Budd-Chiari Syndrome Research Articles

The safety and efficacy of endoscopic ultrasound-guided portal pressure gradient measurement with concomitant endoscopic ultrasound-guided liver biopsy: a systematic review

IntroductionPortal hypertension (PH) is a complication of advanced liver disease. Traditionally, PH has been quantified using hepatic venous pressure gradient (HVPG) through an indirect transjugular approach requiring ionizing radiation exposure. Endoscopic ultrasound-guided porto-systemic pressure gradient (EUS-PPG) measurement is an emerging alternative, minimally invasive technique that provides direct portal pressure measurement. The aim of this systematic review is to evaluate the safety and efficacy of EUS-PPG measurement and concomitant EUS-guided liver biopsy (EUS-LB) in patients with chronic liver disease.MethodsThe preferred reporting items for systematic reviews and meta-analyses method was used. A PubMed, Medline, Web of Science, Google Scholar, and CINAHL search for terms “endoscopic ultrasound,” “EUS,” and “portal pressure gradient” was used to identify qualifying studies. Eligible studies included those which were published before 2022, reporting outcomes of EUS-PPG measurement, simultaneous EUS-LB if applicable, and adverse events rate. Risk of bias was assessed by Egger’s test. Results were synthesized using I2 to test heterogeneity.ResultsFour published studies including 147 patients met inclusion criteria, with mean age 59.6 years, 59% male. Indications for EUS-PPG measurement were history of chronic liver disease or suspected cirrhosis, viral hepatitis, alcohol associated liver disease, hepatic sinusoidal obstruction or Budd Chiari syndrome. The pooled technical success rate of EUS-PPG measurements was 98.61% with 95% confidence interval of 95.20% - 99.82%. A 25-gauge needle was used in 92% (135/147) of patients. EUS-PPG measurement was performed through a transgastric approach in all 147 (100%) patients using a compact manometer with pressure transducer and non-compressible tubing. The mean PPG was 10.07 (range 6.44 – 13.70) mmHg. Ninety-five patients underwent simultaneous EUS-LB using 19G needle with wet suction technique. Technical success rate of EUS-LB was 100% and specimen was adequate in 99% (94/95) patients to establish histological diagnosis. There were no major life-threatening complications of the EUS-PPG procedure. Predominant adverse events were abdominal pain 6.1% (9/147) and sore throat 5.4% (8/147).ConclusionEUS-PPG measurement is safe and useful in providing an assessment of portal pressure in patients with chronic liver disease. Future studies are needed to evaluate whether there is consistent correlation between EUS-PPG measurements and histologic fibrosis stage by liver biopsy.

Clinical outcomes after living donor liver transplantation for Budd-Chiari syndrome

Clinical outcomes after living donor liver transplantation for Budd-Chiari syndrome

Fulminant Hepatitis Revealing Complicated Crohn’s Disease of Budd Chiari Syndrome: About a Case

The Budd-chiari syndrome (BCS) is a rare vascular disease of multifactorial etiopathogenesis, represents a serious thromboembolic complication of Crohn's disease (CD), and may be the revealing mode. This condition results from obstruction of hepatic venous drainage from the hepatic venules to the terminal part of the inferior vena cava, and can be primary or secondary. Here, we report a case of a young woman with fulminant hepatitis whose etiological diagnosis revealed Crohn's disease with acute budd chiari syndrome and who was successfully treated with anticoagulants associated with biotherapy. The patient had a very good clinical evolution, after three months total recovery from the BCS. Very few cases have been published on this association.

Clinical Manifestations and Outcomes of Budd-Chiari Syndrome in Children: A Single-Centre Study

Clinical Manifestations and Outcomes of Budd-Chiari Syndrome in Children: A Single-Centre Study

Clinical Characteristics and Outcomes of Unusual Venous Thrombosis Sites Among Veterans with Myeloproliferative Neoplasms

Myeloproliferative Neoplasms (MPNs) may be complicated by venous thrombosis at unusual sites, which may include portal veins, hepatic veins, and cerebral sinuses. We have previously reported on the association of Agent Orange and the development of MPNs among Veterans. The incidence and timing from Service-Connected Exposures (SCE) of Veterans to the development of MPNs and thrombosis at atypical sites have not been defined. We examine the natural history, clinical characteristics, and outcomes of the Veterans with MPNs, who developed Renal Vein Thrombosis (RVT), Splanchnic Vein Thrombosis (SVT), Budd Chiari Syndrome (BCS), and Cerebral Venous Sinus Thrombosis (CVST). Utilizing the Veterans Affairs Informatics and Computing Infrastructure (VINCI) database from January 1, 2006 - January 26, 2023, Veterans with MPNs, RVT, SVT, BCS, and CVST were identified through ICD-9 and -10 codes, and, subsequently, a retrospective chart review was performed upon those Veterans with MPNs and unusual thrombosis sites. Military toxin exposures were verified through Veterans' duration and location of service. Time to MPN diagnosis refers to time from first year of cumulative SCE until year of MPN diagnosis. Among 12,352,664 Veterans, there were 435 RVT, 156 SVT and BCS, 140 CVST, and 93,269 MPNs identified. Among those with MPNs, there were 9 BCS, 4 RVT, 5 SVT, 2 RVT & SVT, and 9 CVST. CVST was observed only in Vietnam Veterans; 13/20 (65%) of RVT, SVT or BCS cases had SCE from the Gulf War (Table 1). Five/20 of the MPN subjects with RVT, SVT, or BCS had documented pre-existing thrombophilic risk factors, which included malignancy, heparin-induced thrombocytopenia, and splenectomy, whereas 2/9 CVST with MPN had associated alcohol abuse and tooth infection. All non-MPN Veterans with RVT, SVT, or BCS had thrombophilic risk factors, including malignancy, infection, and surgery. One had BCS from Paroxysmal Nocturnal Hemoglobinuria. Median time to MPN diagnosis from SCE was 20 (3-62) years with 13 (65%) thrombotic events occurring at the same time of MPN diagnosis among Veterans with RVT, SVT, or BCS. Six (30%) had their thrombosis recognized prior to their MPN diagnosis, median 6 (1-10) years, while 1 had a thrombotic event 16 years after his MPN diagnosis (Figure 1). Veterans with MPN and CVST had a substantially longer median time from SCE to the time of MPN diagnosis, 42 (24-52) years. No CVST was observed concurrent to the time of MPN diagnosis. Among Veterans with MPN and CVST, 5 (56%) had thrombosis before MPN diagnosis and 4 (44%) had thrombosis after MPN diagnosis, median 5 (2-10) years and 7 (1-21) years, respectively. Among Veterans with MPN and RVT, SVT, or BCS, 17 (85%) had portal hypertension complications, 8 (40%) had undergone endovascular interventions, and all individuals were placed on indefinite systemic anticoagulation, with warfarin most commonly prescribed. Among Veterans with BCS, 3 underwent liver transplantation. Among Veterans with CVST, 2 (22%) had concurrent CNS hemorrhage, 5 (56%) had persistent neurologic symptoms, and 1 had transformation of his MPN to Chronic Myelomonocytic Leukemia. In summary, thrombotic episodes at unusual sites among Veterans with MPNs are rare events but convey substantial morbidity and mortality, particularly among those with RVT, SVT, and BCS. We describe a significantly shorter median time to MPN diagnosis in Veterans with RVT, SVT, and BCS compared to CVST with a difference of 22 years. All MPN subjects with RVT, SVT, or BCS had JAK2 V617F mutations, with majority of thromboses noted at the time of MPN diagnosis. Future studies should include potential role of SCE in acquisition of driver mutations and CHIPs and progression of mutagenesis.

Ahemolytic PNH: Clinical Features of a Distinct Phenotype of Paroxysmal Nocturnal Hemoglobinuria

Introduction: Paroxysmal nocturnal hemoglobinuria (PNH) is a hematopoietic stem/progenitor cell disorder caused by PIGA mutations. All hematopoietic lineages derived from the affected clone exhibit the PNH phenotype [deficiency of glycosyl phosphatidylinositol linked membrane proteins (GPI-APs)]. Diagnosis is made by flow cytometric analysis of red blood cells (RBC), granulocytes, and monocytes. The percentage of granulocytes and monocytes with absent expression of GPI-APs is a measure of the size of the PNH clone. Typically, the percentage of GPI-AP deficient RBCs is somewhat less than that of granulocytes and monocytes due to selective destruction by complement-mediated intravascular hemolysis, manifested by high LDH, bilirubin, reticulocyte count and low haptoglobin. Herein, we report five patients with large PNH clones based on percentage of GPI-AP deficient neutrophils and monocytes, but with absent or near absent PNH RBCs and no biochemical evidence of hemolysis. We call this unique disease subtype, ahemolytic PNH. Methods: The medical records of PNH patients (174 patients with PNH granulocytes >20%) at the University of Texas Southwestern, Cleveland Clinic Foundation, and National Institutes of Health from 2000 to 2022 were examined. Information pertaining to their clinical, laboratory, and molecular attributes (NGS sequencing) was collected. Five patients with granulocyte clones >50% and RBC clones <5% were identified (Table). Results: Patient 1 is a 63-year-old male with a remote history of non-severe aplastic anemia (AA) diagnosed at 11 years old. The first PNH flow cytometry, obtained at age 61 years, showed 0.02% type II PNH RBC, 0.5% type III PNH RBC, 96% PNH monocyte and 97% PNH granulocytes (Table). There was no biochemical evidence of hemolysis at the time of diagnosis or in the preceding 5 years. Patient 2 is a 50-year-old male who presented with pancytopenia and was diagnosed with acute myeloid leukemia with myelodysplastic-related mutations. Flow cytometry showed 85% PNH monocytes and 59% PNH with no detectable PNH RBCs and no biochemical evidence of hemolysis at diagnosis or in 3 years of follow-up (Table). Patient 3 is a 73-year-old female who presented with pancytopenia and marrow aplasia with unremarkable morphology, and a PNH granulocyte clone of 93.6% with 1.3% type III PNH RBCs and 1.3% and 0.5% type II PNH RBCs, with no evidence of hemolysis (Table). Patient 4 is a 21-year-old female who presented with Budd-Chiari syndrome. Flow cytometry showed 73% PNH granulocytes with 3.6% type III PNH RBCs and 1.8% type II PNH RBCs. There was no biochemical evidence of hemolysis, but because of thrombosis, she was treated complement inhibitor therapy (Table). Patient 5 is a 61-year-old female with history of severe aplastic anemia treated with immunosuppression (IST) and eltrombopag. PNH flow cytometry showed PNH granulocytes of 92.4% and 2.1% PNH type II RBCs 2.1% post IST treatment with no biochemical evidence of hemolysis (Table). Conclusion: Herein, we present five patients with a distinct PNH phenotype that we call ahemolytic PNH. Except for the absence or near absence of PNH erythrocytes, this entity shares clinical and pathophysiological features with classic PNH, including thrombophilia (Budd-Chiari Syndrome, Table). The basis of this phenotype is speculative but may be determined by the genotype of the affected HSPC in which the somatic mutation of PIGA first occurred. According to this hypothesis, somatic mutations other than PIGA skew differentiation toward granulocyte/monocyte lineages. The International PNH Interest Group recognizes the following three categories of PNH: classic PNH, PNH in the setting of another define bone marrow abnormality, and subclinical PNH. Like ahemolytic PNH, subclinical PNH has no biochemical evidence of hemolysis. In this case, however, the absence of hemolysis is due to the small size of the PNH clone (median clone size <1%) as determined by flow cytometry analysis of peripheral blood granulocytes and monocytes. We propose that ahemolytic PNH, defined as patients having less than <5% PNH red cells and >50% PNH granulocytes/monocytes, be recognized as a distinct category of PNH.

Clonal Hematopoiesis and Venous Thromboembolism in the UK Biobank

Background: Patients with myeloid malignancies have an increased risk of thrombosis, but less is known about the risk of thrombosis in clonal hematopoiesis of indeterminate potential (CHIP). We evaluated genotype-specific associations between CHIP and both prevalent and incident venous thromboembolism (VTE) in the largest such analysis to date of the UK Biobank. Methods: We examined 425,573 individuals without a prevalent hematologic malignancy diagnosis from the UK Biobank. Whole exome sequencing and single nucleotide polymorphism array data were analyzed to identify individuals with CHIP and/or mosaic chromosomal alterations (mCA), respectively. Prevalent and incident VTE cases were ascertained with respect to the time of DNA sampling. VTE events were identified based on ICD10 codes, including but not limited to, pulmonary embolism, phlebitis/thrombophlebitis of deep veins of lower veins, portal vein thrombosis, and Budd Chiari syndrome. Median follow-up time for incident VTE was 11.8 years. Associations of CHIP and VTE were assessed using logistic regression for prevalent analyses and Cox proportional hazards for incident analyses. The association analyses were adjusted for age at the time of DNA sampling, sex, Caucasian ancestry, genetic principal components, ever smoked status, and body mass index (BMI) as covariates. Results: Prevalent VTE and CHIP: While CHIP overall was not associated with an increased risk of prevalent VTE (odds ratio (OR) 1.06; 95% CI 0.87-1.28, P= 0.56) (Figure 1), JAK2-mutant CHIP and JAK2 CHIP with loss of heterozygosity at the JAK2 locus (LOH 9p) were powerfully associated with increased risk of thrombosis, OR = 7.49 (95% CI 3.02-18.56, P=1.41e-05) and 6.74 (95% CI 1.62-28.08; P=8.71e-03). Having both CHIP and autosomal mCA was associated with a nominally increased risk of VTE (OR 1.76; 95% CI 1.02-3.06, P= 0.04). Otherwise, non- JAK2 CHIP , ASXL1 CHIP , TET2 CHIP , DNMT3A CHIP were not associated with increased prevalent VTE, regardless of clone size. Incident VTE and CHIP: CHIP was modestly associated with incident VTE with a hazard ratio of 1.15 (1.03-1.28; P=0.01), and CHIP with a larger clone size (VAF > 10%) was associated with a hazard ratio of 1.22 (95% CI 1.08-1.39; P= 0.001) (Figure 2). Consistent with our analysis of prevalent VTE, JAK2-CHIP and JAK2-CHIP with LOH 9p were strongly associated with incident VTE with hazard ratios of 3.76 (95% CI 1.79-7.9, P= 0.0004) and 4.89 (95% CI 1.84-13.04; P=0.0015). Other associations, including overall CHIP, CHIP with VAF >10%, CHIP together with autosomal mCA, autosomal mCA, non- JAK2 CHIP, TET2 CHIP with VAF >10%, showed modest increased hazard ratios between 1 and 2. Conclusions: In this study of risk of thrombosis in the UK Biobank, CHIP was not significantly associated with prevalent VTE and was modestly associated incident VTE. Among CHIP genotypes analyzed, only JAK2 mutations were significantly associated with both prevalent and incident VTE risk (OR 7.49 and HR 3.76, respectively). JAK2-mutant CHIP may be an undetected contributor to cases of VTE and may have therapeutic implications.

Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review.

Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high. The aim of this review is to update all the existing information on this pathology by means of an exhaustive analysis covering all the scientific literature produced in the last 5 years. In addition, it has been carried out following the PRISMA model and registered in PROSPERO with code CRD42023394490. One of the main conclusions based on the results obtained in this review is that the origin of the syndrome could have a genetic basis and that the treatment of choice is the decompression of the posterior cerebral fossa.

The utility of testing erythropoietin level in polycythemia diagnosis

ABSTRACTObjectives:Polycythemia vera (PV) is classically thought to be associated with low erythropoietin (EPO) levels. Here, we present a review of the utility of using EPO levels in diagnosing polycythemia.Methods:We conducted a systematic literature review of the Medline data through Pubmed and Google Scholar. We included the articles which described confirmed PV associated with elevated EPO level. Our search strategy included the following terms in Pubmed (((polycythemia vera[MeSH Terms]) OR (jak2 protein tyrosine kinase[MeSH Terms])) OR (Myeloproliferative Disorders[MeSH Terms])) AND (Erythropoietin[MeSH Terms]), and ‘polycythemia vera with erythropoietin’ in Google Scholar.Results:Our research yielded four cases of PV with elevated EPO levels. The most common symptom was a headache. Thrombotic phenomena happened in a single case in the form of Budd-Chiari syndrome. The mean Hb level was 20.2 gm/dl, and the EPO level was 213 mlU/mL.Discussion:Although PV is usually associated with low EPO levels, high levels do not exclude this diagnosis. Workup should include testing for JAK2 mutation and bone marrow biopsy in the presence of suggestive signs and symptoms. Novel biomarkers are also being proposed to aid in the diagnosis.Conclusion:Although elevated EPO levels suggest secondary causes of polycythemia, cases where elevated EPO levels were associated with an underlying PV are reported in the literature, and we have summarized a review of them. Workup for polycythemia should include JAK2 mutation testing if signs and symptoms suggest PV even if EPO is elevated.

Accessory hepatic vein recanalization for Budd-Chiari syndrome: a systematic review and meta-analysis

BackgroundBudd-Chiari syndrome (BCS) results when the outflow of the hepatic vein (HV) is obstructed. BCS patients exhibiting an accessory HV (AHV) that is dilated but obstructed can achieve significant alleviation of liver congestion after undergoing AHV recanalization. This meta-analysis was developed to explore the clinical efficacy of AHV recanalization in patients with BCS.Materials and methodsPubMed, Embase, and Wanfang databases were searched for relevant studies published as of November 2022, and RevMan 5.3 and Stata 12.0 were used for pooled endpoint analyses.ResultsTwelve total studies were identified for analysis. Pooled primary clinical success, re-stenosis, 1- and 5-year primary patency, 1- and 5-year secondary patency, 1-year overall survival (OS), and 5-year OS rates of patients in these studies following AHV recanalization were 96%, 17%, 91%, 75%, 98%, 91%, 97%, and 96%, respectively. Patients also exhibited a significant reduction in AHV pressure after recanalization relative to preoperative levels (P < 0.00001). Endpoints exhibiting significant heterogeneity among these studies included, AHV pressure (I2 = 95%), 1-year primary patency (I2 = 51.2%), and 5-year primary patency (I2 = 62.4%). Relative to HV recanalization, AHV recanalization was related to a lower rate of re-stenosis (P = 0.002) and longer primary patency (P < 0.00001), but was not associated with any improvements in clinical success (P = 0.88) or OS (P = 0.29) relative to HV recanalization.ConclusionsThe present meta-analysis highlights AHV recanalization as an effective means of achieving positive long-term outcomes in patients affected by BCS, potentially achieving better long-term results than those associated with HV recanalization.

S3886 Challenges of Imaging Diagnosis in Budd-Chiari Syndrome

S3886 Challenges of Imaging Diagnosis in Budd-Chiari Syndrome

S3735 Budd-Chiari Syndrome Secondary to Congenital Vascular Malformation: A Novel Complication in a Patient with Trisomy 21

S3735 Budd-Chiari Syndrome Secondary to Congenital Vascular Malformation: A Novel Complication in a Patient with Trisomy 21

S3493 Large Microcystic Appearance of Centilobular Venous Congestion in a Case of Acute on Chronic Budd-Chiari Syndrome

S3493 Large Microcystic Appearance of Centilobular Venous Congestion in a Case of Acute on Chronic Budd-Chiari Syndrome

S3683 Incidental Secondary Budd-Chiari Syndrome as Presenting Finding in a Case of Sarcoidosis With Multi-Organ Involvement

S3683 Incidental Secondary Budd-Chiari Syndrome as Presenting Finding in a Case of Sarcoidosis With Multi-Organ Involvement

S3455 A Rare Case of Small Hepatic Vein Budd Chiari Syndrome Presenting as an Inferior Vena Cava Obstruction Secondary to Compression by Hypertrophied Caudate Lobe

S3455 A Rare Case of Small Hepatic Vein Budd Chiari Syndrome Presenting as an Inferior Vena Cava Obstruction Secondary to Compression by Hypertrophied Caudate Lobe

Yüksek vücut kitle indeksi, sağ lob canlı donörden karaciğer nakli alıcılarında postoperatif kanama ve safra komplikasyon oranlarını artıran bir faktör mü?

Purpose: High Body Mass Index emerges as an important perioperative and postoperative risk factor for liver transplantation We aimed to examine the effect of increased BMI on perioperative follow-up parameters and per-postoperative complications in liver transplant recipients.&#x0D; Materials and methods: One hundred and seventy-two patients who underwent living-donor liver transplantation for end stage liver cirrhosis were included in the study. Whether there was a difference between cold ischemia time, operation time, blood product transfusion rates, hospital, biliary complications, hepatic vein thrombosis, portal vein thrombosis, NASH etiology, postoperative hemorrhage, sepsis, and primary graft dysfunction were analyzed statistically in terms of those with a BMI of 25 and above and those below 25.&#x0D; Results: Anhepatic phase duration (p=0.047) and cold ischemia duration (p=0.009) were statistically longer in patients with BMI &gt;25.&#x0D; Conclusion: Prolonged anhepatic phase and cold ischemia times may be important in terms of the effect of increased BMI on postoperative graft survival.

Portal Hypertension in Children at the Department of Pediatric Gastroenterology &amp; Nutrition, BSMMU, Dhaka, Bangladesh

Background: Portal hypertension is the hemodynamic abnormality frequently associated with serious liver disease, although it is recognized in a variety of extrahepatic diseases also. Portal hypertension is an important cause of morbidity and mortality in Bangladeshi children. Development of esophageal varices and bleeding is one of the major complications of CLD. The mortality from each episode of variceal bleeding is 30-50% depending on the clinical status of the patient. All conditions that interfere with blood flow at any level within the portal system can lead to portal hypertension. For better management of this disorder, it is important to determine the underlying cause. Objective: To assess the portal hypertension in children at the Department of Pediatric Gastroenterology &amp; Nutrition, BSMMU, Dhaka, Bangladesh. Methods: This cross-sectional descriptive study was conducted at the Department of Pediatric Gastroenterology &amp; Nutrition, BSMMU during the period Jan 2018 to July 2019. 50 patients who were diagnosed as portal hypertension were determined by Upper GI Endoscopy. Doppler USG was also done for supporting the diagnosis of Portal HTN as well as to differentiate between extrahepatic Portal HTN and CLD with portal HTN. Demographic data and other related information regarding etiology and complications were recorded in a standard datasheet. Results: Results: A total of 50 cases were included in this study. Their age range was 1.5-16 years. It was observed that 21 (42.0%) patients belonged to age group 6-10 years. The mean age was 9.22±9.85 years with ranged from 2.5 to 16 years. It was observed that almost two third 31 (62.0%) patients were male and 19 (38.0%) were female. Among 50 patients 29 were diagnosed as extrahepatic portal hypertension and 21 were diagnosed as CLD with portal HTN. Shows the etiology of portal hypertension of studied patients. Extrahepatic portal hypertension was the most common etiology (58.0%). Among CLD patients Wilson disease was the most common (13; 26.0%). Two (4.0%) patients were cryptogenic CLD and two (4.0%) were Budd Chiari Syndrome. One patient was Biliary cirrhosis and one patient had Auto immune hepatitis. Conclusion: We concluded that Extrahepatic Portal HTN is the most common cause of portal hypertension in children in this center. Among the intrahepatic causes of portal HTN Wilson disease was the

Intrahepatic Venous Collateral Circulation and Reverse Blood Flow After Main Hepatic Vein Obstruction: A Case Report with Literature Review

Intrahepatic Venous Collateral Circulation and Reverse Blood Flow After Main Hepatic Vein Obstruction: A Case Report with Literature Review

Hemodynamic and Hydrodynamic Pathophysiology in Chiari Type 1 Malformations: Towards Understanding the Genesis of Syrinx.

The pathophysiology of this association of type 1 Chiari malformation (CM1) and syrinxes is still unknown. There is an alteration in the dynamics of neurofluids (cerebrospinal fluid, arterial and venous blood) during the cardiac cycle in CM1. Our objective is to quantify CSF or arterial blood or venous blood flow in patients with Chiari syndrome (CS) with and without syrinxes using phase-contrast MRI (PCMRI). We included 28 patients with CM1 (9 with syrinxes, 19 without). Morphological MRI with complementary PCMRI sequences was performed. We analyzed intraventricular CSF, subarachnoid spaces CSF, blood, and tonsillar pulsatility. There is a highly significant correlation (p < 0.001) between cerebral blood flow, cerebral vascular expansion volume and venous drainage distribution. Venous drainage distribution is significantly inversely correlated with oscillatory CSF volume at the level of the foramen magnum plane [-0.37 (0.04)] and not significantly correlated at the C2C3 level [-0.37 (0.05)] over our entire population. This correlation maintained the same trend in patients with syrinxes [-0.80 (<0.01)] and disappeared in patients without a syrinx [-0.05 (0.81)]. The distribution of venous drainage is an important factor in intracranial homeostasis. Impaired venous drainage would lead to greater involvement of the CSF in compensating for arterial blood influx, thus contributing to syrinx genesis.

Direct oral anticoagulants for the treatment of splanchnic vein thrombosis: A state of art.

Splanchnic vein thrombosis (SVT) is a manifestation of venous thromboembolism in an unusual site. Portal, mesenteric, and splenic veins are the most common vessels involved in SVT which occurs mainly in patients with liver cirrhosis, although non-cirrhotic patients could be affected as well. Thrombosis of hepatic veins, also known as Budd-Chiari syndrome, is another manifestation of SVT. Prompt diagnosis and intervention are mandatory in order to increase the recalization rate and reduce the risk of thrombus progression and hypertensive complications. Traditional anticoagulation with heparin and vitamin-K antagonists is the treatment of choice in these cases. However, recent studies have shown promising results on the efficacy and safety of direct oral anticoagulants (DOACs) in this setting. Available results are mainly based on retrospective studies with small sample size, but first clinical trials have been published in the last years. This manuscript aims to provide an updated overview of the current evidence regarding the role of DOACs for SVT in both cirrhotic and non-cirrhotic patients.