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Аn early consideration in pediatric patients with extrahepatic portal vein obstruction (EHPVO) is to be given for mesoportal shunt. A careful investigation is required to prove intrahepatic portal system patency. Conventional noninvasive imaging is not adequately reliable in assessing patency of the intrahepatic portal venous system. Retrograde portography in children brings additional invasive procedure. Antegrade, direct intraoperative digital subtraction angiography for mesoportal shunt feasibility assessment is poorly presented in literature. Aim - to improve the rate of success of mesoportal shunting in children with EHPVO by analyzing our own experience in intraoperative digital subtraction angiography of the intrahepatic branches of the portal vein for the final assessment of the mesoportal shunting feasibility. Materials and methods. 7 pediatric patients with EHPVO were selected for the study. Angiographies and surgeries in selected patients were performed within single center in a period from May 2022 to July 2023. Mean follow up of successful mesoportal shunting was 12.38±1.46 months. Results. All patients were males. Men age at surgery was 8.71±1.72 years. 5 (71.4%) patients manifested bleeding episodes as the first sign of portal hypertension. In all patients ultrasound revealed splenomegaly and suspected portal hypertension for reduced volumetric portal flow. All 7 patients had high grade. Liver function was normal in all patients, and in none thrombophilia was confirmed. CT scans suspected patent left intrahepatic branch (Rex-zone). Digital subtraction angiography approved mesoportal shunt feasibility in 4 (57.1%) patients. The follow up period was 13.5±2.9 months. Conclusions. Digital subtraction angiography of intrahepatic portal vein is effective visualization method to achieve with radiologic evidence of intrahepatic portal branches patency and make the decision on mesoportal shunting when favorable anatomy proved. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Introduction: Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition.Material and Methods: A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given.Results: According to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children, IT showed hepatic portal circulation. In total shunts in which radiological examination was performed, invasive radiological techniques were able to demonstrate intrahepatic portal vein. In other patients, it was not investigated as they are asymptomatic. A child presented multiorgan failure with fulminant hepatic failure at birth. The shunt was radiologically closed and clinical impairment reversed rapidly. He is now asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven children are asymptomatic at this time and are monitored periodically. Seven children had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis, gastrointestinal bleeding). Of these, in five we performed balloon test occlusion, tolerated in all patients, followed by radiological closure. In our experience, the advancement of interventional radiology techniques avoided surgery to close the shunt.Conclusions: Morphologically, the CPSS is extremely heterogeneous, with multiple possible connections established. CPSS has multiple clinical presentations, from asymptomatic patients to acute liver failure. The therapeutic approach should be individualized and therefore held in overspecialized centers. [ABSTRACT FROM AUTHOR]

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 cm 3 and 26.5 cm 3 for one and two cardiac cycles respectively, whereas in PH patients a median volume of 3.1 cm 3 and 9.0 cm 3 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. [ABSTRACT FROM AUTHOR]

Abstract: 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. [Copyright &y& Elsevier]