Mo1994 ROLE OF ERCP IN THE DIAGNOSIS OF BILIARY ATRESIA: A RETROSPECTIVE SERIES FROM A LARGE HPB CENTRE

Abstract

Cholestatic jaundice affects up to 1 in 2500 newborns. Possible causes include bacterial sepsis, galactosemia, tyrosinemia, panhypo-pituitarism, bile acid synthetic defects, obstructive gallstones and Biliary atresia (BA). BA is a destructive cholangiopathy of neonates, which can lead to portal hypertension and liver failure if not identified early and a Kasai hepatoportoenterostomy performed timely to restore bile flow. Diagnostic algorithms vary substantially between different centres, with some units advocating ERCP be performed in every case. We report outcomes from our large HPB centre where ERCP is performed on a selective basis for suspected biliary atresia. Patients were included in the study who were referred for ERCP with a conjugated hyperbilirubinamia and suspected biliary atresia between January 2008 and December 2018. Patient demographics, laboratory results, imaging, endoscopy and surgical findings were recorded in each case. Of the 374 children undergoing an ERCP during this period, 15% (56) had it performed for conjugated hyperbillirubinaemia with suspected BA. Median age at the time of ERCP was 2 months (0 – 9 months). 82% (46) patients were male. ERCP confirmed diagnosis of BA in 25% (14) cases of which 64% (9) were type 3 BA, 36% (5) type 2 BA and 0% type 1 BA. The majority of patients confirmed with BA were <3 months of age (86% (12)). No endoscopic or anaesthetic adverse events occurred. All patients with BA were surgically managed at a median time of 6 days (1 – 146 days) post ERCP. 93% (13) were managed with Kasai hepatoportoenterotomy, and 1 with orthotopic liver transplantation. ERCP had a diagnostic sensitivity for BA of 74%, with 5 (9%) patients being misdiagnosed with BA on initial assessment, later disproved on liver biopsy; and diagnostic specificity of 71%. Of the 42 (75%) who did not have BA, 36% (15) were diagnosed as conjugated hyperbillirubinaemia of infancy, 36% (35) had Alagille syndrome or alpha1 anti-trypsin, 12% (5) had either choledocholithiasis or biliary sludge, 7% (3) had choledochomalformations, 5% (2) were diagnosed with neonatal hepatitis and 5% (2) with neonatal sclerosing cholangitis. Selective use of ERCP is a sensitive method of diagnosing BA in infants, enabling timely surgery to improve biliary drainage. It is a clinically safe procedure with no endoscopic or anaesthetic adverse events.