The Clinical Analysis And Research For Diagnosis And Treatment Of Congenital Intrahepatic Bile Duct Cysts

Objective:To explore clinical characteristics, imaging features, pathological characteristics, malignant transformation, diagnosis, treatment, prognosis and so on of congenital intrahepatic bile duct cysts.Methods:Retrospectively analyse and review clinical data of 109 congenital intrahepatic bile duct cysts patients (70 cases were type Todani-IV patients,39 cases were type Todani-V patients) who hospitalized in Chinese PLA general hospital from January 2008 to December 2015; summarize their complaints, manifestations, diagnosis, treatments, pathology results, and prognosis.Results:1. Among 109 congenital intrahepatic bile duct cysts patients 42 cases were male,67 cases were female, gender ratio was 1:1.6; with a mean age was 36.35 ± 15.45 years old (age range is:2-73 years old).2. Most patients presented with abdominal pain and fever, no statistical significance between extrahepatic bile duct cysts and clinical manifestation (P>0.05).3.10 type Todani-IV cases’CA19-9 test results were abnormal (14.29%); 6 type Todani-V cases’ CA19-9 test results were abnormal (15.38%).4. Among 109 cases,16 cases received conservative treatment only because of their poor economic situation or cancer metastasis (14.68%),6 cases underwent sclerotherapy and band ligation(5.5%),1 case underwent megaspleenectomy(0.92%),1 case underwent liver transplantation(0.92%),3 cases underwent malignant tissue biopsy(2.75%),1 case underwent percutaneous transhepatic biliary drainage(0.92%),4 cases underwent biliary stone extraction(3.67%),29 cases underwent extrahepatic bile duct cysts resection and choledochojejunostomy anastomosis(25.69%),55 cases underwent partial hepatic resection(45.87%).5.4 cases died after operation,4 cases had abdominal cavity hemorrhage,9 cases had incision liquefaction,2 cases had abdominal fluid,6 cases had bile leaks.6. Pathology results: 6 cases had malignant tissue, no statistical significance between intrahepatic and extrahepatic bile duct cysts (P>0.05).7. All patients were followed except 10 patients were misfollowed, and follow-up time between 0.5--9years;7 patients died,1 case had malignant transformation,8 cases had intermittent fever,3 case had intrahepatic biliary stone,1 case underwent sclerotherapy and band ligation because of because of biliary cirrhosis induced portal hypertension several times.Conclusion:1. Type Todani-IV patients have more organ malformation than type Todani-V.2. Operation treatment has better outcome than conservative treatment. 3. Bile duct stenosis resection can also help Type todani-IV patients relieve symptom.4. Partial hepatic resection can help decrease malignant transformation risk.5. Infection caused by stone is the most important factor contribute to malignant transformation.6. CA19-9 can help predict malignant transformation.7.All patients should be followed period because it is a premalignant disease.