형제 자매간에서 발견된 선천성 담관 확장증 3예 = Three Cases of Congenital Biliary Ectasia in Three Siblings

국문 초록 (Abstract)

저자들은 형제 자매간에서 발견된 3예의 선천성 담관확장증을 경험 하였기에 문헌 고찰과 함께 보고하는 바이다.

다국어 초록 (Multilingual Abstract)

Congenital biliary ectasia is characterized by cystic dilatation of the major intrahepatic radicles (Caroli's disease) or the inter-and intralobular ducts (congenital hepatic fibrosis) or both.
Caroli's disease is characterized by communicating cavernous ectasia of the intrahepaic bile dusts. Congenital hepatic fibrosis is characterized by diffuse portal and perilobular fibrosis and multiple irrebulal buzarreshaped bile ductules in the area of fibrosis.
Both result from similar malformations of the embryonic ductal plate at different levels of the biliary trees.
Endoscopic retrograde cholangiography or percutaneouse transhepatic cholangiography seems to be the ideal method for diagnosis of the congenital biliary ectasia.
Authors experienced 3 cases of congenital biliary ectasia in 3 siblings confirmed by endoscopic retrograde cholangiography and report the cases with the review of the literature.