To compare outcomes of two common surgical procedures for children with liver complications due to Alagille syndrome, a USC researcher at Children’s Hospital of Los Angeles is driving a collaboration with colleagues from 15 hospitals and research centers across the country.
Alagille syndrome is a congenital disorder that can interfere with development of the liver’s bile ducts, preventing normal elimination of the important digestive fluid. A buildup of bile can damage the liver and cause other serious complications.
Fortunately, Alagille syndrome is uncommon, occurring once in approximately 70,000 to 100,000 births. But this rarity has also made the condition difficult to study, explained principal investigator Kasper Wang, MD, associate professor of surgery at the Keck School of Medicine of USC and a pediatric surgeon at Children’s Hospital of Los Angeles.
To prevent a buildup of bile in Alagille patients, surgeons typically employ one of two surgical approaches. In one, surgeons build a system to drain bile from the gall bladder out of the body into an external plastic pouch. For the other approach, surgeons connect the liver to the colon via the small intestine, so bile can be eliminated through normal excretion.
“Naturally, the internal bypass would seem preferable, but it’s not clear that it works as well,” Wang said. “The two have simply never been compared in a study.”
To find enough patients to make a statistically significant comparison, Wang called on gastroenterologists and pediatric surgeons across North America.
“Since the volume of cases is low, no single institution has sufficient experience to know which approach is preferable, or under what conditions one may be better than the other,” Wang said. “A multi-center study is the only way we can gather enough data.”
Institutions contributing data include Texas Children’s Hospital – Baylor College of Medicine; Lurie Children’s Hospital; University of California, San Francisco Medical Center; Washington University of St. Louis; Riley Children’s Hospital; Children’s Hospital of Pittsburgh; Mount Sinai Hospital; Cincinnati Children’s Hospital Medical Center; Children’s Hospital of Philadelphia; Children’s Hospital Colorado; The Hospital for Sick Children, in Toronto, Canada; Johns Hopkins University; and Emory University.
— Paul Karon