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.