Abstract
In order to recognize the present status of surgical treatment of this disease as a whole in Japan, questionnaires were forwarded to 86 main institutions. The questionnaires were filled out and returned by 67 institutions, and the data of the total 653 cases were complied for this study and analysed by computer. The results of this study and the problems in the treatment of this disease were as follows: 1. More than 60% of institutions in Japan had experienced less than 2 cases of biliary atresia in a year. The cases were widely scattered among various institutions. 2. Early diagnosis has not been achieved yet. About 20% of the patients were operated upon 80 days after their births. 3. The surgical results are not satisfactory. Only 46% of the patients are free of jaundice. 4. The results of re-operation is especially poor. Jaundice cleared in only 29% of the re-operated cases. The indication and timing for re-portoenterostomy should be settled. 5. Postoperative cholangitis is still a serious problem. Forty-three % of patients were complicated with postoperative cholangitis. 6. Recurrence of obstructive jaundice frequently occurs. About a half of jaundice free patients have developed recurrence of jaundice during their postoperative courses. 7. Indication of liver transplantation should be established as early as possible. 8. In the United States, Biliary Atresia Registry was introduced in 1978. The necessity of a registration system is also recognized in Japan.
