For many children with biliary atresia (BA), a successful Kasai procedure is a game-changer, potentially delaying the need for a liver transplant for years—even decades. Since January 2022, Cincinnati Children’s pediatric surgeons Alexander Bondoc, MD, and Greg Tiao, MD, have performed 10 Kasai procedures in a row, with a 100% success rate.  

This favorable success rate can be attributed to multiple factors, including the hospital’s emphasis on early diagnosis, a joint medical and surgical co-management strategy, and a standardized clinical care protocol for using postoperative antibiotics and corticosteroids.

A Five-Year Success Rate of 85%

Biliary atresia is a rare, progressive hepatobiliary disorder characterized by the destruction or absence of all or a portion of the extrahepatic bile duct. Bile builds up in the liver, causing scarring, loss of liver tissue and function, and cirrhosis. During a Kasai procedure, a surgeon removes the extrahepatic ducts and replaces them with a segment of the child’s small intestine to re-establish the outflow of bile from the liver.

A successful Kasai restores bile drainage. In the U.S., the overall success rate (defined as Tbili < 2.0mg/dL two months after the procedure) is typically in the 40-50% range for pediatric patients. Cincinnati Children’s has achieved an 85% success rate over the last five years (33 procedures).

A Tailored Postoperative Treatment Protocol

The Liver Care Center medical and surgical teams are highly integrated across the inpatient/outpatient setting. They have developed clinical care protocols utilizing tailored postoperative antibiotics and corticosteroids to optimize the restoration of bile flow with the goal of improving transplant-free survival in early childhood.

Combined with experienced hepatology and pediatric surgery co-management, the protocols have improved post-Kasai success from 40% before implementation to 85%. Additionally, all patients with BA who underwent Kasai in 2022 have had successful bile drainage post-Kasai to date.

A preliminary report published in the Journal of Pediatric Surgery suggests the potential benefit of using the tailored postoperative protocol to improve biliary drainage after Kasai.

Partnering with Community Pediatricians to Identify Jaundice Early

Beyond center expertise, two other factors are closely associated with successfully restoring bile drainage: the child’s age at diagnosis and the extent of cirrhosis at the time of surgery. Early diagnosis is challenging since jaundice, an early symptom of BA, is a relatively common occurrence in newborns, particularly premature babies without liver disease.

“Our team educates community pediatricians to refer families to us as soon as they recognize signs of jaundice,” says pediatric hepatologist Alexander Miethke, MD, medical director of liver transplantation at Cincinnati Children’s. “A fractionated bilirubin test will differentiate between physiological BA and jaundice that is potentially pathophysiological, of which BA is the most common. If a child’s direct bilirubin is greater than 1, the child will need further evaluation.”

A groundbreaking test developed at Cincinnati Children’s can expedite the diagnosis of BA, helping physicians quickly decide whether to perform a liver biopsy followed by an operative cholangiogram, the definitive test for BA. The MMP-7 test offers 98.7% diagnostic sensitivity, with results available in 48 hours or less. The test quantifies the presence of MMP-7 (matrix metalloproteinase-7), an enzyme that researchers at Cincinnati Children’s discovered in 2017 is a biomarker of BA. Other testing methods for BA can take days or even weeks.

A Strategy to Skip Liver Biopsy?

“If a child is diagnosed with biliary atresia, liver transplantation is the only pathway to a cure, but we offer the Kasai procedure unless advanced cirrhosis is present,” says Tiao, division chief for general and thoracic surgery and surgical director of liver transplantation at Cincinnati Children’s. “Having a liver transplant essentially trades one lifelong condition for another, so we want to delay the need for it as long as possible.”He adds that by the time a child is 3 or 4 months old, the Kasai procedure is unlikely to be helpful.

On average, infants who underwent a Kasai procedure at Cincinnati Children’s in 2022 were 55 days old. Miethke, Tiao, and their colleagues at the Liver Care Center hope to lower that average significantly. One strategy includes exploring whether the diagnostic performance of MMP-7 may be superior to that of a liver biopsy in predicting BA. This finding could obviate the need for most liver biopsies in infants with cholestasis, meaning that those with high MMP-7 could proceed directly to intraoperative cholangiogram with possible Kasai.