先天性胆管拡張症と膵・胆管合流異常の診断

Studies of the Congenital Dilatation of the Bile Duct and Abnormal Pancreatico-cholangio Connection

　先天性胆管拡張症（以下，本症）は，Vater¹⁾によって1723年に記載され，1852年にDouglas²⁾により初めて独立疾患として報告されるなど，古くから注目されてきた疾患であるが，欧米では極めてまれな疾患とされていた．しかし，近年，本症の知見の普及と診断法の進歩につれて報告例が増加している一方，未だ本態の解明には多数の問題点を残している．また，その名称も従来は先天性総胆管嚢腫や先天性総胆管拡張症などと呼ばれてきたが，研究が進むにつれて，拡張部が総胆管に必ずしも限局しないことや，拡張部の形状が種々あることもあって，先天性胆管拡張症や先天性胆道拡張症という名称が一般的になってきた．また，Babbitt³⁾の報告以来，本症に高率に合併した膵・胆管合流異常の存在が注目を集め，本症の成因病態に深いかかわり合いを持つものと考えられるようになった．そこで，当教室における先天性胆管拡張症および膵・胆管合流異常の型分類と自験例の概要を述べると共に，その診断に関する問題点について述べる．

　Sixty cases of the congenital dilatation of the bile duct including 48cases with various abnormal pancreatico-cholangio connections were roentgenologically studied and discussed about their diagnostic procedures.

　Eight types (A-H) were classified according to the location of the dilated bile duct. The majority (87%) of the cases showed the type A and B which were accompanied with the abnormal pancreatico-cholangio connection in all cases. But, in the other cases, abnormal pancreatico-cholangio connection were not detected.

　There were such complications as pancreatitis and malignancy of the bile duct in some cases and it was pathogenetically and diagnostically important that the complication of malignancy was only observed in the cases showing type of A and B. Therefore, for the precise detection of the congenital dilatation of the bile duct, not only the demonstration of the entire biliary tract but also the existence of the abnormal pancreatico-cholangio connection should be investigated. First of all, non-invasive examinations such as ultrasound, drip infusion cholangiography and hypotonic duodenography are to be performed, but ERCP should also be done by all means to obtain the analysis of pancreatico-cholangio connection.