Clinical and radiographic features analysis on Caroli's disease

Abstract

Objective To analyze the clinical and radiographic features of Caroli's disease,and to explore the value of CT and MRI in the diagnosis of Caroli's disease,in order to improve diagnostic level of this disease.Methods ① We retrospectively analyzed the clinical data of 11 cases of Caroli's disease patients,which were pathologically confirmed as Caroli's disease,from January 2009 to January 2014 in the First Affiliated Hospital of Bengbu Medical College and the Second Affiliated Hospital of Anhui Medical University.② According to 357 cases reported in the literature we analyzed the clinical and radiological features.Results ① In 11 cases,the male vs female ratio was 1∶1.2,the average age of diagnosis was 46.6 years old,4 of 11 cases were type Ⅰ,the other 7 cases were type Ⅱ; the main clinical manifestations were repeated right upper quadrant pain or discomfort,fever,jaundice,portal hypertension.Overall,4 cases were combined with bile duct stones,2 cases combined with renal cyst,3 cases showed "Sac tail sign",3 cases showed "central dot sign",6 cases had "hanging sign".② Combined with the reported 368 cases of Caroli's disease patients,the male vs female ratio was 1:1.01,the average age of diagnosis was 34.7 years old,48.4％ with type Ⅰ,51.6％ with type Ⅱ; 49.1％ combined with bile duct stones,18.1％ with renal cystic disease; the occurrence rate of "Sac tail sign","central dot sign" and "hanging sign" were 44.3％,43.8％ and 86.2％ respectively.Conclusion Caroli's disease has no significant gender differences,type Ⅱ more slightly than type Ⅰ,the most common complication was duct stones,and the "Sac tail sign","central dot sign" and "hanging sign" are the significant radiographic features of Caroli's disease.These clinical and radiological features can improve the diagnosis of Caroli's disease. Key words: Caroli's disease;  Imaging diagnosis