Advances in Treating Biliary Atresia
Surgeons at NYP Morgan Stanley Children’s Hospital have the highest success rate in the nation for primary repair of biliary atresia (BA), a congenital defect that causes bile to back up into the liver, usually resulting in the need for a liver transplant within the first two years of a child’s life.
“While assembling data for U.S. News & World Report, we found that 70% of the children we operated on for biliary atresia were jaundice-free two years after surgery. That rate was about 15 to 20% higher than we expected,” says Steven Stylianos, MD, Chief of Pediatric Surgery. During the Kasai procedure, a pediatric surgeon removes the infant’s damaged bile ducts and brings up a loop of intestine to replace them. Bile then flows straight to the small intestine.
“We’ve had a passionate interest in BA for three decades,” says Dr. Stylianos. “My predecessor and mentor, R. Peter Altman, MD, was a world authority on the Kasai procedure. He went to Japan to learn the technique from Dr. Kasai, then brought this operation back to the United States." The operation is not only technically demanding, it doesn’t have standard markers, such as where and how much to cut. Success therefore depends upon the surgeon. “Our continuity of training and our pursuit of excellence have put us in the lead,” says Dr. Stylianos. “Our babies are doing well, surpassing all benchmarks for recovery from a life-threatening condition.”
The team at Children’s Hospital has been able to significantly slow the progression of liver disease in babies with BA. Instead of needing at transplant a year or two after birth, children operated on here may not require one until age 10 or 15. “The donor pool will be much different by then,” says Dr. Stylianos. “The child also won’t have to be on immune suppressive medication, and dealing with the problems of liver failure, from such an early age. This is a huge benefit for families.”
Dr. Jean Emond, Director of the Columbia Center for Liver Disease and Transplantation, notes, “Our excellent results in biliary atresia care require seamless collaboration between Pediatric Hepatology, Surgery, and Transplantation. This promotes early diagnosis and treatment of the biliary atresia with expert performance of the Kasai operation that may delay or prevent the need for transplantation. Early detection of the patients in whom the Kasai procedure is insufficient permits early transplantation with the best short and long term outcomes."
More Progress: Living Donor Transplants
“When infants with BA are referred to us from other centers, today we can offer them a new transplant option,” says Alyson Fox, MD, Director of the Living Donor Transplant Program at Columbia. "For years, parents endured the stress of being on the UNOS waitlist, as they watched their babies get sicker. Now they can schedule a life-saving surgery with a living donor.” ABC-TV recently featured a success story from the Columbia Living Donor Program, where a godmother volunteered to give a portion of her liver to save a child with BA.
Sometimes the parents aren’t a match because of health issues, or the size of their liver. Friends and family members want to help and may be potential donors. Says Dr. Fox, "They are more willing to step forward once they learn that the surgery is minimally invasive, and recovery fairly fast. We are able to take the smallest portion of the donor liver—the left lateral segment—to implant in the child. The donor’s liver quickly grows back to normal size. And the transplanted portion will continue to grow with the child. Parents are amazed to see their babies with end- stage organ failure turn around so quickly, their jaundice disappearing in three days.”
Columbia’s outcomes for pediatric living donor liver transplants are outstanding, Dr. Fox reports, with a one-year survival rate of 100%. The Center for Liver Disease and Transplantation (CLDT) is also one of the largest living donor centers in the nation. To date, its surgeons have performed over 300 living donor transplants, more than any other center in the United States. It is also one of a few centers accepting altruistic donors.
“We are particularly proud of our pediatric program,” says Dr. Fox. “Emerging data shows that children with liver transplants may eventually be able to go off immunosuppression medication, allowing for a better quality of life. This seems especially true for those with living donors. We also have a new initiative to provide ongoing follow-up as our young patients grow into adults. Our new transition program offers seamless care, as they go off to college, take their first job, and start navigating fertility.”
Learn more about our exceptional care for pediatric liver disease here:
To refer a patient please call:
Pediatric Center for Liver Disease and Transplantation: 212-305-3000
Short Hills, New Jersey Office: 973-379-5825
Comprehensive Pediatric Transplant Service 866-697-7755, 24 hours, 7 days a week