Congenital hepatic fibrosis (CHF) is an autosomal recessive disease, presenting principally in children or young adults with portal hypertension, and infrequently associated with cholangitis. It is associated with renal malformation and Caroli's disease. The diagnosis of CHF is usually confirmed by its typical histological features. Cholangitis is a severe and frequently fatal complication. We report a 22-year-old man with congenital hepatic fibrosis who showed the cholangitis without radiological features of cystic dilatation or stone of intrahepatic ducts.
Acute Disease ; Adult ; Cholangitis/*complications/diagnosis ; Humans ; Liver Cirrhosis/*complications/*congenital/diagnosis ; Male

Acute cholangitis usually develops in congenital hepatic fibrosis (CHF), accompanied by cystic dilated bile ducts. However, it can also develop in simple CHF and may lead to critical course. A 30-year old man presented with recurrent acute cholangitis without bile duct dilatation. He visited the hospital for febrile sense and abdominal pain in the right upper quadrant. He had been admitted several times for hepatosplenomegaly and cholangitis since childhood and received a liver biopsy 15 years ago. Abdominal computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP) revealed hepatosplenomegaly and a mildly dilated bile duct without stones or biliary cysts. His condition improved after conservative treatment. However, during a two-month follow up period, the patient experienced three episodes of acute cholangitis. A liver biopsy was performed and showed periportal fibrosis and intrahepatic ductular dysplasia, characteristics of congenital hepatic fibrosis. The periportal fibrosis and the infiltration of inflammatory cells were aggravated compared to 15 years ago. There was no evidence of hepatic cirrhosis. He was diagnosed with congenital hepatic fibrosis with recurrent acute cholangitis without intrahepatic duct dilatation, and conservatively treated with antibiotics.
Acute Disease ; Adult ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis/complications/*diagnosis ; Humans ; Liver Cirrhosis/complications/*congenital/pathology ; Male ; Recurrence ; Tomography, X-Ray Computed

Adult ; Bile Ducts, Intrahepatic ; diagnostic imaging ; pathology ; Biopsy, Needle ; Caroli Disease ; complications ; diagnosis ; pathology ; Child ; Diagnosis, Differential ; Female ; Humans ; Liver ; diagnostic imaging ; pathology ; Liver Cirrhosis ; complications ; congenital ; diagnosis ; pathology ; Male ; Spleen ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed