A pathological scoring system in the diagnosis and judgment of prognosis of biliary atresia.

OBJECTIVE

The purpose of this study was to evaluate the diagnostic and prognostic value of a histological scoring system in biliary atresia (BA).

METHODS

From June 2013 to July 2014, 86 wedge liver biopsy specimens were obtained from infants with neonatal cholestasis (58 patients with biliary atresia and 28 patients with non-obstructive cholestasis as control) in our center. A pathologist, single-blinded to the final diagnosis, made the histological diagnosis individually based on an 8-feature (liver fibrosis, portal ductal proliferation, bile plugs in portal ductules, cholestasis, hepatocellular changes inflammatory cells infiltration in portal region, extramedullary hematopoiesis, and ductal plate malformation), 21-point (0 to 21) scoring system.

RESULTS

In this retrospective study, ductular reaction (bile ductular proliferation) and liver fibrosis in the portal area, bile plugs, and ductal plate malformation were the best indicators of BA. With the scoring system, a score of ≥8 had the best diagnostic utility to differentiate BA from other intrahepatic cholestasis histologically (sensitivity 94.7%, specificity 86.2%, accuracy 91.9%). Liver fibrosis and ductal plate malformation were confirmed to be related with the prognosis.

CONCLUSIONS

An 8-feature, 21-point histological scoring system has a good diagnostic accuracy in the interpretation of liver histology in neonatal cholestasis. The use of liver fibrosis and ductal plate malformation are also feasible to assess the prognosis.