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Portal vein aneurysm (PVA) is rarely encountered, and published papers describing this etiology in adults and children typically include only case reports or small case series. We present a clinical case of PVA in a child associated with severe complications, including diffuse thrombosis of the portal venous system. A 10-year-old boy presented with abdominal pain and vomiting, resulting in an initial diagnosis of pancreatic head tumor based on suspicious images on abdominal grayscale ultrasound. Contrast-enhanced computed tomography confirmed a diagnosis of occlusive PVA thrombosis (36 × 37 × 95 mm). Lacking drastic symptoms, the patient was treated with conservative anticoagulant therapy. On follow-up, the thrombosis appeared to shrink gradually and disappeared at 6 months based on Doppler ultrasound imaging. The PVA was reduced in size, and hepatopetal flow was restored. Surgeons and radiologists should be aware of this rare entity to ensure that a precise diagnosis can be established and to provide suitable treatment.

Portal vein thrombosis (PVT) is a rare but severe entity that can cause clinically significant sequela such as worsening portal hypertension or mesenteric ischemia. Those cases refractory to medical management may be referred for endovascular intervention. Several technical considerations have been described in the literature, but a cohesive comparison of these multiple techniques is lacking.The purpose of this article is to review the diagnosis and endovascular management of PVT, including areas in which further research is warranted.Cases of PVT can be readily diagnosed using ultrasound, computed tomography, or magnetic resonance imaging. Treatment often begins with systemic anticoagulation and endovascular interventions may be used in selected cases. Determining the optimal approach to accessing the portal venous system depends on the underlying disease and chronicity of the thrombus and the degree of occlusion. Once access to the portal venous system is established, catheter-directed therapy may be performed to achieve recanalization.Despite the heterogeneity in patient presentation, cases of PVT can be readily diagnosed across several imaging modalities. Strategizing interventional approaches involves evaluation of the underlying disease and the chronicity of the thrombus.· This review will enable interventionalists to establish a framework for treating portal vein thrombosis by identifying patient risk factors and thrombus characteristics that determine patient management.. · The unique risks and benefits for transhepatic, transsplenic, and transmesenteric approaches for establishing portal venous access will be discussed.. · Advantages and complications of thrombolysis, thrombectomy, and transjugular intrahepatic portosystemic shunt creation for treating portal vein thrombosis will be reviewed in detail based on our extensive institutional experience..· Ju C, Li X, Gadani S et al. Portal Vein Thrombosis: Diagnosis and Endovascular Management. Fortschr Röntgenstr 2022; 194: 169 - 180.Die Pfortaderthrombose (PVT) ist eine seltene, aber schwere Erkrankung, die klinisch signifikante Folgeerscheinungen wie eine Verschlechterung der portalen Hypertonie oder eine mesenteriale Ischämie verursachen kann. Fälle, die auf eine medizinische Behandlung nicht ansprechen, können zur endovaskulären Intervention überwiesen werden. In der Literatur sind verschiedene technische Gesichtspunkte beschrieben worden, aber ein umfassender Vergleich dieser verschiedensten Methoden fehlt.In diesem Artikel werden die Diagnose und die endovaskuläre Behandlung der PVT besprochen, einschließlich der Bereiche, in denen weitere Forschung erforderlich ist.PVT-Fälle lassen sich mit Ultraschall, Computertomografie oder Magnetresonanztomografie leicht diagnostizieren. Die Behandlung beginnt häufig mit einer systemischen Antikoagulation, und in ausgewählten Fällen können endovaskuläre Interventionen eingesetzt werden. Die Bestimmung des optimalen Zugangs zum Pfortadersystem hängt von der Grunderkrankung und der Chronizität des Thrombus sowie vom Grad der Okklusion ab. Sobald der Zugang zum Pfortadersystem hergestellt ist, kann eine Katheter-gesteuerte Therapie durchgeführt werden, um eine Rekanalisierung zu erreichen.Trotz der Heterogenität der Patienten bei Vorstellung können Fälle von PVT mit verschiedenen bildgebenden Verfahren leicht diagnostiziert werden. Bei der Planung von Interventionen müssen die Grunderkrankung und die Chronizität des Thrombus berücksichtigt werden.· Diese Übersicht ermöglicht es den interventionell tätigen Ärzten, Rahmenbedingungen für die Behandlung von Pfortaderthrombosen zu schaffen, indem Risikofaktoren für Patienten und Thrombusmerkmale identifiziert werden, die das Patientenmanagement bestimmen.. · Die besonderen Risiken und Vorteile transhepatischer, transsplenischer und transmesenterialer Ansätze zur Etablierung eines portalvenösen Zugangs werden diskutiert.. · Vorteile und Komplikationen der Thrombolyse, der Thrombektomie und der Anlage eines transjugulären intrahepatischen portosystemischen Shunts zur Behandlung von Pfortaderthrombosen werden basierend auf der umfangreichen Erfahrung in unserem Institut im Detail besprochen..

Hemodynamic change after total paracentesis was investigated because it might lead to various complications. Although cell-free and concentrated ascites reinfusion therapy (CART) is safer and more effective than total paracentesis in theory, hemodynamic change after CART has been never reported. And previous studies did not mention hemodynamics of the venous system. We investigated the hemodynamic change, including that of the venous system, before and after CART using color Doppler ultrasonography and fast Fourier transform analysis. Twenty-eight patients with tensive cirrhotic ascites underwent ultrasonography the day before and after total volume CART. The diameter and velocity of the main, right, and left portal vein; inferior vena cava (IVC); and right renal vein were measured using ultrasonography. A total of 11.8 ± 4.4 L of ascites (range 3.6–20.9 L) was filtered and concentrated to 0.85 ± 0.40 L (range 0.36–1.50 L). The diameter of the IVC increased from median 13.5 ± 5.4 mm (range 4–25 mm) to 18.5 ± 4.1 mm (range 7–29 mm) (p = 0.007). The diameter of the right segmental renal vein significantly increased after KM-CART [from 5.0 ± 1.0 (4–8) mm to 7.0 ± 2.0 (3–10) mm] (p = 0.011). Hemodynamic change of the portal venous system was not significant. The time to the next CART in patients with an IVC diameter ≥ 20 mm and

The role of ligation of the portal venous branches to the caudate lobe (cPVL) as preparation for planned major hepatectomy is unclear. The aim of this study was to evaluate the efficacy of laparoscopic cPVL (Lap-cPVL) concomitant with transileocolic portal vein embolization of the right portal venous system (rTIPE), namely, Lap-cPVL/rTIPE, for planned right hemihepatectomy (rHx) in advanced hepatobiliary cancer patients. Thirty-one patients who underwent rHx after rTIPE with/without Lap-cPVL between March 2013 and March 2020 were enrolled in this study. The Lap-cPVL was performed for the portal branches of the right caudate lobe. Eight of the 31 patients underwent Lap-cPVL/rTIPE. The degree of hypertrophy was significantly increased in Lap-cPVL/rTIPE (19.3%, range 6.5–25.6%) as compared to rTIPE (7.2%, range - 1.1 to 21.2%) (p=0.027). The functional kinetic growth rate was also significantly increased in Lap-cPVL/rTIPE (5.40%, range 2.17–5.97) than that in rTIPE (1.85%, range - 0.22 to 6.45%) (p=0.046). Postoperative liver failure ≧ grade B occurred in 21.7% of patients in rTIPE, while there was no postoperative liver failure ≧ grade B in Lap-cPVL/rTIPE. Mortality rates were zero after rHx in this study. Lap-cPVL/rTIPE is safe and provides an additional effect on liver hypertrophy in advanced hepatobiliary cancers.

Purpose Portal venous system thrombosis is a complication of partial splenic artery embolization, and pre-treatment risk assessment is thus important. The purpose of this study was to identify the risk factors for portal venous system thrombosis after partial splenic artery embolization. Materials and methods We retrospectively analyzed 67 consecutive patients who underwent contrast-enhanced computed tomography before and after first partial splenic artery embolization between July 2007 and October 2018. As risk factors, we investigated age, sex, hematological data, liver function, steroid use, heparin use, and findings from pre- and post-treatment computed tomography. Uni- and multivariate analyses were performed to evaluate the relationship between thrombus appearance or growth and these factors. Values of p Results Partial splenic artery embolization was technically successful in all 67 patients. Nine patients showed appearance or growth of thrombus. Univariate analysis showed maximum diameter of the splenic vein before treatment (p = 0.0076), percentage of infarcted spleen (p = 0.017), and volume of infarcted spleen (p = 0.022) as significant risk factors. Multivariate analysis showed significant differences in maximum diameter of the splenic vein before treatment (p = 0.041) and percentage of infarcted spleen (p = 0.023). According to receiver operating characteristic analysis, cutoffs for maximum diameter of the splenic vein and percentage of infarcted spleen for distinguishing the appearance or growth of thrombus were 17 mm and 58.2%. Conclusion Large maximum diameter of the splenic vein before partial splenic artery embolization and high percentage of infarcted spleen after partial splenic artery embolization were identified as risk factors for portal venous system thrombosis. Level of Evidence Level 4, Case Series

Portal vein thrombosis refers to complete or partial obstruction of the portal venous system, in the intrahepatic or extrahepatic venous tract or even in the splenic or superior mesenteric veins. This common and potentially fatal condition can develop in various clinical contexts, especially those of liver cirrhosis, hepatocellular carcinoma, and other solid tumors. Certain characteristics, such as the time since the onset of the thrombus (acute or chronic), its biology (hematic or tumoral), the presence of collateral vessels, and the magnetic resonance imaging aspects, are important components of a thorough, careful analysis, as well as informing decisions regarding the appropriate therapeutic strategy. Here, we present a brief review of the anatomy of the portal venous system and a systematic approach to analyzing the condition, using a mnemonic (ABCD, for age, biology, collaterals, and diffusion). We discuss the various imaging methods and illustrate our discussion with images selected from the case files archived at our facility. Resumo Trombose da veia porta refere-se à obstrução completa ou parcial do sistema venoso portal, localizada nos tratos venosos intra-hepáticos ou extra-hepáticos e até mesmo nas veias esplênica ou mesentérica superior. Vários contextos clínicos podem ser responsáveis pelo desenvolvimento desta condição frequente e potencialmente fatal, especialmente a cirrose hepática, o carcinoma hepatocelular e outros tumores sólidos. Algumas características como o tempo de aparecimento do trombo (agudo ou crônico), sua biologia (hemático ou tumoral), a presença de vasos colaterais e o seu comportamento na ressonância magnética são importantes para uma análise completa e criteriosa, assim como para o gerenciamento adequado da estratégia terapêutica. No presente artigo apresentamos breve revisão da anatomia do trato venoso portal, seguida de uma abordagem sistemática usando um mnemônico (ABCD) para análise da trombose da veia porta por diferentes métodos de imagem, utilizando imagens de casos selecionados do arquivo de ensino do nosso serviço.

Background Portal venous gas (PVG) is a rarely observed clinical finding generally associated with intestinal ischemia. The proper clinical response to the finding of PVG depends somewhat on the setting in which it is observed. Here we describe a case in which extensive arterial gas emboli (AGE) were encountered during point-of-care ultrasound (POCUS) and subsequent computed tomography (CT) identified PVG secondary to gastric wall ischemia as the likely source. Case Report A 69-year-old woman with history of metastatic colon cancer presented to the emergency department (ED) with altered mental status. On arrival, she was hypotensive, hypothermic, cachectic, and with abdominal distension. POCUS was performed to evaluate the source of the patient's hypotension, revealing the presence of PVG, as well as gas bubbles in all four chambers of the heart and the aorta. CT scan revealed gastric wall ischemia and confirmed the presence of significant air emboli throughout the portal venous system. Given the overall poor prognosis, the decision was made to forego further chemotherapy or surgery and the patient died later that week while under hospice care. Why Should an Emergency Physician Be Aware of This? AGE can occur in the setting of PVG. This may cause multi-organ failure by disrupting blood flow to organs, especially in patients with circulatory dysfunction, such as shock. Depending on the setting in which it is diagnosed, early detection of PVG may expedite earlier assessments of a patient's negative prognosis or initiation of attempted life-saving treatment. In this case report, we show that POCUS can be used to obtain an expedited diagnosis in a critically ill patient.

Venous thrombosis within the hepatic vasculature is associated with a distinct array of risk factors, characteristics, and potential complication. As such, it entails unique management considerations and strategies relative to the more common categories of venous thromboembolic disease. Although broadly divided into thrombosis of the afferent vasculature (the portal venous system) and efferent vasculature (the hepatic venous system), presentations and management strategies within these groupings are heterogeneous. Management decisions are influenced by a variety of factors including the chronicity, extent, and etiology of thrombosis. In this review we examine both portal vein thrombosis and hepatic vein thrombosis (and the associated Budd-Chiari Syndrome). We consider those factors which most impact presentation and most influence treatment. In so doing, we see how the particulars of specific cases introduce nuance into clinical decisions. At the same time we attempt to organize our understanding of such cases to help facilitate a more systematic approach. Critically, we must recognize that although increasing evidence is emerging to help guide our management strategies, the available data remain limited and largely retrospective. Indeed, current paradigms are based largely on observational experiences and expert consensus. As new and more rigorous studies emerge, treatment strategies are likely to be continually refined, and paradigm shifts are sure to occur.

This study investigated the morphologic changes of portal cavernoma in children with extrahepatic portal venous obstruction and explored the relationship with prognosis.From February 2008 to October 2017, there were 107 patients with cavernous transformation of the portal vein admitted to our hospital. Rex shunts were performed in 99 cases, Warren shunts in 7 cases, and laparoscopic splenic vessel ligation in 1 case. Intraoperative superior mesenteric venography was used to determine the structure of the portal venous system. According to the morphologic features of the portal vein shown by portal venography, groups were assigned as follows: patients with the cotton form of portal cavernoma; patients with visible collateral veins of portal cavernoma; patients with and without a visible left gastric vein; and patients with and without a clearly visible intrahepatic portal vein. The preoperative and postoperative portal pressure, preoperative incidence of esophageal varices, time at onset, incidence of postoperative rebleeding, preoperative and postoperative size of the spleen, and age at time of operation were compared between these groups.The preoperative incidence of esophageal varices, time at onset, postoperative size of spleen, and age at time of operation were significantly lower in the group with the cotton form than in those with visible collateral veins (P .05). There was a significant correlation between the visible left gastric vein and esophageal varices (P = .002). The time at onset, preoperative and postoperative size of the spleen, and age at time of operation were markedly lower in the group with a good visible intrahepatic portal vein than in those without a clearly visible intrahepatic portal vein (P .05). The visible left gastric vein was notably associated with the performance of a gastroportal shunt (P = .000), and the group with a visible left gastric vein had a higher ratio of children undergoing a gastroportal shunt.The cotton form, an early-stage manifestation of cavernous transformation of the portal vein, typically occurs in younger children with a shorter time to onset. Children with the cotton form of portal cavernoma typically have a better prognosis after Rex shunt.

To outline the use of radiological investigations in patients with congenital portosystemic shunts (CPS) and to assess the degree of portal venous deprivation in relation to the development of liver tumors.Single center retrospective cohort study of all patients with CPS referred from 1990 to 2016. Radiological investigations were reviewed for the presence of intrahepatic portal veins. Two groups were defined: Group 1 - without evidence of preservation of an intrahepatic portal venous system and, Group 2 - with evidence of intrahepatic portal venous flow. These groups were compared for the development of liver tumors, serum ammonia, and nature of subsequent surgical intervention. The ratio of infraceliac to supraceliac aortic diameter and hepatic enhancement in biphasic CT scans were also used to infer hepatic arterial in-flow and compared to age matched controls. Nonparametric tests were used throughout. A P value of 0.05 was considered significant. Data are quoted as median (IQR).45 patients (Group 1, n = 12: Group 2, n = 33) were investigated for CPS at a median age of 8 months (1 month-14 years). Liver tumors were more common in Group 1 than Group 2 [11/12 (92%) versus 10/33 (29%); P 0.001]. Aortic ratio was significantly lower in patients with CPS compared to control (0.82 versus 0.96; P 0.001), but there was no difference between patients with or without tumors (0.82 versus 0.82; P = 0.52). Enhancement of the liver parenchyma was greater in portal venous rather than arterial phases by a median difference of 28 (15-50) Hounsfield units in controls, compared to 15 (8.5-23.5) in CPS patients (P = 0.04). A single stage closure was possible in 2/6 (33%) operated patients in Group 1 and 14/20 (70%) in Group 2 (P = 0.32).Patients without radiological evidence of intrahepatic portal venous flow were significantly more likely to have associated hepatic tumors with a relative risk 3.1.This manuscript includes data that are III and IV Levels-of-Evidence.

Pancreatic pseudocyst and portal vein thrombosis are common conditions resulting from acute and chronic pancreatitis. However, pancreatic pseudocyst-portal vein fistula (PPVF) is a rare and life-threatening complication. In PPVF patients, drainage or operative therapy is necessary under certain conditions, including communication between the pseudocyst and pancreatic duct, pancreatic duct stricture, pancreatic duct stone, and infection that is difficult to control with antibiotics. We describe the case of a 78-year-old woman who presented with an infected pancreatic pseudocyst invading the portal venous system with obstruction due to thromboembolization. Conservative therapy with antibiotics was insufficient. We, therefore, performed endoscopic ultrasound-guided cyst-drainage (EUS-CD). During EUS-CD, PPVF and pseudocyst-pancreatic duct fistula were confirmed by contrast medium injection. Despite the presence of the pseudocyst-pancreatic duct fistula, the pancreatic duct was not visualized in the pancreatic head, suggesting stricture in the main pancreatic duct. We, therefore, performed endoscopic retrograde pancreatography, which revealed pancreatic stone in the main pancreatic duct. Then, we added a 7-Fr pancreatic stent. She was discharged after 89 days of hospitalization. We reviewed the literature on the management of PPVF formation, and EUS-CD is considered to be a good treatment option for PPVF patients without portal vein patency.

Background Portal venous gas has mainly been studied in pediatrics and seen in cases of necrotizing enterocolitis. It is a rare finding in adults and is typically associated with underlying intestinal ischemia or other malignant intra-abdominal pathology. Portal venous gas is seen more readily on ultrasound compared to radiographs in both pediatric and adult patients. Findings include lucencies extending to the periphery of the liver, echogenic bubbles flowing centrifugally throughout the portal venous system, and bidirectional spikes interrupting the monophasic portal venous waveform on spectral analysis. Case Report We present a case of a 36-year-old female who presented with abdominal pain. She had findings consistent with portal venous gas on point-of-care ultrasound, prompting computed tomography of her abdomen and surgical consultation. She was ultimately found to have cecal ischemia from cecal volvulus, had surgical resection and anastomosis, and was able to be discharged from the hospital following recovery. Why Should an Emergency Physician Be Aware of This? With the ever-increasing use of point-of-care ultrasound, emergency physicians should be aware of the findings consistent with portal venous gas as well as its implications. Emergency physicians should know portal venous gas is associated with intestinal ischemia and other malignant pathologies and should prompt more advanced imaging or surgical consultation when observed. Emergency physicians should also understand the distinctions between portal venous gas and pneumobilia found on point-of-care ultrasound, given that portal venous gas is typically a malignant finding and pneumobilia is most frequently benign.

Introduction: The porta hepatis is bounded anteriorly by the quadrate lobe and posterosuperiorly by the caudate lobe. The hepatic artery with the autonomic plexus around it and the portal vein enter the liver through the porta hepatis, while the two hepatic ducts and some lymphatics emerge out from the liver through it. The porta hepatis of the liver is a very important area surgically. The knowledge of the variations in the structures passing through the porta hepatis might be of great help in reducing the risks of surgery in this area. Materials and Methods: The study was conducted in the Department of Anatomy of our institution after obtaining the ethical clearance.87 liver specimens in the Department were used for the study. The liver specimens will be removed during routine dissection for medical undergraduate teaching and would be preserved in 10% formalin. Conclusion: The incidence of variations in the portal venous system can be of prime surgical and radiological importance for portocaval anastomosis and to study hemodynamic flow radiologically. This study highlights the various possible combinations of the structures at the porta hepatis and is thereby useful for the radiologists and surgeons dealing with this region.

A splenic arteriovenous fistula causes a ‘pre-hepatic’ hypertension in the portal venous system with the double mechanism of an increased blood amount and mainly its high pressure inflow. It aggravates for a secondary fibrosis of the portal vein branches and ‘capillarization’ of the hepatic sinusoids, adding a further ‘intra-hepatic’ component. The subsequent development of porto-systemic collaterals induces the risk of gastrointestinal hemorrhages All this suggests to perform a close monitoring of every case of splanchnic aneurysm or pseudo-aneurysm, through the current cross-section imaging tools, for their possible evolution in an arteriovenous fistula, and to consider an early therapy, also endovascular, before any secondary damage of the liver parenchyma. In this case the treatment of the portal vein hypertension can be ‘ethiological’ and resolutive.

Congenital portosystemic shunts (CPSS) may produce a variety of severe, clinically detrimental presentations. When indicated, closure is recommended; however, if the intrahepatic portal venous system (IPVS) is underdeveloped complete closure may not be possible and may result in severe acute portal hypertension. Staged restriction of CPSS flow by both surgical and complex transcatheter interventions has been successful in augmenting development of the IPVS such that complete occlusion of the CPSS can be performed. We report use of a modified microvascular plug to restrict CPSS flow with subsequent IPVS development and safe complete occlusion of CPSS.

Portal vein thrombosis (PVT) is a splanchnic vascular disorder characterised by a recent or chronic thrombotic occlusion of the portal venous system. Its aetiology is miscellaneous, and its management is demanding since PVT can play a critical role as far as morbidity and mortality are concerned. Indeed, PVT can develop as a complication of portal hypertension (PH), in association or not with advanced chronic liver disease, and aggravate its clinical consequences such as variceal bleeding and ascites. Furthermore, a diagnosis of PVT in a non-cirrhotic context can potentially reveal a previously unknown hypercoagulable condition, requiring further diagnostic steps and specific treatment in addition to anticoagulation. In addition to established therapeutic approaches, new strategies, including newer pharmacological treatments and interdisciplinary invasive procedures, gain more attention and have been increasingly introduced into clinical practice. This review aims at discussing the current knowledge in terms of treatment options for PVT.

Hepatocyte transplantation (HcTx) is a promising approach for the treatment of metabolic diseases in newborns and children. The most common application route is the portal vein, which is difficult to access in the newborn. Transfemoral access to the splenic artery for HcTx has been evaluated in adults, with trials suggesting hepatocyte translocation from the spleen to the liver with a reduced risk for thromboembolic complications. Using juvenile Göttingen minipigs, we aimed to evaluate feasibility of hepatocyte transplantation by transfemoral splenic artery catheterization, while providing insight on engraftment, translocation, viability, and thromboembolic complications. Four Göttingen Minipigs weighing 5.6 kg to 12.6 kg were infused with human hepatocytes (two infusions per cycle, 1.00E08 cells per kg body weight). Immunosuppression consisted of tacrolimus and prednisolone. The animals were sacrificed directly after cell infusion ( n=2), 2 days ( n=1), or 14 days after infusion ( n=1). The splenic and portal venous blood flow was controlled via color-coded Doppler sonography. Computed tomography was performed on days 6 and 18 after the first infusion. Tissue samples were stained in search of human hepatocytes. Catheter placement was feasible in all cases without procedure-associated complications. Repetitive cell transplantations were possible without serious adverse effects associated with hepatocyte transplantation. Immunohistochemical staining has proven cell relocation to the portal venous system and liver parenchyma. However, cells were neither present in the liver nor the spleen 18 days after HcTx. Immunological analyses showed a response of the adaptive immune system to the human cells. We show that interventional cell application via the femoral artery is feasible in a juvenile large animal model of HcTx. Moreover, cells are able to pass through the spleen to relocate in the liver after splenic artery infusion. Further studies are necessary to compare this approach with umbilical or transhepatic hepatocyte administration.

Unlike other carcinomas, hepatocellular carcinoma (HCC) metastasizes to distant organs relatively rarely. In contrast, it routinely metastasizes to liver vasculature/liver, affecting portal veins 3-10 times more often than hepatic veins. This portal metastatic predominance is traditionally rationalized within the model of a reverse portal flow, due to accompanying liver cirrhosis. However, this intuitive model is not coherent with facts: 1) reverse portal flow occurs in fewer than 10% of cirrhotic patients, while portal metastasis occurs in 30-100% of HCC cases, and 2) portal vein prevalence of HCC metastasis is also characteristic of HCC in non-cirrhotic livers. Therefore, we must assume that the route for HCC metastatic dissemination is the same as for other carcinomas: systemic dissemination via the draining vessel, i.e., via the hepatic vein. In this light, portal prevalence versus hepatic vein of HCC metastasis appears as a puzzling pattern, particularly in cases when portal HCC metastases have appeared as the sole manifestation of HCC. Considering that other GI carcinomas (colorectal, pancreatic, gastric and small bowel) invariably disseminate via portal vein, but very rarely form portal metastasis, portal prevalence of HCC metastasis appears as a paradox. However, nature does not contradict itself; it is rather our wrong assumptions that create paradoxes. The 'portal paradox' becomes a logical event within the hypothesis that the formation of the unique portal venous system preceded the appearance of liver in evolution of chordates. The analysis suggests that the appearance of the portal venous system, supplying hormones and growth factors of pancreatic family, which includes insulin, glucagon, somatostatin, and pancreatic polypeptide (HGFPF) to midgut diverticulum in the early evolution of chordates (in an Amphioxus-like ancestral animal), promoted differentiation of enterocytes into hepatocytes and their further evolution to the liver of vertebrates. These promotional-dependent interactions are conserved in the vertebrate lineage. I hypothesize that selective homing and proliferation of malignant hepatocytes (i.e., HCC cells) in the portal vein environment are due to a uniquely high concentration of HGFPF in portal blood. HGFPF are also necessary for liver function and renewal and are significantly extracted by hepatocytes from passing blood, creating a concentration gradient of HGFPF between the portal blood and hepatic vein outflow, making post-liver vasculature and remote organs less favorable spaces for HCC growth. It also suggested that the portal vein environment (i.e., HGFPF) promotes the differentiation of more aggressive HCC clones from already-seeded portal metastases, explaining the worse outcome of HCC with the portal metastatic pattern. The analysis also offers new hypothesis on the phylogenetic origin of the hepatic diverticulum of cephalochordates, with certain implications for the modeling of the chordate phylogeny.

Portal venous system evaluation is required in many clinical circumstances, as substantial morbidity and mortality can be associated with a spectrum of portal vein abnormalities. Portal venous system pathologies can be congenital or acquired, and the knowledge of their imaging appearances will allow for a confident diagnosis and appropriate treatment options. In addition, a firm understanding of anatomical variants is important for planning surgical procedures and percutaneous interventions of the liver. This article will review various imaging appearances of portal venous systems abnormalities.

Background Portal venous system thrombosis (PVST) will progress in some cases, indicating worse outcome and the necessity of antithrombotic treatment, but will spontaneously improve in others. It is crucial to understand the natural history of PVST in liver cirrhosis. However, the knowledge regarding how to predict the evolution of PVST in cirrhotic patients is very scant. Methods Sixty-nine cirrhotic patients without malignancy, who had undergone repeated contrast-enhanced computed tomography or MRI to evaluate the severity of PVST at the first and last admissions, were included. Logistic regression analysis was performed to identify the risk factors for the evolution of PVST in liver cirrhosis. Odds ratios (ORs) were calculated. Results Among 42 patients without PVST at the first admission, 10 (23.8%) developed PVST at the last admission. Serum albumin level (OR = 0.873), prothrombin time (OR = 1.619), activated partial thromboplastin time (OR = 1.169), Child-Pugh score (OR = 1.560) and model for end-stage liver disease (MELD) score (OR = 1.292) at the last admission were significant risk factors associated with the development of PVST. Among 27 patients with PVST at the first admission, 11 (40.7%), 4 (14.8%) and 12 (44.4%) had improvement, stabilization and progression of PVST at the last admission, respectively. ΔMELD score (OR = 0.714) was the only significant risk factor associated with the improvement of PVST; additionally, serum albumin level at the first admission (OR = 1.236) was the only significant risk factor associated with the progression of PVST. Conclusion Aggravation and amelioration of liver dysfunction may predict the development and improvement of PVST in liver cirrhosis, respectively.

Patient: Male, 63-year-old Final Diagnosis: Pancreatic undifferentiated carcinoma with expansive metastatic infiltration of the portal venous system Symptoms: Low back pain Medication:— Clinical Procedure: — Specialty: Oncology Objective: Challenging differential diagnosis Background: The portal vein thrombosis caused by a carcinoma of the pancreas or liver could be a diagnostic challenge. There are some cases which could be diagnosed only by observing changes in patients’ clinical manifestations or imaging studies over time. Case Report: A 63-year-old man experienced exacerbation of chronic low back pain for 1 month, with abdominal distention. He was admitted to our hospital because abdominal ultrasonography and abdominal contrast-enhanced computed tomography performed at another hospital revealed an extensive thrombus in the portal, splenic, and superior mesenteric veins. Anticoagulation therapy with warfarin was started immediately on admission. Although no baseline disease, such as malignancies, coagulopathies, infections, or collagen diseases were revealed during the 12 days of his hospitalization, the thrombus gradually expanded, with the appearance of obstructive jaundice 8 months after admission. He was readmitted at that time for biopsy of intrahepatic tumors detected by abdominal computed tomography, which diagnosed metastasis of a carcinoma of unknown origin. Although chemotherapy was started, he died 11 months after his initial admission to our hospital. Autopsy revealed a tumorous lesion in the pancreatic head with almost complete replacement of the portal venous thrombus with a similar-appearing tumor. We confirmed the diagnosis of portal venous metastatic infiltration by undifferentiated pancreatic carcinoma, using histopathological examinations. Conclusions: It is essential to consider portal venous metastatic infiltration of undiagnosed malignancy inducing local hypercoagulopathy as a possible cause of expansive portal venous thrombosis.

We read with interest the review article by Diwan et al. on bariatric surgery in liver transplantation (LT) patients (1). In particular, we wish to highlight portal venous system thrombosis (PVT) among the potential complications after laparoscopic sleeve gastrectomy (LSG).

Portal venous system thrombosis can develop after bariatric surgery. A systematic review and meta-analysis was conducted to evaluate the incidence of portal venous system thrombosis after bariatric surgery and clarify the role of anticoagulation for the prevention of portal venous system thrombosis after bariatric surgery.PubMed, EMBASE, and Cochrane Library databases were searched. The incidence of portal venous system thrombosis after bariatric surgery was pooled by a random-effect model. Subgroup analyses were performed to explore the incidence of portal venous system thrombosis according to the average duration of prophylactic anticoagulation (extended versus short-term). Meta-regression and sensitivity analyses were performed to explore the source of heterogeneity.Among 2,714 papers initially screened, 68 studies were included. Among 100,964 patients undergoing bariatric surgery, 300 developed portal venous system thrombosis. The pooled overall incidence of portal venous system thrombosis after bariatric surgery was 0.419% (95% confidence interval: 0.341%-0.505%). The pooled incidence of portal venous system thrombosis after bariatric surgery was numerically lower in patients who received extended prophylactic anticoagulation protocol after bariatric surgery than those who received short-term prophylactic anticoagulation protocol (0.184% vs 0.459%). Meta-regression analyses demonstrated that sample size (P = .006), type of surgery (P.001), and average duration of prophylactic anticoagulation (P = .024) might be sources of heterogeneity, but not region, publication year, history of bariatric surgery, follow-up duration, or use of prophylactic anticoagulation. Sensitivity analyses could not identify any source of heterogeneity. The estimated mortality of portal venous system thrombosis after bariatric surgery was 1.33%.Portal venous system thrombosis after bariatric surgery is rare, but potentially lethal. Extended prophylactic anticoagulation protocol may be considered in patients at a high risk of developing portal venous system thrombosis after bariatric surgery.

We present a case with a pancreatic neuroendocrine tumor and extensive tumor thrombosis in portal venous system. The tumor was first identified on contrast-enhanced CT and later confirmed using Ga-DOTATATE and Ga-NODAGA-LM3 PET/CT. Both tracers demonstrated similar pattern with higher tumor affinity and tumor-to-background ratio using Ga-NODAGA-LM3.

Neonatal cases of systemic artery to portal venous system arteriovenous malformations (AVMs) can present unique challenges in terms of diagnosis, management, and treatment. Prompt identification of these AVMs is necessary for minimizing long-term sequelae and optimizing prognosis. Our report describes the diagnosis and successful endovascular coil embolization of a congenital right internal mammary artery (IMA) to portal vein AVM in a young infant initially presenting during routine fetal screening with an incidentally discovered congenital thoracic vascular abnormality.

Portal venous system thrombosis (PVST) is a life-threatening complication after splenectomy in cirrhotics patients with portal hypertension, while the application of intervention regimen may prevent the incidence of PVST. The aim of this network meta-analysis was to determine the most appropriate intervention regimen and application time. Several electronic databases were searched up to December 2019. We estimated summary odds ratios (OR) using pairwise and network meta-analyses with random effects for the outcome of occurrence of PVST. This work was registered with PROSPERO (CRD42019161406). The analysis was based on 19 researches, which included 1853 patients. The results drawn from the data in standard meta-analysis indicated that the application of intervention was better than no intervention use, and early application of interventions was better than delayed application in preventing the occurrence of PVST. Subsequent network meta-analysis was performed to determine the most suitable intervention regimen used early post-operation. For separate mono-therapy drug, alprostadil, antithrombin III, low molecular dextran were significantly more efficacious than others. However, mono-therapy analysis was not so close to clinical application. In the follow-up network meta-analysis, low molecular dextran combined with low molecular weight heparin exhibited the largest effect on the preventing the incidence of PVST (0.12, 0.03-0.49), followed by antithrombin III (0.12, 0.04-0.41) with low molecular dextran (0.14, 0.05-0.41). We could draw the conclusion that early application of low molecular weight heparin combined with low molecular dextran seems to be the most satisfactory treatment to prevent the incidence of PVST for patients with cirrhotic portal hypertension after splenectomy.

Introduction Toxic acute liver failure can be fatal even after liver transplantation. Since there are data only in young rats, the aim of our study was to verify the effectiveness of the increase of oxygen to the liver by partial portal vein arterialization (PPVA) in elderly rats with acute liver failure induced by carbon tetrachloride (CCl4) intoxication. Methods Twenty elderly (30 months) Sprague-Dawley rats underwent a CCl4 intoxication (5 mL/kg). Animals were divided after 24 hours (n = 10 per group) to either undergo PPVA (G1, treated group) or be untreated (G2, control group). PPVA consisted of a shunt between the left renal artery and splenic vein after nephrectomy and spelnectomy. The G2 group animals underwent nephrectomy and splenectomy only. The 10-day survival was evaluated. Before euthanasia, blood samples from the portal vein were detected for blood gas analysis. Liver injury was evaluated by the serum alanine aminotransferase (ALT) and prothrombin time levels. Histology was done to evaluate the liver necrosis. Hepatocyte regeneration was assessed by the mitotic index at immunohistochemistry. Results The PPVA has resulted in a significant increase in the oxygen partial pressure and saturation in portal blood. A survival improvement at 10 days was registered in the PPVA-treated rats (90% vs 30%; P = .0065). After 24 hours from intoxication, ALT was high in both groups. A rapid decrease of ALT was observed in G1 as compared to G2. At the same time, livers showed a severe centrolobular necrosis. In the suviving G2 rats, a moderate necrosis was present, while only a mild necrosis was observed in the G1 rats. An higher mitotic index was detected in rats treated with PPVA. Conclusions In our experimental study, the presence of oxygenated blood in the portal venous system following the PPVA procedure had positive effects on liver regeneration and rats’ survival. The PPVA treatment had beneficial effects in elderly rats.

Introduction Occlusive portal venous system thrombosis (PVT) is significantly associated with poor outcomes in cirrhotic patients. Nonselective β-blockers (NSBBs) may be associated with the development of PVT. However, the role of NSBBs in progressing thrombosis remains unclear. Methods Forty-three patients on whom contrast-enhanced computed tomography or magnetic resonance imaging was performed twice, and for whom there was detailed information regarding NSBBs, were eligible in this study, including 16 in the NSBBs group and 27 in the no NSBBs group. A composite endpoint of progressing thrombosis included the development of PVT in patients without PVT and aggravation of PVT in patients with PVT. Logistic regression analysis was employed to identify the effect of NSBBs on the progression of PVT. Results At the last admission, 13 patients had progressing thrombosis. The incidence of progressing thrombosis was significantly higher in the NSBBs group than in the no NSBBs group [50.0% (8/16) vs. 18.5% (5/27), P = 0.030]. The use of NSBBs (odds ratio 4.400, 95% confidence interval 1.107–17.482, P = 0.035) was significantly associated with progressing thrombosis in univariate logistic regression analyses, but not significant (odds ratio 4.084, 95% confidence interval 0.488–34.158, P = 0.194) in multivariate logistic regression analyses. Conclusions NSBBs may play a role in the progression of PVT in liver cirrhosis. The benefits and risks of NSBBs in the management of liver cirrhosis should be fully weighed.

Portal venous interventions comprise a large portion of many Interventional Radiology practices today, and remain some of the more technically challenging cases in one's repertoire of procedures. The patients upon whom these procedures are performed are often critically ill, have decompensated disease, or are burdened with comorbid conditions such that they are poor surgical candidates. This leaves them with few options outside the care of Interventional Radiology. Some portal venous interventions, such as transjugular intrahepatic portosystemic shunt, have an established history of excellent clinical success with numerous technical advancements over the years helping to improve outcomes. Others, like balloon occlusion sclerotherapy or portal venous recanalization, are less well established but are nonetheless invaluable in the treatment of portal venous diseases. The goal of this article is to help dispel some of the anxiety experienced by individuals performing the three main procedures of the portal venous system, namely transjugular intrahepatic portosystemic shunt, balloon-occlusion retrograde transvenous obliteration, and portal vein embolization.

Purpose To evaluate the incidence of bleeding complications between transplenic (TS) and transhepatic (TH) access in portal venous interventions. Materials and Methods Retrospective review of patients who underwent TS or TH access for portal venous system interventions from January 2000 to August 2017. Only procedures with clinical and laboratory follow-up were included (n = 148). Twenty-four TS procedures were performed in 22 patients, and 124 TH procedures were performed in 114 patients. The main indications were for angioplasty/stent, embolization of varices/shunt, or portal vein embolization, with no difference between the groups. Mean patient age and sex were not significantly different between the groups ( P values .445 and .682, respectively). Mean follow up was 2.3 years (range 0.1–14.2). There was no significant difference between the international normalized ratio ( P = .300) and platelets ( P = .234) before the procedure between the 2 cohorts. Results Technical success of vascular access and procedural success was achieved in 22/24 (91.6%) TS procedures and 120/124 (96.8%) TH procedures ( P = .238). There was no significant difference in bleeding complications between the 2 groups (3/24 [12.5%] TS vs 10/124 [8.1%] TH; P = .44). There was no significant difference in major bleeding complications (SIR classification ≥ C; 1/24 [4.2%] TS vs 4/124 [3.2%] TH; P = .789).There was no significant difference in the hemoglobin before or after the procedure (g/dL), with average change −1.1 g/dL (range −3.4 to +1.0) in the TS group and 1.0 g/dL (range −4.5 to +1.9) in the TH group ( P = .540). Finally, there was no significant difference in proportion of patients requiring blood transfusion after the procedure ( P = .520), with 2 (8.3%) in the TS group requiring an average of 4 units (range 2–6) and 17 (13.7%) in the TH group requiring an average of 3.5 units (range 1–26). Conclusions These data suggest no significant difference in bleeding complications between TS and TH access for portal venous interventions.

To evaluate intraabdominal course of umbilical vein, its relation with portal vein and ductus venosus and its anomalies. All pregnant women were examined during midtrimester anomaly scan between January 2015 and December 2017. With evaluation of umbilical vein in its intraabdominal course, size of umbilical vein, its relation to the stomach and the gallbladder and origin of ductus venosus were studied in middle and upper abdominal axial and sagittal planes by two dimensional and colour doppler sonography. Anastomosis of umbilical vein with portal vein and its further course was examined and detailed anatomical survey was made to look for associated anomalies. Fetal and neonatal outcome was assessed after delivery. Twelve cases of umbilical vein abnormalities were evaluated. Of them, there were three cases of umbilical vein varix, four cases of persistent right umbilical vein and five cases of abnormal umbilical–portal–systemic venous shunt were noted. Examination of the intraabdominal course of umbilical vein and ductus venosus should be a part of second trimester anomaly scan. Proper knowledge of umbilical portal venous system is mandatory.

BACKGROUND The purpose of this study was to investigate splanchnic hemodynamic changes and determine an optimal cutoff value for risk factors of portal venous system thrombosis (PVST) after splenectomy with periesophagogastric devascularization (SPD) in cirrhotic patients with esophageal and gastric variceal bleeding (EGVB) and portal hypertension (PH). MATERIAL AND METHODS Data on patients who underwent SPD were collected retrospectively from January 2013 to December 2017. Color Doppler ultrasound was performed to detect hemodynamic changes of the hepatic artery, splenic artery, splenic vein, and portal vein in included patients (n=60) and healthy volunteers (n=30). Outcomes were compared between preoperative and postoperative biochemical indicators. The cutoff values for hemodynamics were identified using receiver operating characteristic (ROC) curve analysis, and univariate and multivariate analyses of risk factors of PVST were performed. RESULTS In our series, hemodynamic indexes of splenic artery, spleen vein, and portal vein in the study group were significantly higher than that of the control group (P

Background and aims Congenital shunts of the portal venous system are rare entities that can present in children with clinical heterogeneity. To evaluate the clinical course of children with uncommon shunts presenting to our institution and examine the available literature on this topic. Medical records of children with rare forms of congenital shunts were retrospectively reviewed for demographics, symptoms, management, and outcome between 2003 and 2016. Results Three female patients with congenital shunts, including a congenital mesenterico-portal Rex shunt (n = 1) and congenital portosystemic shunts (CPSS) (n = 2), were referred for surgical evaluation between ages 4 and 9. Median follow-up was 8 years (range, 6-13 years). One asymptomatic patient did not require treatment and remained disease-free during long-term follow-up. The other 2 patients with CPSS and unusual symptoms, including liver focal nodular hyperplasia (FNH) in infancy (n = 1) and bleeding from esophageal varices (n = 1), showed subsequent progression to liver nodules that were managed by endovascular shunt occlusion. One patient showed symptom resolution and the other showed stable lesions at last follow-up. Literature yielded descriptions of two cases of congenital mesenterico-portal Rex shunt, one case of coincident CPSS and FNH in infancy, but zero reports of bleeding from esophageal varices. Conclusions This case series examines each distinct patient's presentation, discusses the diagnosis, management and outcome and compares findings while discussing literature on this topic. A high index of suspicion and familiarity with unusual forms and treatment options is required to allow timely diagnosis and appropriate treatment.

To measure the distance between the right and middle hepatic veins and portal vein branches, in human cirrhotic liver casts. Was this measure actually smaller in the cirrhotic liver than in normal one? This study was authorized by an area Research Ethics Committee, and each study subject or legal representative granted signed informed consent. Acrylic corrosion casts of 21 resected cirrhotic livers were generated. Diameters of hepatic veins and portal branches and pertinent intervening distances were measured. To assess differences in estimated average (relative to reference values), Student’s t test for one sample was applied. Mean distances from right hepatic vein to the right portal branch and to portal vein bifurcation were 33 ± 6.4 and 36 ± 7.4 mm, respectively, both significantly less than published reference values in healthy human livers (p

The two cornerstones of management for Extrahepatic portal vein obstruction (EHPVO) are endotherapy and surgery [Porto-systemic shunts (PSS)/Mesorex bypass (MRB)]. Endotherapy is the mainstay of treatment for acute variceal bleed control and has also been used extensively for secondary prophylaxis till variceal eradication is achieved. However, long-term follow-up beyond endoscopic eradication of esophageal varices (EEEV) indicates that there are numerous delayed bleed and non bleed sequelae of EHPVO, which merit surgery as a definitive procedure to decompress the hypertensive portal venous system. While endotherapy obliterates natural porto-systemic collaterals in the gastroesophageal region, persistently raised portal pressures manifest as an increase in secondary isolated gastric varices, ectopic varices, portal hypertensive vasculopathy, issues related to massive splenomegaly, portal biliopathy, growth retardation and hence impaired quality of life (QOL). An ideal management strategy should address both bleed and non-bleed consequences of EHPVO and translate into a near normal QOL. Further, MRB has opened up new dimensions to the management philosophy of EHPVO. This review article critically evaluates the role of surgery and endotherapy based on available literature and authors' own experience.Surgery and endotherapy are complementary. However, with increasing duration of follow-up post EEEV, it is evident that there is resurgence in the role of surgery (PSS/MRB) as a single one time definitive procedure for alleviating all bleed and delayed non bleed sequelae of EHPVO.Surgery for EHPVO (PSS/MRB) should not be allowed to become a dying art and future generations of surgeons should continue to receive training in this specialized area of surgery.

Portal venous system thrombosis (PVST) is a common and potentially life-threatening complication of splenectomy for portal hypertension due to cirrhosis.A meta-analysis was conducted to study the necessity of pharmacologic prophylaxis of PVST after splenectomy and how to select the feasible treatment method. Articles were searched through the PubMed, EMBASE, Cochrane Library databases, and CNKI.Overall, 404 articles were initially identified, and 11 of them were eligible. Among these selected articles, 7 articles were associated with the necessity of anticoagulation for prevention of PVST, while 5 were about the drug selection. We first demonstrated that the incidence of PVST after splenectomy was significantly lower in patients who received the preventive measures than in those who did not (odds ratio [OR]: 0.22, 95% confidence interval [CI]: 0.13-0.39, P .00001). Then, we compared the new-style treatment with the conventional treatment and found that patients with new therapy method had lower incidence of PVST than those who received conventional treatment (OR: 0.37, 95% CI: 0.27-0.51, P .00001). Also, some studies (n = 4) reported that early and combination use of anticoagulation drugs can lead to better outcome for patients with splenectomy and devascularization.Preventative use of anticoagulant drugs might decrease the incidence of PVST after splenectomy in patients with portal hypertension, new anticoagulant drugs such as low-molecular-weight heparin should be used, and early or combination use of anticoagulation drugs might lead to lower PVST incidence for patients.

We previously showed an increased number of smaller portal vein (PV) branches in the portal areas of liver biopsy specimens of biliary atresia (BA) patients. We evaluated the correlation between this histopathological feature and the prognosis. Twenty-five consecutive patients with BA encountered between 2000 and 2012 were classified into three prognostic groups based on their postoperative outcomes: Excellent (n = 11) for native-liver survivors with a normal liver function, Good (n = 6) for native-liver survivors with liver dysfunction, and Poor (n = 8) for survivors after liver transplant or on a waiting list. Data from morphometrical analyses, including the fibrotic portal area, numbers of PVs, diameter and total area of PV branches, were statistically compared among the three groups. The number of PV branches per unit area of the whole-liver specimen in the poor prognostic group was significantly lower than that in the excellent group (3.1 ± 0.6 vs. 5.2 ± 2.0/mm2, p = 0.03). There were no significant differences in the other parameters. This is the first report on the relationships between morphometrically analyzed PV branches and the postoperative course in BA patients. The portal venous system is involved as the primary lesion in BA.

PVT is the most frequent vascular complication after LT in small children, and a higher incidence has been observed in those transplanted for biliary atresia or with a LLSG. Thrombosis of the PV causes extrahepatic portal hypertension and is associated with splenomegaly and the development of venous neo-collaterals, including gastro-oesophageal varices and splenorenal shunts. It has also been incidentally suggested in the literature that patients who have had a Roux-en-Y loop for a biliary reconstruction may present with a cavernomatous transformation of the distal portion of the loop. In this study, 13 children with CEPH caused by thrombosis of the PV after LT were analysed. The study evidenced the development of two types of hepatopetal venous networks: (a) a large cavernoma along the Roux loop and around the biliary anastomosis, and (b) a network of neo-collaterals in the gastro-duodeno-pancreatic area that connected to the intrahepatic portal branches directly through the liver capsule. These hepatopetal venous networks between the venous system of the surrounding organs or the omentum and the intrahepatic portal branches can be identified by radiologists. The relevance for the transplanting physician and the transplant surgeon is discussed.

Background Vascular access port (VAP) systems are widely used in human medicine to provide long-term venous access. However, in veterinary medicine the use of VAP systems is not common practice and publications on their potential applications have been limited. A VAP system was used as part of an experimental study on liver regeneration and implanted in the canine portal vein to create direct access to the portal venous circulation of the liver. The aim of the present study is to describe the surgical technique, its use, and the complications of a VAP system in three research dogs. Results The VAP system was successfully used for the intraoperative measurement of portal blood pressure, the administration of cell suspensions, and the collection of portal venous blood samples. Long-term complications consisted of dislocation of the VAP system in one dog (2 months after implantation) and thrombus formation at the catheter tip in two dogs (3 months after implantation). Both complications prevented further use of the VAP but had no adverse clinical implications. Conclusions This pilot study suggests that the VAP system is an effective and safe technique to obtain long term access to the portal venous system in dogs. However, complications with port detachment and thrombosis may limit long term use of VAPs in the portal system of dogs.

This article offers an overview of congenital and acquired vascular anomalies involving the portal venous system in dogs and cats, as determined by multidetector-row computed tomography angiography. Congenital absence of the portal vein, portal vein hypoplasia, portal vein thrombosis and portal collaterals are described. Portal collaterals are further discussed as high- and low-flow connections, and categorized in hepatic arterioportal malformation, arteriovenous fistula, end-to-side and side-to-side congenital portosystemic shunts, acquired portosystemic shunts, cavoportal and porto-portal collaterals. Knowledge of different portal system anomalies helps understand the underlying physiopathological mechanism and is essential for surgical and interventional approaches.