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Biliary atresia in a 19-day-old boy with normal-appearing gallbladder. a Oblique US shows normal aspect of the gallbladder. b Posteroanterior percutaneous cholecysto-cholangiography shows the communication of the gallbladder with the extrahepatic duct and no reflux in the intrahepatic ducts, confirming the diagnosis of biliary atresia
Source publication
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 de...
Citations
... Nuclear medicine using HIDA shows lack of excretion of the tracer into the bowel. In nondiagnostic cases PTC or biopsy may be used for confirmation 5,16,17 (►Fig. 2). ...
This review article discusses the most common benign biliary disorders and the various radiological findings on multiple modalities. A broad spectrum of diseases including various congenital disorders, infective and parasitic etiologies, immunological pathologies such as primary sclerosing cholangitis, and immunoglobulin G4-related sclerosing cholangitis are discussed along with obstructive diseases and ischemic cholangitis.
The article emphasized the imaging differential diagnosis of the above lesions as well as clinical correlates those that are most relevant to radiologists. The article briefly touched upon management and intervention where relevant.
... This modality not only aids in the detection of alternate etiologies. In infants with BA, the gallbladder may appear small, measuring < 15-19 mm in the fasting state [59]. To optimize the accuracy of ultrasonography, the child must have fasted for 3-4 h before the procedure. ...
... In addition, the presence of cysts near the porta hepatis, in the absence of normal CBD, is a specific indicator of BA. Polysplenia has high specificity (100%) but low sensitivity (10%) for BA [59]. It is often associated with other signs of syndromic BA, such as preduodenal portal vein, absence of the inferior vena cava, absence of errant hepatic artery, and abdominal heterotaxy [61] (Table 2 and Figure 3). ...
Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases.
... Correlative Imaging [11][12][13] • US and cross-sectional imaging may show the presence of a cystic mass suspected to represent a choledochal cyst or non-visualization of the gallbladder that may be associated with biliary atresia. • Choledochal cysts are usually first identified with US or CT, but these modalities cannot ascertain the nature of the cyst. ...
Nuclear medicine studies for the evaluation of liver, spleen, and biliary tract pathologies include a number of tests performed with various radiotracers. These imaging modalities are based on an assessment of the various histological components of the liver and spleen and their functional characteristics. Hepatobiliary scintigraphy (HBS) evaluates the function of the hepatocytes and the biliary system. Liver and spleen scintigraphy with [ 99m Tc]-labelled colloid tracks active tracer phagocytosis by the reticuloendothelial system (RES) of the liver, spleen, and bone marrow. Approximately 80–90% of tracer is taken by the liver, 5–10% by the spleen, and the remainder by the bone marrow. The physiologic basis for spleen scintigraphy with heat damaged red blood cells (DRBCs) is the ability of this organ to trap and remove deformed and damaged RBCs from circulation. This method involves drawing blood, in vitro labelling RBCs with [ 99m Tc], and inducing damage to their cell membrane with controlled heating. Following reinjection of DRBCs splenic tissue can be identified with high sensitivity and specificity. Unlike labelled colloid scans and HBS, liver and spleen scintigraphy with [ 99m Tc]-labelled RBCs can be highly specific in the evaluation of vascularity and blood pool of focal liver lesions.
... Abdominal ultrasonography is often the first step in the evaluation of patients with persistent jaundice. Ultrasonographic findings of BA include the triangular cord sign (TCS) in the hilar region and a small or unidentified gallbladder [1,2]. However, a normal gallbladder with an open lumen is present in approximately 10% of cases [3]. ...
... Ultrasonography is frequently the initial imaging test performed in the diagnosis of persistent neonatal jaundice, and it focuses on differentiating between BA and other disorders [6]. Typical ultrasonographic findings indicative of BA are the TCS, a small or unidentified gallbladder with a hazy wall, and the absence of contraction of the gallbladder after feeding [1][2][3]. However, a normal gallbladder with an open lumen is present in approximately 10% of cases [3]. ...
... In the diagnosis of BA, both the sensitivity and specificity of the ultrasonographic findings of gallbladder morphology and post-feeding contraction are reportedly low [2]. Because 10% of BAs show an open gallbladder lumen [3] and the frequency of BA complicated by PM is high, we can assume that the proportion of BA associated with post-feeding contraction of the gallbladder is also high [7]. ...
Ultrasonography is an essential part of the diagnostic process of biliary atresia (BA). The characteristic findings of BA include a hilar hyperechoic zone, the triangular cord sign (TCS), an absence of gallbladder contraction after feeding, and gallbladder atrophy. However, approximately 10% of patients with BA have a normal gallbladder. We herein present two cases of BA with normal morphology of the gallbladder as shown by ultrasonography. In the first case, the patient was positive for the TCS, negative for gallbladder atrophy, and positive for contraction of the gallbladder after feeding; the final diagnosis was hilar obstructive BA complicated by pancreaticobiliary maljunction. In the second case, the patient was positive for the TCS, negative for gallbladder atrophy, and negative for contraction of the gallbladder after feeding; the patient also had common bile duct obstruction and stenosis of the hepatic duct in the hilar region. Based on these two cases, we conclude that gallbladder findings are not diagnostic for BA because in some types, the gallbladder may be normal in morphology and even have the capacity for contraction after feeding.
... The syndromic form is associated with congenital heart disease, polysplenia, heterotaxy, preduodenal portal vein, and inferior vena cava (IVC) anomalies. 23 Clinically, children present acholia, choluria, and jaundice. 7 ABV is the most common cause of obstructive cholestasis in the newborn, in which infants develop jaundice, acholia, and jaundice that persists beyond the first 2 weeks of life. ...
Bile duct atresia (BA) is a severe, progressive cholangiopathy characterized by fibrous and inflammatory obliteration of the intrahepatic and extrahepatic bile ducts. It leads to liver failure, scarring, and end-stage cirrhosis if timely treatment is not achieved. It represents the number one indication for pediatric liver transplantation as a single disease worldwide. Various etiological factors have been associated with BA, such as structural malformations, viral, immune-mediated, and genetic infections. The incidence of BA varies around the world. Untreated BA patients have a 2-year mortality of nearly 100%. The clinical picture is characterized by jaundice, acholia, and jaundice that persists beyond the first 2 weeks of life. Direct or conjugated bilirubin remains the primary screening laboratory test for BA; elevated values occur within the first 2 days of life. Currently, the primary treatment of choice is the Kasai portoenterostomy; the success of surgery has been based on the restoration of bile flow and the elimination of jaundice. However, more than 70% of patients develop liver cirrhosis secondary to persistent liver inflammation, which will require liver transplantation. The following review of the literature aims to collect relevant information from what has been published in recent years on bile duct atresia; focused on the study of etiology, pathophysiology, advances in genetics and immunology. As well as the results associated with surgical treatment and the requirement for liver transplantation.
... Ultrasonography is the primary imaging modality used to evaluate neonatal cholestasis, including biliary atresia. Wellknown imaging findings of biliary atresia include triangular cord sign, abnormal gallbladder morphology, non-visualization of the common bile duct, presence of hepatic subcapsular flow, presence of microcysts or macrocysts near the porta hepatis, and increased hepatic artery diameter [1][2][3]. However, ultrasonography may not show extrahepatic bile ducts well in normal newborns, and some extrahepatic bile ducts may be visible depending on the subtype of biliary atresia [4]. ...
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.
... Ultrasound (US) is recommended for BA diagnosis by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition guidelines (7). The triangular cord (TC) sign and abnormal gallbladder (GB) morphology are the most frequently reported US features in identifying BA (8)(9)(10)(11). However, it is challenging to diagnose BA in infants younger than 60 days old because the TC sign is less common and while abnormalities are more difficult to detect in young patients with BA (12). ...
... Infants fasted for at least 4 hours. An abnormal GB was defined by one of the following US findings: absence of GB, GB with a filled lumen and a length up to 1.5 cm (Figure 2A), GB with a filled lumen and a length over 1.5 cm but a length to width ratio over 5.2, or GB with an incompletely filled lumen and stiff and coarse inner wall (Figure 2A,2B) (9,11,20). A GB found to have an incompletely filled lumen but with a smooth and completely hyperechogenic mucosal lining was considered normal ( Figure 2C) (9). ...
... A normal GB in infants should measure over 15 mm with a thin, defined wall in the fasted state (23). According to the practical approach or recent GB classification (9,11), the absence of GB, a small GB with a cutoff length <15 mm, and abnormal shape and wall of GB were defined as GB abnormalities. The detailed definition of abnormal GBs in our prediction tool may facilitate accurate and reliable GB diagnosis. ...
Background:
Early Kasai surgery before 60 days of life results in better clinical outcomes in patients with biliary atresia (BA). We aimed to develop and validate a prediction tool for the early diagnosis of BA in infants younger than 60 days old.
Methods:
This prospective study recruited consecutive infants younger than 60 days old with conjugated hyperbilirubinemia who were evaluated with an ultrasound (US) scan, including B-mode US with color Doppler flow imaging (CDFI) features and liver two-dimensional shear wave elastography (2D SWE), from March 2017 to July 2021. The reference standard for diagnosis was intraoperative cholangiography, liver biopsy, or the resolution of jaundice. Area under the receiver operating characteristic curve (AUC) analysis, logistic regression analysis, and establishment of a nomogram were performed.
Results:
A total of 174 patients (mean age, 46 days), including 87 infants with BA and 87 non-BA cholestatic infants, were included in the study. The established nomogram based on gallbladder (GB) abnormality, liver 2D SWE, and serum γ-glutamyl transferase (GGT) and alanine aminotransferase (ALT) had an AUC of 0.99 [95% confidence interval (CI): 0.94-1.00], a sensitivity of 92%, and a specificity of 100%. The nomogram in the validation cohort also had good diagnostic performance in the diagnosis of BA, with an AUC of 0.98 (95% CI: 0.95-1.00).
Conclusions:
The new prediction tool had a good diagnostic performance in the early prediction of BA in infants younger than 60 days old and will facilitate timely Kasai surgery.
... For this age group, the differentiation between obstructive and nonobstructive neonatal cholestasis, especially between biliary atresia and neonatal hepatitis, mainly relies on US [14]. The triangular cord sign, an abnormal gallbladder, micro-or macrocysts at the porta hepatis and polysplenia are specific findings associated with biliary atresia [15]. However, a normal US examination does not exclude biliary atresia. ...
Liver cirrhosis in children is a rare disease with multifactorial causes that are distinct from those in adults. Underlying reasons include cholestatic, viral, autoimmune, hereditary, metabolic and cardiac disorders. Early detection of fibrosis is important as clinical stabilization or even reversal of fibrosis can be achieved in some disorders with adequate treatment. This article focuses on the longitudinal evaluation of children with chronic liver disease with noninvasive imaging tools, which play an important role in detecting cirrhosis, defining underlying causes, grading fibrosis and monitoring patients during follow-up. Ultrasound is the primary imaging modality and it is used in a multiparametric fashion. Magnetic resonance imaging and computed tomography are usually applied second line for refined tissue characterization, clarification of nodular lesions and full delineation of abdominal vessels, including portosystemic communications.
... In some specific cases, complementary fetal magnetic resonance imaging (MRI) or early postnatal MRI of the biliary tract might provide additional information. Scintigraphy is useful in select cases [3,4]. ...
... Conversely, it may support suspicion of biliary atresia by showing a large, irregular gallbladder associated with hilar micro-or macrocysts. Finally, fetal MRI might suggest the diagnosis of choledochal cyst by demonstrating dilated biliary ducts connecting to the hilar cyst [3,14]. ...
... Postnatal US and MRI can further differentiate cystic biliary atresia from choledochal cyst by showing intrahepatic bile duct dilatation in cases of cholestasis [3,4,[19][20][21][22]. ...
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.
... In biliary atresia, patients usually present with icterus in neonatal period with development of liver cirrhosis if left untreated; this is a clinical presentation different from our patient's. And also, the presence of dilated intrahepatic ducts in our case is a finding against biliary atresia [17]. Therefore, we believe our patient is a case of biliary agenesis rather than atresia. ...
Background
Cholecystohepatic duct is a rare anomaly of the biliary system which involves drainage of bile into the gallbladder which may be associated with agenesis of the common hepatic duct or common bile duct.
Case presentation
A 2.5-month-old infant presented to our emergency department with icterus. He had a history of esophageal atresia and imperforate anus which had been treated surgically by thoracotomy, esophagostomy, gastrostomy and colostomy placement. Following imaging studies by ultrasound and MRCP, the diagnosis of common hepatic duct agenesis was made. Cholecystohepatic duct was present as the solitary drainage pathway of bile from the intrahepatic to extrahepatic biliary system.
Conclusions
Cholecystohepatic ducts need a high index of suspicion to be diagnosed on preoperative hepatobiliary imaging. As they may be asymptomatic, they are predisposed to iatrogenic injury during hepatobiliary surgeries.
