Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2025 Jun 12;41(1):165.
doi: 10.1007/s00383-025-06066-y.

Cancer surveillance in biliary atresia patients with native liver survival: Standardizing monitoring and follow-up

Affiliations
Review

Cancer surveillance in biliary atresia patients with native liver survival: Standardizing monitoring and follow-up

Omid Madadi-Sanjani et al. Pediatr Surg Int. .

Abstract

Biliary atresia (BA) is a rare cholangiopathy in neonates, leading to end-stage liver failure in the first years of life, when left untreated. Kasai procedure aims to restore biliary drainage to achieve native liver survival. While liver transplantation is the necessary treatment in children with failure of the Kasai procedure, the number of native liver survivors in the long-term remains around 20-30%. Reports on malignancies in native livers of children, adolescents and adults with BA are increasing, including cases of hepatocellular carcinoma, cholangiocarcinoma, hepatoblastoma and small intestinal adenocarcinoma. In this review we summarize the reports, with focus on tumor markers, imaging technologies and reported outcomes. Furthermore, we discuss recent advances in surveillance strategies in adults with chronic liver impairment.

Keywords: Biliary atresia; Cholangiocarcinoma; Hepatoblastoma; Hepatocellular carcinoma; Native liver survival.

PubMed Disclaimer

Conflict of interest statement

Declarations. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
PRISMA flowchart. PRISMA Preferred reported items for systematic reviews and meta-analysis

References

    1. Asai A, Miethke A, Bezerra JA (2015) Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes. Nat Rev Gastroenterol Hepatol 12:342–352. 10.1038/nrgastro.2015.74 - PMC - PubMed
    1. Petersen C, Davenport M (2013) Aetiology of biliary atresia: what is actually known? Orphanet J Rare Dis 8:128. 10.1186/1750-1172-8-128 - PMC - PubMed
    1. Nio M, Wada M, Sasaki H et al (2015) Effects of age at Kasai portoenterostomy on the surgical outcome: a review of the literature. Surg Today 45:813–818. 10.1007/s00595-014-1024-z - PubMed
    1. Kasahara M, Umeshita K, Sakamoto S et al (2017) Liver transplantation for biliary atresia: a systematic review. Pediatr Surg Int 33:1289–1295. 10.1007/s00383-017-4173-5 - PubMed
    1. Rodijk LH, Schins EMW, Witvliet MJ et al (2020) Health-related quality of life in biliary atresia patients with native liver or transplantation. Eur J Pediatr Surg 30:261–272. 10.1055/s-0040-1712932 - PubMed

LinkOut - more resources