Portal-systemic encephalopathy due to a congenital extrahepatic portosystemic shunt: Three cases and literature review

Extrahepatic portal-systemic encephalopathy due to congenital extrahepatic portosystemic shunt has so far been rarely reported in the literature. We h erein report 3 such cases without liver cirrhosis or portal hypertension wh ich were presented with the chief complaint being disturbance of consciousn ess and abnormal behavior. In all cases the brain computed tomography scan revealed no pathological findings, while electroencephalogram showed a diff use slow activity with triphasic waves. The laboratory data revealed a high serum ammonia level. Percutaneous transhepatic portgraphy demonstrated por tosystemic shunts. After these shunts were surgically occluded, the serum a mmonia level reached a normal range and encephalopathy disappeared. A liver biopsy also revealed neither fibrosis nor cirrhosis in any of the cases. T he 23 previously reported cases are also discussed.