CURRENT STATUS OF 21 PATIENTS WHO HAVE SURVIVED MORE THAN 20 YEARS SINCE UNDERGOING SURGERY FOR BILIARY ATRESIA

Between 1952 and 1993, 289 patients with biliary atresia underwent sur gery at the authors' institution. Twenty-two of them survived more tha n 20 years; one has since died of hepatic failure (at age 28 years). O f the 21 current survivors (age range, 20 to 39 years), 13 underwent h epatic portoenterostomy; the others had hepaticoenterostomy. None of t hese patients has undergone liver transplantation. Sixteen patients ha ve led near-normal lives. This includes three married women, one of wh om has given birth to a healthy baby boy. Of the six patients who had portal hypertension, three underwent both splenectomy and proximal spl enorenal shunting in or before 1985. None of these patients has requir ed additional treatment for portal hypertension. The quality of life o f one patient has been severely affected by an unrelated condition (Tu rner's syndrome). A 22-year-old man was diagnosed as having intrahepat ic stones 3 years ago. In another 22-year-old man, hepatic dysfunction developed after frequent episodes of cholangitis. He is now being con sidered for liver transplantation. The majority of the long-term survi vors have good quality of life. However. a few continue to suffer from complications including recurrent cholangitis. Close long-term postop erative follow-up is required for patients with biliary atresia. Copyr ight (C) 1996 by W.B. Saunders Company