卡洛萊氏病

Caroli's Disease

王家槐(Jia-Hwia Wang)

《中華放射線醫學雜誌》 3卷4期 (1978/12) Pp. 297-302

摘要

卡洛萊氏病是一種罕見的疾病，通常除了肝內胆管囊狀擴大外，常合併肝內服結石及腎囊性病變，有時可合併先天性肝纖維化或胰囊性病變。榮民總醫院近年發現一個病例，且合併肝內胆結石及兩側多囊腎。靜脈滴注胆管X光攝影加上斷層攝影對於無黃疸或輕微黃疸病人，在診斷上有幫助，經皮穿肝胆管X光攝影似乎是術前得到正確診斷的檢查方法。將來電腦斷層X光攝影也許會發展成一種對本疾病更安全、更簡便、更正確的診斷方法。保持高度的注意乃是診斷本疾病的第一要件。

並列摘要

Caroli's disease is a rare congenital disease which was first descibed by Caroli et al in 1958 and is characterized by (1) segmental saccular dilatation of the intrahepatic bile ducts; (2) a marked predisposition to biliary calculous disease and cholangitis; (3) sometimes associated with renal cystic diseases or congenital hepatic fibrosis, and occasionally associated with pancreatic cystic disease. A patient with Caroli's disease was diagnosed in Taiwan Veterans General Hospital in 1976 who was presented with renal problems clinically. Its clinical data, laboratory, and roentgenologic findings were presented. In general, patients seek medical advice because of (1) repeated episode of upper abdominal pain with occasional fever and mild jaundice from biliary stone formation, cholangitis and occasionally liver abscess due to ectasia of the intrahepatic bile ducts; (2) symptoms and signs from portal hypertention if hepatic fibrosis is associated and is the predominant lesion, and hematemesis is the most significant symptom; (3) symptoms and signs related to renal calculi and urinary infection, even renal failure, if cystic diseases of kidney are associated. The laboratory findings are non-specific. Infusion intravenous cholangiography with tomography is helpful, if patient is in the state of no jaundice or mild jaundice. Ultrasonic, selective angiographic and liver scan examinations may be helpful in diagnosis. Percutaneous transhepatic cholangiography is the decisive examination for diagnosis. Computerized tomographic scan may be a safe, convenient and useful diagnostic tool in the near future. Roux-en-Y intrahepatic cystojejunostomy is a better choice of surgical treatment. Maintaining high index of suspicion is essential to gain diagnosis.