【摘要】 目的:总结Caroli综合征患者的临床表现以及相关的辅助检查结果,探讨Caroli综合征的临床特点及超声诊断价值。方法:1例8岁小儿因发热、腹痛和肝大入院,超声检查发现肝内胆管囊状扩张,伴有中心圆点征,超声诊断为Caroli综合征。回顾性分析Caroli综合征患者的临床表现、生化检查及超声诊断,并综合相关文献报道。结果:超声检查Caroli综合征的"中心圆点征"为该病的特征性声像图表现。肝内胆管囊状扩张与先天性肝纤维化有关,临床表现为胆汁异常、门静脉高压和肾小管囊性扩张。通常的临床症状为黄疸、胆管炎,实验室检查有转氨酶、尿素氮和肌酐升高。结论:Caroli综合征临床表现多样,诊断主要依据临床表现及影像学检查。更多还原

【Abstract】 Objective: To explore the clinical manifestations,physical examination,laboratory investigations,and ultrasound examination of caroli’s syndrome Methods: An 8- year- old girl was admitted with haematemesis,abdominal pain,and hepatomegaly. Ultrasonographic examination showed dilatation of intrahepatic biliary radicals with central dot sign. A diagnosis of caroli’s syndrome was rendered. The clinical and laboratory findings of caroli’s syndrome were retrospectively review. Results:The central dot sign on ultrasound examination is indicative of caroli’s syndrome,which is the most sensitive radiographic study. The more common disorder of the bile duct dilatation is associated with congenital hepatic fibrosis. The clinical manifestations of caroli’s syndrome are related to the biliary abnormalities,portal hypertension,and renal tubular ectasia. Jaundice and cholangitis are the more common manifestations of caroli’s syndrome. The laboratory findings reveal elevated aminotransferase,blood urea nitrogenand creatinine levels. Conclusion: Abdominal ultrasound are the common modalities for evaluation of these lesions. The clinical features of aroli’s syndrome reflect the characteristics of congenital hepatic fibrosis such as portal hypertension.更多还原