Comparison Of Clinical And Histological Parameters In Biliary Atresia Patients With And Without Ductal Plate Malformation

Background And Aim: Biliary atresia (BA) can be associated with ductal plate malformation (DPM) like changes in as high as 40% of patients. It is associated with variable prognosis, with most of the studies suggesting poor overall outcome. Aim isto study the clinical and histological parameters in biliary atresia patients with and without DPM. Methods: Medical records of patients diagnosed with Biliary atresia from January 2021 to September 2022 were analyzed. Histology of every patient was reviewed by expert pathologist classifying them into BA with and without DPM. Results: A total of 84 patients were included with the median age of 3 months. 66.7% were males. 35 cases (41.66%) had associated DPM like changes. Age at onset of jaundice and physical examination findings were similar among two groups. Overall, 13(15.5%) patients had ascites at presentation. Biochemical parameters and synthetic liver functions were no different between the groups. In histopathology, BA with DPM group showed higher degrees of ductular reaction and higher grades of fibrosis in comparison to BA without DPM (p=0.003). 41 (48.8%) of cases underwent portoenterostomy with overall survival of 12.65%. Among patients who underwent PE, short term survival (<3 years) rate was 25%. Survival rate was no different between BA with or without DPM. When key histological features were analysed, we observed advanced fibrosis group (Grade 3 or 4) was associated with a higher degree of ductular reaction which was statistically significant (p=0.002). None of the patients with mild-moderate fibrosis had associated ductal plate malformation (0.003). Patients who underwent PE had lower grades of fibrosis (p=0.019) and higher degrees of portal inflammation(p=0.007). Conclusions: Presence of DPM with BA is not uncommon with no differences in clinical features and overall outcome in our cohort. The exuberant ductular reaction was a key histopathology feature which induces DPM-like arrays in biliary atresia.