title: Hepatobiliary scintigraphy in arteriohepatic dysplasia (Alagille's syndrome). A report of two cases.
abstract: Hepatobiliary scintigraphy has proven to be of great utility in distinguishing biliary atresia from other causes of neonatal cholestasis. Arteriohepatic dysplasia (Alagille's syndrome) is an uncommon entity characterized by typical facial features, pulmonary artery stenosis, and a liver disorder which presents during the neonatal period as progressive jaundice. Two neonates, who were later shown to have Alagille's syndrome, underwent hepatobiliary scintigraphy to rule out biliary atresia. Findings on the hepatobiliary scans from the two patients were similar to those usually associated with biliary atresia and both finally required surgical exploration to rule out biliary atresia. The findings on hepatobiliary scans in these patients with Alagille's syndrome are discussed and compared with those associated with other forms of neonatal cholestasis.
authors.affiliation: Department of Radiology, Children's Hospital, Boston, Massachusetts.
authors.forename: D A
authors.initials: DA
authors.lastname: Summerville
authors.forename: M
authors.initials: M
authors.lastname: Marks
authors.forename: S T
authors.initials: ST
authors.lastname: Treves
mesh_terms: D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities*; D001656:Biliary Atresia / Q000000981:diagnostic imaging; D001659:Biliary Tract / Q000000981:diagnostic imaging*; D002648:Child; D002780:Cholestasis, Intrahepatic / Q000000981:diagnostic imaging*; D003251:Constriction, Pathologic / Q000150:complications; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007098:Imino Acids; D007223:Infant; D008297:Male; D009942:Organometallic Compounds; D015609:Organotechnetium Compounds; D011651:Pulmonary Artery / Q000473:pathology; D011877:Radionuclide Imaging; D013577:Syndrome; D019785:Technetium Tc 99m Disofenin
chemical_list: D007098:Imino Acids; D009942:Organometallic Compounds; D015609:Organotechnetium Compounds; C024340:technetium Tc 99m (para-butyl)iminodiacetic acid; D019785:Technetium Tc 99m Disofenin