Doppler Sonographic Findings in Children With Idiopathic Portal Vein Cavernous Deformity and Variceal Hemorrhage

OBJECTIVE: To describe the flow patterns in the portal vascular territory in children with portal vein cavernous deformity. METHODS: The study included 12 children (age 4-10 years) with hematemesis, melena, or both in whom B-mode gray scale sonography revealed small anechoic spaces replacing the site of the portal vein. The portal vein cavernous deformity was present either alone (in 8 patients) or with congenital hepatic fibrosis (in 4). Doppler sonography (color and spectral) was performed to assess the flow in the portal vascular territory, splenic vein, intrasplenic veins, and abdominal collaterals. RESULTS: Doppler sonography confirmed the venous flow waveform in the cavernous portal vein in all children with normal flow direction in the few intrahepatic portal vein branches and also in the intrahepatic veins. Splenomegaly was present in all. The intrasplenic veins were dilated in all but had normal flow direction except in 2 with spontaneous trans-splenic shunts. Gallbladder varices were shown in 4 patients, and perisplenic collaterals were shown in 3. CONCLUSIONS: Doppler sonography is a valuable noninvasive imaging technique for assessment of the portal hemodynamic profile in patients with portal vein cavernous deformity, which can affect subsequent treatment decision making. Trans-splenic shunts are uncommon, but this Doppler sonographic report documents such shunts in children with portal hypertension.