Biliary atresia and pregnancy: Puberty may be an important point for predicting the outcome

Purpose: The aim of this study was to study the late clinical features of biliary atresia in relation to pregnancy. Materials and Methods: Of the 175 patients with biliary atresia operated on at our department, the data for 49 patients (18 males, 31 females) older than 15 years were reviewed and analyzed. Results: Six patients delivered 7 newborns (5 with their native liver and 1 with a transplanted liver). Although one of the patients delivered twice after transplantation, another underwent transplantation after delivery. None of the 7 newborns showed any congenital anomalies; however, 6 were small-for-date newborns. Their birth weight tended to be inversely related to the mothers' age at delivery. An additional 13 patients required liver transplantation, of whom 1 died after transplantation and 1 died before transplantation. The remaining 30 patients showed uneventful courses without transplantation. The age at Kasai operation was significantly higher in the 5 patients who delivered babies with their native liver compared with that in these 30 patients (88.0 vs 68.6 days). A significant difference between these 5 patients and those who died or required transplantation before pregnancy was recognized even at the age of puberty in the alanine aminotransferase (36.5 ± 26.5 vs 126.4 ± 56.1 IU/L), aspartate aminotransferase (36.2 ± 26.5 vs 114.2 ± 48.9 IU/L), and choline esterase (249.7 ± 64.8 vs 163.5 ± 40.2 IU/L). Conclusions: The age at Kasai operation may not necessarily impact the late clinical course; however, liver function at puberty may predict the subsequent clinical course, including the safety of pregnancy. The management strategies should be revised after puberty.