儿童Caroli′s病8例

Eight Cases of Caroli′s Disease in Children

目的探讨儿童Caroli′s病的临床特点及诊断方法,以提高对该病的认识。方法回顾性分析本院1998年2月-2007年12月收治的8例Caroli′s病患儿临床资料、实验室检查及影像学表现,8例均行腹部B超及CT检查。结果Caroli′s患儿男5例,女3例;平均年龄6.3岁。病史5 d～4个月。临床表现分别为肝脾大5例,呕血伴腹痛3例,发热伴尿色混浊1例。8例中5例属门静脉周围纤维化型,患儿有不同程度的肝硬化,5例肝脾大,其中3例出现门静脉高压,1例有胆囊炎。余3例为单纯型,其中1例并婴儿型多囊肾。实验室检查2例肝功能异常,1例肾功能异常。影像学检查示肝大,肝实质内散在多发不规则无回声区,与胆管相连。CT检查可见"中心点"征,为特异性征象。1例可见食管胃底静脉明显扩张。本组8例均采用保守治疗,无1例手术治疗。结论Caroli′s病临床表现多样,对于腹痛及肝大的患儿尤应注意。B超及CT检查对诊断Caroli′s病具有重要价值,有助于早期明确诊断。可采取保守或手术治疗。

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 Dee. 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 intrabepatic 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.