Segmental Atrophy of Explanted Livers in Biliary Atresia: Pathological Data from 63 Cases of Failed Portoenterostomy

Objectives: Atrophy of the left lateral segment (LLS) is often encountered in liver transplantation (LT) for biliary atresia (BA). To clarify the meaning of the heterogeneous atrophy, we compared the pathological characteristics of the LLS with the right posterior segment (RPS) of BA livers obtained during LT. Methods: Among the 116 patients with BA who underwent LT at our hospital between 2014 and 2018, 63 patients with persistent cholestasis after the Kasai portoenterostomy (KP) were selected. Three pathologists evaluated tissues from the LLS and RPS for five pathological parameters. Positive areas in whole-slide image observed as portal inflammation, fibrosis, cholestasis, and ductular reaction, were analyzed with automated image quantitation. Moreover, we examined the relationship between the pathological score and the Pediatric End-stage Liver Disease (PELD) score. Results: The median age at LT was 7 months (range 4-26 months). Inflammation and fibrosis were significantly greater in the LLS than in the RPS (p < 0.001, for both); however, there were no differences in cholestasis, ductular reaction, and hepatocellular damage (p = 0.3, 0.3, and 0.82). The same results were obtained in automated image quantitation. Moreover, the sums of the five pathological scores in the LLS showed a significant positive correlation with the PELD score (p = 0.016, rs = 0.3). Conclusions: More severe inflammation and fibrosis without cholestasis were observed in the LLS. The segmental atrophy may not be associated with poor bile drainage, but with etiopathogenesis of BA. Moreover, the proper site for biopsy during KP could be the LLS.

DOI: 10.1097/MPG.0000000000002929