Intrahepatic portosystemic venous shunt: occurrence in a child patient without liver cirrhosis.

: There are multiple reports of intrahepatic portosystemic venous shunt (PSVS) cases in adult patients. We report the case of a 4-year-old child with PSVS and pulmonary arteriovenous malformation (PAVM). Abdominal sonography and computed tomography (CT) revealed the presence of PSVS. T1-weighted magnetic resonance imaging (MRI) demonstrated multiple intracranial hyperintense lesions, mainly in the globus pallidus, which suggested portosystemic encephalopathy. Tc-99m labeled microsphere study showed diffusely increased uptake in the thyroid and kidneys. The scan suggested the existence of PAVM. Pulmonary angiography was performed in order to evaluate pulmonary hypertension, and pulmonary venous pressure was slightly elevated. Contrast echocardiography suggested the presence of an intrapulmonary arteriovenous malformation with significant right-to-left shunt, as evidenced by rapid filling of the left atrium with dissolved bubbles. In this case, contrast echocardiography was helpful in diagnosing the patient's PAVM. In conclusion, we present the case of PSVS with PAVM in childhood. The incidence of PSVS is low, and data from the literature remain limited. However, further investigation is required to clarify the possible correlation between PSVS and PAVM.