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Erschienen in:
01.11.2010 | Review
Management of Large Hepatocellular Carcinoma in Adult Patients with Alagille Syndrome: A Case Report and Review of Literature
Erschienen in: Digestive Diseases and Sciences | Ausgabe 11/2010
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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.