Secondary Budd-Chiari Syndrome Research Articles

Case Report: Budd-Chiari Syndrome (BCS) on Hepatocellular Carcinoma (HCC): Primary or Secondary Cause?

This is the case of a 63-year-old female patient admitted for management of a liver mass discovered incidentally on imaging. The patient presented with weight loss and prutitus, and on clinical examination had collateral venous circulation and scraping lesions. Abdominal ultrasound coupled with Doppler showed a hepatic tissue mass with left-sided portal thrombosis. Hepatic MRI confirmed the diagnosis and also revealed SBC with infiltration of the left and median hepatic veins. Liver biopsy confirmed diagnosis of hepatocellular carcinoma (HCC)in cirrhotic liver. Etiological work-up for prothrombotic factors and cirrhosis was negative. The patient was a candidate for palliative treatment. But our dilemma: is this a case of primary or secondary BCS? Scholars Middle East Publishers

Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers.

Management of Budd-Chiari syndrome (BCS) has improved over the last decades. The main aim was to evaluate the contemporary post-liver transplantant (post-LT) outcomes in Europe. Data from all patients who underwent transplantation from 1976 to 2020 was obtained from the European Liver Transplant Registry (ELTR). Patients < 16 years with secondary BCS or HCC were excluded. Patient survival (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression analysis identified predictors of PS and GS after 2000. Supplemental data was requested from all ELTR-affiliated centers and received from 44. In all, 808 patients underwent transplantation between 2000 and 2020. One-, 5- and 10-year PS was 84%, 77%, and 68%, and GS was 79%, 70%, and 62%, respectively. Both significantly improved compared to outcomes before 2000 ( p < 0.001). Median follow-up was 50 months and retransplantation rate was 12%. Recipient age (aHR:1.04,95%CI:1.02-1.06) and MELD score (aHR:1.04,95%CI:1.01-1.06), especially above 30, were associated with worse PS, while male sex had better outcomes (aHR:0.63,95%CI:0.41-0.96). Donor age was associated with worse PS (aHR:1.01,95%CI:1.00-1.03) and GS (aHR:1.02,95%CI:1.01-1.03). In 353 patients (44%) with supplemental data, 33% had myeloproliferative neoplasm, 20% underwent TIPS pre-LT, and 85% used anticoagulation post-LT. Post-LT anticoagulation was associated with improved PS (aHR:0.29,95%CI:0.16-0.54) and GS (aHR:0.48,95%CI:0.29-0.81). Hepatic artery thrombosis and portal vein thrombosis (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). LT for BCS results in excellent patient- and graft-survival. Older recipient or donor age and higher MELD are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.

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

Budd-Chiari Syndrome Imaging Diagnosis: State of the Art and Future Perspectives.

Budd-Chiari syndrome (BCS) is a rare hepatic vascular disorder defined by the presence of partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Several conditions can lead to BCS, from hypercoagulable states to malignancies. Primary BCS is the most common subtype, and usually bartends hypercoagulability states, while secondary BCS involves tumor invasion or extrinsic compression. A combination of clinical and imaging features leads to the diagnosis of BCS, including (1) direct signs: occlusion or compression of the hepatic veins and/or inferior vena cava, and the presence of venous collaterals; (2) indirect signs: morphological hepatic changes with caudate lobe enlargement; inhomogeneous enhancement, and hypervascular nodules. From a clinicopathological point of view, two forms of BCS can be distinguished: acute and subacute/chronic BCS, although asymptomatic and fulminant forms are also possible. Acute presentations are rare, and symptoms include hepatomegaly, ascites, and hepatic insufficiency. Subacute/chronic forms are the most common presentation, with dysmorphic liver and variable degrees of fibrosis deposition. Patients with chronic BCS can develop benign regenerative nodules (large regenerative nodules or FNH [Focal Nodular Hyperplasia]-like lesions), but are also at a higher risk of hepatocellular carcinoma (HCC). The radiologist role is therefore fundamental in both diagnosis and surveillance of BCS. The aim of this review is to present all clinical and imaging signs that can help to reach the diagnosis of BCS, with their clinical significance, providing tips and tricks for the cross-sectional diagnosis of this condition.

Secondary Budd-Chiari syndrome occurred after adjuvant radiotherapy for perihilar cholangiocarcinoma: a case report

BackgroundBudd–Chiari syndrome (BCS) is a rare vascular disorder of the liver, and acute and secondary BCS is even rarer.Case presentationA 62-year-old man with perihilar cholangiocarcinoma of Bismuth type IIIa underwent right hemi-hepatectomy with caudate lobectomy and pancreatoduodenectomy. Adjuvant chemoradiotherapy was performed due to a positive hepatic ductal margin. Subsequently, the disease passed without recurrence. The patient visited for acute onset abdominal pain at the 32nd postoperative month. Multidetector-row computed tomography (MDCT) showed stenosis of the left hepatic vein (LHV) root, which was the irradiated field, and thrombotic occlusion of the LHV. The patient was diagnosed with acute BCS caused by adjuvant radiotherapy. Although anticoagulation therapy was performed, the patient complained of sudden upper abdominal pain again. MDCT showed an enlarged LHV thrombus and hepatomegaly. The patient was diagnosed with exacerbated acute BCS, and stenting for the stenotic LHV root was performed with a bare stent. Although stenting for the LHV root was very effective, restenosis occurred twice due to thrombus in the existing stent, so re-stenting was performed twice. The subsequent clinical course was acceptable without recurrence or restenosis of the LHV root as of 6 months after the last stenting using a stent graft.ConclusionAlthough no case of BCS caused by radiotherapy has yet been reported, the present case showed that late side effect of radiotherapy can cause hepatic vein stenosis and secondary BCS.

Percutaneous Sclerotherapy for Budd-Chiari Syndrome Secondary to Giant Hepatic Venous Malformations (Hemangiomas)

This prospective study evaluated the safety and effectiveness of percutaneous sclerotherapy in the treatment of secondary Budd-Chiari syndrome due to hepatic venous malformations (HVMs). Four patients (mean age, 40 years; 3 women) with 5 HVMs underwent 7 sessions of percutaneous sclerotherapy with a mixture of bleomycin and lipiodol. All patients had chronic Budd-Chiari syndrome, determined based on imaging findings, with the main symptom being abdominal discomfort and distention. On physical examination, 2 patients had ascites and the other 2 had an epigastric mass. The indication for treatment was intractable abdominal symptoms due to hepatic and/or inferior vena cava (IVC) outflow compression. All procedures were technically successful, with no major complications. Three patients underwent a second session because of incomplete IVC decompression. The patients’ symptoms completely resolved at 6 and 12 months of follow-up. There was a significant reduction in lesion volume (P = .007) and an increase in IVC luminal area (P = .018) at 12 months of follow-up.

Living Donor Liver Transplantation with Left Lobe for Budd-Chiari Syndrome - A Challenging Procedure

Budd-Chiari syndrome (BCS) represents a rare medical entity which has an estimated incidence of 0.1 to 10 people per million every year. It is defined by the obstruction of the flow in the inferior vena cava or the hepatic veins. Various classifications have been proposed. So, it can be acute or chronic and primary or secondary. The chronic form is more frequent and is characterized by signs of portal hypertension. Liver transplantation is the ultimate therapeutic management of Budd-Chiari syndrome. Primary BCS is mostly a result of hematological disorders and hypercoagulable conditions. Secondary BCS appears due to invasion or extrinsic pressure of the veins from various reasons, including hepatocellular carcinoma (HCC), liver abscesses and cysts. We presented a rare case of a young lady with Budd Chiari syndrome and IVC thrombosis who were transplanted with hepatic left lobe from her sister.

Application of ex vivo liver resection and autotransplantation in treating Budd-Chiari syndrome secondary to end-stage hepatic alveolar echinococcosis: A case series.

Background:Secondary Budd-Chiari syndrome (BCS) occurs due to a blockage in the liver caused by invasion or compression by a large lesion. Conventional treatments for BCS do not solve practical problems, wherease liver transplantation has been only applied as a last-resort therapy and as the only opportunity for a radical cure. We explored the feasibility of applying ex vivo liver resection and autotransplantation (ELRA) for the new indications of treating patients with end-stage hepatic alveolar echinococcosis (HAE). Our center has firstly proposed the idea and successfully treated the 49 patients with HAE. This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE.Methods:According to the degree of lesion invasion and surgical options, 11 patients were divided into 4 types. These 11 patients had large lesions that invaded the second and third hilum of the caudate lobe and involved the confluence of the hepatic vein and the inferior vena cava, suprahepatic vena cava, or at least 2 hepatic veins and led to secondary BCS. The aim of the present work was to report 11 patients with life-threatening diseases who underwent ELRA (ex vivo liver resection and autotransplantation) for secondary BCS, to propose a classification system for secondary BCS, and to suggest that secondary BCS is an indication for ELRA.Results:Eleven patients successfully underwent ELRA without intraoperative mortality. The median autograft weight was 690 g (440–950 g); operative time was 12.5 hours (9.4–16.5 hours); Postoperative hospital stay was 15 days (7–21 days). Clavien-Dindo grade IIIa or higher postoperative complications occurred in only 5 patients.Conclusions:This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE, not only provides new ideas for alternative treatments of secondary BCS, but also provides a classification system for secondary BCS. This article describes the technical process of outflow tract reconstruction and the experience for expanding the indications for ELRA. Our study demonstrated that ELRA is well feasible for treatment of BCS secondary to advanced HAE.

Paroxysmal nocturnal hemoglobinuria and liver transplantation, a new paradigm.

Paroxysmal nocturnal hemoglobinuria (PNH) is a type of hemolytic anemia acquired by the PIG-A gene mutation. This causes a deficiency of a complement regulatory protein, CD59, which results in hemolysis, hemoglobinuria and thrombosis (due to the release of procoagulant factors). Budd-Chiari syndrome is characteristic in these patients and has classically been considered as a contraindication for liver transplantation (LT) due to post-transplant recurrence. Since the approval of eculizumab for the treatment of PHN, disease control is possible and therefore the post-transplant recurrence of thrombotic phenomena involving the liver is avoided.

Budd Chiari Syndrome and Intrahepatic Cholangiocarcinoma, An Unusual Combination: Case Report and Review of the Literature

Intrahepatic cholangiocarcinoma arising in the setting of Budd Chiari syndrome is uncommon and its prognostic and management implications differ from hepatocellular carcinoma. We report a case of intrahepatic cholangiocarcinoma in a patient with primary Budd Chiari syndrome. Hepatocellular carcinoma is known to occur with Budd Chiari syndrome. It was difficult to differentiate from hepatocellular carcinoma in the presence of increased alfa-fetoprotein levels. The contrast imaging showed features of progressive enhancement in the arterial, portal, and venous phases. A targeted liver biopsy showed histological features typical for cholangiocarcinoma. Immunostaining for cytokeratin 7 and cytokeratin 20 were positive, whereas that for arginase was negative, suggesting an intrahepatic cholangiocarcinoma. The patient was planned for inferior vena cava angioplasty followed by resection for intrahepatic cholangiocarcinoma. Previously, only secondary Budd Chiari syndrome developing in the background of primary liver tumor has been described; no report exists of intrahepatic cholangiocarcinoma arising in background of primary Budd Chiari syndrome.

Síndrome de Budd Chiari de aparición en el puerperio: Reporte de caso y revisión de la literatura

El síndrome de Budd-Chiari es una entidad definida como la obstrucción al flujo sanguíneo en el tracto de salida hepático. Esta obstrucción se puede localizar desde las pequeñas venas hepáticas hasta la entrada de la vena cava inferior a la aurícula derecha. En la mayoría de los casos, se ocasiona por una trombosis endoluminal conocida como Budd-Chiari primario y secundario, cuando ocurre por una compresión extrínseca asociada a las lesiones que ocupan espacio como malignidad. Por tanto, los estados trombofílicos hereditarios se conocen como los principales factores de riesgo. El embarazo y el puerperio, al ser estados hipercoagulables, se pueden asociar al síndrome de Budd-Chiari, sin embargo, la prevalencia reportada en la literatura es muy variable dependiendo de la población estudiada. En Colombia no hay estudios de incidencia o prevalencia de la enfermedad; existen varios reportes de caso, pero no relacionados con el embarazo.&#x0D; Reportamos el caso de una paciente, que 12 semanas posparto presentó un síndrome de Budd-Chiari; además presentamos el manejo ofrecido, la evolución clínica y una revisión de la literatura de los casos asociados con el embarazo.

Budd-Chiari syndrome - various etiologies and imagistic findings. A pictorial review.

Budd Chiari syndrome defines an obstruction of the hepatic venous outflow. Primary causes include pro-coagulant states resulting in venous thrombosis, while secondary Budd Chiari syndrome appears in conditions associated with extrinsic compression of the hepatic veins or tumor invasion. Clinical presentation is greatly varied, from incidentally discovered asymptomatic thrombosis to fulminant liver failure due to hepatic congestion. Abdominal ultrasonography is the key diagnostic tool of Budd Chiari syndrome. This pictorial essay aims to show the ultrasonographic aspect of Budd-Chiari syndrome associated with other medical conditions (abdominal malignancy, hematologic disorders and abdominal surgery).

Usability of Inferior Vena Cava Interposition Graft During Living Donor Liver Transplantation: Is This Approach Always Necessary?

PurposeTo share the outcome of caval reconstruction technique in patients who underwent living donor liver transplantation (LDLT) with inferior vena cava (IVC) interposition grafting. MethodsBetween January 2009 and December 2018, an artificial or homologous interposition vascular graft was used for the continuity of resected native (IVC) due to various reasons in 29 of 1740 patients who underwent LDLT at our institute. Demographic, clinical, and radiological data were prospectively collected and retrospectively analyzed. ResultsSixteen female and 13 male patients ranging 6–67 years of age were included. Right, left, and left lobe lateral segments were used in 22, 5, and 2 patients, respectively. The three leading LDLT indications were primary or idiopathic Budd-Chiari syndrome (BCS) (n = 12), alveolar echinococcosis (n = 7), and secondary BCS (n = 5). The three leading indications for IVC interposition grafting were thrombosis, dense fibrosis, and IVC invasion caused by tumor or echinococcosis. Homologous IVC graft was used in 17, homologous aortic graft in 7, and Dacron graft in 5 patients. Throughout the follow-up period, ascites ± pleural effusion and elevated liver enzymes were detected in 12 and 4 patients, respectively. Stenosis and/or thrombosis requiring one or more procedures such as 1–6 sessions balloon angioplasty, stent, and thrombus aspiration were observed in half of the patients. ConclusionRetrohepatic IVC damages are not a contraindication for LDLT. The presence or absence of venous collateral circulation is an important indicator of the need for IVC interposition graft use.

Transthoracic cholecystectomy following biliary pancreatitis arising from a thoracic herniated gallbladder

Asymptomatic right diaphragmatic rupture with liver and gallbladder herniation and secondary Budd-Chiari syndrome is a rare complication of abdominal trauma. In this setting, the management of gallbladder stones remains poorly described and may require a thoracic approach.

Diagnosis of Budd-Chiari syndrome.

Budd-Chiari syndrome (BCS) is defined by clinical and laboratory signs associated with partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Primary BCS is the most frequent type and is a complication of hypercoagulable states, in particular myeloproliferative neoplasms. Secondary BCS involves tumor invasion or extrinsic compression. Most patients present with chronic BCS including a non-cirrhotic, dysmorphic, chronic liver disease with various degrees of fibrosis deposition. Acute BCS is rare, and patients present with hepatomegaly, ascites, and hepatic insufficiency. The diagnosis is based on imaging. Imaging features include (1) direct signs, in particular occlusion or compression of the hepatic veins and/or the inferior vena cava and venous collaterals and (2) indirect signs, in particular morphological changes in the liver with hypertrophy of the caudate lobe and delayed nodule formation. Ultrasound and magnetic resonance imaging are the gold standard for diagnosis. The aim of this review is to provide an overview of the role of imaging in the diagnosis of BCS.

Early radiological intervention and haematology screening is associated with excellent outcomes in Budd-Chiari syndrome.

Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder, resulting from thrombosis of the hepatic veins. Various treatments, including pharmacological, radiological and surgical interventions, have been used. To describe retrospectively our institution's experience with management of patients with BCS. A retrospective study of all cases of primary Budd-Chiari syndrome presenting to our institution between January 2000 and August 2012 was performed. Patients with secondary Budd-Chiari syndrome due to malignancy or local mass compression were excluded. Between 2000 and 2012, 27 patients with primary BCS presented with a median Rotterdam score of 1.16 (range: 0.07-2.11). A total of 24 patients (89%) had at least one risk factor, with the commonest being myeloproliferative neoplasm (MPN), detected in 17 of 24 (71%) of the tested patients, including four patients with normal blood counts at diagnosis. All patients were anticoagulated with warfarin or low-molecular-weight heparin (LMWH). A total of 25 (92.6%) patients also had primary radiological interventions, consisting of transjugular intrahepatic portosystemic shunt (TIPS) in 18 (67%) patients and/or angioplasty/stenting in 11 (40%). A total of 14 patients developed TIPS stenoses, requiring a median of 1.5 (range: 1-14) revisions. No patient developed TIPS failure requiring alternative therapy. Two patients were lost to follow-up. At a median follow up of 59 months (range: 2-248 months), the overall survival was 96% at 1 year and 81% at 5 years, much greater than predicted by the Rotterdam score. No patients required liver transplantation. There is a high incidence of MPN in patients with primary BCS, including patients with normal peripheral blood counts at the time of diagnosis. Our approach of anticoagulation, aggressive and early radiological intervention aimed at rapid decompression of the congested liver resulted in excellent medium-term outcomes.

Good outcome following liver transplantation using pericardial-peritoneum window for hepato-atrial anastomosis to overcome advanced hepatic alveolar echinococcosis and secondary Budd-Chiari Syndrome - a case report

BackgroundThis report presents a case of a 57- year old female with advanced Hepatic Alveolar Echinococcosis causing a secondary Budd-Chiari Syndrome due to infiltration of the suprahepatic inferior vena cava treated successfully by liver transplantation.Case presentationA temporary veno-venous bypass was introduced, but a typical end to end cavo-caval anastomosis wasn’t possible in this case. In order to access a disease free part of the inferior vena cava, an oval window of the diaphragm was excised, providing communication between the peritoneum and pericardium. A vascular clamp was placed onto the right atrium which allowed for an atrial-caval anastomosis. The remainder of hepatectomy was performed in a conventional manner. In the post-operative period and during the 18 month follow-up there were no complications. The patient remains in good general condition with optimal graft function.ConclusionsA hepato-atrial anastomosis with a pericardial-peritoneum window during liver transplantation is feasible and extends the curability potential for patients with advanced Hepatic Alveolar Echinococcosis considered for liver transplantation.

Trans-arterial Embolization of Focal Nodular Hyperplasia in a Teenager: Beyond Pain Control

Background: Focal nodular hyperplasia (FNH) is the most common benign non-vascular liver tumor. It is predominantly found in women (9:1 ratio) between ages 30 to 50 years. Intervention is usually reserved for symptomatic FNH lesions. Trans-arterial embolization has been shown to be a reasonable alternative to surgical resection. Budd-Chiari syndrome is a potentially deadly phenomenon in which the hepatic venous outflow tract is obstructed, which can lead to liver failure. Secondary Budd-Chiari is caused by compression of the hepatic veins or inferior vena cava (IVC) by lesions originating outside of the vein (e.g. tumors). Case: A 19 year old girl was referred to Hepatology Clinic for management of symptomatic FNH, progressive in size and associated right upper quadrant pain and fullness over a period of five years. At the time of presentation she had two MRIs of the abdomen over a three month interval, showing an 3.5 X 5.7 cm enlarging mass abutting the IVC involving segment VIII, extending into the caudate lobe. Physical exam was significant for reproducible right upper quadrant tenderness. Labs including liver function tests were normal. After an unremarkable EGD was performed to evaluate for alternative causes of abdominal pain, and Hepatobiliary Surgery had deemed resection a highrisk intervention, the patient was referred to Interventional Radiology for embolization of her FNH. During the procedure, by arteriogram, a hypertrophied branch of right hepatic artery was found to be the primary vascular supply of the FNH. This branch was embolized with lipiodiol and ethanol which until cessation of flow into the artery supplying the targeted mass. Follow up MRI imaging of abdomen at 6 months post-procedure show positive response with progressively diminishing size of residual FNH (2.8 X 1.7 cm). At 6 month post-procedural follow up patient reported complete resolution of symptoms at rest. Discussion: Although typically presenting in adult women ages 30-50, this case highlights the need to recognize FNH as a potential cause for right upper quadrant in teenage girls. In this case the medical teams decided to pursue embolization not only because of the mass effect, but also because of the abutment on the hepatic veins and IVC. Early recognition and therapeutic embolization of FNH can not only safely lead to significant symptomatic pain relief, it can also decrease risk of venous outflow obstruction and by extension prevent secondary Budd-Chiari syndrome.

A rare case of hepatocellular carcinoma with right atrial invasion

An elderly male came with h/o loss of appetite and weight loss since 3 months. On evaluation, prothrombin time (PT), activated partial thromboplastin time (aPTT), and international normalized ratio (INR) values were high. Hepatitis B surface antigen (HbSAg) screening was reactive and serum alfa-fetoprotein (AFP) was raised. Ultrasound examination revealed a hepatic mass with inferior vena caval extension and direct invasion of the right atrium. Computed tomography (CT) scan of the lower thorax and abdomen was performed, which confirmed the ultrasound diagnosis. The patient had a brief stay in the hospital and got discharged against medical advice; he was not available for follow-up. Hepatocellular carcinomas (HCCs) commonly metastasizes to the lung, bone, brain, and adrenal glands. About 70% of the patients with HCCs have hepatic and portal vein invasions but encroachment into the right atrium is very rare. The most common symptoms of cardiac metastasis include asymptomatic cases, bilateral lower leg edema, and exertional dyspnea in that order. Typical complications of intravascular tumor extension lead to secondary Budd-Chiari syndrome, right heart insufficiency, or massive pulmonary embolism. The prognosis of HCC with atrial invasion is poor, with median survival ranging from 1 month to 4 months. HCC in these cases may be more aggressive, with a shorter doubling time. The risk for cardiopulmonary collapse is higher, with heart failure or sudden death. Surgical interventions as well as nonsurgical approaches have been used in the treatment of patients with symptomatic inferior vena cava (IVC)/right atrial tumor thrombi. There are few reports of en bloc hepatectomy and resection of the right atrial thrombus under cardiopulmonary bypass. Routine screening in patients with HCC, such as including chest imaging as part of surveillance computed tomographic scans, may facilitate earlier detection and treatment.

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.