Hepatic encephalopathy and hepatopulmonary syndrome are classically seen in patients with cirrhosis and hepatic synthetic dysfunction. However, they can be caused by vascular anomalies that shunt blood away from the liver. In these settings, the vascular anomalies most often require surgical correction or liver transplantation. However, we report a case of a 43-year-old male with hepatic encephalopathy and hepatopulmonary syndrome from a type 2 Abernethy malformation that was corrected with an inferior vena cava (IVC) stent.
CASE REPORT
A 43-year-old male presented to an outside hospital in 2005 with altered mental status. His serum aminotransferases were normal, and he had no history or physical evidence of underlying liver disease. His urine toxicology screen was negative. He was evaluated by the neurology department and found to have normal magnetic resonance imaging/magnetic resonance angiography of the brain and a normal lumbar puncture. His electroencephalogram showed "systemic slowing," but no seizure activity was found. His ammonia level was incidentally found to be elevated at 140 mol/L, and he was empirically treated with lactulose with resolution of his symptoms. Magnetic resonance cholangiopancreatography was normal. Liver biopsy was performed and showed mild nonspecific portal lymphocytic infiltrate (grade 1) without fibrosis. The cause of his mental status changes remained elusive.