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

Susan Tsai, Ahmet Gurakar, Robert Anders, Dora Lam-Himlin, John Boitnott, Timothy M. Pawlik

Research output: Contribution to journalReview articlepeer-review

17 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)3052-3058
Number of pages7
JournalDigestive diseases and sciences
Issue number11
StatePublished - Nov 2010


  • Adult
  • Alagille syndrome
  • Hanging technique
  • Hepatocellular carcinoma
  • Surgery

ASJC Scopus subject areas

  • Physiology
  • Gastroenterology


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