The family of a 5-month-old male infant was referred to Duke Children’s Hospital & Health Center. He had been recently diagnosed with biliary atresia at another regional medical center and, at 4 months of age, underwent a Kasai procedure (portoenterostomy) that involved removing the affected bile ducts and gallbladder with anastomosis of the small intestine to the liver so that bile could be secreted. However, at the time of the surgery, the patient had evidence of bridging fibrosis and cirrhosis. Upon presentation at Duke, his bilirubin level was elevated; he was also jaundiced, had a fluid-filled abdomen, and was underweight for his age.
Question: What is the definitive treatment for biliary atresia?
Answer: Liver transplant is the definitive treatment for biliary atresia.
Without transplantation, most children with biliary atresia will die from hepatic failure, even after undergoing portoenterostomy. In general, outcomes for liver transplantation in children are excellent, with 5- and 10-year survival rates now approaching 95%.
“When we met the patient, he was still jaundiced and had ascites. He was small and wasn’t growing very well,” says Megan W. Butler, MD, a pediatric gastroenterologist at Duke. Butler was one of several specialists involved in the care of the infant.
Although the Kasai procedure had helped somewhat in lowering the patient’s bilirubin levels, the operation is most effective when performed on patients younger than 2 months of age (ideally younger than 45 days of age), Butler explains. However, even with a successful initial surgery, approximately 80% of children with biliary atresia will require a liver transplant at some point.
The patient was placed on the deceased donor liver transplant list. But, at the time, other children were also on the list, so specialists at Duke Medicine recommended simultaneously evaluating potential living donors. Typically, a family member or family acquaintance becomes the donor, but, in this case, no suitable related donors were identified.
The patient’s mother decided to enlist the support of the community to find a viable living donor for her son. In an overwhelming response, 250 individuals came forward and offered to be evaluated as potential donors.
“This outpouring posed some challenges for Duke’s living donor coordinators and hepatologists to narrow down this huge field of good Samaritans to determine who would be the best match,” recounts Butler, “but that’s eventually how he got his liver transplant.”
The transplant was performed on May 15, 2015, which was 5 months after his initial evaluation at Duke.
To date, the patient is doing well. He has not been readmitted since the transplant, and his bilirubin level is normal. He has also gained 2 pounds.
Notably, some potential donors said they were willing to be a living donor to other children in need of a liver transplant. Butler explains that this generated substantial discussion at Duke about living donor protocols in which donors were required to specify an individual patient to whom they were donating.
“After this case, we are thinking maybe living donation for liver transplantation can be more like bone marrow donation,” Butler says, “where you can donate for whoever needs the transplant.”
Duke is the only center in North Carolina with specialists who perform transplants in children younger than 1 year of age and the only center in the state that performs living donor liver transplants.