title: Common misdiagnoses of biliary atresia.
abstract: OBJECTIVE
  Discrimination of biliary atresia (BA) from other causes of neonatal cholestasis (NC) is challenging. We aimed to analyze the clinicopathological findings in cholestatic infants who were provisionally diagnosed with BA and then excluded by intraoperative cholangiography compared with those with a definitive diagnosis of BA and to shed light on common misdiagnoses of BA.
  
  
  METHODS
  We retrospectively analyzed the data of infants diagnosed preoperatively with BA and referred to surgery between the years 2009 and 2013. On the basis of intraoperative cholangiography results, infants were divided into those with a definitive diagnosis of BA and those misdiagnosed with BA.
  
  
  RESULTS
  Out of 147 infants, there was a misdiagnosis of BA in 10 (6.8%) infants. Alanine transaminase was significantly higher in the non-BA group, whereas other clinical and laboratory findings were comparable in both groups. Hepatomegaly and abnormal gallbladder in ultrasound, and ductular proliferation and advanced grades of portal fibrosis in liver biopsy were significantly higher in infants with BA. However, giant cells were more common in the non-BA infants. Nonetheless, the frequency of clay stool, hepatomegaly, abnormal gallbladder, ductular proliferation, and advanced portal fibrosis was remarkable (100, 70, 40, 70, and 50%, respectively) in the misdiagnosed infants. The misdiagnoses were idiopathic neonatal hepatitis, progressive familial intrahepatic cholestasis type 3, cytomegalovirus hepatitis, Alagille syndrome, and a cholangitic form of congenital hepatic fibrosis.
  
  
  CONCLUSIONS
  A meticulous preoperative workup should be performed to exclude other causes of NC even if signs of BA are present, especially if features such as giant cells in histopathology are present. This involves completing the NC workup in parallel involving all common causes of NC rather than performing them in series to avoid loss of valuable time and efforts.
authors.affiliation: Departments of aPediatric Hepatology bHepatobiliary Surgery, National Liver Institute, Menofiya University, 32511, Shebin El-koom, Menofiya, Egypt.
authors.forename: Mostafa M
authors.initials: MM
authors.lastname: Sira
authors.forename: Mohammad
authors.initials: M
authors.lastname: Taha
authors.forename: Ahmad M
authors.initials: AM
authors.lastname: Sira
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging; D001706:Biopsy; D002758:Cholangiography / Q000379:methods; D002779:Cholestasis / Q000151:congenital / Q000209:etiology; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D004534:Egypt; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007430:Intraoperative Care / Q000379:methods; D008099:Liver / Q000473:pathology; D008297:Male; D012189:Retrospective Studies; D014463:Ultrasonography