Cord Sign Research Articles

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.

Internal Jugular Vein Thrombosis: Etiology, Symptomatology, Diagnosis and Current Treatment.

(1) Background: internal jugular vein thrombosis (IJVthr) is a potentially life-threating disease but no comprehensive reviews on etiology, symptomatology, diagnosis and current treatment guidelines are yet available; (2) Methods: we prospectively developed a protocol that defined objectives, search strategy for study identification, criteria for study selection, data extraction, study outcomes, and statistical methodology, according to the PRISMA standard. We performed a computerized search of English-language publications listed in the various electronic databases. We also retrieved relevant reports from other sources, especially by the means of hand search in the Glauco Bassi Library of the University of Ferrara; (3) Results: using the predefined search strategy, we retrieved and screened 1490 titles. Data from randomized control trials were few and limited to the central vein catheterization and to the IJVthr anticoagulation treatment. Systematic reviews were found just for Lemierre syndrome, the risk of pulmonary embolism, and the IJVthr following catheterization. The majority of the information required in our pre-defined objectives comes from perspectives observational studies and case reports. The methodological quality of the included studies was from moderate to good. After title and abstract evaluation, 1251 papers were excluded, leaving 239 manuscripts available. Finally, just 123 studies were eligible for inclusion. We found out the description of 30 different signs, symptoms, and blood biomarkers related to this condition, as well as 24 different reported causes of IJVthr. (4) Conclusions: IJVthr is often an underestimated clinical problem despite being one of the major sources of pulmonary embolism as well as a potential cause of stroke in the case of the upward propagation of the thrombus. More common symptoms are neck pain and headache, whereas swelling, erythema and the palpable cord sign beneath the sternocleidomastoid muscle, frequently associated with fever, are the most reported clinical signs. An ultrasound of the neck, even limited to the simple and rapid assessment of the compression maneuver, is a quick, economic, cost-effective, noninvasive tool. High quality studies are currently lacking.

MOGAD: How It Differs From and Resembles Other Neuroinflammatory Disorders.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a distinct CNS inflammatory disease with symptoms and imaging findings that overlap other neuroinflammatory disorders. We highlight the imaging characteristics of MOGAD and contrast them with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS). Intracranial features that suggest MOGAD include childhood acute disseminated encephalomyelitis pattern with diffuse signal abnormality in the cortical gray matter, subcortical white matter, deep white matter, and deep gray matter on T2-weighted and FLAIR images; few bilateral T2-hyperintense fluffy and poorly demarcated lesions; pontine or thalamic involvement (or both); and cerebellar peduncle lesions in children. Intraorbitally, one sees edematous, enlarged, tortuous optic nerve or nerves; bilateral long-segment T2 hyperintensity of anterior segments of the optic nerve; sparing of the optic chiasm and retrochiasmatic pathways; and perioptic nerve sheath and surrounding orbital fat enhancement. Spinal involvement is seen as longitudinally extensive transverse myelitis with a sagittal T2-hyperintense intramedullary spinal line, the axial "H" spinal cord sign (central cord gray matter T2 hyperintensity), and conus medullaris involvement. Early accurate diagnosis of MOGAD is important because prognosis and treatment differ from those for NMOSD and MS.

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.

Accuracy of Noncontrast CT Brain in Detection of Cerebral Venous Sinus Thrombosis

Background and Purpose: Increasing concern of cerebral venous thrombosis due to treatable and curable causes of stroke. The diagnosis of cerebral venous sinus thrombosis is challenged due to nonspecific clinical symptomatology. Patients may present at an emergency room with a variety of neurological conditions such as severe headache, weakness, seizure, etc. Neuroimaging, particularly noncontrast cranial computed tomography (NCCT), is an investigation of choice in differentiation and triage the patients for further treatment. CT is sensitive in the detection of acute thrombosis or blood clots in all regions of the body. We hypothesize that NCCT might be sensitive to diagnose cerebral venous thrombosis immediately. Materials and Methods: Retrospectively review the electronic database of our patients, there are 27 patients with cerebral sinus venous thrombosis (SVT) and 4 patients with cerebral deep venous thrombosis (DVT). Other 79 patients present with clinically diagnosed cerebral venous thrombosis but the final result can exclude cerebral venous thrombosis. We use MR imaging and CT venography as the gold standard. Independently reviewed by two neuroradiologists for CT direct sign and CT indirect signs that suggest SVT or DVT. CT direct signs for SVT and DVT are the presence of hyperdensity in the sinus venous or deep venous system (cord sign, attenuated vein sign) and CT indirect signs are the changes in brain parenchyma (brain edema, hemorrhagic infarction). Results: Sensitivity and specificity of NCCT in detection attenuated vein sign and diagnosis DVT are 75% and 100% whereas the sensitivity and specificity of NCCT in detection cord sign and diagnosis SVT are 43.8% and 99.7% as respectively. Conclusions: NCCT might not sensitive in detection of SVT without CT direct sign which needs further investigation. However, NCCT might beneficial for emergency conditions such as DVT patients, cortical vein thrombosis and also in SVT patients with the positive CT direct sign.

Clinical Manifestation of Biliary Atresia in Bali

Biliary atresia is a rare disease of the liver and bile ducts that occurs in infants. Biliary atresia is a condition in which the absence of a lumen in the extrahepatic biliary tract causes obstruction of bile flow. Symptoms of the disease appear or develop about two to eight weeks after birth. This study aims to describe the clinical manifestations of patients with Biliary Atresia at Sanglah General Hospital, Bali. This cross-sectional study was performed at the Sanglah Hospital Denpasar, Bali. Data was taken from medical record from January 2015 - December 2020. Study population were children who diagnosed as Biliary Atresia. Diagnosis was confirmed by cholangiography and liver biopsy. The study included 30 infants with Biliary Atresia, mostly (70%) were girl. Average age at admission was 3 months. Ninety percent subject had hepatomegaly in physical examination. Acholic stool were found in 80% subject. The average serum levels of conjugated bilirubin were 9.8 mg/dL, alanine aminotransferase (ALT) was 182 u/L, aspartate transaminase (AST) was 324 u/L, gamma-glutamyl transpeptidase (GGT) level was 671 u/L and albumin was 3.8 g/dL. Seventy percent patient showed triangular cord sign in abdominal ultrasonography. Kasai procedure was performed to all those infants. The most common clinical manifestation was icteric, dark urine, acholic stool and hepatomegaly. Triangular cord sign was the most common finding in abdomen ultrasonography.

Evaluation of Visual Examination of Stool as A Screening Test for Infant with Prolonged Neonatal Cholestasis Namely Biliary Atresia

Introduction: Neonatal cholestasis is a hepatobiliay disease characterized by biliary obstruction in the neonatal period. Biochemically it is evidenced by prolonged elevation of serum conjugated bilirubin beyond the first 14 days of life.1 Most common causes are biliary atresia and idiopathic neonatal hepatitis.3, 4&#x0D; Objective: To evaluate stool color as a screening test by visual inspection in infants with prolonged neonatal cholestasis.&#x0D; Methodology: This was a cross-sectional analytic study, conducted in Pediatric Gastroenterology and Nutrition Department,BSMMU, Dhaka, from 3 September 2012 to 3 February 2013 about 6 month duration. Statistically calculated 38 infants with prolonged neonatal direct hyperbilirubinaemia beyond their 14 days of age were included in this study.&#x0D; Results: The mean age of the subjects was 62.3 days with a standard deviation (SD) ±13.7 days. Male to female ratio was 1.2:1. All (100%) the subjects were icteric and hepatomegaly was found in 94.7% subjects. Dark urine (84.2%), pale stool (78.5%), bleeding manifestations (31.8%) and infection (29%) were also observed. Thirty (78.5%) subjects had pale colored stool. Mean (± SD) albumin and conjugated bilirubin levels were 3.68 (±1.88)gm/dl and 5.29 (±1.31)mg/dl respectively. ALT and GGT level of the study subjects were 346.19±124.28 u/dl and 315±198.91 u/l respectively. Common ultrasonographic findings of the patients were non visualization of gallbladder 60.5%, non-visualization of common bileduct 50%, hepatomegaly 92.1%, and triangular cord sign in portahepatis 7.9%. Scintigraphy revealed impaired excretion into intestine 88.9% in majority of the subjects. Liver biopsy revealed liver architecture was preseved 65.8% bile duct proliferation 52.6%, regenarating nodule was absent 65.8% gaint cell was present 52.6% portal tract inflammation was found in 47.4%. Sensitivity of stool color in the diagnosis of neonatal cholestasis was found 90.6%, specificity 83.3%, accuracy 89.5%, positive predictive value 96.7% and negative predictive value 62.5%.&#x0D; Conclusion: It can be concluded that stool color might be reliable indicator for screening of prolonged neonatal cholestasis namely biliary atresia.&#x0D; J Bangladesh Coll Phys Surg 2021; 39(1): 46-52

Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound.

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.

Effects of cytomegalovirus infection on the differential diagnosis between biliary atresia and intrahepatic cholestasis in a Chinese large cohort study

Introduction and objectivesDifferentiating biliary atresia from other causes of neonatal cholestasis is challenging, particularly when cytomegalovirus (CMV) and biliary atresia occur simultaneously. We aimed to elucidate whether CMV infection would affect the differential diagnosis of biliary atresia and intrahepatic cholestasis. Patients and methodsThis retrospective study was conducted among patients with neonatal cholestasis admitted to three tertiary hospitals between January 2010 and August 2019. The clinical characteristics, laboratory, and imaging findings were recorded. On the basis of the CMV serology results, the infants were classified into CMV-IgM (+) and CMV-IgM (−) groups. The clinical differences and diagnostic performances of routine predictors between biliary atresia and intrahepatic cholestasis were analyzed in each group. Finally, we compared the diagnostic performances of various tests in the two groups. ResultsA total of 705 patients with neonatal cholestasis were enrolled: 215 (30.5%) patients were positive for CMV-IgM, among whom 97 had biliary atresia and 118 had CMV hepatitis; 490 infants were CMV-IgM (−), among whom 240 had biliary atresia and 250 had intrahepatic cholestasis. The diagnostic performances of stool color, direct bilirubin level, γ-glutamyl transpeptidase level, abnormal gallbladder, triangular cord sign, and hepatobiliary scintigraphy between CMV hepatitis and CMV-IgM (+) biliary atresia were similar to those between CMV-IgM (−) biliary atresia and CMV-IgM (−) intrahepatic cholestasis groups. ConclusionsOur large-scale study showed a high prevalence of CMV infection in patients with neonatal cholestasis in China. The presence of CMV infection did not affect the routine predictors to discriminate biliary atresia and intrahepatic cholestasis.

Three-color risk stratification for improving the diagnostic accuracy for biliary atresia.

It is challenging to early differentiate biliary atresia from other causes of cholestasis. We aimed to develop an algorithm with risk stratification to distinguish biliary atresia from infantile cholestasis. In this study, we enrolled infants with cholestasis into 2 subgroups from January 2010 to April 2019. A prospective cohort (subgroup 2) of 187 patients (107 with biliary atresia and 80 without biliary atresia) underwent acoustic radiation force impulse elastography. Stepwise regression was used to identify significant predictors of biliary atresia. A sequential algorithm with risk stratification was constructed. Among 187 patients, shear wave speed > 1.35m/s and presence of the triangular cord sign were considered high risk for biliary atresia (red), in which 73 of 78 patients (accuracy of 93.6%) with biliary atresia were identified. Afterwards, γ-GT, abnormal gallbladder, and clay stool were introduced into the algorithm and 55 intermediate-risk infants were identified (yellow) with a diagnostic accuracy of 60% for biliary atresia. Of the remaining 54 infants who were classified as low-risk patients (green), the accuracy for excluding biliary atresia was 98.1%. By applying a three-color risk stratification tool, 70.6% patients were identified as either high risk or low risk for biliary atresia (area under the curve, 0.983; sensitivity, 98.7%; specificity, 91.4%). We also estimated the risk of biliary atresia in different color groups, which was 94.7% (95%CI, 94.3-95.5%) in the red group and 7.2% (95%CI, 6.6-8.3%) in the green group. Our simple noninvasive approach was able to identify biliary atresia with high accuracy. • Five predictors, namely shear wave speed, triangle cord sign, γ-glutamyl transferase, abnormal gallbladder, and clay stool, were selected to identify biliary atresia in cholestasis. • Shear wave speed > 1.35m/s and presence of the triangle cord sign were considered high-risk patients with a diagnostic accuracy of 93.6% for biliary atresia. • Risk for biliary atresia was high (red), intermediate (yellow), or low (green). In the red and green group, we achieved an extremely high diagnostic performance (area under the curve, 0.983; sensitivity, 98.7%; specificity, 91.4%).

Re-do Kasai procedure in a preterm infant

We report the case of a 10-day old boy with biliary atresia (BA) who was delivered by caesarean section at 33 weeks 4 days of gestation (birth weight, 2135 g). At birth, his direct bilirubin level was high, and stools were light yellow. Abdominal ultrasonography showed no triangular cord sign, and duodenal fluid examination showed no bile. We performed a Kasai procedure (KP) 27 days after birth. His liver biopsy showed intrahepatic bile duct reduction. No bile excretion occurred postoperatively. We performed a redo KP 83 days after birth. Postoperative bile excretion was good, and his jaundice promptly resolved. The patient's liver biopsy showed bile duct development. Jaundice recurred at 8 months of age, and brain-dead-donor liver transplantation was required. The patient died of liver failure at 23 months of age. Surgery for BA during the neonatal period is usually effective, and the results of re-operation in cases with poor postoperative biliary excretion are poor. Here, we present a novel case that illustrates the progression and timing of the pathological hepatic changes in BA and consider the indications for and timing of re-operation in preterm infants. • The standard treatment for biliary atresia is the Kasai procedure. • All patients including preterm infants should undergo surgery as soon as possible. • The outcome is poor after a redo Kasai procedure for bile excretion failure. • Outcomes can be improved by limiting the indications for a redo Kasai procedure.

Neonatal Jaundice.

Hyperbilirubinemia is a common occurrence in neonates; it may be physiological or pathological. Conjugated hyperbilirubinemia may result from medical or surgical causes, and can result in irreversible liver damage if untreated. The aim of imaging is the timely diagnosis of surgical conditions like biliary atresia and choledochal cysts. Abdominal ultrasound is the first line imaging modality, and Magnetic resonance cholangiopancreatography (MRCP) also has a role, especially in pre-operative assessment of choledochal cysts (CDCs). For biliary atresia, the triangular cord sign and gallbladder abnormalities are the two most useful ultrasound features, with a combined sensitivity of 95%. Liver biopsy has an important role in pre-operative evaluation; however, the gold standard for diagnosis of biliary atresia remains an intra-operative cholangiogram. Choledochal cysts are classified into types according to the number, location, extent and morphology of the areas of cystic dilatation. They are often associated with an abnormal pancreaticobiliary junction, which is best assessed on MRCP. Caroli's disease or type 5 CDC comprises of multiple intrahepatic cysts. CDCs, though benign, require surgery as they may be associated with complications like cholelithiasis, cholangitis and development of malignancy. Severe unconjugated hyperbilirubinemia puts neonates at high risk of developing bilirubin induced brain injury, which may be acute or chronic. Magnetic resonance imaging of the brain is the preferred modality for evaluation, and shows characteristic involvement of the globus pallidi, subthalamic nuclei and cerebellum - in acute cases, these areas show T1 hyperintensity, while chronic cases typically show hyperintensity on T2 weighted images.

Value of shear wave elastography in diagnosing biliary atresia by ultrasonography

Objective To investigate the value of the shear wave elastography (SWE) in the diagnosis of biliary atresia (BA) in infants by ultrasonography. Methods SWE was used to measure the liver stiffness measurement(LSM) of 138 infants. Following surgical or pathological diagnosis, the infants were subclassified into BA and non-biliary atresia (non-BA) groups. ROC analysis was used to determine the sensitivity and specificity of LSM, the ultrasonic findings in the differential diagnosis of suspected BA, and the cut-off value to diagnose BA. Results Of the 138 infants with cholestatic hepatitis, 51 were diagnosed as BA group and 87 cases were assigned to the non-BA group. The ROC analysis showed the cut-off value of LSM for diagnosis of biliary atresia in children with jaundice was 12.35 kPa, the AUC was 0.936, and the sensitivity and specificity were 84.3% and 89.7%, respectively.In the parallel test, the triangular cord (TC) sign combined with abnormal gallbladder (AbGB) had the best diagnostic performance in all patients. However, in patients older than 30 days, the LSM combined with TC had the best AUC(0.987) and diagnostic performance(98.6%) like with TC combined with AbGB. Conclusions Both SWE and gray scale ultrasound have higher diagnostic performance for BA. LSM can be used as a valuable index for diagnosing BA by ultrasound. Key words: Ultrasonography; Shear wave elastography; Infant; Liver; Biliary atresia

Risk Estimation for Biliary Atresia in Patients with Neonatal Cholestasis: Development and Validation of a Risk Score.

Purpose To develop and validate a scoring system based on clinical and imaging features to predict the risk for biliary atresia in patients with neonatal cholestasis. Materials and Methods Patients with neonatal cholestasis who underwent both ultrasonography (US) and hepatobiliary scintigraphy (n = 480) were retrospectively identified from two tertiary referral hospitals from January 2000 to February 2017. Patients from one hospital were classified as the derivation cohort (n = 371), and those from the other hospital were classified as the validation cohort (n = 109). Clinical and imaging features associated with biliary atresia were assessed. Histopathologic or intraoperative cholangiographic findings served as the reference standard for biliary atresia. A prediction model was developed by using logistic regression and was then transformed into a scoring system. The scoring system was internally and externally validated. Results Among the 371 patients in the derivation cohort, 97 (26.15%) had biliary atresia. A scoring system was constructed with the following variables: full-term birth, presence of the triangular cord sign at US, abnormal gallbladder morphology at US, and failure of radioisotope excretion to the small bowel at hepatobiliary scintigraphy. The maximum possible score with this system is 7 points. This system enabled differentiation of biliary atresia in the derivation cohort (C statistic, 0.981; 95% confidence interval [CI]: 0.970, 0.992) and the validation cohort (C statistic, 0.995; 95% CI: 0.987, 1.000). The risk score also showed good calibration in both the derivation and the validation cohorts (P = .328 and P = .281, respectively). Conclusion A simple scoring system combining clinical and imaging features can help accurately estimate the risk for biliary atresia in patients with neonatal cholestasis.

Evaluation of Cases with Neonatal Cholestasis

Amaç: Çalışmanın amacı, neonatal kolestaz nedeniyle takip edilen çocuk hastaların etiyoloji, tanı yöntemleri, laboratuvar bulguları, tedavi şekilleri ve uzun dönem prognozları açısından geriye dönük olarak incelenmesi amaçlandı. Gereç ve Yöntem: Yaşamın ilk üç ayında kolestazı olan hastalar çalışmaya alınmıştır. Hastalar ekstrahepatik kolestaz ve intrahepatik kolestaz olarak gruplandırılmıştır. Ekstrahepatik kolestaz grubu içerisine biliyer atrezi, koyulaşmış safra sendromu ve koledok kisti dâhil edilmiştir. Bunların dışında kalan hastalar intrahepatik kolestaz grubu içerisinde değerlendirilmiştir. Hastaların tanıları, yakınmaları, klinik seyirleri, laboratuvar bulguları ve tedaviye yanıtları incelenerek geriye dönük olarak prognozları değerlendirilmiştir. Bulgular: Çalışma grubu kolestaz tanısı alan hastalar 43’ü kız (%41), 62’si erkek (%59) cinsiyete sahip olmak üzere toplam 105 hastadan oluştu. Vakaların 46’sı (%43.8) prematüre doğmuştu. Otuz altı hasta (%34.3) 30-37 haftalar arasında; 10 hasta (%9.5) 30 haftanın altında olup, 39’unda ise (%37.1) düşük doğum ağırlığı mevcuttu. Hastaların %81’inde intrahepatik, %19’unda ekstrahepatik nedenlere bağlı kolestaz geliştiği saptandı. Akolik dışkı ekstrahepatik kolestaz vakaların %90’ında (18 vaka), intrahepatik vakaların %15.3’ünde (13 vaka) olmak üzere toplam 31 vakada görülmüştür. İntrahepatik kolestaz vakalarında doğum ağırlığının ekstrahepatik kolestaz vakalarına göre daha düşük olduğu saptandı. Klinik seyirlerinde intrahepatik kolestaz hastası 38 vaka (%44.7), ekstrahepatik kolestaz hastası 7 vaka (%35) düzeldi. Sonuç: Çalışmamızda, hastaların literatür oranlarından daha geç tanı aldığı görülmüştür. Bu gecikme özellikle biliyer atrezi vakalarının erken cerrahi müdahale şansını azaltmaktadır. Ultasonografik görüntülemede “triangular cord sign” belirtisinin literatürden farklı olarak hiç görülmemesi bu konuda eğitim ve tecrübe eksikliğinin olduğunu düşündürmüştür. Bunun dışında, GGT yüksekliği, spenomegali, doğum ağırlığı, akolik dışkılama gibi karaciğer hastalığı için anlamlı olacak bulguların gözden kaçması, tanı için hızlı davranılamaması çocuk hekimlerinin farkındalığını artırılması gerektiğine işaret etmektedir.

Cord sign in cerebral venous thrombosis.

Cord sign in cerebral venous thrombosis.

Early US findings of biliary atresia in infants younger than 30 days

To investigate and compare ultrasound (US) findings for the diagnosis of biliary atresia (BA) in infants younger than 30 days with those of infants older than 30 days. From 2000 to 2015, we reviewed hepatobiliary US images in 12 BA infants younger than 30 days (younger BA group) and 62 BA infants older than 30 days (older BA group) before Kasai procedure. Eight (67%) of younger BA group underwent follow-up US examinations before Kasai procedure. Our review of the images focused on triangular cord sign, gallbladder (GB) abnormalities, vascular changes, and signs of portal hypertension. The triangular cord sign was present in 17% of younger BA group and in 56% of older BA group (P=.024). GB abnormalities were commonly identified in both groups. The hepatic artery diameter was significantly smaller in younger BA group than in older BA group (P<.001). Signs of portal hypertension were less common in younger BA group (17%) than in older BA group (84%) (P<.001). Follow-up US of two infants in younger BA group showed a new appearance of the triangular cord sign. BA infants younger than 30 days showed atypical US findings compared with those older than 30 days. • BA infants younger than 30 days show atypical US findings. • GB abnormalities were common in both younger and older BA group. • Subsequent US examination may be helpful to diagnose BA in young infants.

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

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

A case of biliary atresia with pancreaticobiliary maljunction

BackgroundThe pathogenesis of biliary atresia (BA) is still unknown. There are several reports on the etiology of BA, including pancreaticobiliary maljunction (PBM). We experienced a case of Kasai type IIIa BA with PBM, in which we found elevation of pancreatic enzymes in the gallbladder. We evaluated whether PBM is related to the pathogenesis of BA based on our findings.Case presentationThe patient was born at 40 weeks of gestation. His body weight at birth was 2850 g. At the age of 4 days, he had an acholic stool and was referred to our hospital. Abdominal ultrasonography showed that triangular cord sign was negative. The gallbladder was isolated with a diameter of 19 mm, and it contracted in response to oral feeding. His ultrasonographic findings were atypical for BA, but his jaundice did not improve. Therefore, we performed an operation at the age of 56 days. Intraoperative cholangiography showed a common bile duct and pancreatic duct and a common channel patent, while the common hepatic duct or intrahepatic duct was not visualized. Bile in the gallbladder contained colorless fluid, which showed elevated lipase level (34,100 IU/L). We performed Kasai portoenterostomy under the diagnosis of Kasai type IIIa BA with PBM. The patient’s postoperative course was uneventful, and he was discharged on day 30 after the operation. Histopathological evaluation showed that the lumens of the common bile duct and cystic duct were patent. However, the common hepatic duct was closed, and only bile ductules with diameters of less than 50 μm were isolated. Infiltration of lymphocytes was detected in the porta hepatis. No apparent inflammation was observed around the cystic duct, which was constantly exposed to pancreatic juice because of reflux through PBM.ConclusionsReflux of pancreatic juice through PBM might not be an etiological factor for BA, but might be associated with patency of the common and cystic bile ducts in Kasai type IIIa BA.