Medium-term outcomes after laparoscopic revision of laparoscopic Kasai portoenterostomy in patients with biliary atresia

Orphanet J Rare Dis. 2021 Apr 30;16(1):193. doi: 10.1186/s13023-021-01835-z.

Abstract

Objective: To determine whether revision laparoscopic Kasai portoenterostomy (RLKPE) is a viable treatment option for patients with biliary atresia (BA) who had undergone initially successful laparoscopic Kasai portoenterostomy (ILKPE).

Methods: The medical records of 312 patients with nonsyndromic BA who had undergone ILKPE between May 2009 and May 2017 were retrospectively reviewed. The patients were divided into three groups according to their outcomes after ILKPE: group A: 25 patients who had undergone RLKPE; group B: 203 patients who had undergone ILKPE and required no further surgical intervention; group C: 84 patients with failed ILKPE who had either died or required liver transplantation for survival. The 3-year and 5-year survival with native liver (SNL) rates were compared between groups A and B and between groups A and C. Among the 25 patients in group A, the perioperative data of RLKPE were compared with those of ILKPE.

Results: Of the 312 patients who had undergone ILKPE, 228 reached the normal bilirubin concentration range within 6 months postoperatively. Among them, 25 patients with a sudden cessation of bile flow had undergone RLKPE. Adequate biliary drainage, as evidenced by normalized conjugated bilirubin levels, was achieved in 80% of patients who had undergone RLKPE. The perioperative variables, including the operative time, blood loss, rate of conversion to open surgery and complications of RLKPE, were not significantly different between RLKPE and ILKPE. The 3-year and 5-year SNL rates in patients after RLKPE were 64.0% and 52.0%, respectively, which were not significantly different from the corresponding rates of 86.2% and 73.9%, respectively, in patients after unrevised ILKPE (P > 0.05).

Conclusion: Our data demonstrated that RPLKE is a viable and effective treatment option in patients with sudden cessation of bile drainage after ILKPE. RPLKE can delay the need for liver transplantation, yielding encouraging medium-term patient outcomes.

Keywords: Biliary atresia; Laparoscopic Kasai portoenterostomy; Outcomes; Revision.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biliary Atresia* / surgery
  • Humans
  • Infant
  • Laparoscopy*
  • Liver Transplantation*
  • Portoenterostomy, Hepatic
  • Retrospective Studies
  • Treatment Outcome