M. Nio et al., THE OUTCOME OF SURGERY FOR BILIARY ATRESIA AND THE CURRENT STATUS OF LONG-TERM SURVIVORS, Tohoku Journal of Experimental Medicine, 181(1), 1997, pp. 235-244
Between 1953 and 1995, 300 patients with biliary atresia underwent sur
gery at Tohoku University Hospital. The 10-year survival of patients w
ho were operated on in or before 1965 was 9%. But the survival rate we
nt up ta 61% in patients operated on between 1976 and 1985. Eighty-fiv
e patients including 2 who developed liver failure after Kasai operati
on and underwent liver transplantation have survived more than 10 year
s. Eleven of them (13%) have recurrent or persistent jaundice. Of the
30 patients who have survived more than 20 years (10 males and 20 fema
les, age range; 20 to 41 years), 20 underwent hepatic portoenterostomy
, 8 underwent hepaticoenterostomy and the remaining 2 underwent hepati
c portocholecystostomy. None of these patients has undergone liver tra
nsplantation Twenty-two patients have led near-normal lives. The remai
ning 8 patients have experienced some troubles due to cholangitis, por
tal hypertension intrahepatic gallstones and so on. TWO Of them are co
nsidered as candidates for liver transplantation. While the majority o
f long-term survivors of biliary atresia have good quality of life, cl
ose long-term follow-up is essential even in patients with biliary atr
esia aged 20 years or more.