Unusual hepatic‐portal‐systemic shunting demonstrated by Doppler sonography in children with congenital hepatic vein ostial occlusion

PURPOSE: This report describes unusual changes in the hepatic vasculature in 3 children presenting with upper gastrointestinal hemorrhage. METHODS: The study included 3 children (ages 5-8 years) who presented with hematemesis. All had mild hepatosplenomegaly and normal liver function. Esophageal varices were demonstrated in all on upper endoscopy. Color and spectral Doppler sonography was performed to assess the hepatic vasculature, including the hepatic veins (HVs), portal vein (PV), hepatic artery (HA), and inferior vena cava (IVC). RESULTS: The HVs were all patent but with ostial occlusion at the point of their communication with the IVC. Complete flow reversal was shown inside the HVs, with blood draining into collateral vessels at the liver surface and paraumbilical vein. In one patient, the paraumbilical vein could be traced to its communication with the right external iliac vein. In all children, the direction of flow in the PV, HA, and IVC was normal. After endoscopic sclerotherapy, all children were shown to be in good general condition and to have normal liver function for a follow-up period of 15-36 months. CONCLUSIONS: Ostial occlusion of the HV is a rare cause of hepatic outflow obstruction in children. Doppler sonography is a valuable, noninvasive imaging technique for evaluation of the hepatic vasculature and the accompanying shunting pathways in such cases.