Eight Cases of Caroli′s Disease in Children / 实用儿科临床杂志

ABSTRACT

Objective To explore the clinical characteristics and treatment of Caroli′s disease in children.Methods The clinical data,laboratory examination and radiological feature of 8 children with Caroli′s disease between Feb.1998 and Dec.2007 were analyzed retrospectively.All children underwent CT and abdominal ultrasonogram.Results Five cases of the 8 children were male and 3 cases were female.The mean age was 6.3 years old.The cases′ history were from 5 days to 4 months.The clinical symptoms showed that 3 cases had hematemesis,5 cases had hepatosplenomegaly,and 1 case had fever and turbid urine.Of the total 8 cases,5 cases were hepatic fibrosis and liver cirrhosis and hepatosplenomegaly,3 cases were portal hypertension,and 1 case had cholangitis.The other 3 cases were simple types.One case had infantile polycystic kidney disease.Laboratory analysis revealed 2 cases had dysfunction of liver and 1 dysfunction of renal.The imaging characteristics showed multiplied irregular dilatation of the intrahepatic bile ducts in enlarged liver,with central dot sign on CT scan.One case presented enlarged gastroesophageal vein.The 8 cases undertook conservative treatment,with no surgery.Conclusions The symptoms of Caroli′s disease are highly variable.Caroli′s disease should be focused especially on children with abdominal pain and hepatomegaly.CT is important for diagnosis of Caroli′s disease at earlier stage.The disease can be conservatively treated,and(or) surgically operated.