third-Trimester Ultrasound Characteristics.

OBJECTIVE: To explore the diagnostic performance of prenatal ultrasound in the prediction of biliary atresia (BA). METHODS: We prospectively collected cases of suspected biliary abnormalities in the 2nd trimester of pregnancy and performed a series (at least 3) of prenatal ultrasound examinations in the 2nd and 3rd trimester. The presence of the gallbladder was examined each time, and its size and shape were assessed if the gallbladder was visible. The existence of other abnormalities was carefully evaluated. Neonatal ultrasound examination was conducted within 1 month after birth, and clinical data were followed-up for 6 months after birth. RESULTS: Among the 41 895 patients, 298 were suspected to have biliary abnormalities, while 82 patients were excluded due to loss to follow-up or induced labor caused by other abnormalities. A total of 216 patients were included in this study, and 15 were diagnosed with BA. We summarized the ultrasound findings of the gallbladders and defined a high-risk gallbladder for the prenatal diagnosis of BA. This was demonstrated to have the best diagnostic performance as a single parameter, with an area under the curve of 0.914 (95 %CI: 0.869-0.948). In addition, higher incidences of biliary cysts, right hepatic artery dilation, echogenic bowel, and ascites were observed in BA fetuses. Logistic regression analysis showed that the combination of 5 parameters had better diagnostic performance, with an area under the curve of 0.995 (95 %CI: 0.973-0.999). CONCLUSION: The fetal gallbladder was found to be a critical feature for the identification of BA. Concomitant abnormalities could be helpful to improve the accuracy of the diagnosis.

The accuracy of magnetic resonance cholangiopancreatography in the diagnosis of biliary atresia in preterm infants with cholestasis.

BACKGROUND: Magnetic resonance cholangiopancreatography (MRCP) is a useful and non-invasive method to diagnose biliary atresia (BA) in term infants, however few studies have investigated its use in preterm infants. This study aimed to evaluate the accuracy of MRCP in the diagnosis of BA in preterm infants with cholestasis. METHODS: Infants aged less than 6 months who received MRCP for cholestasis at a tertiary medical center were enrolled from 2011 to 2020. Demographic and laboratory data were retrospectively obtained. One pediatric radiologist reviewed the MRCP images. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of MRCP to diagnose BA based on surgical proof or at least 6 months of follow-up were assessed. RESULTS: A total of 80 infants (36 preterm and 44 term) were analyzed. The mean post-chronological age was 1.8 months, and the female-to-male ratio was 0.78. Six (16.7%) preterm and 16 (36.4%) term infants were confirmed to have BA. BA was obscured by a choledochal cyst preoperatively in two term infants. In the preterm infants, the sensitivity, specificity, PPV, NPV, and accuracy of MRCP to diagnose BA were 100%, 77%, 46%, 100%, and 81%, respectively, compared to 81%, 86%, 76%, 89%, and 84% in the term infants. Using MRCP to differentiate BA from other cholestasis in the preterm infants had superior sensitivity (100% vs. 81%) and NPV (100% vs. 89%), and lower specificity (77% vs. 86%) and PPV (46% vs. 76%) than in the term infants. CONCLUSIONS: Negative MRCP findings can be used to exclude BA in preterm infants with cholestasis based on a favorable NPV.

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. All patients received indocyanine green (ICG) intravenously at 0.05 mg/Kg. The first 6 patients in the case group underwent IOC and LFC simultaneously, and the control group completed LFC. The imaging characteristics of LFC were recorded and summarized by the conventional and fluorescence mode of the endoscopic fluorescence imaging system (DPM-ENDOCAM-03). On this basis, 12 patients in the case group were diagnosed as BA according to LFC without IOC, and all 18 patients completed open Kasai surgery to confirm the diagnosis. RESULTS: Laparoscopic fluorescence mode in BA detected liver fluorescence but no visualization of the extrahepatic bile ducts. However, the extrahepatic bile ducts in the control group were visible. Based on the imaging characteristics summarized from the LFC of the first 6 cases with BA in the case group, the remaining 12 cases who only underwent LFC were also successfully diagnosed with BA. Furthermore, the formation of hepatic hilar fibrous mass was found in all the patients during the open Kasai procedure, which confirmed the BA diagnosis. CONCLUSIONS: LFC appears as a specific pattern in BA and may be used for intraoperative diagnosis of BA. It has the advantages of simplicity, short time-consuming, and no radiation damage.

Insight into microvascular adaptive alterations in the Glisson system of biliary atresia after Kasai portoenterostomy using X-ray phase-contrast CT.

OBJECTIVES: To investigate microvascular alterations in the Glisson system of biliary atresia (BA) patients after Kasai portoenterostomy (KP) using three-dimensional (3D) virtual histopathology based on X-ray phase-contrast CT (PCCT). METHODS: Liver explants from BA patients were imaged using PCCT, and 32 subjects were included and divided into two groups: KP (n = 16) and non-KP (n = 16). Combined with histological analysis and 3D visualization technology, 3D virtual histopathological assessment of the biliary, arterial, and portal venous systems was performed. According to loop volume ratio, 3D spatial density, relative surface area, tortuosity, and other parameters, pathological changes of microvasculature in the Glisson system were investigated. RESULTS: In the non-KP group, bile ducts mostly manifested as radial multifurcated hyperplasia and twisted into loops. In the KP group, the bile duct hyperplasia was less, and the loop volume ratio of bile ducts decreased by 13.89%. Simultaneously, the arterial and portal venous systems presented adaptive alterations in response to degrees of bile duct hyperplasia. Compared with the non-KP group, the 3D spatial density of arteries in the KP group decreased by 3.53%, and the relative surface area decreased from 0.088 ± 0.035 to 0.039 ± 0.015 (p < .01). Deformed portal branches gradually recovered after KP, with a 2.93% increase in 3D spatial density and a decrease in tortuosity from 1.17 ± 0.06 to 1.14 ± 0.04 (p < .01) compared to the non-KP group. CONCLUSION: 3D virtual histopathology via PCCT clearly reveals the microvascular structures in the Glisson system of BA patients and provides key insights into the morphological mechanism of microvascular adaptation induced by biliary tract dredging after KP in BA disease. KEY POINTS: • 3D virtual histopathology via X-ray phase-contrast computed tomography clearly presented the morphological structures and pathological changes of microvasculature in the Glisson system of biliary atresia patients. • The morphological alterations of microvasculature in the Glisson system followed the competitive occupancy mechanism in the process of biliary atresia.

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. There were statistically significant differences between BA group and non-BA group in maximum length, maximum width and LTWR of gallbladder (P < 0.001). In all age groups (I: ≤ 30 days; II: 31-60 days; III: 61-90 days; IV: 91-120 days; V: ≥ 121 days), in which group III (61-90 days) had the highest area under the curve (AUC) of 0.843, and group V (≥121 days) had the lowest AUC of 0.548. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of gallbladder LTWR > 3.26 for BA in group II (31-60 days) were 78.9%, 75.0%, 75.0%, 78.9% and 76.9%, respectively. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of gallbladder LTWR > 3.69 for BA in group III (61-90 days) were 76.6%, 84.6%, 92.5%, 59.5% and 78.9%, respectively. Ultrasonography LTWR of gallbladder has certain value in the diagnosis of BA. The diagnostic value of gallbladder LTWR in infants with different ages was quite different, and it was relatively high in infants with 31-90 days.

The application of artificial intelligence to support biliary atresia screening by ultrasound images: A study based on deep learning models.

PURPOSE: Early confirmation or ruling out biliary atresia (BA) is essential for infants with delayed onset of jaundice. In the current practice, percutaneous liver biopsy and intraoperative cholangiography (IOC) remain the golden standards for diagnosis. In Taiwan, the diagnostic methods are invasive and can only be performed in selective medical centers. However, referrals from primary physicians and local pediatricians are often delayed because of lacking clinical suspicions. Ultrasounds (US) are common screening tools in local hospitals and clinics, but the pediatric hepatobiliary US particularly requires well-trained imaging personnel. The meaningful comprehension of US is highly dependent on individual experience. For screening BA through human observation on US images, the reported sensitivity and specificity were achieved by pediatric radiologists, pediatric hepatobiliary experts, or pediatric surgeons. Therefore, this research developed a tool based on deep learning models for screening BA to assist pediatric US image reading by general physicians and pediatricians. METHODS: De-identified hepatobiliary US images of 180 patients from Taichung Veterans General Hospital were retrospectively collected under the approval of the Institutional Review Board. Herein, the top network models of ImageNet Large Scale Visual Recognition Competition and other network models commonly used for US image recognition were included for further study to classify US images as BA or non-BA. The performance of different network models was expressed by the confusion matrix and receiver operating characteristic curve. There were two methods proposed to solve disagreement by US image classification of a single patient. The first and second methods were the positive-dominance law and threshold law. During the study, the US images of three successive patients suspected to have BA were classified by the trained models. RESULTS: Among all included patients contributing US images, 41 patients were diagnosed with BA by surgical intervention and 139 patients were either healthy controls or had non-BA diagnoses. In this study, a total of 1,976 original US images were enrolled. Among them, 417 and 1,559 raw images were from patients with BA and without BA, respectively. Meanwhile, ShuffleNet achieved the highest accuracy of 90.56% using the same training parameters as compared with other network models. The sensitivity and specificity were 67.83% and 96.76%, respectively. In addition, the undesired false-negative prediction was prevented by applying positive-dominance law to interpret different images of a single patient with an acceptable false-positive rate, which was 13.64%. For the three consecutive patients with delayed obstructive jaundice with IOC confirmed diagnoses, ShuffleNet achieved accurate diagnoses in two patients. CONCLUSION: The current study provides a screening tool for identifying possible BA by hepatobiliary US images. The method was not designed to replace liver biopsy or IOC, but to decrease human error for interpretations of US. By applying the positive-dominance law to ShuffleNet, the false-negative rate and the specificities were 0 and 86.36%, respectively. The trained deep learning models could aid physicians other than pediatric surgeons, pediatric gastroenterologists, or pediatric radiologists, to prevent misreading pediatric hepatobiliary US images. The current artificial intelligence (AI) tool is helpful for screening BA in the real world.

Selective enlargement of left lateral segment liver volume as a potential diagnostic predictor for biliary atresia.

PURPOSE: To determine an early diagnostic indicator of biliary atresia (BA), we focused on morphological left-right differences of BA livers. METHODS: Of 74 infants with suspected BA at our hospital in the last 12 years, 25 met the conditions for investigation: 15 infants with BA (BA group) and 10 with other pathologies (non-BA group). CT volumetry of the liver in each patient was performed using a 3D image analysis system. Patient characteristics, blood data, and proportion of the left lateral segment to the total liver volume (LLS ratio) were compared between the two groups. RESULTS: Among the patient characteristics and liver function tests, only γ-glutamyl transpeptidase (GGT) were significantly higher in the BA group (p < 0.001). The LLS ratio was 0.321 (0.227-0.382) in the BA group and 0.243 (0.193-0.289) in the non-BA group (p = 0.01). The summary cut-off, area under the curve, sensitivity, and specificity were 0.322, 0.813, 53.3, and 100% for the LLS ratio and 94.26, 0.95, 86.7, and 100% for the GGT × LLS ratio, respectively. CONCLUSIONS: The LLS ratio is highly specific and may be an early diagnostic predictor of BA. Moreover, this segmental LLS enlargement may be associated with the etiology of BA.

Use of shear wave elastography for the diagnosis and follow-up of biliary atresia: A meta-analysis.

BACKGROUND: Timely differentiation of biliary atresia (BA) from other infantile cholestatic diseases can impact patient outcomes. Additionally, non-invasive staging of fibrosis after Kasai hepatoportoenterostomy has not been widely standardized. Shear wave elastography is an ultrasound modality that detects changes in tissue stiffness. The authors propose that the utility of elastography in BA can be elucidated through meta-analysis of existing studies. AIM: To assess the utility of elastography in: (1) BA diagnosis, and (2) post-Kasai fibrosis surveillance. METHODS: A literature search identified articles that evaluated elastography for BA diagnosis and for post-Kasai follow-up. Twenty studies met criteria for meta-analysis: Eleven for diagnosis and nine for follow-up post-Kasai. Estimated diagnostic odds ratio (DOR), sensitivity, and specificity of elastography were calculated through a random-effects model using Meta-DiSc software. RESULTS: Mean liver stiffness in BA infants at diagnosis was significantly higher than in non-BA, with overall DOR 24.61, sensitivity 83%, and specificity 79%. Post-Kasai, mean liver stiffness was significantly higher in BA patients with varices than in patients without, with DOR 16.36, sensitivity 85%, and specificity 76%. Elastography differentiated stage F4 fibrosis from F0-F3 with DOR of 70.03, sensitivity 96%, and specificity 89%. Elastography also differentiated F3-F4 fibrosis from F0-F2 with DOR of 24.68, sensitivity 85%, and specificity 81%. CONCLUSION: Elastography has potential as a non-invasive modality for BA diagnosis and surveillance post-Kasai. This paper's limitations include inter-study method heterogeneity and small sample sizes. Future, standardized, multi-center studies are recommended.

Imaging in Pediatric Obstructive Jaundice.

Cholestatic jaundice characterized by elevated conjugated bilirubin can be due to multitude of factors in neonates and childhood. Extrahepatic biliary atresia (EHBA), choledochal cyst, neonatal hepatitis, cytomegalovirus (CMV), and biliary plug are some of the common causes in neonate and early infancy. Causes in late infancy and childhood comprises viral hepatitis, choledochal cyst, cholelithiasis, worm infestation, and biliary compression secondary to extrinsic causes (node, collection, tumor). Some serious disorders like biliary atresia must be considered with the emphasis on early diagnosis of treatable causes. In the modern era, with multiple diagnostic modalities available including high-resolution ultrasonography, magnetic resonance imaging (MRI), CT scan, and nuclear imaging [hepatobiliary iminodiacetic acid (HIDA) scan], rapid diagnosis can be made in many surgically treatable cases. The authors will discuss the imaging modality available with advantages, disadvantages, and common indications of each modality, and overview of obstructive jaundice discussing the wide spectrum of causes in neonates and late childhood. Combining available knowledge with careful and meticulous search can help narrow down the diagnosis and initiate prompt treatment.

Long-term follow-up of biliary atresia using liver transient elastography.

OBJECTIVE: Liver transient elastography (TE) using FibroScan® has gained popularity as a non-invasive technique to assess hepatic fibrosis by measuring liver stiffness. This study focused on biliary atresia patients post Kasai operation for more than 10 years to prospectively correlate the hepatic fibrosis score to the biochemical changes of liver fibrosis and clinical development of portal hypertensive complications. METHODS: TE was performed in 37 patients who had biliary atresia post Kasai operation done at median age of 60 days. Biochemical indices of liver fibrosis including aspartate aminotransferase/platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score based on age, platelet count, alanine aminotransferase and aspartate aminotransferase level were calculated at the time of TE. Platelet count, spleen size, varices, ascites and hepatic encephalopathy were evaluated as clinical markers of portal hypertension. RESULTS: There were 22 female and 15 male with TE done at median age of 17.0 years. Median FibroScan® fibrosis score was 11.4. Fibrosis score of 6.8 kilopascal (kPa) was taken as the upper reference limit of normal. Nine patients (24%) had normal fibrosis score. Score above or equal to 6.8 kPa was significantly associated with lower platelet level (p = 0.001), higher INR (p = 0.043), higher APRI (p = 0.021), higher FIB-4 score (p = 0.013), and larger splenic diameter (p = 0.004). Higher FibroScan® fibrosis score was also significantly associated with portal hypertensive complications (p = 0.001). CONCLUSIONS: The FibroScan® fibrosis score correlated well with the biochemical changes of liver fibrosis and development of portal hypertensive complications clinically. Screening of portal hypertensive complications such as varices is recommended for patients with raised fibrosis score upon long-term follow-up. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Prenatally diagnosed biliary cysts: A spectrum from congenital biliary dilatation to biliary atresia from the same origin.

Kato and colleagues present prenatal cases with transitional features between congenital biliary dilatation and biliary atresia. They propose that all prenatal biliary cysts originate from choledochal cysts with a narrow segment, but present on a spectrum from congenital biliary dilatation to biliary atresia depending on the severity of reactive biliary sclerosis.

Percutaneous ultrasound-guided cholecystocholangiography with microbubbles combined with liver biopsy for the assessment of suspected biliary atresia.

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.