Anticoagulating Budd-Chiari syndrome patients presenting with variceal bleed: A retrospective study.

BACKGROUND AND AIM: This aims to study incidence of re-bleeding on anticoagulation and survival of Budd-Chiari syndrome (BCS) patients presenting with variceal bleeding. METHODS: Budd-Chiari syndrome patients presenting with variceal bleed between 01/01/2007 and 01/05/2019 were retrospectively studied. Patients underwent endoscopic treatment ± endovascular therapy, followed by anticoagulation. Variceal re-bleed (on anticoagulation) and survival were studied. RESULTS: Of 376 BCS patients diagnosed during the study period, 40 (10.7%) patients, presenting with variceal bleed (age 33 [25-40] years; male patients 70%; Rotterdam score 1.13 [0.63-1.22]), Group 1 were compared with 40 randomly selected age-matched BCS patients presenting with ascites, no bleeds (40 [23-42] years; male patients 42.5%; Rotterdam score 1.11 [1.09-1.16]), Group 2. The commonest site of obstruction was hepatic vein (65%) in Group 1 and combined hepatic veins and inferior vena cava (57.5%) in Group 2 (P < 0.01). Thirty-six Group 1 patients underwent endoscopic intervention (variceal ligation, 33; sclerotherapy, 2; glue injection, 1). Endovascular intervention was performed in 30 Group 1 patients (angioplasty ± stent, 22; endovascular shunt, 8) and in 34 Group 2 patients (angioplasty ± stent, 26; endovascular shunt, 8). All 80 patients were started on anticoagulation. Variceal bleed on anticoagulation occurred in five patients in Group 1 and three patients in Group 2. One-year and 5-year survival were 94.2% and 87.5%, respectively, in Group 1 and 100% and 80%, respectively, in Group 2. CONCLUSIONS: About one-tenth of BCS patients present with variceal bleed. On management with endoscopic ± endovascular therapy, followed by anticoagulation, variceal re-bleed in these patients were comparable with those in BCS patients presenting with ascites and survival was excellent at 1 and 5 years.

Sonographic classification of draining pathways of obstructed hepatic veins in Budd-Chiari syndrome.

PURPOSE: To describe and propose a sonographic classification of the blood-draining pathways of obstructed hepatic veins in Budd-Chiari syndrome. METHODS: This retrospective study included 206 patients with hepatic vein obstructions who underwent sonographic examination. We evaluated the afflicted hepatic veins, as well as the course, orifice, blood flow direction of draining veins, and communicating branches. Results were classified and compared with digital subtraction angiography and computed tomography angiography. RESULTS: Of 618 hepatic veins in 206 patients, 542 were obstructed. The blood-draining pathways were classified as hepatic vein-accessory hepatic vein (131/206), hepatic vein-hepatic vein/accessory hepatic+hepatic vein (49/206), and, less frequently, collateral pathways (26/206). Blood was drained from obstructed hepatic veins to the inferior vena cava, right atrium, para-umbilical veins, or hepatic subcapsular veins through communicating branches of various number and diameters. Doppler signals were obtained from the draining veins. CONCLUSIONS: Sonography provides accurate information regarding the blood-draining pathways of obstructed hepatic veins in Budd-Chiari syndrome, which may be helpful for treatment and follow-up.

Long term impact of transjugular intrahepatic portosystemic shunt (TIPS) creation on hepatic morphology.

PURPOSE: The purpose of this study was to evaluate long-term morphologic changes occurring in the liver after TIPS creation with correlation with hepatic function to gain insight on the physiologic impact of TIPS on the liver. METHODS: This retrospective study included patients who underwent TIPS creation between 2005 and 2022 and had contrasted CT or MRI studies prior to and between 1 and 2 years post procedure. Strict exclusion criteria were applied to avoid confounding. Parenchymal volume and vessel measurements were assessed on the pre- and post-TIPS CT or MRI and MELD scores calculated. RESULTS: Of 580 patients undergoing TIPS creation, 65 patients (mean age, 55 years; 36 males) had pre-TIPS and post-TIPS imaging meeting inclusion criteria at median 16.5 months. After TIPS, the mean MELD score increased (12.9 to 15.4; p = 0.008) and total liver volume decreased (1730 to 1432 mL; p < 0.001). However, the magnitude of volume change did not correlate with MELD change. Neither portosystemic gradient nor TIPS laterality correlated with total or lobar hepatic volume changes or MELD changes. The main portal vein diameter increased (15.0 to 18.7 mm; p < 0.001). Thrombosis of the hepatic vein used for TIPS creation resulted in a mean increase in MELD of +4.1 compared to -2.1 in patients who had a patent and normal hepatic vein (p = 0.007). CONCLUSIONS: Given lack of correlation between portosystemic gradient, hepatic atrophy, hepatic function, and TIPS laterality, the alterations in portal flow dynamics after TIPS may not be impactful to hepatic function. However, hepatic vein patency after TIPS correlated with improved hepatic function.

Budd-Chiari Syndrome Caused by Polycythemia Vera: A Case Report.

Budd-Chiari syndrome (BCS) is a rare condition characterized by the obstruction of hepatic venous outflow. It has various potential etiologies, with myeloproliferative neoplasms representing the most prevalent pathogenic association. Here, we present the case of a 51-year-old male who manifested abdominal pain and ascites. Subsequent clinical investigation revealed the presence of BCS secondary to a myeloproliferative syndrome, specifically polycythemia vera. This case emphasizes the importance of diagnosing BCS and conducting a thorough investigation into its underlying etiology.

Adult-onset still's disease and budd-chiari syndrome: A case report.

Adult onset still's disease (AOSD) is a rare autoinflammatory disease displaying with a wide range of non-specific symptoms and budd-chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow. We present the case of a young patient who presented with persistent fever, sore throat, elbow, hand fingers and knees arthralgia with abdominal pain. The patient's symptoms had started 7 days before the referral. Imaging and laboratory data led to the diagnosis of BCS in the context of AOSD. The patient treated with corticosteroid in combination of warfarin with favorable outcome and complete improvement of signs and symptoms. We came to this conclusion AOSD complicated with BCS is a rare but potentially life-threatening entity. Clinicians should be aware of this complication.

Improving Outcomes in Catheter-Directed Thrombolysis for the Management of Acute Budd-Chiari Syndrome: A Case Report.

Traditionally catheter-directed thrombolysis is performed for recanalization of hepatic vein thrombosis in acute Budd-Chiari syndrome. Successful recanalization of the hepatic veins requires a continuous infusion of the thrombolytic agent for an adequate duration due to increased resistance to blood flow in the setting of luminal thrombosis. Here, we describe a case of acute Budd-Chiari syndrome in a young female in whom prolonged catheter-directed thrombolysis of the right hepatic vein was performed for a duration of 84 hours using alteplase as the thrombolytic agent. This was followed by angioplasty and stent placement. We observed that prolonged catheter-directed thrombolysis was associated with a progressive reduction in clot burden with improved luminal patency of the hepatic vein and improved outcome of subsequent angioplasty and stenting. There was a rapid improvement in liver function tests after the procedure and liver enzymes returned to baseline within a week. A follow-up ultrasound scan showed normal blood flow and a patent lumen of the right hepatic vein. In the absence of complications, prolonged catheter-directed thrombolysis in acute Budd-Chiari syndrome can achieve adequate recanalization of the hepatic veins and improved long-term clinical outcomes. This may obviate the need for other invasive procedures like TIPS (transjugular intrahepatic portosystemic shunt)/DIPS (direct intrahepatic portosystemic shunt) and liver transplantation.

Iatrogenic Budd-Chiari Syndrome Secondary to Transhepatic Hemodialysis Catheterization.

Hepatic vein thrombosis (HVT), or Budd-Chiari syndrome, is a rare disorder resulting from the obstruction of blood flow from the liver to the inferior vena cava and eventually back to the heart. HVT can lead to extensive hepatocellular injury and portal hypertension and may require liver transplantation in patients. We present a rare cause of HVT and subsequent liver failure secondary to transhepatic venous catheterization for hemodialysis (HD) in a patient with end-stage renal disease (ESRD).

Gonadal dysfunction in male patients with Budd Chiari syndrome and its reversibility with treatment.

BACKGROUND AND AIMS: Budd Chiari syndrome (BCS) commonly affects adolescents and adults. With improved survival, important quality-of-life parameters such as sexual life and fertility become more relevant. This study was aimed to assess the gonadal function in male patients with BCS and the effect of treatment on gonadal function. METHODS: Thirty male patients with newly diagnosed BCS were prospectively assessed for the presence of gonadal dysfunction. Erectile function was assessed using standardized International Index of Erectile Function questionnaire (IIEF). Follicular stimulating hormone (FSH), luteinizing hormone (LH), sex hormone-binding globulin (SHBG), estradiol, total testosterone (TT), calculated free testosterone (cFT), calculated bioavailable testosterone (cBT), sperm count, and sperm motility were compared at baseline and at 6 months of treatment for the assessment of gonadal function. RESULTS: Sixteen (53.3%) out of 30 patients were sexually active at the time of study and 5/16 (31%) had erectile dysfunction. Hypogonadotropic hypogonadism (HH) was the most common pattern seen in 50% cases followed by hypergonadotropic hypogonadism (HyH) in 23% cases. 27% patients had eugonadism. At 6 months of treatment, 60% of patients in HH group became eugonadal as compared to only 14% in HyH group. Proportion of patients with erectile dysfunction reduced (5/16 vs 1/16) after 6 months of therapy. The improvement in sperm count and sperm motility was not significant. CONCLUSION: Gonadal dysfunction is common in male patients with BCS. HH remains the most common type of hypogonadism BCS and the type which improves significantly after treatment.

State of the Art, Current Perspectives, and Controversies of Budd-Chiari Syndrome: A Review.

Background: Budd-Chiari syndrome (BCS) is an eponym that includes a group of conditions characterized by partial or complete hepatic venous tract outflow obstruction, and the site of obstruction may involve one or more hepatic veins, inferior vena cava, or the right atrium. The classification of BCS is based on etiology, site of obstruction, and duration. Its etiology is very heterogeneous; in particular, hepatic vein thrombosis is the most common type of obstruction and myeloproliferative disorder, the most common thrombophilic disorder, in the West. In Asian countries, the type of obstruction, thrombophilic disorders, clinical features, and treatment strategies vary widely from region to region. Although the cause can be identified in 90% of patients with the help of gene mutation testing, BCS remains under-recognized in many countries. A higher prevalence of acute cases has been reported in the West than in the East. This global and regional heterogeneity raises several challenges regarding the evaluation, management strategy, and individualized approach of BCS. This study aimed to conduct a systematic review of BCS to elucidate treatment strategy options. Methods: PubMed, Embase, Cochrane Library, and Google Scholar databases were searched systematically. Results: Sixty-nine pertinent articles were retrieved and included in the present study. Conclusions: Further research on the following three topics would help define individualized treatment strategies. The first is a better understanding of the molecular pathways underlying the thrombophilic conditions implicated in the pathogenesis of BCS. The second is the role of the genotype and gene mutations in the determination of coagulation status of patients with BCS. The third is the definition of clear criteria and development of a common prognostic index to risk stratify the patients at presentation and consequently detect candidates for invasive therapies.

Hepatic vein tumor thrombosis in patients with hepatocellular carcinoma: Prevalence and clinical significance.

BACKGROUND: There is strong evidence that portal vein tumor thrombosis (PVTT) is associated with poor survival in patients with hepatocellular carcinoma (HCC). However, data regarding the clinical significance of hepatic vein tumor thrombosis (HVTT) is rare, particularly in Western patients. OBJECTIVE: To determine the HVTT prevalence in a Western patient population and its impact on survival. METHODS: We included 1310 patients with HCC treated in our tertiary referral center between January 2005 and December 2016. HVTT and PVTT were diagnosed with contrast-enhanced cross-sectional imaging. Overall survival (OS) was calculated starting from the initial HCC diagnosis, and in a second step, starting from the first appearance of vascular invasion. RESULTS: We observed macrovascular invasion (MVI) in 519 patients who suffered from either isolated HVTT (n = 40), isolated PVTT (n = 352), or both combined (HVTT + PVTT) (n = 127). Calculated from the initial HCC diagnosis, the median OS for patients with isolated HVTT was significantly shorter than that of patients without MVI (13.3 vs. 32.5 months, p < 0.001). Calculated from the first appearance of MVI, the median OS was similar among patients with isolated HVTT (6.5 months), isolated PVTT (5 months), and HVTT + PVTT (5 months). Multivariate analysis confirmed HVTT as an independent risk factor for poor survival. CONCLUSIONS: HVTT may be more common than typically reported. In most patients, it was accompanied by PVTT. Isolated HVTT occurred less frequently and later than isolated PVTT; however, once developed, it had the same deleterious impact on survival. Therefore, patients with HVTT should be classified as advanced stage of HCC.

Vascular Complications in Patients with Chronic Pancreatitis.

INTRODUCTION: Chronic pancreatitis (CP) is a long-standing progressive inflammation of the pancreas, which can lead to a variety of vascular complications, such as splanchnic venous thrombosis (VT) and arterial pseudoaneurysm (PA). There is a lack of studies on vascular complications in Scandinavian countries. METHODS: We performed a retrospective analysis of medical records of patients with CP identified from the Karolinska University Hospital database between 2003 and 2018. A total of 394 patients with definite CP were included in the study. RESULTS: There were 33 patients with vascular complications, with a median age of 62 (IQR 55-72) years. The cumulative incidence of vascular events was 3.2% at 5 years. Thirty patients had isolated VT, whereas three patients had PA (7.6% and 0.8%, respectively). Isolated splenic vein thrombosis was most common (53.3%), followed by a combination with other splanchnic veins. PA was found in the splenic artery in two patients and in the left gastric artery in one patient. Varices were present in three (10%) patients; variceal bleeding was not recorded. All patients had asymptomatic splanchnic VT, most with chronic VT with developed collaterals (83.3% had abdominal collateral vessels). Nearly two-thirds of patients with VT (63.3%) received no treatment, whereas 11 (36.6%) were treated with anticoagulants. Pseudocysts and alcoholic etiology of CP are risk factors for vascular complications. CONCLUSIONS: The cumulative incidence of vascular complications was 3.2% at 5 years. Splanchnic VT is more common than PA. Patients were asymptomatic with no variceal bleeding, explained by well-developed collateral vessels and strong study inclusion criteria.

Budd-Chiari syndrome on background of JAK2V617F mutation with no hematologic abnormalities.

Whenever considering idiopathic Budd-Chiari syndrome, consider the possibility of JAK2 mutation even if clinical parameters are within normal range.

Identification of RPL5 gene variants and the risk of hepatic vein thrombosis in Saudi patients.

OBJECTIVES: To identify ribosome protein L5 gene variants and the risk of hepatic vein thrombosis in Saudi patients. METHODS: A case-control study was conducted during the period of May 2018 to September 2019. Sixty-five patient cases of hepatic vein thrombosis (HVT) were chosen, and 50 healthy individuals of the same ages and both gender were set as a control group. The genotype of the gene RPL5 was determined by PCR please provide abbreviation in full and capillary electrophoresis. Sanger sequencing for genetically screened variants was applied for the RPL5 gene. RESULTS: Alleles A at variant rs182018447 and T allele at variant rs559377519 were strongly corelated (p=0.009 and p=0.037, respectively) with the risk of HVT. The genotype frequencies of the RPL5 gene, the A/A genotypes at rs182018447 and T/T at rs559377519 were associated with HVT (p=0.000 and p=0.004; respectively) and an increase in risk for HVT among these patients. Please rephrase the highlighted text without using the word respectively. CONCLUSION: Our findings indicate that the 5 genetic novel variants examined in the RPL5 gene were associated with a risk of HVT in all our Saudi cases. Additionally, the A/A at rs182018447 and T/T at rs559377519 genotypes were substantially susceptible to HVT in all these patients.

Limited added value of Doppler ultrasound of the liver after recent contrast-enhanced computed tomography.

PURPOSE: The aim of this study is to assess the added diagnostic value of Doppler ultrasound of the liver (DUL) performed within 3 days of contrast-enhanced CT (CECT) for the diagnosis of portal vein (PV) or hepatic vein (HV) thrombosis. METHODS: Adult patients were included if they underwent DUL within three days after a CECT of the abdomen in the emergency or inpatient setting. Retrospective review of clinical data and imaging reports was performed. In patients with discrepant or positive findings on CECT and/or DUL with respect to PV or HV thrombosis, image review was performed by three fellowship-trained abdominal radiologists in consensus. RESULTS: The final cohort consisted of 468 patients. Of these, 26 (5.6%) patients had equivocal findings for thrombosis on CECT, and DUL could make a confident diagnosis of positive or negative in 18 (69%) patients. Additionally, there were 2 (0.4%) patients with PV or HV thrombosis on DUL following a limited CECT, and 2 (0.4%) patients who developed interval PV thrombosis between CECT and DUL. CONCLUSION: DUL after CECT added diagnostic value for PV and/or HV thrombosis in less than 5% of patients. The patency of PV and HV is often not explicitly mentioned in CECT reports at our institution, which may lead to uncertainty for the referring provider as to whether the PV and HV were adequately evaluated. Few CECT have false positive or missed or underreported findings, and a careful review of the original CECT should be performed if DUL is requested.

Pleural Effusion Presenting in a Young Man With Behcet's Disease.

Behcet's disease (BD) is a rare multisystem chronic vasculitis of variable clinical presentation and unknown origin. Pulmonary involvement in BD is uncommon, with pleural effusion being an even rarer and difficult to diagnose manifestation. Herein, we report a challenging case of a young man who presented with recurrent pleural effusion and hepatic vein thrombosis and a recent history of papilledema with idiopathic intracranial hypertension. The patient was hospitalized for diagnostic and therapeutic thoracocentesis. Biochemistry and cytological analysis showed an exudative aspect of the collected pleural fluid with lymphocyte-dominated cytology. A multidisciplinary discussion was held, and thorough investigation was carried out to rule out malignant and infectious etiologies, among other differentials. During the second hospitalization, the patient complained of genital ulcers, which were verified to be recurrent along with oral ulcers. The diagnosis of BD was established based on the International Classification Criteria for BD, and the symptoms improved significantly upon using colchicine and immune-suppressive drugs.

Acute Budd-Chiari syndrome during hepatic vein catheterization.

This case report describes a 4-year-old male with a history of hepatoblastoma, treated with chemotherapy followed by a right extended hepatectomy. Secondary to this, the patient experienced recurrent hepatic vein stenosis. He was treated initially with conventional angioplasty, followed by paclitaxel-coated balloon dilatations in an attempt to prevent episodes of re-stenosis. During the catheterization of the hepatic vein in one of the treatments, hemodynamic instability due to an acute Budd-Chiari syndrome occurred. The hemodynamic compromise became unresponsive to intravascular resuscitation and inotropic support. The patient was then treated with an emergency conventional angioplasty of the hepatic vein, which resulted in a rapid response and eventually in a full recovery. The etiology of this complication remains unclear; however, it may have been secondary to endothelial damage leading to acute thrombosis and/or venous spasm. Conventional angioplasty was successful in managing this complication. Awareness of iatrogenic acute Budd-Chiari syndrome as a potentially fatal complication during hepatic catheterization/dilatation, especially in the posthepatectomy setting, successful management of this complication, and the importance of a multidisciplinary and rapid response, is emphasized.

Venous thrombosis and segmental hypoperfusion in amebic liver abscess: MDCT demonstration and its implications.

PURPOSE: To report venous thrombosis and associated perfusion defect in amebic liver abscess (ALA) using MDCT. METHOD: MDCT images of 62 patients with ALA were reviewed for venous thrombosis and associated perfusion abnormalities. RESULT: The study found 43 (69%) patients with venous thrombosis: portal vein thrombosis (PVT) occurred in 39, hepatic vein thrombosis (HVT) in 37 and inferior vena cava (IVC) thrombosis in 4. Combined PVT and HVT occurred in 33 (77%) patients. The portal vein thrombi remained localized in subsegmental branches in 25 patients and extended to segmental branches in 14. The hepatic vein thrombi were confined to peripheral branches in 18 patients; they progressed to the main trunk in 19 and to the IVC in 4. A wedge-shaped hypoattenuating zone suggesting ischemia was identified in 33 (77%) patients in portal phase: 31 had combined PVT and HVT, 2 had HVT alone, but none had PVT alone. It occurred significantly more often with combined PVT and HVT than HVT alone (p = 0.05). Arterial phase enhancement occurred in 2 of 13 patients with multiphasic CT. All patients were symptomatic despite medical therapy and therefore required percutaneous drainage. About half of the patients were identified with ruptured abscesses. Segmental atrophy was observed in seven of nine patients who underwent follow-up CT. CONCLUSION: Combined PVT and HVT commonly occur with ALA and often manifests as segmental hypoperfusion in portal venous phase, indicating ischemia. The detection of such events by CT may be indicative of severe disease that requires aggressive management involving percutaneous drainage.

Venous thrombosis of the liver: current and emerging concepts in management.

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

Prognosticators of hepatocellular carcinoma with intrahepatic vascular invasion.

OBJECTIVE: The prognosis of intrahepatic vascular invasion, including unilateral or main portal vein tumor thrombosis (PVTT) and hepatic vein thrombosis, is still poor. Many patients with intrahepatic vascular invasions never receive radiotherapy (RT). In recent years, more conformal RT techniques such as intensity-modulated RT (IMRT) have been developed and applied to treat other cancers and have significantly improved treatment results and decreased side effects. The purpose of this study is to evaluate the treatment results in patients with intrahepatic vascular invasion and explore the role of IMRT in these treatments. MATERIALS AND METHODS: There were a total of 73 patients with newly diagnosed AJCC stage IIIB hepatocellular carcinoma (HCC), with either PVTT or hepatic vein tumor thrombosis between 2007 and 2015 in our hospital. IMRT was used for all patients who received RT. Prognostic factors, including treatment modalities, liver function, and comorbidities, were analyzed using univariate and multivariate analysis with the Cox model. Survival time was analyzed using the Kaplan-Meier method. RESULTS: The longest follow-up time was 45.3 months. The median age was 67 years. Univariate analyses indicated that IMRT, transarterial chemoembolization (TACE), target therapy (sorafenib), tumor size, Child-Pugh class, and ascites were significantly associated with overall survival (OS). In multivariate analysis, IMRT (hazard ratio [HR], 0.495; P = 0.019), sorafenib (HR, 0.340; P = 0.013), tumor size (HR, 2.085; P = 0.020), and Child-Pugh class (P = 0.004), were independent prognostic predictors for patients with intrahepatic vessel invasion, but TACE and ascites were not. The outcomes of patients who had different treatment modalities were significantly different (P < 0.001). Patients who received IMRT with TACE had the best outcomes. Patients who received an RT dose above 5400 cGy had better outcomes than those who with a dose below 5400 cGy, although the results were not significantly different (P = 0.248). CONCLUSION: IMRT is an important treatment component for patients with intrahepatic vascular invasion. Combined treatment modalities, such as IMRT with TACE, could improve the outcomes of HCC patients with intrahepatic vessel invasion.