title: The Role of Endoscopic Retrograde Cholangiopancreatography in the Diagnosis of Biliary Atresia: 14 Years' Experience.
abstract: BACKGROUND
  Biliary atresia (BA) is a rare destructive inflammatory obliterative cholangiopathy of neonates. Early diagnosis is important in disease management. The aim was to evaluate the role of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing BA in a large cohort. In addition, we evaluated whether parameters such as bile trace, GGT, bilirubin, and laboratory values in combination can be used to develop a risk score that could indicate the referral to specialized centers.
  
  
  METHODS
  All infants with neonatal cholestasis (2000-2014) who presented to our endoscopy unit for suspected BA were included. Demographics, laboratory parameters, ultrasound findings, liver biopsy results, ERCP diagnosis, and surgical outcome were collected. Value and safety of ERCP and risk factors for BA were retrospectively analyzed.
  
  
  RESULTS
  We included 251 infants in our cohort (55% males, median age: 53 days). BA was intraoperatively diagnosed in 155 (83.4%) patients and was excluded in 30 (16.2%). Fifty-six cases were not operated due to the ERCP findings. ERCP was successful in 224/251 patients (89.2%) with no procedure-related complications. The operative and endoscopic diagnosis matched in 96.6% of the patients (positive predictive value: 92.2%, negative predictive value: 97.1%). In comparison to cases with excluded BA, the ones with this disease were significantly associated with absence of duodenal bile traces (98.4 vs. 1.6%,p< 0.001), higher bilirubin (p< 0.001, cutoff 7.3 mg/dL), and higher GGT (p< 0.001, cutoff 250 U/L).
  
  
  CONCLUSIONS
  ERCP is safe and accurate in the hands of experts in diagnosing BA if the cause of cholestasis is unclear. While evaluating the role of ERCP for diagnosing this disease, we found that the secondary parameters GGT > 250 U/L, bilirubin > 7.3 mg/dL (125 μmol/L), and the absence of bile traces are risk factors.
authors.affiliation: Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, Hannover, Germany.
authors.forename: Ahmed A
authors.initials: AA
authors.lastname: Negm
authors.affiliation: Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany.
authors.forename: Claus
authors.initials: C
authors.lastname: Petersen
authors.affiliation: Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, Hannover, Germany.
authors.forename: Andrea
authors.initials: A
authors.lastname: Markowski
authors.affiliation: Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, Hannover, Germany.
authors.forename: Birgit
authors.initials: B
authors.lastname: Luettig
authors.affiliation: Department of Diagnostic and Interventional Radiology, Hannover Medical School, Hannover, Germany.
authors.forename: Kristina I
authors.initials: KI
authors.lastname: Ringe
authors.affiliation: Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, Hannover, Germany.
authors.forename: Tim O
authors.initials: TO
authors.lastname: Lankisch
authors.affiliation: Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, Hannover, Germany.
authors.forename: Michael P
authors.initials: MP
authors.lastname: Manns
authors.affiliation: Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany.
authors.forename: Benno
authors.initials: B
authors.lastname: Ure
authors.affiliation: Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, Hannover, Germany.
authors.forename: Andrea S
authors.initials: AS
authors.lastname: Schneider
mesh_terms: D001656:Biliary Atresia / Q000000981:diagnostic imaging* / Q000209:etiology / Q000628:therapy; D002760:Cholangiopancreatography, Endoscopic Retrograde; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012017:Referral and Consultation; D012189:Retrospective Studies; D012307:Risk Factors