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Introduction Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear. Methods Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography. Results Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time. Conclusions These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension.

Purpose To investigate the feasibility of estimating the portal vein blood volume that flows into the intrahepatic volume (IHPVBV) in each cardiac cycle using non-contrast MR venography technique as a surrogate marker of portal hypertension (PH). Materials and methods Ten patients with chronic liver disease and clinical symptoms of PH (40% males, median age: 54.0, range: 44–73 years old) and ten healthy volunteers (80% males, median age: 54.0, range: 44–66 years old) were included in this study. A non-contrast Triple-Inversion-Recovery Arterial-Spin-Labeling (TIR-ASL) technique was used to quantify the IHPVBV in one and two cardiac cycles. Liver (LV) and spleen volumes (SV) were measured by manual segmentation from anatomical MR images as morphological markers of PH. All images were acquired in a 1.5 T Philips Achieva MR scanner. Results PH patients had larger SV (P = 0.02) and lower liver-to-spleen ratio (P = 0.02) compared with healthy volunteers. The median IHPVBV in healthy volunteers was 13.5 cm3 and 26.5 cm3 for one and two cardiac cycles respectively, whereas in PH patients a median volume of 3.1 cm3 and 9.0 cm3 was observed. When correcting by LV, the IHPVBV was significantly higher in healthy volunteers than PH patients for one and two cardiac cycles. The combination of morphological information (liver-to-spleen ratio) and functional information (IHPVBV/LV) can accurately identify the PH patients with a sensitivity of 90% and specificity of 100%. Conclusion Results show that the portal vein blood volume that flows into the intrahepatic volume in one and two cardiac cycles is significantly lower in PH patients than in healthy volunteers and can be quantified with non-contrast MRI techniques.