Answered and unanswered controversies in the surgical management of extra hepatic biliary atresia

Almost a half-century since Kasai described the portoentersotomy for extrahepatic biliary atresia (EHBA), some questions about the management of this condition have been resolved and many are unanswered. The most useful diagnostic steps to aid in the diagnosis are debated. Sonography can be helpful but its sensitivity and selectivity are arguable with strong advocates for its effectiveness. Likewise, the magnetic resonance imaging has forceful advocates but also has not been universalized. The liver biopsy, done commonly before an operation for cholestasis, is often not discriminating. The radionuclide scan hepatobiliary iminodiacetic acid (HIDA) scan after phenobarbital stimulation is helpful if negative, but false positive results are common. It is agreed to proceed expeditiously to the operation in the cholestatic infant after a prompt investigation. The proposal to avoid this step and provide liver transplantation as initial management for EHBA has been suppressed by several clinical findings. The Kasai procedure has not worsened the outcome of eventual liver transplantation. The Kasai, even if it eventually fails, will often buy time and allow the child to grow before transplantation is needed. Multiple reoperations prior to the transplant are discouraged. Revisions to improve bile flow have not gained wide popularity. Use of a stoma to divert the bile has been largely abandoned. The need for frozen section examination of the liver at the site chosen for the portoenterostomy is no longer demanded. The preferred type of intestinal conduit is argued. Unanswered questions about the post-operative management include the role of steroids and of prophylactic antibiotics. The Biliary Atresia Research Consortium, a multi-institutional National Institutes of Health (NIH)-supported project, will address many of the unanswered issues.