Late cholangitis after kasai procedure detected with magnetic resonance cholangiopancreaticography: a case report.

Ascending cholangitis occurs in about 30 to 50% of patients after Kasai hepatoportoenterostomy for extrahepatic biliary atresia (1,2). It generally occurs within the first few years after surgery (3). Late cholangitis seems to be extremely rare (3,4). Patients with cholangitis usually have high fever with chills and jaundice, reflecting worsening of liver functions in about half of the patients (5). Blood tests may show leucocytosis. Diagnosis is best made by liver biopsy, showing histologic features of cholangitis. In addition, cultures of liver tissue and blood may be positive. Ultrasound and computer tomography (CT) scan usually reveal distended intrahepatic bile ducts. Postoperative ascending cholangitis is an important prognostic factor and is associated with liver cirrhosis, portal hypertension, and premature death. Because of the risk of permanent liver damage, aggressive therapy is fully justified. We report the case of a girl who underwent a Kasai procedure at the age of 8 weeks and had a fully uneventful follow-up with normal liver functions in the following years. She was considered cured until the age of 8 years when she had a first episode of fever, tentatively diagnosed as cholangitis. We were only able to actually diagnose cholangitis when magnetic resonance cholangiopancreaticography (MRCP) showed abnormalities consistent with sclerosing cholangitis during the fourth episode, which occurred when the patient was 17 years old.