Sistema Gastrointestinal e Digestivo

Abstrato

Case Report: A Rare and Unusual Cause of Acute Liver Failure

Nwe Ni Than, Frederick Chen, Simon Olliff, Dhiraj Tripathi1

Budd-Chiari syndrome (BCS) is a rare condition caused by hepatic outflow obstruction. Typical clinical features include right upper quadrant pain, ascites and hepatosplenomegaly. We report a case of a 26 year old female who presented with jaundice, worsening abdominal swelling and abdominal pain. She subsequently developed acute liver failure and spontaneous bacterial peritonitis (SBP) with significant renal impairment requiring organ support. Computed tomography (CT) scan showed congested liver and occlusion of three hepatic veins consistent with Budd- Chiari Syndrome. Transjugular intra hepatic portosystemic stent/shunt (TIPSS) was inserted which resulted in complete resolution liver and renal function. She was subsequently found to be positive for the JAK-2 V617F mutation suggesting an underlying myeloproliferative disorder. This is a rare case in which a patient developed spontaneous bacterial peritonitis (SBP) in the context of BCS. The patient had excellent outcome from TIPSS procedure, which rescued her from undergoing liver transplantation.