A prognostic analysis of cystic and non-cystic biliary atresia

Cystic biliary atresia (CBA) is a rare variant of type III biliary atresia (BA). This study aimed to investigate the clinical features and prognosis of CBA in comparison to non-CBA cases.

A retrospective analysis was conducted on 196 children diagnosed with type III BA between December 2016 and December 2020. This cohort included 22 cases in the CBA group and 174 cases in the non-CBA group. All children underwent Kasai portoenterostomy (KPE) and were subjected to standardized follow-up with a maximum of 5 years. We compared and analyzed the age at surgery, postoperative jaundice resolution rates, native liver survival rates, and complication rates between the two groups.

Patients in the CBA group underwent surgery at a significantly younger age compared to those in the non-CBA group (P < 0.001). There were no statistically significant differences in preoperative liver function indices, such as ALT and AST, between the two groups (P > 0.05). Postoperative cholangitis occurred less frequently in the CBA group than in the non-CBA group (P = 0.009). The rates of jaundice resolution at 6 and 12 months post-surgery were significantly higher in the CBA group than in the non-CBA group (P < 0.05). The five-year native liver survival rate was 67.87% for the CBA group, compared to 40.10% for the non-CBA group (P < 0.0233).

The overall prognosis for CBA is superior to that of non-CBA. Children with CBA received surgical intervention earlier; the postoperative jaundice resolution rate and five-year native liver survival rate of the children were higher than that of non-CBA. These findings provide valuable clinical guidance for the individualized treatment and follow-up management strategies for CBA.