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

J Pediatr Surg. 2020 May;55(5):866-872. doi: 10.1016/j.jpedsurg.2020.01.027. Epub 2020 Mar 6.

Abstract

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.

Level of evidence: IV.

Keywords: Biliary atresia; Histology findings; Liver biopsy.

MeSH terms

  • Biliary Atresia / diagnosis*
  • Biopsy, Needle
  • Cholangiography
  • Delayed Diagnosis
  • Female
  • Graft Survival
  • Humans
  • Infant
  • Infant, Newborn
  • Jaundice / etiology
  • Jaundice, Neonatal / etiology*
  • Liver / pathology*
  • Liver Transplantation
  • Male
  • Portoenterostomy, Hepatic