Long-term survivors in biliary atresia--findings for a 20-year survival group

Recently, there has been remarkable increase in the survival rate in cases of biliary atresia (BA). However, long-term survivors are as yet a small population. Of the total of 152 patients undergoing surgery for BA during the period from 1969 to 1995 in our institution, 39 of them were operated on more than 20 years ago with follow up for all but one of these, who can no longer be accounted for. Six are still alive, 1 male, and 5 females, two cases being of type I BA, and the other 4 of type III and 1 is unaccounted for. The prognosis of these individuals was clinically evaluated. At the present time, none of them is receiving hospital care, but 3 have experienced sequelae. Two patients required partial splenic embolization (PSE), endoscopic infusion sclerotherapy (EIS), and/or gastroesophageal decongestion and splenectomy (Hassab's operation) (Hassab 1967) for hypersplenism and/or portal hypertension. The other has needed hospital care for recurrent cholangitis. Laboratory investigations revealed a serum total bilirubin (TB) of less than 1.0 mg/100 ml in 3 of the 5 patients for which samples could be obtained, between 1.0 and 2.0 mg/100 ml in 1, and in excess of this in the remaining case. The 1-alanine 2 oxoglutarate aminotransferase (ALT) level was within the normal range in only 1, and was mildly to moderately elevated in 4. The white blood cell count (WBC) was less than 3,000/microliter and the platelet count was less than 10 x 10(4)/microliter in 1, and within the normal ranges in the other 4 patients. The results thus indicate that occult and progressive liver damage may occur in long-term survivors of BA.