title: Management of large hepatocellular carcinoma in adult patients with Alagille syndrome: a case report and review of literature.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Surgery, The Johns Hopkins University School of Medicine, 600 N Wolfe Street, Baltimore, MD 21287, USA.
authors.forename: Susan
authors.initials: S
authors.lastname: Tsai
authors.forename: Ahmet
authors.initials: A
authors.lastname: Gurakar
authors.forename: Robert
authors.initials: R
authors.lastname: Anders
authors.forename: Dora
authors.initials: D
authors.lastname: Lam-Himlin
authors.forename: John
authors.initials: J
authors.lastname: Boitnott
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Pawlik
mesh_terms: D000328:Adult; D016738:Alagille Syndrome / Q000453:epidemiology*; D006528:Carcinoma, Hepatocellular / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery*; D015897:Comorbidity; D005260:Female; D006498:Hepatectomy / Q000379:methods*; D006801:Humans; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D014057:Tomography, X-Ray Computed