Cholangitis is associated with liver fibrosis in choledochal malformations

Most patients with choledochal malformations carry favourable prognosis after surgery and a minority may experience liver fibrosis. This study utilized liver transient elastography to monitor the progression of liver fibrosis and evaluated potential risk factors.

Seventy patients with choledochal malformations received surgery between 2000 and 2022. Operative details, biochemical profiles, episodes of cholangitis, and contrast studies for biliary reflux were evaluated. The degree of liver fibrosis was measured by a single experienced operator using FibroScan for all patients, and 6.7 kPa (kPa) was taken as the upper limit of normal.

At least one set of liver fibrosis score by FibroScan was obtained from 42 patients. Median fibrosis score was 4.26 kPa (IQR 3.5-5.3 kPa). Four patients (9.5 %) had measurements compatible with fibrosis (6.9-10.8 kPa) and post-operative episodes of cholangitis (OR 18, p = 0.004) were found to be associated. The liver was found to be more stiff in patients with reflux into biliary tree upon contrast studies (3.87 vs 4.89, p = 0.049) and those with post-operative episodes of cholangitis (4.38 vs 7.47, p < 0.001). Comparable liver stiffness scores were achieved in hepaticoduodenostomy and hepaticojejunostomy groups (3.94 vs 4.93, p = 0.129). No significant correlation was found with the age of operation (p = 0.278). Serial liver stiffness measurements were performed for seventeen patients, none has shown progression to liver fibrosis.

Cholangitis imposes a higher risk of fibrosis in choledochal malformations as evidenced by the detrimental effect on liver stiffness. Effort should be made to prevent cholangitis. Serial testing of transient liver elastography can act as a non-invasive tool to closely monitor any progression to liver fibrosis.