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 article

15 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)3052-3058
Number of pages7
JournalDigestive Diseases and Sciences
Volume55
Issue number11
DOIs
StatePublished - Nov 2010
Externally publishedYes

Fingerprint

Alagille Syndrome
Hepatocellular Carcinoma
Liver
Hepatic Insufficiency
N,N-dimethyl-3,3-diphenyl-1-methylallylamine
Biopsy
Cone-Beam Computed Tomography
Hyperbilirubinemia
Cholestasis
Hepatectomy
Fibrosis
Hemorrhage
Morbidity

Keywords

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

ASJC Scopus subject areas

  • Gastroenterology
  • Physiology

Cite this

Management of large hepatocellular carcinoma in adult patients with alagille syndrome : A case report and review of literature. / Tsai, Susan; Gurakar, Ahmet; Anders, Robert; Lam-Himlin, Dora; Boitnott, John; Pawlik, Timothy M.

In: Digestive Diseases and Sciences, Vol. 55, No. 11, 11.2010, p. 3052-3058.

Research output: Contribution to journalReview article

Tsai, Susan ; Gurakar, Ahmet ; Anders, Robert ; Lam-Himlin, Dora ; Boitnott, John ; Pawlik, Timothy M. / Management of large hepatocellular carcinoma in adult patients with alagille syndrome : A case report and review of literature. In: Digestive Diseases and Sciences. 2010 ; Vol. 55, No. 11. pp. 3052-3058.
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