Biliary Atresia; Do Histopathological Insights Predict Clinical Outcomes?

Background & Objectives: Biliary atresia(BA) is an idiopathic, progressive inflammatory disease of the biliary tract. Kasai Procedure (KP-Hepatoportoenterostomy) should be performed to maintain biliary drainage and decelerate cirrhotic progression. Here, we aimed to evaluate the histopathological findings in liver related to clinical outcomes.

Methods: 26 BA cases with clinical follow-up were reevaluated. Portal fibrosis, edema and expansion, severity and distribution of biliary duct proliferation, bile plugs, pseudorosette formation, giant cell transformation(GCT), hepatocyte damage, extramedullary hematopoesis, ductal plate malformation were evaluated in liver biopsies which were obtained during KP. Clinical information about attacks of cholangitis, native-liver survival, need for transplantation were obtained from the database.

Results: Among the 26 cases, 23 had Kasai in 90 days after birth, involved in the statistical analysis. 8 (%34.8) cases were operated in the first 45 days (Group 1) whereas 15 (Group 2) between 45-90 days. Severe fibrosis was significantly more common in Group 2(p=0,009). Biliary duct proliferation was intense(p=0,008) and diffuse(p=0,032) in Group 2. There was no significant difference between groups for GTC. Additionally, no difference was found between the groups in terms of clinical outcomes. However, patients with shorter than two-years native-liver survival(n=6) were all in Group 2. Besides, patients with at least four years native-liver survival had had Kasai before 60 days.

Conclusion: BA is, by its nature, a progressive disease that results in liver cirrhosis. It has been demonstrated by clinical outcomes and pathological parameters that surgery performed at an early age offers a longer native-liver survival. It is seen that findings from pathological examination are directly related to time until KP is performed, these findings can provide preliminary information about the course of the disease.