Prognostic impact of hepatobiliary scintigraphy in diagnosis and postoperative follow-up of newborns with biliary atresia

Aim: To investigate the prognostic relevance of hepatobiliary scintigraphy (HBS) in newborns suffering from biliary atresia (BA) for establishing the primary diagnosis and in the postoperative follow-up after portoenterostomy (Kasai). Methods: Twenty newborns with direct hyperbilirubinemia and 6 chi ldren after operative treatment of BA (Kasai) underwent HBS with Tc-99m-DEI DA. In patients without intestinal drainage, hepatocellular extraction was estimated visually and calculated semiquantitatively by means of liver/hear t-ratio 5 min p.i. Results: 10/20 patients with hyperbilirubinemia did not display biliary drainage; 6 had BA, 3 intrahepatic hypoplasia, and one show ed a bile plug syndrom. 4/6 with BA but none of the 4 children with diagnos es other than BA presented with a good extraction. All of the 4 children wi th BA, who had either pre- or postoperatively a bad extraction, needed live r transplantation due to liver failure. Both of the two newborns with BA an d favourable outcome after Kasai had a good extraction in the preoperative HBS and demonstrated good intestinal drainage in the postoperative scan. Co nclusion: HBS rules out BA with high accuracy by demonstrating drainage of bile into the intestine. In newborns without drainage a good extraction fav ours the diagnosis of BA. In newborns with BA a bad extraction seems to ind icate a poor postoperative prognosis after Kasai operation. HBS might there fore help to select those children who will not benefit from portoenterosto my. Postoperatively, HBS can easily and quickly confirm the successful hepa tobiliary anastomosis by demonstrating biliary drainage into the intestine.