EXTRAHEPATIC BILIARY ATRESIA Aetiology and physiol

EXTRAHEPATIC BILIARY ATRESIA Aetiology and physiology

Biliary atresia is present in approximately 1 in 12 /uni00A0 000 live births and a ff ects males and females equally . The aetiology is unclear; the extrahepatic bile ducts are progressively destroyed by an inflammatory process that starts around the time of birth. Intrahepatic changes also occur and eventually result in biliary cirrhosis and portal hypertension. Untreated, death from the consequences of liver failure occurs before the age of 3 years. The Japanese and Anglo-Saxon classification describes three main types (Kasai) ( Figure 71.23 ): /uni25CF type I: atresia restricted to the CBD; /uni25CF type II: atresia of the common hepatic duct: /uni25CF type IIa: a patent gallbladder and a patent CBD are present; /uni25CF type IIb: the gallbladder, cystic duct and CBD are also obliterated; /uni25CF type III: atresia of the right and left hepatic ducts and the entire extrahepatic biliary tree. Hats worn by the people of Phrygia , an ancient country in Asia Minor; they resemble the liberté cap of the French Revolution. Pablo Luis Mirizzi , 1893–1964, surgeon, Córdoba, Argentina. Morio Kasai , 1922–2008, Professor of Surgery , Tokyo University , Tokyo, Japan. - ).

RP RP Figure 71.21 Patterns of cystic duct anatomy. Note segment VI drain

age into the cystic duct and drainage of the right posterior sectorial duct (RP) into the neck of the gallbladder or an accessory duct (duct of Luschka). Figure 71.22 Magnetic r esonance cholangiopancr eatography demon

strating low insertion of the cystic duct (thick arrow) into the common bile duct (thin arrow).