Diagnosis Of Biliary Hypoplasia By Laparoscopic Cholangiography And Hepatic Histopathology

Objectives: To discuss the significance of diagnosis of biliary hypoplasia by clinical feature,laparoscopic cholangiography and hepatic histopathology,and conclude the diagnostic criteria.Materials and Methods: A retrospective review was carried out between April 2009 and January 2016.77 consecutive patients with obstructive jaundice who suspected biliary atresia were underwent laparoscopic cholecystocholangiography and liver biopsy.These patients were admitted into Neonatal Surgical Intensive Care Unit and General Surgery Dpartment of Ba Yi Children’s Hospital affiliated to Clinical Medical College in Beijing Military General Hospital of People’s Liberation Army.Among them,11 cases were diagnosed in biliary hypoplasia(BH),50 cases were diagnosed in biliary atresia(BA),16 cases were diagnosed in cholestasis.Variance analysis the biochemical results of these groups preoperative within 7 days,comparison of the three groups’ intraoperative cholangiography,liver histopathology results and the long time survival with native liver.Conclude the characteristics of biliary hypoplasia disease.Results: 1.Biochemical results :(1)Alanine aminotransferase(ALT)preoperative within 7 days: BH group ALT range from 80 U/L to 380 U/L,with an average of 154.75 U/L;BA group ALT range from 44 U/L to 630 U/L,with an average of 146.3 U/L;Cholestasis group ALT range from 56 U/L to 1238 U/L,with an average of 225.8 U/L.(2)Total Bilirubin and direct biirubin preoperative within 7 days:1)BH group total bilirubin range from 121.5μmol/L to 214.7μmol/L,with an average of 153.1μmol/L;direct bilirubin range from 78.2μmol/L to 155.1μmol/L,with an average of 111.8μmol/L.2)Cholestasis group total bilirubin range from 120.2μmol/L to 286.8μmol/L,with an average of 189.0μmol/L;direct bilirubin range from 68.8μmol/L to 152.6μmol/L,with an average of 103.1μmol/L.3)BA group total bilirubin range from 127.1μmol/L to 310.2μmol/L,with an average of 201.6μmol/L;direct bilirubin range from 75.7μmol/L to 236.8μmol/L,an average of 128.2μmol/L。The BH group,BA group and Cholestasis group Total Bilirubin(P 0,060)and direct biirubin(0.418)preoperative within 7 days.There was no statistically significant difference.2.Cholangiography:(1)BH group: The cholangiography shows that the gallbladder,right hepatic duct,left hepatic duct and common bile duct were visible but extremely thin.intraheptic bile ducts were also visible,but there was no structure of ‘biliary tree’.duodenum was visible.(2)BA group: Atrophic gallbladder.1)Ⅰtype BA: The cholangiography shows that the gallbladder,cystic duct,right hepatic duct,left hepatic duct and intrahepatic bile ducts were visible,but commom bile duct and duodenum were disappered.2)Ⅱtype BA: The cholangiography shows that the gallbladder,common bile duct and duodenum were visible,but right hepatic duct,left hepatic duct and intrahepatic bile ducts were disappered.3)Ⅲ type BA: The cholangiography shows that only the gall bladder was visible,others were disappered.(3)Cholestasis group: 16 cases had good sized gallbladders,both intrahepatic and extrahepatic bile ducts were visible.3.Hepatic histopathology results: All patients’ liver had intrahepatic cholestasis,but there has significant difference in the three groups.(1)BH group: The liver histopathology shows that portal and interlobular bile ducts reduce or loss.(2)BA group: The liver histopathology shows that fibrous tissue hyperplasia at portal and around the interlobular,proliferating bile ductules.(3)Cholestasis group: The liver histopathology shows that the portal area had chronic inflammatory cell infiltration,with or without interlobular bile ducts hyperplasia.4.Follow-up:(1)BH group: 11 cases were followed up for 6 months to 4 years postoperative,2 cases died at the age of 6 to 8 months,1 case died at the age of 7 years old during waiting for liver transplantation.8 cases still survival but accompanied by progressive jaundice,growth retardation,abdominal distension ventosity,and so on.Among the 8cases,1 cases was underwent gallbladder-colonic anastomosis,1case was underwent liver transplantation after 2 years postoperative.(2)BA group: 50 cases were followed up for 1 month to 6 years postoperative,4 cases death due to the surgery complications,7 cases died at the age of 8 months to 12 months,these patients was only underwent laparoscopic cholecystocholangiography and liver biopsy,but refuse to accepted kasai operation.38 cases were underwent laparoscopic cholecystocholangiography,liver biopsy and kasai operation in one surgery,35 cases the jaundice disappered soon,3 cases still had jaundice and accompanied by liver cirrhosis.1 cases was only underwent laparoscopic cholecystocholangiography and liver biopsy,and then accepted liver transplantation.(3)Cholestasis group: 16 cases were followed up for 3 month to 4 years postoperative,14 cases have no jaundice,2 cases had liver cirrhosis would need to accept liver transplantation.Conclusions:Biliary hypoplasia diseases in children are similar in clinical feature,cholangiography and pathology.The treatment and prognosis are different from other obstructive jaundice diseases.It is significant to make definite diagnosis as early as possible.Diagnostic criteria: 1.Clinical feature: obstructive jaundice occurs in the early stage after birth and progressively;2.Cholangiography: extrahepatic bile ducts extremely thin,the diameter less than or equal to 2 mm,duodenum was visible,intraheptic bile ducts were visible but not clearly;3.Hepatic histopathology: intrahepatic cholestasis,portal and interlobular bile ducts reduce or loss;4.There were little bile drainaged after gallbladder fistulation.