Abstract
A case of mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) in a female child who developed bacterial translocation from a congenital portal vein hypoplasia is reported. The patient was diagnosed as having portal hypertension after examinations and laboratory results showing splenomegaly and thrombocytopenia at the age of 1 year. The patient required three endoscopic variceal ligation (EVL) surgeries before the age of 9 due to development of multiple esophageal varices. After the second and third EVL procedures, she developed septicemia, possibly due to bacterial translocation associated with the administration of general anesthesia. The day after the third EVL, the patient presented with high fever and neurological disturbances (altered consciousness). Magnetic resonance imaging detected abnormal intensities in the corpus callosum ampulla and cerebral white matter, which suggested a diagnosis of MERS type 2. Considering this clinical course, the possible association between bacterial translocation and MERS in a patient with congenital portal vein hypoplasia and portal hypertension is discussed.