The evolution of early liver biopsy findings in babies with jaundice may delay the diagnosis and treatment of biliary atresia

Background: The early diagnosis of biliary atresia (BA) is associated with a better outcome after portoenterostomy. However, very early liver biopsy findings may appear atypical for BA and delay diagnosis. Repeat biopsy histology may change rapidly to show more typical features. Methods: Between 1997 and 2018, 6 babies with jaundice had more than one biopsy to establish diagnosis. Clinical and histologic data were collected. chi-Square was used for analysis (p < 0.05 significant). Results: Five patients had two biopsies, and one had three. Median age at first, second, and third biopsy was 40 (13–57), 68.5 (35–78), and 133 days, respectively. Biopsy readings showed no portal edema initially (0/6), but in all 6 on repeat biopsy (p = 0.001). Bile duct proliferation was seen in 6/6 final biopsies, but in only 1/6 initially (p = 0.003). All patients underwent a portoenterostomy (median age 75 days (43–113)). Median delay between initial biopsy and Kasai was 29 days (14–67). Transplant free survival (n = 5 patients) ranged from 184 to 716 days (median 309 days). One patient died before being transplanted. Conclusion: Early biopsies may not display characteristic findings of BA, but these can appear quickly on subsequent evaluation. The interval needed to repeat a biopsy may have an adverse effect on bile drainage.