Biliary atresia in infants with prolonged cholestatic jaundice: diagnostic accuracy of hepatobiliary scintigraphy

Abstract

Cholestatic jaundice during infancy is one of the most problematic challenges for pediatricians. Biliary atresia (BA) and neonatal hepatitis syndrome (NHS) are major causes of cholestatic jaundices. Our aim was to compare the diagnostic accuracy of hepatobiliary scintigraphy with liver biopsy and ultrasonography in excluding BA. Seventy consecutive patients, all suffering from prolonged cholestatic jaundice (>1 month), were included. Laparotomy with surgical cholangiography was considered as the gold standard; however, in nine patients, based on the patient's recovery from jaundice and the normalization of laboratory values during the clinical follow-up period (=6-12 months), the diagnosis of NHS was verified and performing laparotomy was unnecessary. All patients underwent hepatobiliary scintigraphy, liver biopsy and ultrasonography and their results were compared. Based on the gold standards mentioned above, 46 patients (46/70 = 65.7%) had BA. The sensitivity, specificity, PPV, NPV, and accuracy of the hepatobiliary scintigraphy in diagnosis of BA were 90%, 80%, 91.8%, 76.2%, and 84.5%, respectively. The respective values for liver biopsy were 92.5%, 88.9%, 94.9%, 84.2%, and 90.1% and for ultrasonographic analysis were 41.7%, 90.9%, 90.9%, 41.7%, and 66.3%. Hepatobiliary scintigraphy is an important imaging technique in the diagnostic evaluation of infants with prolonged cholestatic jaundice. It is a convenient and reliable method of differentiating BA from NHS, with a diagnostic accuracy superior to that of US but slightly inferior to that of liver biopsy. Ultrasonography is the least sensitive and specific available modality and its findings should be confirmed by scintigraphy or liver biopsy.