Surgical modifications, additions, and alternatives to Kasai hepato-portoenterostomy to improve the outcome in biliary atresia

Pediatr Surg Int. 2017 Dec;33(12):1275-1282. doi: 10.1007/s00383-017-4162-8. Epub 2017 Oct 4.

Abstract

Kasai hepato-portoenterostomy (HPE) is the most widely used surgical technique to restore bile flow in biliary atresia (BA). We aimed to review literature on HPE substitutes and additions to Kasai especially in advanced BA (ABA). A PubMed search was done for surgical procedures apart from or along with Kasai HPE for BA. Additional procedures to prevent cholangitis were also reviewed. Procedures and outcome were analysed. Alternative procedures done by the authors have also been described briefly. Results have been compiled in this review article. In ABA, with portal hypertension and liver cirrhosis, Kasai HPE is associated with poor outcome, increased morbidity, and even mortality. Most require liver transplant (LT). Some alternatives to HPE include exploration at porta hepatis to assess the bile flow yet avoid the major surgery (HPE) as a bridge to LT. Conduit diversion may help to combat cholangitis resistant to steroid therapy. Stoma formation is not preferred in cases listed for LT due to high risk of bleeding. Hepatocyte infusion, stem cell therapy, and synthetic liver are the future options to meet the challenges in BA. Various alternative procedures may become handy in the future especially in ABA.

Keywords: Biliary atresia; Hepatoenterostomy; Kasai modifications; Stem cells.

Publication types

  • Review

MeSH terms

  • Biliary Atresia / surgery*
  • Humans
  • Infant, Newborn
  • Liver Transplantation / methods*
  • Portoenterostomy, Hepatic / methods*
  • Treatment Outcome