title: Ductal paucity and Warkany syndrome in a patient with congenital extrahepatic portocaval shunt.
abstract: An eleven-year-old clinically dysmorphic and developmentally retarded male child presenting with complaints of 5 episodes of recurrent cholestatic jaundice since 3 years of age was evaluated. Imaging revealed features consistent with congenital extrahepatic portocaval shunt (Abernethy type 1b), multiple regenerative liver nodules and intrahepatic biliary radical dilatation. The presence of ductal paucity and trisomy 8 were confirmed on liver biopsy and karyotyping. The explanation for unusual and previously unreported features in the present case has been proposed.
authors.affiliation: Vikrant Sood, Rajeev Khanna, Seema Alam, Dinesh Rawat, Shorav Bhatnagar, Archana Rastogi, Departments of Pediatric Hepatology, Radiology and Pathology, Institute of Liver and Biliary Sciences, New Delhi 110070, India.
authors.forename: Vikrant
authors.initials: V
authors.lastname: Sood
authors.affiliation: Vikrant Sood, Rajeev Khanna, Seema Alam, Dinesh Rawat, Shorav Bhatnagar, Archana Rastogi, Departments of Pediatric Hepatology, Radiology and Pathology, Institute of Liver and Biliary Sciences, New Delhi 110070, India.
authors.forename: Rajeev
authors.initials: R
authors.lastname: Khanna
authors.affiliation: Vikrant Sood, Rajeev Khanna, Seema Alam, Dinesh Rawat, Shorav Bhatnagar, Archana Rastogi, Departments of Pediatric Hepatology, Radiology and Pathology, Institute of Liver and Biliary Sciences, New Delhi 110070, India.
authors.forename: Seema
authors.initials: S
authors.lastname: Alam
authors.affiliation: Vikrant Sood, Rajeev Khanna, Seema Alam, Dinesh Rawat, Shorav Bhatnagar, Archana Rastogi, Departments of Pediatric Hepatology, Radiology and Pathology, Institute of Liver and Biliary Sciences, New Delhi 110070, India.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Rawat
authors.affiliation: Vikrant Sood, Rajeev Khanna, Seema Alam, Dinesh Rawat, Shorav Bhatnagar, Archana Rastogi, Departments of Pediatric Hepatology, Radiology and Pathology, Institute of Liver and Biliary Sciences, New Delhi 110070, India.
authors.forename: Shorav
authors.initials: S
authors.lastname: Bhatnagar
authors.affiliation: Vikrant Sood, Rajeev Khanna, Seema Alam, Dinesh Rawat, Shorav Bhatnagar, Archana Rastogi, Departments of Pediatric Hepatology, Radiology and Pathology, Institute of Liver and Biliary Sciences, New Delhi 110070, India.
authors.forename: Archana
authors.initials: A
authors.lastname: Rastogi
keywords: Congenital extrahepatic portocaval shunt; Ductal paucity; Trisomy 8; Warkany syndrome