논문 상세보기
pp.105-110
담도 과오종과 선천성 간섬유증은 태생기에 간판 기형에 의해 생기는 섬유 낭성 질환에 속한다. 담도 과오종은 증상 을 유발하지 않고 우연히 발견되는 질환이지만, 선천성 간섬 유증은 25%에서 담관염이 발생하고 증상이 발생하면 항생 제 투여부터 수술까지 적극적 치료가 필요하다. 저자들은 복부 영상 소견으로 담도 과오종으로 진단하고 경과 관찰하던 64세 남자가 2년간 반복적인 담관염이 발생한 원인을 찾기 위해 시행한 간 생검에서 담도 과오종과 선천성 간섬유증이 병합된 것으로 진단한 증례를 경험하여 문헌고찰과 함께 보고한다.
Biliary hamartoma and congenital hepatic fibrosis belong to fibrocystic disorders originating from ductal plate malformation. A 66-year-old man who had incidentally been diagnosed with biliary hamartoma two years ago presented to us with recurrent acute cholangitis. In the first episode, he had presented with septic shock and was treated with endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy under the diagnosis of acute cholecystitis and cholangitis. However, during a two-month follow-up period, the patient experienced four episodes of acute cholangitis. Because he showed normal ERCP, and biliary hamartoma is usually asymptomatic, a liver biopsy was performed. Pathology revealed combined features of biliary hamartoma and congenital hepatic fibrosis, characterized as periportal fibrosis and intrahepatic ductular dysplasia. During follow-up for the last six months, he had experienced two episodes of acute cholangitis and was treated with antibiotics. A follow-up abdominal CT scan revealed aggravated hepatosplenomegaly compared to that of two years ago. We report a case of combined congenital hepatic fibrosis and biliary hamartoma and a literature review.
