Retrospection Study On The Clinical Diagnosis And Treatment Of 243 Cases In Human Congenital Bile Duct Cyst As Well As The Rediscovery Of Its Classification And Its Clinical Significance

Congenital Bile Duct Cyst(CBDC) is a congenital disease,the typical symptoms are abdominal pain,abdominal mass and jaundice.Abdominal pain is the major symptom,sometimes with fever and vomit.The first treatment is operation,conservation medical treatment is inefficient.Currently,the classification of CBDC is still the Todani's(1977) grouping. Because of the existing of some special types can not be included by the Todani's grouping and the Caroli's disease is hard to definition in this grouping,we present a new classification case based on Todani's grouping.Objective:This topic includes two parts.The first part is beginning with the clinic.Through a retrospective analysis of clinical data of CBDC in 243 patients, we find the disadvantage in tradition treatment and provide the proof medical evidence for the new classification and optimizing operation program.The second part is to summarize these cases to prefer a new classification plan and discuss its' clinical significance.Methods:Through a retrospective analysis of clinical data of CBDC in 243 patients from 1992 to 2008,according the imaging data,clinical classification, operation modus,therapeutic and follow-up results etc,we will analysis it and do some research.Results:1.60 male,183 female and the ratio is 1:3.05.The youngest is only 50 days, the oldest is 79 years,the mean age is 26±21.67 years.2.according to Todani's grouping,typeⅠ195,typeⅡ1,typeⅢ0,typeⅣ-A 31,typeⅣ-B 1,typeⅤ15.3.The first operation modus:①typeⅠ:external drainage operation 12 cases, internal drainage operation 42 cases,excision of bile duct cysts 127 cases。②typeⅡ:excision of bile duct cysts with T tube drainage.1 case。③typeⅣ-A:external 2 cases;internal 6 cases;excision 22 cases;typeⅣ-B: none.④typeⅤ:external-1;internal 3;partial hepatectomy 3;partial hepatectomy with internal 2,exploration of the common bile duct and calculus removed operation 1 case,ERCP and EST1。4.twice or multiple operation modus:including 82 patients,the reoperation ratio is 33.74%.①typeⅠ(64 patients):external drainage operation 7 cases,internal drainage operation 2 cases,excision of bile duct cysts 36 cases.②typeⅣ-A(10):external 1 case,Excision 8,stoma reconstruction 19.③typeⅤ:external 1;internal 4;partial hepatectomy 2;partial hepatectomy with internal 2,exploration of the common bile duct and calculus removed operation 1 case.5.complication:In short term after operation,there were abdominal pain 3 cases,bile leaking 3 cases,pancreatic juice leaking 3 cases,acute pancreatitis 2 cases,infection of wound 6 cases,bleeding in abdominal cavity 1 case,pulmonary infection 1 case,no death.Following-up 218 patients in 6 months to 9 years,is 89.7%.Including cholangitis 8 cases, cholangitis with calculus of intrahepatic duct 14 cases,above-mentioned with stenostomia and jaundice 23 cases.Cyst recur 3 cases.Cancerization 17 cases(15 died).Conclusion:1.The ultrasound,CT,ERCP,MRCP are help to the diagnosis and classification.The B ultrasound could be the early way of the diagnosis.2.Classification:TypeⅠ:Cyst in choledoch(Ⅰa:Choledochal cyst;Ⅰb: diffuse or cylindrical dilatation;Ⅰc:The multi-cyst in choledoch).TypeⅡ: Diverticulum type in the whole extrahepatic duct.TypeⅢ:Choledochcele. TypeⅣ:Mutiple cysts at the intra- and extrahepatic ducts.(Ⅳ-A:without bile stricture near porta hepatis;Ⅳ- B:with bile stricture near porta hepatic.Gourd type).TypeⅤ:Cysts at the intrahepatic ducts.(Ⅴa:Limits in the left intrahepatic ducts;Ⅴb:Limits in the right intrahepatic ducts;Ⅴc:Limits in the centrally intrahepatic ducts;Ⅴd:diffused intrahepatic ducts).3.Once the congenital bile duct cyst got the diagnosis,the surgery treat should be done as soon as possible.Only the individuation and the usable radical cure excision could get the best effect.The cyst excision, hepaticojejunostomy(Roux-en-Y) is the first choice treatment,the suitable surgery opportunity and the modus were the key of the perfect surgery progress.