The epidemiology of Budd–Chiari syndrome in France

Ollivier-Hourmand, Isabelle; Allaire, Manon; Goutté, Nathalie; Morello, Rémy; Chagneau‐Derrode, Carine; Goria, Odile; Dumortier, Jérôme; Cervoni, Jean Paul; Dharancy, Sébastien; Ganne-Carrié, Nathalie; Bureau, Christophe; Carbonell, Nicolas; Abergel, Armand; Nousbaum, Jean Baptiste; Anty, Rodolphe; Barraud, Hélène; Ripault, Marie Pierre; Lédinghen, Victor de; Minello, Anne; Oberti, Frédéric; Radenne, Sylvie; Bendersky, Noelle; Farges, Olivier; Archambeaud, Isabelle; Guillygomarc’h, Anne; Ecochard, Marie; Ozenne, Violaine; Hilleret, Marie Noelle; Nguyen‐Khac, Eric; Dauvois, Barbara; Perarnau, Jean Marc; Lefilliatre, Pascale; Raabe, J.-J.; Doffoël, Michel; Becquart, J; Saillard, Eric; Valla, Dominique; Dao, Thông; Plessier, Aurélie

doi:10.1016/j.dld.2018.04.004

Cited by 32 publications

(22 citation statements)

References 19 publications

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“…BCS is the least common manifestation of SVT, with reported incidence rates of around 1–2 cases per million inhabitants per year 8,9. The estimated prevalence of BCS varies across different countries, ranging from around 1–4 cases per million inhabitants in the West10,11 up to 5–7 cases per million inhabitants in the East 12,13…”

Section: Epidemiologymentioning

confidence: 99%

<p>Splanchnic Vein Thrombosis: Current Perspectives</p>

Valeriani¹,

Riva²,

Nisio³

et al. 2019

VHRM

110

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Splanchnic vein thrombosis (SVT) including portal, mesenteric, splenic vein thrombosis and the Budd-Chiari syndrome, is a manifestation of unusual site venous thromboembolism. SVT presents with a lower incidence than deep vein thrombosis of the lower limbs and pulmonary embolism, with portal vein thrombosis and Budd-Chiari syndrome being respectively the most and the least common presentations of SVT. SVT is classified as provoked if secondary to a local or systemic risk factor, or unprovoked if the causative trigger cannot be identified. Diagnostic evaluation is often affected by the lack of specificity of clinical manifestations: the presence of one or more risk factors in a patient with a high clinical suspicion may suggest the execution of diagnostic tests. Doppler ultrasonography represents the first line diagnostic tool because of its accuracy and wide availability. Further investigations, such as computed tomography and magnetic resonance angiography, should be executed in case of suspected thrombosis of the mesenteric veins, suspicion of SVT-related complications, or to complete information after Doppler ultrasonography. Once SVT diagnosis is established, a careful patient evaluation should be performed in order to assess the risks and benefits of the anticoagulant therapy and to drive the optimal treatment intensity. Due to the low quality and large heterogeneity of published data, guidance documents and expert opinion could direct therapeutic decision, suggesting which patients to treat, which anticoagulant to use and the duration of treatment.

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Section: Epidemiologymentioning

confidence: 99%

<p>Splanchnic Vein Thrombosis: Current Perspectives</p>

Valeriani¹,

Riva²,

Nisio³

et al. 2019

VHRM

110

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“…Interestingly, when these patients were re-analysed during follow-up, additional aetiological factors were diagnosed in 12 previously unclassified patients. Nonetheless, recent data from France and Italy describe the absence of a prothrombotic factor in 30% 1 and 61% 2 of patients respectively, maybe due to the intrinsic limitations of population studies. Differences between Eastern and Western countries are found and the prevalence of prothrombotic disorders in China seems to be very low.…”

Section: Bcs and Ncpvt Risk Factorsmentioning

confidence: 99%

“…The estimated incidence of BCS and NCPVT in the absence of cirrhosis and cancer is 1 per million per year and 0.35-2.5 cases per 100,000 per year, respectively. [1][2][3] Most patients with BCS and NCPVT have identifiable thrombotic risk factors. However, both entities represent only $1% of all venous thromboembolic events, implying other local factors besides thrombophilic disorders for developing BCS or NCPVT.…”

Section: Bcs and Ncpvt Risk Factorsmentioning

confidence: 99%

“…In the European BCS cohort, the presence of local trauma, inflammatory diseases and abdominal infections was only reported in 11% of patients, 8 although in more recent data from the French survey it was found in 25% of patients. 1 In patients with NCPVT, a local cause should be exhaustively investigated, as it may be present in approximately 30% of cases. Initial imaging studies (CT or MRI) performed during the diagnostic work-up should be examined carefully as they may reveal signs of gastrointestinal (appendicitis, diverticulitis, intra-abdominal abscesses or infections) or biliopancreatic pathologies including pancreatic pseudocysts and gallbladder alterations.…”

Section: Local Risk Factorsmentioning

confidence: 99%

“…Concomitant splanchnic vein thrombosis and BCS has also being described although the incidence varies from 3.8-21% in epidemiological studies. 1 Another clinical finding in patients with chronic BCS is the presence of benign hepatic regenerative nodules. 72,73 Although the pathogenesis remains unclear, the coexistence of focal defects of portal perfusion and hypervascularized areas of preserved venous outflow may be involved in their development.…”

Section: Natural History Bcsmentioning

confidence: 99%

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Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis

Hernández‐Gea

Gottardi

Leebeek

et al. 2019

Journal of Hepatology

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Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities.

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