Review of transplant issues in patients with biliary atresia

The most common indication worldwide for pediatric transplantation, biliary atresia is also the most common cause of chronic liver disease in newborns.

Recently, Children's Hospital of Pittsburgh of UPMC physicians completed a review of issues that children with biliary atresia face that lead to the need for transplantation.

Nationally about 42 percent of children undergoing their first liver transplant are diagnosed with biliary atresia. Through the review, which is published in the November issue of Liver Transplantation, doctors at Children's propose that carefully planned medical management of children with biliary atresia is essential to minimize morbidity and mortality. Optimal outcomes for liver transplantation are reached by carefully examining indications for and alternative to transplantation and through the management of clinical problems for children with biliary atresia.

“Careful assessment of the clinical status of children with biliary atresia is essential,” said co-author Benjamin L. Shneider, MD, director, Pediatric Hepatology Program at Children's. “It is so important to optimize medical and surgical management and carefully determine the timing for liver transplantation, if it is needed.”

At Children's, 308 primary liver transplants were performed between 1995 and 2006. Of these, 103, or 33 percent, were performed for biliary atresia. Overall patient survival and graft survival rates at more than 10 years were 88 percent and 81 percent, respectfully, among the highest long term survival rates in the world. These patients were enrolled in the Studies on Pediatric Liver Transplantation (SPLIT) --- a multi-center, prospective study to collect scientific data on pediatric liver transplantation.

“Surgical outcomes after liver transplantation for biliary atresia are outstanding in spite of complex anatomic issues and the small size of many of our patients,” said co-author George Mazarigeos, MD, director of the Hillman Center for Pediatric Transplantation. “By using a full complement of surgical techniques, an experienced center can minimize mortality for children awaiting transplantation. In addition, this can lead to excellent long-term outcomes.”

Biliary atresia, which occurs once in every 15,000 births, is an irreversible problem that is fatal without treatment. However, surgical intervention (the Kasai hepatoportoenterostomy) may allow a child with biliary atresia to live longer and have a better quality of life. In order to have optimal results, infants should undergo surgery before 2 months of age, necessitating early referral. Symptoms of biliary atresia occur between 2 weeks to 2 months of life, and may include: jaundice that persists beyond 3 weeks of age, dark urine, light colored stools and failure to thrive.