간문장관 문합술을 시행받은 선천성 간외담관폐쇄증 환아의 예후

Abstract

Purpose : The surgical results for congenital extrahepatic biliary atresia have been improved since Kasai first described hepatic portoenterostomy(Kasai operation) in 1959. This study was conducted to evaluate the postoperative prognosis of congenital extrahepatic biliary atresia after hepatic portoenterostomy. Methods : This clinical study was done by reviewing the medical records of 30 patients who have undergone hepatic portoenterostomy at the Severance Hospital, Yonsei University College of Medicine between January 1984 to June 1994 due to congenital extrahepatic biliary atresia. Results : 1) The ages of the 30 patients ranged from 45 to 242 days and the mean age at operation was 90.8 days. Seventeen patients were male and 13 were female, and the male to female ratio was 1.3:1. 2) Of the total 30 patients, 9 cases(30%) had died, 12 cases(40%) were still being followed up, and 9 cases(30%) never returned for during follow-up. The calculated survival rate of more than 3 years was 53%. 3) Jaundice was improved in 8 patients(27%) after portoenterostomy,but the remaining 22 patients(73%) showed no improvement. The two year survival rate of the jaundice-free group was 100%, compared with 42% in the jaundice-persistent group, which showed a statistically significant difference (p<0.05). 4) Hepatic portoenterostomy was performed in 7 patients(23%) before the age of 60 days, and done in the remaining 23 patients(77%) after 60days. The two year survival rate of the former group was 54%, compared with 70% in the latter group, which did not show a statistically significant difference. 5) Portal hypertension was complicated in 17 patients(57%), but not in the remaining 13 patients(43%). The two year survival rate of the former group was 54%, compared with 100% in the latter group, which showed a statistically significant difference between the two groups(p<0.05). 6) Cholangitis was complicated in 15 patients(50%), but not in the remaining 15 patients(50%). The two year survival rate of the former group was 53%, compared with 81% in the latter group, which did not show a statistically significant difference. Conclusion : The 3 yearsurvivalrate in patients who performed hepatic portoenterostomy due to congenital extrahepatic biliary atresia was 53%. The persistent jaundice and the development of portal hypertension after surgery were important determinants of long term survival of exteahepatic biliary atresia in this study.