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
Case Reports
. 2010 Nov;55(11):3052-8.
doi: 10.1007/s10620-009-1123-7. Epub 2010 Jan 27.

Management of large hepatocellular carcinoma in adult patients with Alagille syndrome: a case report and review of literature

Susan Tsai  1 , Ahmet Gurakar, Robert Anders, Dora Lam-Himlin, John Boitnott, Timothy M Pawlik
Affiliations
Case Reports

Management of large hepatocellular carcinoma in adult patients with Alagille syndrome: a case report and review of literature

Susan Tsai et al. Dig Dis Sci. 2010 Nov.

Abstract

Background: Alagille syndrome is a multi-system developmental disorder associated with paucity of interlobular bile ducts and cholestasis, rarely associated with hepatocellular carcinoma. Associated syndromic co-morbidities may complicate surgical management. As such, we herein review the modern management of a large hepatocellular carcinoma in an adult patient with Alagille syndrome and review the literature of adult Alagille patients with hepatocellular carcinoma.

Case presentation: A 29-year-old woman with a history of Alagille syndrome was referred with biopsy-proven 12 × 8 cm hepatocellular carcinoma replacing her right liver. Biopsy of the contralateral liver demonstrated findings consistent with Alagille syndrome, but no underlying cirrhosis. CT volumetrics demonstrated a future liver remnant of 40%. Extensive hematologic and cardiac work-up was performed pre-operatively, given the syndrome's associated bleeding dyscrasias and cardiac abnormalities. The patient underwent a margin-negative right hepatectomy using the "hanging" technique through a thoracoabdominal approach. The patient developed a transient hyperbilirubinemia but no hepatic insufficiency and did well post-operatively.

Conclusion: Since Alagille syndrome affects multiple organ systems, preoperative evaluation of cardiac, hematologic, and hepatic function should be considered. This case illustrates the peri-operative management of an Alagille patient, and highlights several key technical points that contributed to a successful resection.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Nat Genet. 1997 Jul;16(3):235-42 - PubMed
    1. Anesth Analg. 1999 Nov;89(5):1137-42 - PubMed
    1. Am J Gastroenterol. 1993 Sep;88(9):1446-9 - PubMed
    1. Transplantation. 2001 Jul 27;72(2):345-7 - PubMed
    1. N Engl J Med. 2004 Sep 30;351(14):1425-36 - PubMed

Publication types

MeSH terms

-