Extra-hepatic biliary atresia: diagnostic methods (original) (raw)
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Journal of Pediatric and Neonatal Individualized Medicine, 2014
Extrahepatic biliary atresia (EHBA) refers to stenosis or atresia of the extrahepatic biliary tree. It accounts for 25-30% of cases of neonatal cholestasis. If left untreated, EHBA progresses to biliary cirrhosis and is universally fatal within the first 2 years of life. Early diagnosis is crucial since surgical treatment (Kasai procedure) is the only treatment option. Histopathologic examination of liver biopsy specimens is a key element in the diagnostic work-up of infants with suspected EHBA. Pathologic diagnosis aims at excluding non-surgically correctable causes of neonatal cholestasis thereby leading to surgical exploration for confirmation of the diagnosis. All published data indicate that pathologists can diagnose EHBA with high sensitivity, high specificity and reasonable interobserver agreement. The most useful histologic features in the diagnosis of EHBA are portal tract changes including ductular proliferation and bile plugs in ducts and ductules. These lesions are not pathognomonic but can be seen in extrahepatic obstruction of any cause. Total parenteral nutrition (TPN)associated cholestasis and alpha1-antitrypsin (A1AT) deficiency cannot be differentiated from EHBA without access to clinical data and may lead to false-positive diagnosis. False-negative interpretation may be caused by early age at diagnosis or by small/indequate specimens. The pathologist also plays a role in the examination of the resected fibrotic segment and Review
Tropical Gastroenterology, 2012
Aim: To determine the utility of Tc99m-Mebrofenin hepato-biliary scintigraphy (HIDA scan) for diagnosis of biliary atresia in patients with neonatal cholestasis. Methods: Our study involves the retrospective analysis of 46 patients with neonatal cholestasis who underwent HIDA scans at the Pediatric Hepatobiliary Clinic, BJ Wadia Hospital for Children from May 2005 to July 2007. Biliary atresia (BA) was diagnosed on the basis of intra-operative cholangiogram. Non-BA patients were included in the neonatal hepatitis (NH) group. All patients received phenobarbitone and ursodeoxycholic acid for 5 days, prior to the HIDA scan. The HIDA scan was evaluated on the basis of uptake of the radioactive tracer by the liver at 5 minutes after intravenous injection; retention of radioactive tracer within the liver at 24 hours after injection and visualization of excretion of tracer into the intestine upto 24 hours after administration. The results of the HIDA scans were analyzed and correlated with the final diagnosis, gender and age of the patients. Chi-square test was employed for statistical analysis. Results: The age of presentation of our patients ranged from 5 days to 6 months. The male: female ratio was 37:9. Of the total 46 patients, 28 had BA and 18 had NH. All 28 (100%) patients diagnosed with BA showed persistent radiotracer in the liver at 24 hours whereas 17 (94.4%) of the 18 NH patients showed hepatic radiotracer retention (p=0.207), the difference being statistically insignificant. Twenty two (78.6%) patients of BA showed no excretion of the radiotracer at 24 hours whereas only 7 (38.9%) of the NH group did not excrete the radiotracer (p=0.007), which was statistically significant. Neither the sex nor the age of the child contributed to any difference on the hepatic retention (p=0.618 and 0.235, respectively) or on the intestinal excretion (p=0.307 and 0.9, respectively) of the radiotracer. Conclusion: HIDA scan is a useful tool for screening of biliary atresia in patients with neonatal cholestasis. Non excretion of the radioactive radiotracer into the intestines even after 24 hours of radiotracer administration can suggest biliary atresia in majority of patients.
American Journal of Roentgenology, 2011
dence of neonatal cholestasis is estimated to be approximately 1 in 2500 live births [2]. Although its cause is varied, extrahepatic biliary atresia (EHBA) or idiopathic neonatal hepatitis (NH) is seen in 60-90% of infants with conjugated hyperbilirubinemia [3, 4]. These two entities have similar clinical and biochemical findings. However, they have completely different pathogenesis and management. Therefore, it is very important to accurately diagnose these conditions. EHBA is an obliterative cholangiopathy of unknown cause affecting both the intra-and extrahepatic bile ducts. If left untreated, EHBA has a progressive course to cirrhosis and endstage liver failure. Portoenterostomy, the surgical treatment of choice for EHBA, is unlikely to be of value if performed after the patient is 3 months old [3]. Currently, the reference standard for the diagnosis of EHBA is laparotomy with intraoperative cholangiography because
MR cholangiography in children and young adults with biliary disease
American Journal of Roentgenology, 1999
Our objective was to describe the MR cholangiography findings for young patients with suspected biliary disease who underwent half-Fourier acquisition fast spin-echo technique with respiratory triggering. SUBJECTS AND METHODS. Twenty-eight MR cholangiography studies were performed in 22 patients on a l.5-T MR unit. Ten of these 22 patients had undergone liver transplantation. RESULTS. MR cholangiography revealed abnormalities of both the extrahepatic and the intrahepatic major and minor bile duct systems, despite the small diameter of the duct system in this group of patients. Four patterns of biliary disease were shown: global dilatation of extrahepatic or intrahepatic ducts (n = 7); segmental, uniform dilatation of central or peripheral intrahepatic ducts (n = 9); segmental, nonuniform dilatation of central or peripheral intrahepatic ducts (1? = 2); and fusiform ectasia with segmental, irregular intrahepatic dilatation and bile lakes (n = 2). The findings of eight studies were interpreted as normal. The four patterns of abnormalities were correlated with the results from percutaneous transhepatic cholangiography. T-tube cholangiography, and liver biopsy and with clinical and surgical information, as available. CONCLUSION. MR cholangiography is a noninva.sive technique for evaluation ofbiliary disease. The improved resolution afforded by respiratory triggering permits evaluation of both major and minor bile ducts, even in young, uncooperative subjects. Four patterns of abnormalities were prospectively identified, correlated with other information, and used to direct clinical treatment.
Journal of Pediatric Surgery, 1999
Purpose: The aim of this study was to evaluate the importance of the ultrasonographic “triangular cord” (TC) coupled with gallbladder images in the diagnostic prediction of biliary atresia (BA) from infantile intrahepatic cholestasis.Methods: Seventy-nine infants with cholestatic jaundice underwent ultrasound examinations, focusing on the TC and gallbladder images. The TC was defined as visualization of a triangular or bandlike periportal echogenicity (3 mm or greater in thickness), which represents a cone-shaped fibrotic mass cranial to the portal vein in infants with BA. An abnormal gallbladder (nonvisualized or small) was thought to be more suggestive of BA than infantile intrahepatic cholestasis.Results: Among 25 infants with BA, 21 showed TC, whereas 4 had no TC. Fifty-three of 54 infants with infantile intrahepatic cholestasis had no TC, showing a diagnostic accuracy of 94% with 84% sensitivity and 98% specificity. As for positive predictive value in the diagnosis of BA by the TC coupled with gallbladder images, it was 100% when a positive TC was coupled with an abnormal gallbladder and 88% when a positive TC was coupled with a normal gallbladder. It decreased to 25% when a negative TC was coupled with an abnormal gallbladder.Conclusions: The TC appears to be a very specific and definite ultrasonographic finding in the early diagnosis of BA. Positive TC regardless of gallbladder images is highly suggestive of BA, showing a 95% positive predictive value, but BA cannot be ruled out when negative TC is coupled with an abnormal gallbladder, requiring further diagnostic modalities such as liver needle biopsy or hepatobiliary scintigraphy.
Post-Graduate Medical Journal of NAMS, 2007
Background: Noninvasive methods that have been used for diagnosis of bile duct stones include ultrasonography, computed tomography and magnetic resonance cholangiopancreatography without use of contrast media. Ultrasonography is the popular, safe, cost effective and effective initial modality for the investigation of obstructive jaundice. Objectives: To identify the magnitude of calculus disease as the cause of extra hepatic biliary obstructive jaundice and role of ultrasonography in the diagnosis. Methodology: One year (Aug 2005-July 2006) case records of obstructive jaundice attended in Bir Hospital for ERCP were analyzed. Those all cases were evaluated by ultrasonography prior to admission in the hospital. Among them, the record of those cases carrying or suspected choledochocholithiasis was identified and their USG findings correlated with the ERCP results. Results: Among the 73 case records enrolled in study, 20 cases were diagnosed choledocholithiasis, and suspected same in other 4 with other differential diagnosis. ERCP diagnosis was similar in 20 cases, among other four cases with differential diagnosis, two were found to be stone in distal CBD and there was periampulary mass in one. Another one case was negative for calculus disease but it was diagnosed juxta papillary diverticulum, otherwise the ERCP was reported no obstructing lesion. This result shows USG diagnosis was correct in 83.33%, supportive in two and different in remaining two cases in comparison to ERCP. Choledocholithiasis was more common in female(N=15, 75.0%) than the male (N=4, 25.0%). Conclusion: Our result indicate that the majority of cases of choledocholithiasis can be diagnosed with ultrasonography.
E-ISSN: 2308-6483/13 © 2013 Synergy Publishers Biliary Atresia: A Challenging Diagnosis
Biliary atresia (BA) constitutes about one third of all neonatal cholestasis (NC) and the most common indication (up to 50%) of liver transplantation (LTx) in children. Despite extensive studies, its etiopathogenesis has not been clearly revealed. Treatment is primarily surgical based on reinstitution of bile flow by Kasai portoenterostomy, the success of which is largely dependent on the early diagnosis before 60 days of age. If portoenterostomy is not successful or not performed, LTx is the only life-saving alternative. Accurate diagnosis of BA, particularly distinguishing it from other causes of liver injury in the neonatal period, is challenging as there is a high degree of overlap in clinical, biochemical, imaging, and histological characteristics. There is no single preoperative investigation that enables the diagnosis of BA to be made with certainty. Liver biochemistry assessment, biliary radionuclide excretion scanning, magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), percutaneous needle liver biopsy, and laparoscopy can all be helpful, but their results are not individually diagnostic. The current review presents an overview of BA with emphasis on the recent diagnostic modalities.