The clinical analysis of infantile biliary hypoplasia diagnosed by laparoscopy cholangiography

Objective To analyze the characteristics of cholangiography and pathology of biliary hypoplasia diagnosed by laparoscopic cholangiography.Methods Medical records of 8 patients with biliary hypoplasia diagnosed by laparoscopic cholangiography during the last 4 years were reviewed.The clinical features,cholangiography,pathology and prognosis were analyzed.Results Two specimens from elder cases showed enlargement of liver with nodosity.The specimens from the other 6 cases showed almost normal liver.The gallbladders of 7 cases were poorly developed and smaller than normal.Cholangiography showed that in all cases the hepatic ducts and choledochus were extremely thin,the intrahepatic bile ducts were unwell developed.There was a little bile drainage from gallbladder fistulation,and in some cases the bile drainage increased after orally intaking ursodeoxycholic acid.Intrahepatic cholestasis and the paucity of interlobular bile ducts were pathologically detected in all cases.The specimens of 2 elder cases showed pseudolobule,the rest of cases showed slight fibrosis.The patients were followed up from 6 months to 4 years.Two patients died at the age of 6-8 months,the other 6 patients survived with persistent cholestasis,retarded growth,abdominal distension.Conclusions Biliary hypoplasia is a type of disease that show common features in clinical features,cholangiography and pathology.But its treatment and prognosis are different from other obstructive jaundice diseases of infants.It is important to make an early and definite diagnosis,so that Kasai procedure would not be misused.We propose that the diagnostic criteria for biliary hypoplasia should include:Obstructive jaundice appears soon after birth and continue aggravating; Cholangiography shows the hepatic ducts and choledochus are extremely thin (≤2 mm),the intrahepatic bile ducts are not well developed; Pathology shows intrahepatic cholestasis and paucity of interlobular bile ducts; A little bile drainage can be seen through gallbladder fistulation. Key words: Biliary hypoplasia;  Laparoscopy;  Cholangiography