J Korean Pediatr Soc.
1979 May;22(5):384-390.
Caroli's disease
- Affiliations
-
- 1Dept. of Pediatrics, College of Medicine, Kyung Hee University, Korea.
- 2Dept. of Radiology, College of Medicine, Kyung Hee University, Seoul, Korea.
Abstract
- Congenital dilatation of the biliary tract(Caroli's disease) is uncommon condition. The condition was first described by Caroli and associates in 1958. The location, degree and type of dilatation vary. The disease entity is characterized by: (1) congenital saccular dilatation of the intrahepatic bile ducts; (2) high incidence of stone formation and cholangitis; (3) absence of hepatic cirrhosis and portal hypertensin; (4) association with renal tubular ectasia or other forms of cystic disease of kidneys, and possibly, of the pancreas. However, the pure form, as described above, has been uncommon. Since the original description of this entity in 1958, there have been approximately 30 documented cases described in the world literature which may not be entirely due to the rarity of the condition but may be a reflection of difficulties in clinical diagnosis and differentiation from other developmental abnormalities. In none of the reported cases has the correct diagnosis been established preoperatively. A case of congenital dilatation of the biliary tree in 12years old Female child was presented. The diagnosis was suggested by Computerized Tomographic Scanning and was subsequently confirmed by liver biopsy and operative cholangiogram at surgery. The review of literature on Caroli's disease was made briefly.
