小児の胆管異常―特に膵・胆管合流異常症の観点から先天性胆道拡張症を中心に

Biliary Tract Abnormalities in Children: From the view-point of choledocho-pancreatic elongated common channel disorders

　小児の胆管異常疾患として主なものは，先天性胆道拡張症（CBD），先天性胆道閉鎖症（CBA）および種々の胆管形成不全である．このうち後2者は生後問もない乳児期に発症し，小児科ないし小児外科医により取り扱われる．一方，前者のCBDはその症例の2/3は小児期に発症するが，1/3は思春期以後の成人になって発症，一般内科や一般外科医の手で加療されるものも少なくない．

　更に本症では本特集で取り上げている，胆管・膵管合流異常が高頻度にみられ，その病因・病態との関連性が現在のトピックとして注目されている．今回，この限られた紙面で小児胆管異常のすべてを述べることは難しいので，本稿では特に胆管・膵管合流異常の観点から先天性胆道拡張症（CBD）を中心に，小児胆管異常疾患についてわれわれの臨床経験ならびに研究の一端を述べてみたい．

　As for the etiology and pathogenesis, cholangitis due to the pancreatic juice reflux and also the anatomical (mechanical) and functional stenosis of the distal common bile duct, caused by the abnormality of the choledocho-pancreatic elongated common channel formation, are considered to be most important factors.

　The type of congenital biliary dilatation (C.B.D.) is classified into two groups according to the shape of the dilated biliary tract. Type I is that the shape of dilated common bile duct is cystic, while fusiform dilatation is seen in type II.

　The so-called “infantile jaundice type”, which is seen in babies under one year of age with persistent jaundice after birth, presents similar clinical feature to congenital biliary atresia.

　Concerning the treatment of C.B.D., the necessity of primary excision is stressed. When the excision is impossible to perform, the internal biliary drainage operation without excision of the cyst should never be attempted because of so much postoperative serious complications. We, tress the necessity of early-staged excision after temporary external biliary drainage procedure in these cases.