Cause Of Budd-Chiari Syndrome Research Articles

Hepatocellular carcinoma with metastasis to the inferior cava vein and right atrium, an unusual cause of Budd Chiari Syndrome

Hepatocellular carcinoma with metastasis to the inferior cava vein and right atrium, an unusual cause of Budd Chiari Syndrome

Inferior vena caval web causing Budd Chiari syndrome

Budd Chiari syndrome (BCS) is a condition resulting from obstruction of hepatic venous outflow at various levels, regardless of the cause of obstruction. Manifestations can range from asymptomatic state to fulminant hepatic failure or cirrhosis depending on how acutely the obstruction developed. IVC web is an unusual yet potentially treatable cause of BCS. This case report throws light on a rare cause of BCS, which is an IVC web and how endovascular technique helped in patient recovery.

Splanchnic Vein Thrombosis in Overt Myeloproliferative Neoplasms: The Mayo Clinic Experience with 107 Unselected Consecutive Cases

Background:Splanchnic vein thrombosis (SVT) is a characteristic feature of JAK2 mutation-enriched myeloproliferative neoplasms (MPN). MPN constitutes the most frequent cause of Budd-Chiari syndrome (BCS) and non-malignant, non-cirrhotic portal vein thrombosis (Blood. 2012;120:4921). Reported risk factors for recurrent SVT included BCS, thrombosis history, splenomegaly, leukocytosis and absence of warfarin therapy (Blood Cancer J. 2016;6:e493). The main objective of the current study was to share the experience from a single tertiary center.Methods:Study patients were selected from institutional databases, with additional cases identified through Advanced Cohort Explorer Tool. Conventional diagnostic criteria were utilized (Blood 2016;127:2391) Diagnosis of SVT in all cases was confirmed by imaging studies. Cases with latent MPN (MPN-U) or evidence of other cancer were excluded. All analyses considered clinical and laboratory parameters obtained at time of SVT.Results:i) Presenting features107 patients (median age 56 years; range 22-84; 62% females) were considered; specific MPN diagnosis was primary myelofibrosis (PMF) 36%, polycythemia vera (PV) 29%, essential thrombocythemia (ET) 29%, post-PV 6% and post-ET myelofibrosis (MF) 1%. Among evaluable cases, JAK2 mutation was detected in 85% and abnormal karyotype in 33%. At the time of SVT, 80% were not receiving aspirin (ASA) therapy and 4% had history of OCP use. Thrombosis history was recorded in 51% and included history of SVT in 38%. 39 (36%) patients had pre-SVT history of splenectomy, including 16 (15%) in the month before. Presentation included abdominal pain 62%, ascites 31% and GI bleed 15%.ii) Type of SVT and clinical correlatesSVT types included BCS ± other vessels (n=9; 6 PV, 2 ET and 1MF), portal ± splenic (n=73; 38 MF, 20 PV, 15 ET), portal + mesenteric ± splenic (n=23; 14 ET, 5 MF and 4 PV). Comparison of these three SVT categories disclosed significant associations with MPN sub-category (p<0.01; BCS with PV and “portal + mesenteric” with ET), abdominal pain (p=0.02; with BCS and “portal + mesenteric”) and ascites (p=0.007; with BCS).iii) Initial management of SVTInitial treatment included systemic anticoagulation (SA) only in 39% of the patients, SA + ASA 4%, SA + cytoreductive drug 21%, SA + ASA + cytoreductive drug 4%, and no SA 33%. In addition, trans-jugular intrahepatic portosystemic (TIPS) or other surgical shunting was undertaken in 13% and variceal banding in 14%.iv) Post-SVT survival, causes of death and prevalence of liver failureAt median post-SVT follow up of 2.6 years (range .02-17.5), 35 (33%) deaths and 5 (5%) leukemic transformations were noted; the corresponding percentages for PV/ET vs MF were 18% vs 53% and 1.6% vs 9%. Causes of death were unrelated to SVT in 17 instances, unknown in 6, liver failure in 5 (all MF), upper gastrointestinal bleed in 4 (3 MF 1 PV), AML in 2 MF cases and thrombosis in one ET patient.v) Risk factors for post-SVT survival and recurrent SVTAs expected, post-SVT survival was significantly shorter in patients with MF vs PV/ET (HR 4.6, 95% CI 2.2-9.6). In multivariable analysis, older age (p<0.01), MF diagnosis (vs PV/ET) (HR 4.0; 95% CI 1.9-8.4) and presentation with ascites (HR 2.7, 95% CI 1.3-5.8) were independently associated with shorter survival; the latter was also associated with post-SVT complications (p=0.01). When analysis was restricted to the 62 patients with PV/ET, median post-SVT survival was not reached and advanced age was the only predictor of shortened survival (p<0.01), while associations of BCS with PV and “portal + mesenteric” with ET were confirmed (p<0.01). Seven of 8 BCS cases occurred in patients younger than age 60 (p=0.07) and 7 of 8 BCS presented with ascites (p<0.01). The only predictor of SVT relapse was male sex (p<0.01). In general, post-SVT survival and recurrence rate were not affected by type of SVT or initial treatment strategy.Conclusions:Outcome in MPN-associated SVT mostly depends on the underlying MPN type, with limited influence from SVT-directed treatment strategies; importantly, there was no overt evidence of adverse effect, from SVT, on overall or leukemia-free survival in PV or ET; liver failure and GI bleed were relatively frequent causes of death in MF-associated SVT. Other noteworthy observations from the current study included association of BCS with PV, ascites and younger age; and recurrent SVT with male sex. DisclosuresNo relevant conflicts of interest to declare.

Budd-Chiari Syndrome – The Forgotten Danger of Repeat Liver Surgery

Introduction: Budd-Chiari syndrome (BCS) is defined as obstruction of the hepatic outflow anywhere along the hepatic venous course. With certain liver resections, a risk exists for inadvertent disruption to the main venous outflow tracts predisposing to iatrogenic BCS in return resections. Specified pre-operative imaging of the hepatic outflow is generally not undertaken prior to repeat hepatic resections, meaning the hepatic venous outflow can remain unclassified in operative planning. We present the death of a man whose main venous outflow was inadvertently divided during a right hepatectomy following a previous segment 4 metastectomy. Discussion: Our case report describes an iatrogenic cause of BCS after routine stapling of the right hepatic vein eliminated all hepatic outflow due to the patient’s abnormal venous vasculature following previous resection. The patient’s previous hepatic surgery had inadvertently disrupted the hepatic outflow at the confluence of the left and middle hepatic veins, making the right hepatic vein the main outflow route. This was not identified pre-operatively despite the patient undergoing innumerable imaging procedures including portal vein embolization. He subsequently died immediately following surgery from a combination of blood loss and acute liver failure. Conclusion: In the setting of previous hepatic surgery, it is imperative to assess in detail the pre-operative hepatic venous vasculature. Furthermore, when unusual intraoperative hepatic congestion and bleeding occurs, operators need to strongly consider the possibility of obstruction to the venous outflow tracts and thus make every effort to return venous outflow to the remnant liver.

Budd-Chiari Syndrome as A Manifestation of Right Atrium Myxoma

Budd-Chiari syndrome (BCS) is a rare condition marked by a number of symptoms due to hepatic venous obstruction. A 40-year-old female patient presented with abdominal pain, distension, and dyspnea. An abdominal ultrasonogram showed hepatomegaly, splenomegaly and ascites. A hypodense mass obstructed hepatic flow suggested thrombus formation in the hepatic and portal veins and suprahepatic part of the inferior vena cava on computed tomography. Transthoracic echocardiography was performed to assess thrombus extension to inferior vena cava and underlying pathological processes. It showed a mass lesion in the right atrium near intra atrial septum about 4 × 3 cm in diameter. The lesion manifested as an intracardiac thrombus extending from the inferior vena cava. After the successful surgical removal, the diagnosis of the mass was confirmed as atrial myxoma by histopathological examinations. We conclude that right atrial myxoma is a rare cause of Budd-Chiari syndrome.

Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome

Behçet's syndrome (BS) is a known cause of Budd-Chiari syndrome (BCS). We aimed at identifying the prevalence of BS in patients with BCS, analyzing different clinical presentations, treatment modalities and outcome of these patients. We conducted a retrospective cohort study, in which all medical records of patients who were presented to Tropical Medicine Department, Ain Shams University with a confirmed diagnosis of primary BCS from May 2005 to December 2016 were collected and analyzed. In total, 271 patients had a confirmed diagnosis of primary BCS, included Group I: 232 (85.6%) patients with BCS without BS and Group II: 39 patients (14.4%) with BCS due to BS. Male gender (P=0.000), oral ulcers, genital ulcers, Prominent abdominal veins, lower limb swellings, lower extremity deep venous thrombosis (P=0.000) and jaundice (P=0.003) were more frequent in group II patients. The presence of intrahepatic collaterals (P=0.004) and IVC thrombosis (P=0.000) was significant in group II. Medical treatment alone in the form of immunosuppressive drugs and anticoagulation (66.7% vs. 24.1%)±IVC stenting (23% vs. 1.3%) (P=0.000) were the main treatment modalities for BCS related to BS. The frequency of HCC in BS was significantly higher (10.26% vs. 2.59%) (P=0.013). The prevalence of BS in Egyptian patients with BCS is considerably high. The clinical presentation of these patients was different from those without BS. Besides, the incidence of HCC was higher in patients with BS, whereas the mortality did not differ between the two groups.

1297 Cheers to Budd: Pyogenic Liver Abscess Complicated by Acute Budd-Chiari Syndrome

INTRODUCTION: Amebic liver abscess (ALA) is the most common extraintestinal manifestation of amebiasis with high mortality rates (20%). Complications include rupture; superinfection; anemia; and rarely vascular thrombosis. Budd-Chiari syndrome is characterized by post-sinusoidal portal hypertension caused by obstruction of either major hepatic veins or the inferior vena cava. CASE DESCRIPTION/METHODS: A 54-year-old alcoholic male, with a history of recent travel to India, presented with 2 weeks of fever, vomiting and abdominal pain. On exam, he was febrile, icteric, with generalized abdominal tenderness. Chemistries revealed WBCs 12000/mm3, total/direct bilirubin 6.5/3 mg/dL, AST/ALT 410/318 U/L, and ALP 310 U/L. USG RUQ showed hepatomegaly with multiple, thick-walled areas of central low attenuation, suspicious for LA. CT confirmed the presence of multiple ALAs along with rupture and delayed filling in the left and middle hepatic veins. Under USG guidance, brownish fluid (anchovy sauce-like) was aspirated and a pigtail catheter was placed. He was started on ceftriaxone and metronidazole for the treatment of pyogenic LA. Fluid serology was positive for Entamoeba histolytica and only metronidazole was continued for 21 days, along with rivaroxaban for six months for the treatment of Budd Chiari syndrome. His liver enzymes normalized in 21 days and a follow-up USG after 48 days of treatment with rivaroxaban showed no evidence of thrombosis in hepatic veins and IVC. DISCUSSION: ALA should be suspected in patients with fever &amp; jaundice with a history of recent travel to an endemic area. The rates of complications are high, commonest being an abscess rupture. Thrombosis of IVC, hepatic and sometimes right atrium, are rare complications, caused by either direct spread of inflammation or external mechanical compression by the abscess. A diagnosis of Budd-Chiari syndrome should be considered in cases of prolonged fever unresponsive to treatment or ascites with leg edema and collateral abdominal venous circulation. Underlying prothrombotic affection must be excluded. To conclude, identifying a treatable cause of Budd-Chiari syndrome, such as liver abscess, can result in early, effective, and life-saving treatment.

Budd-Chiari syndrome secondary to polycythemia vera with inferior vena cava thrombosis

A middle aged male presented with abdominal distension since one month. Further workup showed plasma hemoglobin of 18.1 g/dL with a high pack cell volume (PCV), raised urea, creatinine and disturbed liver function tests. Abdominal ultrasonography showed an enlarged caudate lobe with thrombi in the inferior vena cava while CT scan of abdomen confirmed the same findings and was suggestive of Budd-Chiari syndrome. Further workup was conducted to rule out other causes and to find out the possible cause of Budd-Chiari syndrome. A peripheral film was requested, which showed hyper-segmented neutrophils. Later on JAK2 mutation and thrombophilia profile was ordered, which was positive for JAK2 mutation. Even though the patient was started on low molecular weight heparin but he eventually passed away. Key point Budd-Chiari syndrome is due to the obstruction of the hepatic efferent outflow tract from either the hepatic veins to where the inferior vena cava meets the right atrium. Its pathogenesis is due to the decreased outflow of the liver, leading to increase in pressure, stasis and later on damage induced by hypoxia and thrombus in the portal veins. Citation: Majid Z, Tasneem AA, Luck NH, Ul Haque MM, Mandhwani RK, Laeeq SM, et al. Budd-Chiari syndrome secondary to polycythemia vera with inferior vena cava thrombosis Immunopathol Persa. 2018;4(1):e04. DOI: 10.15171/ipp.2018.04.

Acute Liver Failure as a First Manifestation of Polycythemia Vera (PCV)

This is a case of extensive vein thrombosis including Budd Chiari syndrome (BCS) that led to acute liver failure and diagnosis of Polycythemia Vera. It is important to recognize hypercoagulable conditions in patients presenting with BCS. Polycythemia Vera (PCV) is increasingly reported as a culprit in BCS patients. 71-year-old female with recent history of right Internal jugular (IJ) thrombosis, brachiocephalic thrombosis. She presented with two-week history of worsening abdominal swelling, peripheral edema. Patient was diagnosed previously with right IJ thrombus after she presented with neck pain one month ago. Hypercoagulable work-up was nonsignificant then. She was started on anticoagulation with Warfarin. CT scan of her abdomen in clinic follow-ups showed small ascites and multiple liver masses. MRI abdomen was suggestive of Budd Chiari syndrome. Patient presents now to our care with worsening ascites, impending acute liver failure. Multidisciplinary care initiated with Hepatology, Hematology/oncology and radiology. JAK2 V617F mutation was sent given thrombosis with high hematocrit and came back positive. Her course in hospital then included interventions to relieve obstruction via angioplasty by radiology, anticoagulation and management of acute liver failure. Phlebotomies were performed to keep hematocrit within limits set by hematologist. Patient deemed not a candidate for liver transplant. Management plan was to continue on anticoagulation with regular phlebotomies. Budd Chiari syndrome (BCS) characterized by thrombosis of hepatic vein and occasionally supra-hepatic part of Inferior Vena Cava (IVC). This syndrome often occurs in hypercoagulable states especially when oral contraceptives are on board. Polycythemia Vera, described as clonal proliferation of myeloid cells distinguished by elevated red cell mass, has been reported in literature as a cause of BCS. Venous thrombosis is not infrequent in PCV. Studies showing prevalence of JAK2 activation in BCS. While primary myeloproliferative diseases were leading causes of portal and hepatic vein thromboses in other studies. This case sheds the light on how drastic PCV can present. Acute liver failure caused by BCS might warrant work up for hypercoagulable status and PCV. Especially that interventions initiated further on can decrease the risk of recurrence once we know the cause.

Association of factor V Leiden, Janus kinase 2, prothrombin, and MTHFR mutations with primary Budd-Chiari syndrome in Egyptian patients.

Budd-Chiari syndrome (BCS) is defined as obstruction of hepatic venous outflow anywhere from the small hepatic veins to the suprahepatic inferior vena cava. The pathogenesis of BCS is still not fully understood. This study aimed to evaluate the association of factor V Leiden (FVL), Janus kinase 2 (JAK2), prothrombin, and methylene tetrahydrofolate reductase (MTHFR) mutations with primary BCS. The study was carried out on 35 patients with primary BCS and 15 age and gender matched healthy individuals as a control group. Genotyping of FVL, prothrombin, and MTHFR mutations was determined by GENEQUALITY AB-THROMBO TYPE kit based on the reverse hybridization principle. JAK2 mutation was determined by polymerase chain reaction-restriction fragment length polymorphism. There was a statistically significant difference between patients and controls regarding FVL, MTHFR C677T, and MTHFR A1298C mutations with odds ratio of 1.83, 2.0, and 1.79, respectively. Hetero MTHFR C677T, hetero FVL, and hetero MTHFR A1298C were the most common etiological factors being responsible for 57.1, 42.9, and 42.9% of primary BCS cases, respectively. It could be concluded that BCS is a multifactorial disease; in the current study, MTHFR C677T mutation was the most common cause of disease. Identification of one cause of BCS should not eliminate investigations for detection of other etiological factors.

Liver Transplantation for Budd-Chiari Syndrome With Large Solitary Focal Nodular Hyperplasia of the Liver in a Patient With Essential Thrombocytemia: Case Report

Budd-Chiari syndrome is a rare condition caused by interrupted hepatic venous outflow in the hepatic veins, inferior vena cava, or right atrium. Reports from the literature have delineated on focal nodular hyperplasia (FNH)–like lesions in association with Budd-Chiari Syndrome. To our knowledge, there are no reports about true FNH lesions in patients with Budd-Chiari Syndrome. Focal nodular hyperplasia develops in disorders with aberrant circulation and vasculature. We report a case of Budd-Chiari syndrome in association with large solitary FNH in a 22-year-old man who was referred to our institution with sudden intermittent right upper quadrant abdominal pain, vomiting, diarrhea with pale stool, decreased appetite, dark urine, and abdominal distention for 15 days. Laboratory investigations revealed anemia, thrombocytosis, and abnormal liver function tests and coagulation profile. Imaging revealed hepatic vein thrombosis, confirming Budd-Chiari syndrome, and a 6.2 × 6.1 × 6.8 cm lesion in segment 8 of the liver. Primary cause of Budd-Chiari syndrome was essential thrombocythemia according to bone marrow biopsy and molecular testing results. The patient was treated medically and underwent transjugular intrahepatic portosystemic shunt insertion. The lesion in segment 8 continued to enlarge. Cadaveric liver transplantation was carried out. On gross and histologic examination of the explanted liver, the lesion was found to be a true FNH.

Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus

Introduction: Hepatic venous outflow block or Budd-Chiari syndrome is a severe liver disease with a 3 years survival rate of 50%. Several conditions have been implicated as a cause of Budd-Chiari syndrome, including myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, the presence of lupus anti-coagulant, oral contraceptives, pregnancy, and others. In a small number of cases, Budd-Chiari syndrome is associated with the presence of lupus anticoagulant. Anticardiolipin antibodies (ACA) are similar to lupus anti-coagulant antiphospholipid antibodies (APLAs), which have been described in patients with recurrent arterial and venous thrombosis, thrombocytopenia, fetal loss, or miscarriage. Case Report: A 23-year-old woman is reported with Budd-Chiari syndrome in whom lupus anticoagulant and anticardiolipin antibodies were shown; 9 months after diagnosis of systemic lupus erythematosus (SLE) treatment with steroids admitted with gastrointestinal problems, abdominal pain and ascites and treated oral anticoagulants induced a considerable improvement. This treatment was continued after 1 year, but interruption was followed by redevelopment of ascites. Further treatment with anticoagulants was continued for 5 years with noticeable improvement. Conclusion: Patients with Budd-Chiari syndrome should be tested for lupus anticoagulants and anticardiolipin antibodies, Budd-Chiari syndrome resulting from this cause may have a good response to treatment with oral anticoagulants; this treatment should be maintained permanently, and pregnancy in such patients may initiate serious difficulties. The condition of the patient at follow-up was good.

Aetiological factors of Budd-Chiari syndrome in Algeria.

To study the clinical presentation of Budd-Chiari syndrome (BCS) and identify the aetiologies of this disease in Algeria. Patients with BCS, hospitalised in our unit from January 2004 until June 2010 were included and the aetiological factors were assessed. Patients presenting a BCS in the setting of advanced-stage cirrhosis or a liver transplantation were excluded from the study. The diagnosis was established when an obstruction of hepatic venous outflow (thrombosis, stenosis or compression) was demonstrated. We diagnosed myeloproliferative disease (MPD) by bone marrow biopsy and V617F JAK2 mutation. Anti-phospholipid syndrome (APLS) was detected by the presence of anticardiolipin antibodies, anti-β2 glycoprotein antibodies and Lupus anticoagulant. We also detected paroxysmal nocturnal haemoglobinuria (PNH) by flow cytometry. Celiac disease and Behçet disease were systematically investigated in our patients. Hereditary anticoagulant protein deficiencies were also assessed. We tested our patients for the G20210A mutation at Beaujon Hospital. Imaging procedures were performed to determine a local cause of BCS, such as a hydatid cyst or a liver tumour. One hundred and fifteen patients were included. Mean follow up: 32.12 mo. Mean age: 34.41 years, M/F = 0.64. Chronic presentation was frequent: 63.5%. The revealing symptoms for the BCS were ascites (74.8%) and abdominal pain (42.6%). The most common site of thrombosis was the hepatic veins (72.2%). Involvement of the inferior vena cava alone was observed in 3 patients. According to the radiological investigations, BCS was primary in 94.7% of the cases (n = 109) and secondary in 5.2% (n = 6). An aetiology was identified in 77.4% of the patients (n = 89); it was multifactorial in 27% (n = 31). The predominant aetiology of BCS in our patients was a myeloproliferative disease, observed in 34.6% of cases. APLS was found in 21.7% and celiac disease in 11.4%. Other acquired conditions were: PNH (n = 4), systemic disease (n = 6) and inflammatory bowel disease (n = 5). Anticoagulant protein deficiency was diagnosed in 28% of the patients (n = 18), dominated by protein C deficiency (n = 13). Secondary BCS was caused by a compressing hydatic cyst (n = 5) and hepatocellular carcinoma (n = 1). The main aetiologic factor of BCS in Algeria is MPD. The frequency of celiac disease justifies its consideration when BCS is diagnosed in our region.

Association between JAK2 rs4495487 Polymorphism and Risk of Budd-Chiari Syndrome in China.

Myeloproliferative neoplasms (MPNs) are the leading cause of Budd-Chiari syndrome (BCS), and the C allele of JAK2 rs4495487 was reported to be an additional candidate locus that contributed to MPNs. In the present study, we examined the role of JAK2 rs4495487 in the etiology and clinical presentation of Chinese BCS patients. 300 primary BCS patients and 311 healthy controls were enrolled to evaluate the association between JAK2 rs4495487 polymorphism and risk of BCS. All subjects were detected for JAK2 rs4495487 by real-time PCR. Results. The JAK2 rs4495487 polymorphism was associated with JAK2 V617F-positive BCS patients compared with controls (P < 0.01). The CC genotype increased the risk of BCS in patients with JAK2 V617F mutation compared with individuals presenting TT genotype (OR = 13.60, 95% CI = 2.04–90.79) and non-CC genotype (OR = 12.00, 95% CI = 2.07–69.52). We also observed a significantly elevated risk of combined-type BCS associated with CC genotype in the recessive model (OR = 4.44, 95% CI = 1.31–15.12). This study provides statistical evidence that the JAK2 rs4495487 polymorphism is susceptibility factor JAK2 V617F positive BCS and combined BCS in China. Further larger studies are required to confirm these findings.

Behcet's disease in Budd-Chiari syndrome.

BackgroundBehcet’s disease (BD) is a well-known cause of Budd-Chiari syndrome (BCS). Data are lacking on the presentation and outcome of BCS related to BD.MethodsWe investigated the relationship between BD and BCS in 14 patients with both diseases and compared the results to 92 BCS patients without BD.ResultsMale gender (p = 0.003), North African origin (P = 0.007) and inferior vena cava obstruction (P < 0.0001) were more frequent in patients with BD and BCS than in those with BCS alone and the plasma C-reactive protein level was higher (p = 0.003). Two of the patients with the combined diseases underwent recanalization of the vena cava and the hepatic veins, none received transjugular intrahepatic portosystemic shunts (TIPS), one received a surgical shunt and one underwent liver transplantation. TIPS were less frequent in patients with BD and BCS than in those with BCS alone (P = 0.019). Eighty six per cent of patients with BCS and BD received corticosteroids and immunosuppressive therapy. The 5-year transplantation-free survival rate was 63% in patients with BCS alone and 91% in those without BD (P = 0.11). In our series and in the literature, a high number of patients [12 (61.5%) and 11 (64.7%) respectively] treated with anticoagulation and corticosteroids and/or immunosuppressants did not require invasive treatment.ConclusionThis study shows a higher frequency of IVC obstruction in patients with BCS and BD. Medical treatment with anticoagulation and immunosuppressive agents may improve the symptoms of BCS. Therefore early management with immunosuppressive and anticoagulation therapy appears to be the treatment of choice in patients with BCS and BD.

Acute Budd-Chiari Syndrome with Suspicious Behçet’s Disease: Successful Catheter-Directed Thrombolysis after the Failure of Anticoagulation

Budd-Chiari syndrome (BCS) with occlusion of the major hepatic veins is a rare and serious complication of Behçet’s disease. We report the case of a 38-year-old female who presented with abdominal pain recently. Computerized tomography of the abdomen revealed acute thrombus in the supra- to intrahepatic IVC and thrombotic occlusion of the middle hepatic vein, which is a cardinal sign of acute BCS. The patient had suffered from recurrent oral ulcers, multiple arthralgia, and erythema nodosum, but was not diagnosed with Behçet’s disease based on the international diagnostic criteria. We also tested for other autoimmune diseases, hereditary thrombophilia, antiphopholipid syndrome, myeloproliferative neoplasm and paroxysmal nocturnal hematuria, the results of which were all negative. Therefore, Behçet’s disease was suspected clinically and was also thought to be the cause of BCS. She was treated with heparin for a week, but edema of lower extremities and the abdominal wall were aggravated. We performed catheter- directed thrombolysis and thrombectomy followed by insertion of a stent. Abdominal distension and edema of the legs were improved immediately after the procedure. The patient has exhibited no signs of recurred BCS during 3 months of follow-up with warfarin treatment. Keywords: Acute Budd-Chiari syndrome, Behçet’s disease, Catheter-directed, Thrombolysis

Behcet¿s disease in Budd-Chiari syndrome

Behcet’s disease (BD) is a well-known cause of Budd-Chiari syndrome (BCS). Data are lacking on the presentation and outcome of BCS related to BD. We investigated the relationship between BD and BCS in 14 patients with both diseases and compared the results to 92 BCS patients without BD. Male gender (p = 0.003), North African origin (P = 0.007) and inferior vena cava obstruction (P < 0.0001) were more frequent in patients with BD and BCS than in those with BCS alone and the plasma C-reactive protein level was higher (p = 0.003). Two of the patients with the combined diseases underwent recanalization of the vena cava and the hepatic veins, none received transjugular intrahepatic portosystemic shunts (TIPS), one received a surgical shunt and one underwent liver transplantation. TIPS were less frequent in patients with BD and BCS than in those with BCS alone (P = 0.019). Eighty six per cent of patients with BCS and BD received corticosteroids and immunosuppressive therapy. The 5-year transplantation-free survival rate was 63% in patients with BCS alone and 91% in those without BD (P = 0.11). In our series and in the literature, a high number of patients [12 (61.5%) and 11 (64.7%) respectively] treated with anticoagulation and corticosteroids and/or immunosuppressants did not require invasive treatment. This study shows a higher frequency of IVC obstruction in patients with BCS and BD. Medical treatment with anticoagulation and immunosuppressive agents may improve the symptoms of BCS. Therefore early management with immunosuppressive and anticoagulation therapy appears to be the treatment of choice in patients with BCS and BD.

Budd-Chiari syndrome secondary to toxic pyrrolizidine alkaloid exposure

In this report, we describe a case of pyrrolizidine alkaloid-related Budd-Chiari syndrome in Hong Kong. A 10-month-old boy presented with ascites, right pleural effusion, and hepatomegaly after consumption of herbal drinks for 3 months. His clinical (including imaging) features were compatible with Budd-Chiari syndrome. Budd-Chiari syndrome is a rare disease entity in paediatric patients. In our case, extensive workup performed to look for the underlying cause of Budd-Chiari syndrome was unrevealing, except for toxic pyrrolizidine alkaloid exposure in his herbal drinks.

Long-term Follow-up Study in Budd-Chiari Syndrome

We aimed to present our long-term surveillance experience in patients with Budd-Chiari syndrome (BCS), and we retrospectively evaluated the natural history, results of thrombophilia studies, and the factors related to mortality. Primary BCS is a rare form of vascular disease, secondary to underlying thrombophilia. Because of its rarity and heterogeneous nature, there is a scarcity of knowledge about the natural history of the disease. In 22 years, a total of 62 patients with primary BCS were followed in our tertiary hospital. We identified an acquired cause of BCS in 40 out of 62 patients (64.5%), whereas in 6 patients (9.7%), we found no identifiable cause. One or more thrombophilia causes were identified in 56 patients (90.3%). In 19 patients with myeloproliferative disease, 15 had Janus tyrosine kinase 2 mutation analysis and Janus tyrosine kinase 2 positivity was found in 10 patients. In regression analysis, portal vein thrombosis was found to be the only indicator of mortality, with an estimated instantaneous risk of 8.4. In this study, we present one of the largest series of BCS in the English literature. We have shown that the multifactorial nature of underlying thrombophilia should be thoroughly investigated. In a patient with BCS, a clinician should be alert for the development or coexistence of portal vein thrombosis due to its deleterious effect on mortality.

Hydatid Cyst of Liver: A Rare Cause of Secondary Budd-Chiari Syndrome

ABSTRACT Hydatid cyst of the liver, causing compression of the inferior vena cava (IVC) and hepatic veins is a rare cause of secondary Budd-Chiari syndrome (BCS). As the hydatid disease is endemic in India, it is a rare but treatable cause of BCS. The early diagnosis and timely intervention can prevent hepatic complications leading to BCS in the affected patients. How to cite this article Mahajan D, Kang M, Gupta R, Khandelwal N. Hydatid Cyst of Liver: A Rare Cause of Secondary Budd-Chiari Syndrome. J Postgrad Med Edu Res 2013;47(3): 159-161.