Predicting factors on the occurrence of cystic dilatation of intrahepatic biliary system in biliary atresia.

Purpose: Cystic dilatation of intrahepatic biliary system (CDIB) is an intractable complication of biliary atresia (BA). In this study, we investigated the predicting factors of CDIB development after jaundice resolved following hepatoportoenterostomy (HPE).

Methods: From 1988 to 2008, 28 (80.0%) of 35 uncorrectable type of BA patients became jaundice-free after HPE. Of these 28 patients, this retrospective study included comparisons of the preoperative characteristics, postoperative jaundice period, cumulative steroid dose, outcome, and liver fibrosis grade at the time of HPE between CDIB-positive and -negative groups, divided by postoperative ultrasonography findings.

Results: There were no differences between groups in perioperative characteristics. Liver fibrosis in the CDIB-positive group (n = 7) included grade II in two patients, grade III in four patients, and grade IV in one patient. On the contrary, the CDIB-negative group (n = 21) included 8, 11, 2, and 0 patients with grades I-IV, respectively, with a significant difference (p < 0.05) between groups. Survival rate with native liver in CDIB-positive group was significantly lower than that in CDIB-negative group (p < 0.05).

Conclusion: Postoperative CDIB might be a poor prognostic factor, and the liver fibrosis grade at the time of HPE could be helpful to predict the development of CDIB after HPE.