Diagnosis Of Extrahepatic Biliary Atresia Research Articles

ERCP in the diagnosis of extrahepatic biliary atresia

This study assessed the usefulness of ERCP in the diagnosis of biliary atresia. We evaluated 57 infants with prolonged cholestasis with abdominal ultrasound, liver biopsy, and ERCP. Using clinical observations alone, 22 infants were thought to have biliary atresia; whereas 35 children were thought to have neonatal hepatitis. The ERCP was performed with a prototype duodenoscope and was successful in all infants except two with biliary atresia. In 20 infants three types of radiological findings consistent with biliary atresia were seen: type 1, no visualization of biliary tree (35%); type 2, opacification of the distal common duct and gallbladder without visualization of the main hepatic duct (35%); and type 3, opacification of the distal common duct, the gallbladder, and a segment of the main hepatic duct with biliary lakes at the porta hepatis (30%). Twenty-five of 35 infants with suspected neonatal hepatitis were excluded because of a liver biopsy that was diagnostic. In the remaining 10 infants the liver biopsy had some features of extrahepatic biliary atresia and ERCP was performed prior to surgery. A normal extrahepatic biliary tree was obtained in all of them. In conclusion, ERCP permits the visualization of the biliary tree in young infants and is useful in selecting those infants who should be considered for exploratory laparotomy.

IMPROVEMENT OF CHOLESCINTICRAPHY IN THE DIFFERENTIAL DIACNOSIS OF EXTRAHEPATIC BILIARY ATRESIA

The introduction of halogenated analogs of iminodiacetic acid (IDA) has increased the effectivity of cholescintigraphy in the differential diagnosis of extrahepatic biliary atresia (EHBA). We attempted to evaluate cholescintigraphy with regard to its potential in increasing the effectivity and predictive value in the diagnosis of EHBA. We studied 16 patients (3 with EHBA, weight = 2,45-6 kg, direct bilirubin 1,6-17,9 mg/dl) by 18 tests. 99mTc mebrofenin (n=5) and 99mTc lodida (n=13) were used as tracers in intermittant sequential scintigraphy to 24 hrs. The data were evaluated by a computer program to obtain 13 kinetic parameters, and were compared with the results of the conventional scintigraphic interpretation. The positive predictive value of the test increased to 80 % by measurement of the maximum intestinal activity, and to 100 % by evaluation of the hepatic tracer clearance. Combining the maximum intestinal activity with the hepatic tracer retention or clearance led to an effectivity and positive predictive value of 100 %. The examination of liver indices (± adjustment for background activity) had no value. Conclusions: An automated evaluation of data characterizing the excretion of halogenated IDA derivatives into the intestine has the potential of allowing the differentiation of EHBA from intrahepatic diseases with high probability. We plan to perform prospective studies on larger numbers of infants.

Support for a conservative approach to mixed intrahepatic and extrahepatic biliary hypoplasia

This report describes the pitfalls in the diagnosis of extrahepatic biliary atresia in an infant with paucity of the interlobular bile ducts.

The place of percutaneous liver biopsies in the early preoperative differential diagnosis of extrahepatic biliary atresia

The place of percutaneous liver biopsies in the early preoperative differential diagnosis of extrahepatic biliary atresia

Biliary atresia in the newborn

A prenatal sonographic diagnosis of extrahepatic biliary atresia was made and, 76 hours after birth, operatively confirmed. A standard Kasai operation was performed, with the exception of the use of an ancillary appendiceal conduit to provide biliary drainage of an independent bile duct draining the right anterior hepatic segment. Hepatic and ductal histology were identical to those usually found in biliary atresia in a 6- to 8-week-old infant. The child is well at 16 months.

The prelaparotomy diagnosis of extrahepatic biliary atresia: A. G. Manolaki, V. F. Larcher, A. P. Mowat, et al. Arch Dis Child 58:591–594 (August), 1983

The prelaparotomy diagnosis of extrahepatic biliary atresia: A. G. Manolaki, V. F. Larcher, A. P. Mowat, et al. Arch Dis Child 58:591–594 (August), 1983

The prelaparotomy diagnosis of extrahepatic biliary atresia.

The diagnostic accuracy of laboratory investigations in the prelaparotomy differentiation between extrahepatic biliary atresia (EHBA) and intrahepatic disease (IHD) was assessed in 86 consecutive infants presenting with conjugated hyperbilirubinaemia. Forty five infants had EHBA and 41 IHD. The mean serum bilirubin concentration, gamma-glutamyltranspeptidase (GGT) activity, and the GGT/aspartate transaminase (AST) ratio were appreciably higher in infants with EHBA than in those with IHD. In infants with IHD, however, serum bilirubin concentrations were in the EHBA range in 19 (47%), as were GGT values in 29 (71%), and GGT/AST ratios in 33 (80%). In individual patients neither increasing nor decreasing GGT values were of diagnostic importance. Failure of biliary excretion of 99Tcm-p-Butyl-ida occurred in 29 of 30 (97%) patients with EHBA but also in 22 of 23 (67%) with IHD. In all 5 patients with IHD associated with alpha 1 antitrypsin deficiency these 4 investigations gave results in the EHBA range. Liver biopsy specimen interpretation, correct in 38 of 42 infants with EHBA, gave an overall accuracy of diagnosis of 86%: the results of 3 further biopsies were equivocal. In 33 of 40 infants with IHD bile duct obstruction was excluded; the remaining 7, including 4 with alpha 1 antitrypsin deficiency, showed equivocal changes. Faecal excretion of 131I rose bengal faecal excretion was less than 10% in 36 of 37 patients with EHBA and in 9 of 26 with IHD, giving an overall accuracy of diagnosis of 84%. In patients in whom genetic disorders, such as alpha 1 antitrypsin deficiency had been excluded, interpretation of liver biopsy specimens together with 131I rose bengal faecal excretion remain the most accurate means of identifying those who need surgery for EHBA and of avoiding unnecessary laparotomy in infants with IHD.

Endoscopic Retrograde Cholangiopancreatography in the Diagnosis of Extrahepatic Biliary Atresia

In the evaluation of jaundice in infancy, the differentiation between neonatal and biliary atresia may be difficult since there is considerable overlap in the clinical features and the liver biopsy findings. Visualization of the biliary system is desirable and may be diagnostic. This has been accomplished in most cases by operative cholangiography. We report preoperative confirmation of the diagnosis of extrahepatic biliary atresia by endoscopic retrograde cholangiopancreatography (ERCP) in a 4-month-old infant. Report of a Case .—A 3-week-old female infant, the product of an uncomplicated pregnancy and full-term delivery, was noted to be jaundiced. Laboratory values at five weeks included the following: total serum bilirubin, 9.8 mg/dL; direct bilirubin, 2.5 mg/dL; SGOT, 70 IU/L; and SGPT, 50 IU/L. A liver biopsy performed at ten weeks was interpreted as showing chronic active hepatitis and the child was treated with corticosteroids. One month later, the infant was taken to New York

Cholangiocarcinoma Associated With Biliary Cirrhosis Due to Congenital Biliary Atresia

An 11-year-old girl developed cholangiocellular carcinoma in association with biliary cirrhosis due to congenital biliary atresia. An exploratory laparotomy and an operative cholangiogram at 3 months of age had confirmed the diagnosis of extrahepatic biliary atresia. A liver biopsy specimen taken at 6 months of age showed biliary cirrhosis. The subsequent clinical course was characterized by persistent moderate jaundice, anemia, malnutrition, rickets, pathologic fractures, and recurrent gastrointestinal bleeding. The presence of cholangiocellular carcinoma of the liver with advanced biliary cirrhosis was established at an exploratory laparotomy a week before her death. We discuss here the pathogenesis of biliary cirrhosis and carcinoma of the liver; there may be a relation between the two in the childhood population.