Treatment of extrahepatic bile duct atresia: results and long-term prognosis after hepatic portoenterostomy

Abstract

Since 1972, 64 infants suffering from extrahepatic bile duct atresia have been examined in our Department. Between 1975 and 1984 conventional or extended hepatic-portoenterostomy was performed on 41 patients. These infants were classified into two groups on the basis of age at the time of surgery in relation to the duration of cholestasis. Of 12 patients operated upon 5 or more years ago, 8 (66%) are still alive with favorable bile flow and 4 (34%) have died from hepatic failure caused by biliary cirrhosis. Of 22 infants above 8 weeks of age who have been treated so far, 7 (31%) are alive with good bile flow and 15 (69%) have died within the past 3 years. In contrast, 12 (63%) of 19 infants operated upon before 8 weeks of age are healthy with good bile flow and only 7 (37%) have died from cirrhosis. The course of the disease was analyzed with regard to the following findings: (1) age at surgery with respect to the duration of cholestasis; (2) extent of liver fibrosis at surgery; (3) cross-section of ductular structures in the porta hepatis; (4) numerical and morphological changes in the interlobular bile ducts; (5) extent of dissection of the porta hepatis; (6) postoperative hepatoportitis. Principles determining the long-term follow-up of this dynamic obliterative process can be deduced from these investigations. Accordingly, a favorable prognosis can be expected if the following conditions are fulfilled: no cholestasis for more than 6 weeks; low-grade fibrosis at the time of surgery, with a mean diameter of the ductular structures in the porta hepatis of more than 450 μm; no numerical or morphological alterations in the interlobular bile ducts; no postoperative “hepatoportitis”.