Computed tomography enterography, magnetic resonance enterography, and bowel ultrasound are complementary tools central to diagnosing and monitoring Crohn disease. These modalities can identify active inflammation, penetrating, and stricturing disease. Crohn disease must be monitored frequently to guide therapy, and resolution of inflammation on imaging correlates directly with steroid-free clinical remission. While disease activity assessment is qualitative, newer quantitative techniques to assess active inflammation are emerging. These as well as other techniques, such as contrast-enhanced ultrasound (US) and US elastography, will offer new tools for future radiologists.

Portal vein thromboses (PVT) is a common clotting disorder that can be seen in patients with and without cirrhosis. There are no current clinical guidelines on management of portal vein thromboses in these two distinct populations given most studies are retrospective and comprised of heterogenous cohorts.

When evaluating PVT, patients must first be stratified into those with cirrhosis and those without cirrhosis. In addition, a novel nomenclature can help categorize specific PVT types and determine the need and response to anticoagulation. The management of PVT in patients with cirrhosis varies and is primarily dependent on whether the PVT is recent or chronic. In contrast, patients without cirrhosis are almost always anticoagulated to avoid complications of PVT. Direct oral anticoagulants, low-molecular weight heparin, and vitamin-K antagonists have all been used in patients with and without cirrhosis, without clear guidance on optimal treatment duration and surveillance.

Direct oral anticoagulants are increasingly used for patients with PVT though there is limited data on the safety and efficacy of these medications. The risk/benefit profiles of various anticoagulants must be considered when choosing a therapeutic anticoagulant. There are ongoing studies evaluating outcome measures of different anticoagulants in patients with PVT. Large, multicenter, randomized controlled trials may help elucidate the efficacy of anticoagulants on various outcome measures in PVT, including recanalization, bleeding, and survival.

ObjectiveThis study aimed to evaluate the potential role of the whole-exome sequencing (WES) technique in screening pathogenic genes of non-cirrhotic and non-malignant portal vein thrombosis (PVT) and the clinical efficacy of AngioJet-assisted pharmacomechanical thrombectomy (PMT) with transjugular intrahepatic portosystemic stent shunt (TIPS) to treat this disease.Methods16 patients with acute non-cirrhotic and non-malignant PVT were retrospectively analyzed. 14 patients received genetically diagnosed using the WES technique, ten patients received PMT with or without TIPS and 6 cases received anticoagulation alone. Changes in clinical symptoms, and recanalization of the portal vein (PV) were also recorded.Results4 patients (28.6%) had JAK2 V617 F mutation, 4 patients (28.6%) had PROC mutations, 3 patients (21.4%) had SERPINC1mutations. Among patients treated with anticoagulation alone, 5 patients (83.3%, 5/6) developed cavernous transformation of PV and one patient with JAK2 V617 F mutation achieved complete recanalization of PV. One patient treated with PMT without TIPS developed PV cavernous transformation, eight patients (80%) treated with PMT and TIPS achieved complete or partial recanalization of PV. Three patients who developed intestinal necrosis all had SERPINC1 mutations and one of them died of intestinal necrosis. No recurrence was found during follow-up (1-36 months).ConclusionsThe WES technique offers a promising way to screen for thrombophilia in patients with non-cirrhotic and non-malignant PVT. Patients with SERPINC1 mutations are more inclined to develop intestinal necrosis than others. PMT combined with TIPS provides a safe and effective therapeutic alternative.

Splanchnic venous thrombosis (SVT) is a frequent complication of acute pancreatitis (AP) and may worsen the prognosis. There are no definite recommendations for the management of SVT. Our objectives were to analyze the recanalization rate and short and long-term complications of SVT.

Single-center retrospective cohort study, which included patients hospitalized for AP complicated by SVT within the first 30 days. The primary endpoint was venous recanalization. Secondary endpoints were portal hypertension, gastrointestinal bleeding, complications of anticoagulation, and mortality.

Between 2014 and 2018, 711 patients were hospitalized with AP; 121 (17 %) developed SVT and 88 were included in the analysis. Venous thrombosis involved the portal vein (27 %), splenic vein (21 %), superior mesenteric vein (16 %), 2 sites (19 %) or 3 sites (14 %). Of them, 78 % of patients received anticoagulation. After a median follow-up of 30 months, thrombosed vessel(s) got recanalized in 42 % of patients within a median of 7 months. Thrombosis extension was observed in 18 % of patients. The only factor associated with non-recanalization was portal vein or multi-site involvement (p = 0.004). Anticoagulation treatment was not associated with a higher rate of recanalization. Portal hypertension developed in 56 % of patients and varices rupture occurred in 9 % of patients. The only predictive factor of portal hypertension was non-recanalization of the thrombosed vessel (p < 0,001).

Recanalization was observed in 42 % of patients with SVT complicating in AP. Non-recanalization was the only risk for portal hypertension. Our study suggests an absence of benefit of anticoagulation.

Acute mesenteric ischemia (AMI) is associated with high mortality rates. There are multiple challenges to establishing an accurate early diagnosis and providing state-of-the-art care for AMI patients. A high index of suspicion is key for early diagnosis. Once suspicion is raised, a triphasic computed tomography angiography is the essential diagnostic tool. Avoiding delays, using hybrid operating rooms and contemporary revascularization techniques for arterial occlusive AMI, can significantly improve the prognosis. Regional health care systems should be developed to direct AMI patients into centers with sufficient capabilities for providing all aspects of care at all hours. The acute care surgeon has a central role in performing laparotomies and bowel resections when needed and coordinating the management flow in close collaboration with vascular surgeons and interventional radiologists for prompt and effective revascularization. A significant share of patients with an arterial occlusive AMI can be managed by endovascular revascularization without the need for a laparotomy. There are no reliable tools for predicting transmural bowel necrosis, and individual assessment and clinical experience are very important in decision-making when choosing between laparotomy and close observation. During laparotomy, an atherosclerotic occlusion at the root of the superior mesenteric artery can be stented by using a retrograde open mesenteric or percutaneous approach, and surgical bypass is seldom needed. Using hospital-specific management pathways is very useful for the standardization of care in arterial occlusive AMI. In venous AMI, systemic anticoagulation is sufficient in most cases. In patients whose symptoms do not resolve, there are various options for endovascular and surgical revascularization. In nonocclusive mesenteric ischemia, prevention by maintaining sufficient abdominal perfusion pressure is key. High-level evidence is scarce, but with current knowledge, the prognosis of AMI patients has plenty of room for improvement.

Portal vein thrombosis in non-cirrhotic individuals, although uncommon, is an increasingly explored condition that affects mainly young people, consequently representing a significant disease burden. Reports primarily including western European populations have recently shed light regarding the pathophysiology, risk factors, natural history, treatment, and prognosis of this entity. Underlying predisposing conditions are documented in ~70% of cases, encompassing local risk factors, inherited and acquired thrombophilia, cancer, and systemic inflammatory conditions. Non-cirrhotic portal vein thrombosis can cause significant portal hypertension in the acute setting, but, more frequently, significant portal hypertension-related complications arise when the condition becomes chronic and portosystemic collaterals develop, increasing the risk for variceal bleeding and ascites. The diagnostic approach to screen for underlying thrombophilia remains a challenge, and recommendations in this regard, although scarce and backed by scarce evidence, have changed notably in the last years, leaning toward a universal screen in patients who develop this condition without a clear provoking factor. Recently, studies have shown that long-term anticoagulation may be appropriate even in the absence of clear provoking factors or underlying thrombophilia. Future studies should address which patients may benefit from this approach, which patients may not need it, and what the most appropriate strategies are to approach patients who do not recover portal vein patency with anticoagulation to further prevent portal hypertension-related complications.

This study assesses the efficacy and safety of Portal Vein Recanalization with Intrahepatic Portosystemic Shunt (PVR-TIPS) in non-cirrhotic patients with chronic portal vein occlusion (CPVO), cavernomatous transformation, and symptomatic portal hypertension (PH) and/or portal vein thrombotic progression.

Medical records of 21 non-cirrhotic patients with CPVO and portal cavernoma undergoing PVR-TIPS were analyzed. Hemodynamic (intraprocedural reduction in portosystemic pressure gradient), clinical (data on gastrointestinal bleeding, abdominal pain, ascites, and presence of esophageal varices from imaging exams) and technical success (PVR-TIPS) assessed efficacy. Safety was determined through complications classified according to the CIRSE Classification System.

PVR-TIPS was successfully performed in all patients, resulting in a significant reduction in portal pressure gradient by 10 mmHg (21.475 ± 9.7 mmHg - 11.454 ± 5,4 mmHg, p < 0.001), alleviating portal hypertension symptoms without thrombotic progression. Clinical success included resolution or reduction of ascites (p = 0.016), gastroesophageal varices (p = 0.004), abdominal pain (p = 0.0021), and cessation of gastrointestinal bleeding (p = 0.021). Complications occurred in 33% of patients, including six grade III events (1 perioperative liver bleeding, 5 delayed stent occlusions) and one grade VI event resulting in death (4.8%). Primary patency rate was 76% (21.3 months, range:0.2-82), secondary patency 100% (4 months, range:3.8-40.8). Survival at follow-up was 90.4%, with one unrelated death. One patient underwent liver transplantation, three became eligible post-recanalization.

PVR-TIPS proves effective and safe in reducing portal pressure gradient, thereby alleviating PH symptoms without evidence of portal thrombosis progression in non-cirrhotic patients with CPVO and portal cavernoma. It expands therapeutic options, including liver transplantation.

Portal venous system thrombosis (PVT) outside the setting of cirrhosis is uncommon with limited information available about the etiological and clinical characteristics across varied racial and ethnic groups.

This retrospective cohort study examines the long-term outcomes of non-cirrhotic, Hispanic adults diagnosed with PVT at a single center in Mexico City between January 2000 and August 2023. Patients with conditions predisposing to PVT were excluded.

We included 100 Hispanic adults diagnosed with non-cirrhotic PVT. Thrombophilia was identified in 49 %, with antiphospholipid syndrome (APS) being the most prevalent thrombophilia (23 %), followed by JAK2 mutation (18 %). Chronic PVT, observed in 70 % of cases, predominantly affected the portal vein (50 %), followed by porto-mesenteric (41 %) and porto-splenic (9 %) territories. At diagnosis, 55 % had esophageal varices. Anticoagulant therapy was administered to over half of the patients for >12 months. Over a median follow-up of 55 months, the 5-year risk of re-thrombosis was 24 %, and the 5-year risk of variceal bleeding (VB) was 45 %. The 4-year overall survival (OS) was 97 %. Comparative analysis between thrombophilia-associated and idiopathic PVT did not reveal significant differences in VB, re-thrombosis, and OS.

This study underscores the unique clinical profile of Hispanic patients with non-cirrhotic PVT, highlighting a high prevalence of APS and substantial risks of VB. These findings contribute to a better understanding of PVT in Hispanics and emphasize the importance of tailored management strategies. The generalizability of our results may be limited by the thrombophilia testing approach, the excluded populations, and its retrospective, single center nature.

Splanchnic vein thrombosis (SpVT) is an uncommon site of venous thrombosis that is associated with complications including portal hypertension and hepatic dysfunction. The evaluation and management of this patient population has evolved in recent years, but there are limited data from clinical trials to guide management. Given the various acquired and biologic risk factors leading to the development of SpVT and the potential complications that can arise from it, input from a multidisciplinary team can be valuable in managing such patients, including thrombosis specialists or hematologists, gastroenterologists or hepatologists, interventional radiologists, and surgeons. In this article, we present 4 cases that highlight important issues and considerations in the evaluation of SpVT, including initial diagnostic approach in a patient with a new diagnosis of SpVT, considerations for anticoagulant therapy, management of SpVT in patients with myeloproliferative neoplasms, and the role of interventional vascular procedures in the management of SpVT. By reviewing the current literature, we address clinically relevant questions that are posed to clinicians managing patients with SpVT; we also point out gaps in our current knowledge that merit future investigation.

Portal vein thromboses (PVTs) are common in patients with cirrhosis and are associated with advanced portal hypertension and mortality. The treatment of PVTs remains a clinical challenge due to limited evidence and competing risks of PVT-associated complications vs bleeding risk of anticoagulation. Significant heterogeneity in PVT phenotype based on anatomic, host, and disease characteristics, and an emerging spectrum of therapeutic options further complicate PVT management. This Clinical Practice Update (CPU) aims to provide best practice advice for the evaluation and management of PVT in cirrhosis, including the role of direct oral anticoagulants and endovascular interventions.

This expert review was commissioned and approved by the American Gastroenterological Association (AGA) Institute CPU Committee and the AGA Governing Board to provide timely guidance on a topic of high clinical importance to the AGA membership, and underwent internal peer review by the CPU Committee and external peer review through standard procedures of Gastroenterology. These Best Practice Advice statements were drawn from a review of the published literature and from expert opinion. Because systematic reviews were not performed, these Best Practice Advice statements do not carry formal ratings regarding the quality of evidence or strength of the presented considerations. Best Practice Advice Statements BEST PRACTICE ADVICE 1: Asymptomatic patients with compensated cirrhosis do not require routine screening for PVT. BEST PRACTICE ADVICE 2: Patients with cirrhosis with PVTs identified on Doppler ultrasound should undergo cross-sectional imaging with computed tomography or magnetic resonance imaging to confirm the diagnosis, evaluate for malignancy, and document the degree of lumen occlusion, clot extent, and chronicity. BEST PRACTICE ADVICE 3: Patients with cirrhosis and PVT do not require a hypercoagulable workup in the absence of additional thromboemboli or laboratory abnormalities or family history suggestive of thrombophilia. BEST PRACTICE ADVICE 4: Patients with cirrhosis and PVT with evidence of intestinal ischemia require urgent anticoagulation to minimize ischemic injury. If available, these patients should be managed by a multidisciplinary team, including gastroenterology and hepatology, interventional radiology, hematology, and surgery. BEST PRACTICE ADVICE 5: Consider observation, with repeat imaging every 3 months until clot regression, in patients with cirrhosis without intestinal ischemia and recent (<6 months) thrombosis involving the intrahepatic portal vein branches or when there is <50% occlusion of the main portal vein, splenic vein, or mesenteric veins. BEST PRACTICE ADVICE 6: Anticoagulation should be considered in patients with cirrhosis without intestinal ischemia who develop recent (<6 months) PVT that is >50% occlusive or involves the main portal vein or mesenteric vessels. Patients who have increased benefit of recanalization include those with involvement of more than 1 vascular bed, those with thrombus progression, potential liver transplantation candidates, and those with inherited thrombophilia. BEST PRACTICE ADVICE 7: Anticoagulation is not advised for patients with cirrhosis with chronic (>6 months) PVT with complete occlusion with collateralization (cavernous transformation). BEST PRACTICE ADVICE 8: Patients with cirrhosis and PVT warrant endoscopic variceal screening if they are not already on nonselective beta-blocker therapy for bleeding prophylaxis. Avoid delays in the initiation of anticoagulation for PVT, as this decreases the odds of portal vein recanalization. BEST PRACTICE ADVICE 9: Vitamin K antagonists, low-molecular-weight heparin, and direct oral anticoagulants are all reasonable anticoagulant options for patients with cirrhosis and PVT. Decision making should be individualized and informed by patient preference and Child-Turcotte-Pugh class. Direct oral anticoagulants may be considered in patients with compensated Child-Turcotte-Pugh class A and Child-Turcotte-Pugh class B cirrhosis and offer convenience as their dosages are independent of international normalized ratio monitoring. BEST PRACTICE ADVICE 10: Patients with cirrhosis on anticoagulation for PVT should have cross-sectional imaging every 3 months to assess response to treatment. If clot regresses, anticoagulation should be continued until transplantation or at least clot resolution in nontransplantation patients. BEST PRACTICE ADVICE 11: Portal vein revascularization with transjugular intrahepatic portosystemic shunting may be considered for selected patients with cirrhosis and PVT who have additional indications for transjugular intrahepatic portosystemic shunting, such as those with refractory ascites or variceal bleeding. Portal vein revascularization with transjugular intrahepatic portosystemic shunting may also be considered for transplantation candidates if recanalization can facilitate the technical feasibility of transplantation.

Non-cirrhotic portal vein thrombosis (PVT) is rare, and early initiation of anticoagulation therapy is crucial for recanalization and preventing complications. A man in his 50s presented to the emergency department with acute back pain. His vital signs and laboratory results were normal, showing no signs of infection. An initial computed tomography (CT) scan with intravenous contrast in the arterial phase showed no abnormalities. However, on the third day, a CT scan in the late phase confirmed PVT in the left branch, along with gallbladder infarction. This case highlights the limitations of arterial-phase CT in diagnosing acute PVT, which often extends to the splenic vein or superior mesenteric venous arches and may lead to intestinal infarction, although gallbladder infarctions remain rare.

The role of anticoagulation in asymptomatic cirrhotic patients with portal vein thrombosis (PVT) remains unclear. This study aims to evaluate the efficacy and safety of anticoagulation in this patient population.

We systematically searched PubMed, Web of Science, Cochrane Library, and Embase up to August 2024. The primary outcomes analyzed were PVT recanalization, progression of PVT, bleeding events, and mortality. Odds ratios (OR) with 95% confidence intervals (CI) were calculated for dichotomous variables.

Seventeen studies, including randomized controlled trials (RCTs) and observational studies, were included in the analysis. Compared to no intervention, anticoagulation significantly increased the PVT recanalization rate (OR = 3.89, p < .001) and decreased the PVT progression rate (OR = 0.28, p < .001) as well as overall mortality (OR = 0.66, p = .008). Importantly, anticoagulation did not significantly increase the bleeding rate (OR = 1.21, p = .41). Subgroup analysis revealed a greater benefit in PVT recanalization within the short-term treatment subgroup (≤ 6 months) compared to long-term treatment subgroup (> 6 months), and in the Asian subgroup compared to the European or United States of America (USA) subgroup. In the Warfarin subgroup, while the total bleeding rate increased significantly, there was no significant rise in major bleeding events. Additionally, a downward trend in variceal bleeding was observed in the Asian subgroup (OR = 0.44; 95% CI: 0.19-1.04; p = .06).

Anticoagulation is both safe and effective for asymptomatic cirrhotic patients with PVT. It not only treats PVT and reduces all-cause mortality, but also does so without significantly increasing the risk of bleeding events.

Acute portomesenteric venous thrombosis (PVT) is a rare but potentially life-threatening condition in individuals without cirrhosis. Initial management typically involves anticoagulation therapy, but the optimal approach to interventional treatment remains a topic of ongoing research. This article explores both traditional and emerging endovascular techniques, providing an overview of the existing evidence supporting their use. Additionally, it delves into the significance of acute PVT in the context of contemporary pathologies, notably coronavirus disease 2019 infection, vaccine-induced immune thrombotic thrombocytopenia, and liver transplantation.

In patients with noncirrhotic chronic extrahepatic portal vein obstruction (EHPVO), data on the morbimortality of abdominal surgery are scarce.

We retrospectively analyzed the charts of 76 patients (78 interventions) with EHPVO undergoing abdominal surgery within the Vascular Disease Interest Group network. Fourteen percent of the patients had ≥1 major bleeding (unrelated to portal hypertension) and 21% had ≥1 Dindo-Clavien grade ≥3 postoperative complications within 1 month after surgery. Fifteen percent had ≥1 portal hypertension-related complication within 3 months after surgery. Three patients died within 12 months after surgery. An unfavorable outcome (ie, ≥1 abovementioned complication or death) occurred in 37% of the patients and was associated with a history of ascites and with nonwall, noncholecystectomy surgical intervention: 17% of the patients with none of these features had an unfavorable outcome, versus 48% and 100% when one or both features were present, respectively. We then compared 63/76 patients with EHPVO with 126 matched (2:1) control patients without EHPVO but with similar surgical interventions. As compared with control patients, the incidence of major bleeding ( p <0.001) and portal hypertension-related complication ( p <0.001) was significantly higher in patients with EHPVO, but not that of grade ≥3 postoperative complications nor of death. The incidence of unfavorable postoperative outcomes was significantly higher in patients with EHPVO than in those without (33% vs. 18%, p =0.01).

Patients with EHPVO are at high risk of major perioperative or postoperative bleeding and postoperative complications, especially in those with ascites or undergoing surgery other than wall surgery or cholecystectomy.

Portal hypertension is a hemodynamic abnormality that complicates the course of cirrhosis, as well as other diseases that affect the portal venous circulation. The development of portal hypertension compromises prognosis, especially when it rises above a certain threshold known as clinically significant portal hypertension (CSPH). In the consensus conference on Portal Hypertension promoted by the Spanish Association for the Study of the Liver and the Hepatic and Digestive diseases area of the Biomedical Research Networking Center (CIBERehd), different aspects of the diagnosis and treatment of portal hypertension caused by cirrhosis or other diseases were discussed. The outcome of this discussion was a set of recommendations that achieved varying degrees of consensus among panelists and are reflected in this consensus document. The six areas under discussion were: the relevance of CSPH and the non-invasive methods used for its diagnosis and that of cirrhosis, the prevention of the first episode of decompensation and its recurrence, the treatment of acute variceal bleeding and other complications of portal hypertension, the indications for the use of TIPS, and finally, the diagnosis and treatment of liver vascular diseases.

Portal hypertension is a hemodynamic abnormality that complicates the course of cirrhosis, as well as other diseases that affect the portal venous circulation. The development of portal hypertension compromises prognosis, especially when it rises above a certain threshold known as clinically significant portal hypertension (CSPH). In the consensus conference on Portal Hypertension promoted by the Spanish Association for the Study of the Liver and the Hepatic and Digestive diseases area of the Biomedical Research Networking Center (CIBERehd), different aspects of the diagnosis and treatment of portal hypertension caused by cirrhosis or other diseases were discussed. The outcome of this discussion was a set of recommendations that achieved varying degrees of consensus among panelists and are reflected in this consensus document. The six areas under discussion were: the relevance of clinically significant portal hypertension and the non-invasive methods used for its diagnosis and that of cirrhosis, the prevention of the first episode of decompensation and its recurrence, the treatment of acute variceal bleeding and other complications of portal hypertension, the indications for the use of TIPS, and finally, the diagnosis and treatment of liver vascular diseases.

Background: Obesity and type 2 diabetes mellitus (T2DM) are modern-day pandemics that have a significant impact on global healthcare. The glucagon-like peptide-1 receptor agonist (GLP1-RA) semaglutide is a novel treatment for both tbl2DM and obesity, but can be associated with an increased risk of venous thromboembolism. Case presentation: This case report describes a 59-year-old woman with tbl2DM who received semaglutide to manage glycemic levels, and also experienced the additional benefit of weight reduction. Within six months of initiating GLP1-RA, the patient experienced low back pain associated with nausea and poor oral intake. She had no known risk factors for venous thromboembolism or thrombophilia and had no history of significant illness in her family. Her physical examination revealed no significant findings. Only mild leukocytosis and neutrophilia were noted. She underwent an abdominal computed tomography scan, which revealed intrahepatic portal vein thrombosis without evidence of liver cirrhosis or abdominal malignancy. Her symptoms improved with oral anticoagulation (rivaroxaban). The result of thrombophilia examination was negative for inherited or acquired thrombophilia, except for a Janus kinase 2 mutation, which may increase the risk of thrombosis. Conclusions: The use of GLP1-RA is increasing due to the growing desire for weight loss medications. Therefore, it is important that physicians better understand the possible risks of thrombosis before initiating GLP1-RA treatment.

Extrahepatic portal vein obstruction (EHPVO) is a rare disease-causing form of portal hypertension. Myeloproliferative neoplasm (MPN) including essential thrombocythemia (ET) is a reported risk factor for EHPVO due to underlying persistent thrombophilia. A Japanese woman in her 40s was referred to our hospital with a 1-month history of gastric variceal bleeding due to EHPVO. Laboratory investigation showed thrombocytosis despite portal hypertension. She had a mutation in clonal marker JAK2V617F with EHPVO, which prompted us to consult a hematologist. A bone marrow biopsy revealed megakaryocyte lineage proliferation, which confirmed a diagnosis of ET. Esophagogastroduodenoscopy revealed esophagogastric varices (LsF2CbRC2, Lg-cF1RC1), and abdominal computed tomography and angiography revealed splenomegaly and portal vein thrombosis with cavernous transformation, which suggested EHPVO. The patient had a history of ruptured esophagogastric varices and required prophylaxis against further variceal bleeding before antithrombotic therapy for EHPVO with ET. We performed laparoscopic Hassab's operation followed by endoscopic variceal ligation (EVL) and hematological cytoreduction therapy. Laparoscopic Hassab's operation and three bi-monthly EVL procedures improved the esophagogastric varix (LmF0RC0, Lg-f F0RC0) at 6 months after surgery. Cytoreduction therapy reduced platelet count to 60.1 × 104/uL, and the patient was very healthy at 7 months after surgery. Patients with EHPVO are traditionally referred to a gastroenterologist for abdominal pain, intestinal bleeding, or refractory ascites; however, hypercoagulative disease may be occult in such patients and require the attention of a hematologist. When treating patients with EHPVO, gastroenterologists should screen for hematological disease, including MPN.

 Portal vein system thrombosis (PVST) is a frequent and possibly fatal concurrent disorder following splenectomy. The optimal anticoagulant to prevent PVST following splenectomy remains unclear.

 The purpose of this study was to compare the safety and efficacy of apixaban versus aspirin in preventing PVST after laparoscopic splenectomy (LS) for cirrhotic hypersplenism.

 In this single-center randomized controlled trial, 80 patients with liver cirrhosis who received LS were randomly allocated to two treatment arms that were treated with apixaban or aspirin for 6 months. The primary effectiveness outcome was PVST formation after LS.

 We excluded four patients who withdrew from the study. The dynamic incidence of PVST, main and intrahepatic branches of PVST, and splenic vein thrombosis in the 6 postoperative months were all significantly lower in the apixaban treatment arm compared to the aspirin treatment arm (all P <0.001). Significantly lower incidences of PVST, main and intrahepatic branches of PVST, and splenic vein thrombosis in apixaban treatment arm started from postoperative day 7, month 1, and day 7 compared to the aspirin treatment arm respectively (all P <0.05). Multiple logistic regression analysis revealed that apixaban was an independent protective factor for PVST at postoperative month 3, as compared with aspirin (relative risk, 0.057; 95% confidence interval, 0.013-0.248; P <0.001).

 Compared with aspirin, apixaban could earlier and more effectively prevent PVST following LS for cirrhotic hypersplenism. Apixaban can be chosen as a priority treatment option versus aspirin, contributing to a lower risk of PVST.

Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy. An international working group with extensive expertise in portal hypertension was assigned with formulating consensus guidelines to clarify the definition, diagnosis, histological features, natural history, and management of NCPF/IPH, especially in the context of PSVD. The guidelines were prepared based on evidence from existing published literature. Whenever there was paucity of evidence, expert opinion was included after detailed deliberation. The goal of this manuscript, therefore, is to enhance the current understanding and help create global consensus on the issues surrounding NCPF/IPH.

Since the introduction of SARS-CoV-2 vaccines, several cases of vaccine-induced immune thrombocytopenia and thrombosis (VITT) have been described, especially cerebral vein thrombosis. We aimed to retrospectively collect all new cases of acute onset first or recurrent splanchnic vein thrombosis (SVT) following a recent SARS-CoV-2 vaccination within the Vascular Liver Disease Group network.

New cases of SVT were identified from April 2021 to April 2022; follow-up was completed on December 31, 2022. Criteria to define VITT were derived from previous studies. Data from a pre-COVID cohort of patients with SVT (N=436) were used for comparison of clinical presentation, etiology, and outcome. Twenty-nine patients were identified with SVT occurring with a median of 11 days (range 2-76) after the first (48%), second (41%), or third (10%) vaccination (ChAdOx1 nCov-19 (n=12) or BNT162b2 (n=14), other (n=3) Only 2 patients(7%) fulfilled criteria for definite VITT. Twenty (69%) had SVT at multiple sites, including 4 (14%) with concomitant extra-abdominal thrombosis. Only 28% had an underlying prothrombotic condition, compared to 52% in the pre-COVID SVT cohort ( p =0.01). Five patients (17%) underwent bowel resection for mesenteric ischemia, compared with 3% in pre-COVID SVT ( p <0.001). Two patients died shortly after diagnosis (7%).

Although definite VITT was rare, in 72% of cases, no other cause for SVT could be identified following SARS-CoV-2 vaccination. These cases were different from patients with nonvaccine-related SVT, with lower incidence of prothrombotic conditions, higher rates of bowel ischemia, and poorer outcome. Although SVT after SARS-CoV-2 vaccination is rare in absolute terms, these data remain relevant considering ongoing revaccination programs.

One-third of non-cirrhotic portal vein thrombosis (NCPVT) cases are associated with local factors. The risk of rethrombosis after anticoagulation withdrawal is unknown. We aimed to determine factors associated with new splanchnic or extrasplanchnic thrombotic events in this setting.

We performed a retrospective study including cases of recent NCPVT associated with local factors. High- and low-risk prothrombotic factors, prespecified according to RIPORT study criteria, were assessed. Univariate and multivariate Cox models assessed the influence of different variables on the occurrence of new thrombotic events.

At baseline, 83/154 (53.9%) patients had at least one prothrombotic factor including 50 (32.5%) with a high-risk and 33 (21.4%) with a low-risk prothrombotic factor. Oestrogen-containing contraception was discontinued in all patients. During follow-up, 63/140 (45%) patients had at least one prothrombotic factor, including 47 (33.6%) with a high-risk and 16 (11.4%) with a low-risk prothrombotic factor. Seventeen new thrombotic events occurred after a median follow-up of 52 (IQR 14-62) (min-max 3.0-69.0) months. New thromboses were associated with high-risk factors (hazard ratio [HR] 3.817, 95% CI 1.303-11.180, p = 0.015), but were inversely related to recanalization (HR 0.222, 95% CI 0.078-0.635, p = 0.005) and anticoagulation (HR 0.976, 95% CI 0.956-0.995, p = 0.016). When a high-risk factor was present a new thrombotic event occurred in 7.4%, 14.6%, 14.6% and 28.8% of patients at 1, 3, 5 and 7 years under anticoagulants, respectively, compared to 21.2%, 21.2%, 58% and 58% without anticoagulants, respectively.

In cases of recent NCPVT associated with local factors, high-risk factors for thrombosis are associated with new thrombotic events. Permanent anticoagulation appears beneficial in this high-risk situation.

In non-cirrhotic portal vein thrombosis (NCPVT) associated with local factors, systematic screening for prothrombotic factors is recommended, but the prevalence of the latter is not clearly established, and the risk of recurrent intra or extrasplanchnic thromboembolism is poorly described. Thus, interest in permanent anticoagulation remains. NCPVT associated with local factors is a matter of concern for hepatologists, gastroenterologists and digestive surgeons. Due to a lack of knowledge, practices are heterogeneous. Our findings highlight that systematic screening for prothrombotic factors in NCPVT is needed even when associated with local factors, as it may justify long-term anticoagulation for the prevention of new intra or extrasplanchnic thrombotic events in at least one-third of cases. The interest in long-term anticoagulation should be investigated prospectively in the absence of high-risk prothrombotic factors.

NCT0536064.

Portal vein thrombosis (PVT) refers to the development of a non-malignant obstruction of the portal vein, its branches, its radicles, or a combination. This Review first provides a comprehensive overview of all aspects of PVT, namely the specifics of the portal venous system, the risk factors for PVT, the pathophysiology of portal hypertension in PVT, the interest in non-invasive tests, as well as therapeutic approaches including the effect of treating risk factors for PVT or cause of cirrhosis, anticoagulation, portal vein recanalisation by interventional radiology, and prevention and management of variceal bleeding in patients with PVT. Specific issues are also addressed including portal cholangiopathy, mesenteric ischaemia and intestinal necrosis, quality of life, fertility, contraception and pregnancy, and PVT in children. This Review will then present endpoints for future clinical studies in PVT, both in patients with and without cirrhosis, agreed by a large panel of experts through a Delphi consensus process. These endpoints include classification of portal vein thrombus extension, classification of PVT evolution, timing of assessment of PVT, and global endpoints for studies on PVT including clinical outcomes. These endpoints will help homogenise studies on PVT and thus facilitate reporting, comparison between studies, and validation of future studies and trials on PVT.

Extrahepatic portal vein obstruction (EHPVO) is a rare disease with myeloproliferative neoplasm (MPN) as the most common cause. We report that hypersplenic hematologic changes in EHPVO might be eliminated by MPN. Through experience with splenectomy for variceal control with EHPVO, we suspected that spleen might mask MPN-induced thrombocytosis, and that MPN might have a significant influence on excessive thrombocytosis after splenectomy. To clarify the influence of MPN and spleen on platelet trends, we conducted a retrospective hospital database analysis, evaluating 8 EHPVO patients with splenectomy (2 males, 6 females; from 17 years to 64 years, mean 38.3 years). Three (37.5%) of 8 were diagnosed as MPN by JAK2V617F mutation. The perioperative serum platelet counts in EHPVO without MPN were 10.5, 35.4, and 36.6 (x104/μL) preoperatively, after 1 week and 3 weeks, respectively. The platelet counts in EHPVO with MPN were 34.2, 86.4, and 137.0 (x104/μL), respectively. Splenectomy and MPN showed positive interaction on platelet increasing with statistical significance. We also examined the spleen volume index (SpVI: splenic volume (cm3) / body surface area (m2) and postoperative platelet elevations ratio (PER: 3-week postoperative platelet counts / preoperative platelet counts). However, both SpVI and PER showed no significant difference with or without MPN. Histological examination revealed splenic congestion in all 8 EHPVO cases, and splenic extramedullary hematopoiesis in 2 of 3 MPN. In EHPVO with MPN, hypersplenism causes feigned normalization of platelet count by masking MPN-induced thrombocytosis; however, splenectomy unveils postoperative thrombocytosis. Spleen in EHPVO with MPN also participates in extramedullary hematopoiesis.

Portal vein thrombosis (PVT) is a common complication of liver cirrhosis, yet there are fewer studies about predictors of PVT recanalization. We aimed to further explore the predictors of recanalization in cirrhotic PVT to facilitate accurate prediction of patients' clinical status and timely initiation of appropriate treatment and interventions. To further investigate the benefits and risks of anticoagulant therapy in cirrhotic PVT patients.

A retrospective cohort study of patients with cirrhotic PVT in our hospital between January 2016 and December 2022, The primary endpoint was to analyze predictors of PVT recanalization by COX regression. Others included bleeding rate, liver function, and mortality.

This study included a total of 82 patients, with 30 in the recanalization group and 52 in the non-recanalization group. Anticoagulation therapy was the only independent protective factor for portal vein thrombosis recanalization and the independent risk factors included massive ascites, history of splenectomy, Child-Pugh B/C class, and main trunk width of the portal vein. Anticoagulation therapy was associated with a significantly higher rate of PVT recanalization (75.9% vs. 20%, log-rank P < 0.001) and a lower rate of PVT progression (6.9% vs. 54.7%, log-rank P = 0.002). There was no significant difference between different anticoagulation regimens for PVT recanalization. Anticoagulation therapy did not increase the incidence of bleeding complications(P = 0.407). At the end of the study follow-up, Child-Pugh classification, MELD score, and albumin level were better in the anticoagulation group than in the non-anticoagulation group. There was no significant difference in 2-year survival between the two groups.

Anticoagulation, massive ascites, history of splenectomy, Child-Pugh B/C class, and main portal vein width were associated with portal vein thrombosis recanalization. Anticoagulation may increase the rate of PVT recanalization and decrease the rate of PVT progression without increasing the rate of bleeding. Anticoagulation may be beneficial in improving liver function in patients with PVT in cirrhosis.

The aims of this study were to determine whether human umbilical cord mesenchymal stem cells (hucMSCs) modified by miRNA-25-3p (miR-25-3p) overexpression could promote venous endothelial cell proliferation and attenuate portal endothelial cell injury. HucMSCs and human umbilical vein endothelial cells (HUVEC) were isolated and cultured from human umbilical cord and characterized. Lentiviral vectors expressing miRNA-25-3p were transfected into hucMSCs and confirmed by PCR. We verified the effect of miR-25-3p-modified hucMSCs on HUVEC by cell co-culture and cell supernatant experiments. Subsequently, exosomes of miR-25-3p-modified hucMSCs were isolated from cell culture supernatants and characterized by WB, NTA and TEM. We verified the effects of miR-25-3p-modified exosomes derived from hucMSCs on HUVEC proliferation, migration, and angiogenesis by in vitro cellular function experiments. Meanwhile, we further examined the downstream target genes and signaling pathways potentially affected by miR-25-3p-modified hucMSC-derived exosomes in HUVEC. Finally, we established a rat portal vein venous thrombosis model by injecting CM-DiR-labeled hucMSCs intravenously into rats and examining the homing of cells in the portal vein by fluorescence microscopy. Histological and immunohistochemical experiments were used to examine the effects of miRNA-25-3p-modified hucMSCs on the proliferation and damage of portal vein endothelial cells. Primary hucMSCs and HUVECs were successfully isolated, cultured and characterized. Primary hucMSCs were modified with a lentiviral vector carrying miR-25-3p at MOI 80. Co-culture and cell supernatant intervention experiments showed that overexpression of miRNA-25-3p in hucMSCs enhanced HUVEC proliferation, migration and tube formation in vitro. We successfully isolated and characterized exosomes of miR-25-3p-modified hucMSCs, and exosome intervention experiments demonstrated that miR-25-3p-modified exosomes derived from hucMSCs similarly enhanced the proliferation, migration, and angiogenesis of HUVECs. Subsequent PCR and WB analyses indicated PTEN/KLF4/AKT/ERK1/2 as potential pathways of action. Analysis in a rat portal vein thrombosis model showed that miR-25-3p-modified hucMSCs could homing to damaged portal veins. Subsequent histological and immunohistochemical examinations demonstrated that intervention with miR-25-3p overexpression-modified hucMSCs significantly reduced damage and attenuated thrombosis in rat portal veins. The above findings indicate suggest that hucMSCs based on miR-25-3p modification may be a promising therapeutic approach for use in venous thrombotic diseases.

Alveolar Echinococcosis (AE) is a malignant zoonotic disease caused by Echinococcus multilocularis infection. Considering whether the lesion is accompanied by vascular invasion (VI) is crucial for treatment strategies. A cost-effective and convenient clinical diagnostic method is urgently needed to supplement current techniques. Consequently, we detected soluble CD155 (sCD155) as a potential biomarker for diagnosing VI in hepatic alveolar echinococcosis (HAE).

Blood samples were from 42 AE patients and 49 healthy controls (HCs). Based on the computed tomography (CT) and contrast-enhanced CT, AE patients were further categorized into HAE with VI (VIAE; 27 cases) and HAE without VI (NVAE; 15 cases). The sCD155 concentration was measured by an enzyme-linked immunosorbent assay (ELISA). Correlations between sCD155 expression levels and clinicopathological features of AE patients were analyzed using SPSS and GraphPad Prism software.

The sCD155 concentrations in AE patients were significantly higher than in HCs. The serum sCD155 level significantly differed between the VIAE and NVAE groups. The univariate analysis showed that VI of AE was significantly correlated with the sCD155 level when the sCD155 was greater than 11 ng/mL. After adjusting for potential confounding factors, the multivariable analysis showed that sCD155 had an independent effect on VI of HAE. The receiver operating characteristic (ROC) curve showed that sCD155 could differentially diagnose VI of HAE at the cut-off value of 11.08 ng/mL with an area under the curve (AUC) value of 0.75. The sensitivity and specificity were 74.07 % and 66.67 %, respectively; the positive and negative predictive values were 74.07 % and 60.00 %, respectively.

The sCD155 could be a VI biomarker for HAE. Elevated sCD155 levels are indicative of an increased likelihood of concomitant VI in HAE patients, necessitating a thorough evaluation of vascular impairment and the formulation of individualized management strategies.

Portal vein thrombosis (PVT) is a rare condition, particularly in non-cirrhotic patients. Anticoagulation remains the mainstay of the treatment. Extensive PVT can lead to variceal bleeding, ascites, bowel ischemia, and hypersplenism. The role of thrombolysis and thrombectomy in these patients remains unclear. However, there is evidence that local thrombolysis and thrombectomy should be considered in those who remain symptomatic on anticoagulation and are at risk of complications with acute PVT.

Anticoagulant therapy with heparin is the first-line treatment for acute mesenteric vein thrombosis and is effective in improving outcomes. Conversely, patients with failed early anticoagulant therapy occasionally develop bowel infarction requiring surgery. The efficacy of long-term anticoagulant therapy on recanalizing mesenteric vein thrombosis in patients with failed early anticoagulant therapy remains unclear. Herein, we report a patient who achieved recanalization of port-superior mesenteric vein thrombosis treated with anticoagulant therapy for 10 years after failed early anticoagulant therapy, followed by bowel resection.

A 38-year-old male patient visited an outpatient clinic due to acute exacerbation of abdominal pain that had persisted for a month. He was diagnosed with port-superior mesenteric vein thrombosis on contrast-enhanced computed tomography (CT) scan and was transferred to our institution. Although he presented with abdominal pain, his respiration and circulation were stable upon hospital arrival. Anticoagulant therapy with heparin was started, and the patient was admitted to the intensive care unit. However, the patient's abdominal pain worsened, and he began to develop signs of peritonitis. Repeat CT scan revealed bowel infarction. Thus, the patient underwent bowel resection 6 h after admission. The initial surgery was completed with open abdomen management. Bowel anastomosis was performed on the second-look surgery on the first postoperative day. Finally, the abdomen was closed on the third postoperative day after confirming the absence of bowel ischemia progression. The patient had prolonged impaired bowel function with paralytic ileus, but was discharged on the 60th postoperative day. He was then diagnosed with protein C and S deficiency based on the tests performed. Anticoagulant therapy with warfarin was initiated. He also received anticoagulant therapy in the outpatient setting. The patient's port-superior mesenteric vein thrombosis had improved gradually with warfarin during the follow-up period. At 10 years after surgery, total occlusion of the port-superior mesenteric vein was recanalized with improvement of the portal collateral vessels. In addition, no gastric or esophageal varices were observed.

Long-term anticoagulation therapy could affect the recanalization of extensive thrombus in multiple segments in patients with mesenteric venous thrombosis.

Non-cirrhotic non-malignant portal vein thrombosis (NCPVT) is an uncommon condition characterised by thrombosis of the portal vein, with or without extension into other mesenteric veins, in the absence of cirrhosis or intra-abdominal malignancy. Complications can include intestinal infarction, variceal bleeding and portal biliopathy. In this article, we address current concepts in the management of NCPVT including identification of risk factors, classification and treatment, and review the latest evidence on medical and interventional management options.

Isolated splenic vein thrombosis (iSVT) is a common complication of pancreatic disease. Whilst patients remain asymptomatic, there is a risk of sinistral portal hypertension and subsequent bleeding from gastric varices if recanalisation does not occur. There is wide variation of iSVT treatment, even within single centres. We report outcomes of iSVT from tertiary referral hepatobiliary and pancreatic (HPB) units including the impact of anticoagulation on recanalisation rates and subsequent variceal bleeding risk.

A retrospective cohort study including all patients diagnosed with iSVT on contrast-enhanced CT scan abdomen and pelvis between 2011 and 2019 from two institutions. Patients with both SVT and portal vein thrombosis at diagnosis and isolated splenic vein thrombosis secondary to malignancy were excluded. The outcomes of anticoagulation, recanalisation rates, risk of bleeding and progression to portal vein thrombosis were examined using CT scan abdomen and pelvis with contrast.

Ninety-eight patients with iSVT were included, of which 39 patients received anticoagulation (40%). The most common cause of iSVT was acute pancreatitis n = 88 (90%). The recanalisation rate in the anticoagulation group was 46% vs 15% in patients receiving no anticoagulation (p = 0.0008, OR = 4.7, 95% CI 1.775 to 11.72). Upper abdominal vascular collaterals (demonstrated on CT scan angiography) were significantly less amongst patients who received anticoagulation treatment (p = 0.03, OR = 0.4, 95% CI 0.1736 to 0.9288). The overall rate of upper GI variceal-related bleeding was 3% (n = 3/98) and it was independent of anticoagulation treatment. Two of the patients received therapeutic anticoagulation.

The current data supports that therapeutic anticoagulation is associated with a statistically significant increase in recanalisation rates of the splenic vein, with a subsequent reduction in radiological left-sided portal hypertension. However, all patients had a very low risk of variceal bleeding regardless of anticoagulation. The findings from this retrospective study should merit further investigation in large-scale randomised clinical trials.

Splanchnic or visceral vein thromboses (VVTs) are atypical thrombotic entities and include thrombosis of the portal vein, hepatic veins (Budd-Chiari syndrome), mesenteric veins, and splenic vein. All VVTs have in common high 30-day mortality up to 20% and it seems to be difficult to diagnose VVT early because of their rarity and their wide spectrum of unspecific symptoms. VVTs are often associated with myeloproliferative neoplasia, thrombophilia, and liver cirrhosis. VVT is primarily diagnosed by sonography and/or computed tomography. In contrast to venous thromboembolism, D-dimer testing is neither established nor helpful. Anticoagulation is the first-line therapy in patients with stable circulation and no evidence of organ complications. Anticoagulation improves significantly recanalization rates and stops the progress of thrombosis. Low-molecular-weight heparin, vitamin K antagonists, as well as direct-acting oral anticoagulants are possible anticoagulants, but it is noteworthy to be aware that all recommendations supporting the off-label use of anticoagulants are based on poor evidence and consist predominantly of case series, observational studies, or studies with small case numbers. When choosing a suitable anticoagulation, the individual risk of bleeding and thrombosis must be weighted very carefully. In cases of bleeding, bowel infarction, or other complications, the optimal therapy should be determined on a case-by-case basis by an experienced multidisciplinary team involving a surgeon. Besides anticoagulation, there are therapeutic options including thrombectomy, balloon angioplasty, stenting, transjugular placement of an intrahepatic portosystemic shunt, liver transplantation, and ischemic bowel resection. This article gives an overview of current diagnostic and therapeutic strategies.

Essential thrombocythemia is a Janus kinase 2 (JAK2) mutation-prevalent myeloproliferative neoplasm characterized by clonal thrombocytosis; clinical course is often indolent but might be interrupted by thrombotic or hemorrhagic complications, microcirculatory symptoms (e.g., headaches, lightheadedness, and acral paresthesias), and, less frequently, by disease transformation into myelofibrosis (MF) or acute myeloid leukemia.

In addition to thrombocytosis (platelets ≥450 × 109 /L), formal diagnosis requires the exclusion of other myeloid neoplasms, including prefibrotic MF, polycythemia vera, chronic myeloid leukemia, and myelodysplastic syndromes with ring sideroblasts and thrombocytosis. Bone marrow morphology typically shows increased number of mature-appearing megakaryocytes distributed in loose clusters.

Approximately 80% of patients express myeloproliferative neoplasm driver mutations (JAK2, CALR, MPL), in a mutually exclusive manner; in addition, about 50% harbor other mutations, the most frequent being TET2 (9%-11%), ASXL1 (7%-20%), DNMT3A (7%), and SF3B1 (5%). Abnormal karyotype is seen in <10% of patients and includes +9/20q-/13q-.

Life expectancy is less than that of the control population. Median survival is approximately 18 years but exceeds >35 years in younger patients. The triple A survival risk model, based on Age, Absolute neutrophil count, and Absolute lymphocyte count, effectively delineates high-, intermediate-1-, intermediate-2-, and low-risk disease with corresponding median survivals of 8, 14, 21, and 47 years.

Four risk categories are considered: very low (age ≤60 years, no thrombosis history, JAK2 wild-type), low (same as very low but JAK2 mutation present), intermediate (same as low but age >60 years), and high (thrombosis history or age >60 years with JAK2 mutation).

MPL and CALR-1 mutations have been associated with increased risk of MF transformation; spliceosome with inferior overall and MF-free survival; TP53 with leukemic transformation, and JAK2V617F with thrombosis. Leukemic transformation rate at 10 years is <1% but might be higher in JAK2-mutated patients with extreme thrombocytosis and those with abnormal karyotype.

The main goal of therapy is to prevent thrombosis. In this regard, once-daily low-dose aspirin is advised for all patients and twice daily for low-risk disease. Cytoreductive therapy is advised for high-risk and optional for intermediate-risk disease. First-line cytoreductive drugs of choice are hydroxyurea and pegylated interferon-α and second-line busulfan.

The current review includes specific treatment strategies in the context of extreme thrombocytosis, pregnancy, splanchnic vein thrombosis, perioperative care, and post-essential thrombocythemia MF, as well as new investigational drugs.

Background: Portal vein thrombosis (PVT) is a rare disease with an estimated incidence of 2 to 4 cases per 100,000 inhabitants. The most common predisposing conditions for PVT are chronic liver diseases (cirrhosis), primary or secondary hepatobiliary malignancy, major infectious or inflammatory abdominal disease, or myeloproliferative disorders. Methods: PVT can be classified on the basis of the anatomical site, the degree of venous occlusion, and the timing and type of presentation. The main differential diagnosis of PVT, both acute and chronic, is malignant portal vein invasion, most frequently by hepatocarcinoma, or constriction (typically by pancreatic cancer or cholangiocarcinoma). Results: The management of PVT is based on anticoagulation and the treatment of predisposing conditions. The aim of anticoagulation in acute thrombosis is to prevent the extension of the clot and enable the recanalization of the vein to avoid the development of complications, such as intestinal infarction and portal hypertension. Conclusions: The treatment with anticoagulant therapy favors the reduction of portal hypertension, and this allows for a decrease in the risk of bleeding, especially in patients with esophageal varices. The anticoagulant treatment is generally recommended for at least three to six months. Prosecution of anticoagulation is advised until recanalization or lifelong if the patient has an underlying permanent pro-coagulant condition that cannot be corrected or if there is thrombosis extending to the mesenteric veins.

Transjugular intrahepatic portosystemic shunt (TIPS) emerges as a key treatment for portal hypertension (PH) complications. While international guidelines provide clear indications for its use in cirrhosis, empirical knowledge is notably scarcer in non-cirrhotic PH, particularly in nonmalignant noncirrhotic portal vein thrombosis (NNPVT) and in patients with portosinusoidal vascular disorder (PSVD). Patients afflicted by these rare diseases exhibit distinct clinical profiles compared to their cirrhotic counterparts, often characterized by a younger age, predominantly preserved hepatic functionality even in cases of severe PH, and a higher propensity for extensive splanchnic thrombosis, which intricately complicates TIPS placement, posing unique challenges for its creation. The objective of this review is to synthesize existing literature on the effectiveness, safety, specific indications, and clinical outcomes of TIPS in adult patients with NNPVT or PSVD, focusing also on the technical challenges of TIPS insertion in the presence of portal cavernoma.

Autosomal dominant polycystic kidney disease (ADPKD) often involves polycystic liver disease (PLD). In severe cases, PLD can develop various complications. However, fatal acute portal vein thrombosis (APVT) associated with PLD has not been reported. A 64-year-old male reported mild consciousness disorder. He had been under maintenance hemodialysis for end-stage renal disease due to ADPKD with PLD. Because of recurring hepatic cyst infections, he had sustained high levels of C-reactive protein. Regarding the mild consciousness disorder, a diagnosis of hepatic encephalopathy was made based on an elevation of serum ammonia without any other abnormal liver function tests. Several days after his admission, hepatobiliary enzymes elevated, and acute liver failure progressed. Enhanced abdominal computed tomography suggested the possibility of complete occlusion of the portal vein by a thrombus. Based on an absence of obvious portosystemic collaterals, a diagnosis of APVT was made. The patient died 19 days after admission. Patients with PLD with repeated cystic infections have been seen to develop liver failure, and APVT formation may be one cause of the rapid progression of fatal liver failure. In conclusion, this is the first paper to report on the involvement of APVT in patients with PLD.

This study aims to evaluate the long-term patency of transjugular intrahepatic portosystemic shunt (TIPS) and determine the predictors of shunt dysfunction in patients with chronic portal vein occlusion (CPVO).

This retrospective study was conducted from December 2010 to December 2020 in patients with portal hypertension and CPVO. Patients were followed up from initial TIPS insertion to December 2022 or death. Details of TIPS procedure, adverse events and clinical outcomes were recorded. The cumulative rate of shunt patency was calculated by the Kaplan-Meier method and compared by using the log-rank test. Independent predictors of shunt dysfunction were calculated with the Cox regression model. A nomogram comprising independent variables was developed to enhance the predictive accuracy of shunt patency.

One hundred six patients (mean age, 45.3 years ± 13.6; 71 males and 35 females) were enrolled in the study. TIPS procedure was technically successful in 100 of 106 patients (94.3 %). The primary shunt patency rates for all 100 patients were 78.9 %, 74.7 %, 67.2 %, and 62.4 % at 6, 12, 24, and 36 months, respectively, and the overall shunt patency rates were 88.9 %, 86.8 %, 83.6 %, and 81.2 % at 6, 12, 24, and 36 months, respectively. Independent predictor of shunt dysfunction were inadequate inflow from superior mesenteric vein or splenic vein (the maximum diameter < 8 mm) and platelet count ≥ 300 × 109/L. The developed nomogram is a simple tool for accurately predicting shunt patency.

In patients with CPVO, inadequate inflow and high platelet count are important factors for TIPS dysfunction.

Extrahepatic portal vein obstruction (EHPVO) is a rare disease. Most EHPVO patients are usually referred to a gastroenterologist for intestinal bleeding and hypersplenic thrombocytopenia; however, hypercoagulative diseases may be occult in these patients and require anticoagulation. The purpose of this study was to elucidate the clinical characteristics of EHPVO. We conducted a retrospective analysis of the hospital database, evaluating the medical records of 15 patients (7 males, 8 females, mean age of onset 42.0 years, range 5-74 years). Thirteen of 15 EHPVO patients (86.7%) had intestinal varices. These included 10 esophageal (66.7%), 12 gastric (80.0%), and 6 ectopic varices (40.0%). Nine (60.0%) of 15 had a history of intestinal bleeding. Regarding comorbidities, 5 of 15 (33.3%) suffered from vascular diseases, including acute myocardial infarction, cerebral infarction, pulmonary embolism, Budd-Chiari syndrome, and mesenteric vein thrombosis. The former 3 vascular commodities manifested at less than 32 years of age. Four patients (26.7%) with JAK2V617F mutation were diagnosed as myeloproliferative neoplasm (MPN). 72.3% of EHPVO patients without MPN experienced thrombocytopenic state. No EHPVO patients with MPN experienced thrombo-leukocytopenia. The elevation of white blood cell and platelet counts, and decrease of protein S were seen in EHPVO with MPN, compared with EHPVO without MPN. EHPVO is frequently associated with underlying hypercoagulative factors, causing a dilemma between thrombotic complications and portal hypertensive bleeding. Most EHPVO patients experience an evident thrombocytopenic state due to severe hypersplenism; however, hypersplenic hematologic changes are eliminated in EHPVO with MPN. MPN should be suspected in EHPVO patients negative for thrombo-leukocytopenia.