Budd-Chiari syndrome is a rare cause of hepatic venous outflow obstruction, with heterogenous etiologies. We report the case of a 35-year-old man with chronic, compensated Budd-Chiari syndrome due to retrohepatic inferior vena cava (IVC) occlusion, likely resulting from early-life thrombosis. Imaging and venography confirmed a 3-cm IVC interruption with extensive alternative venous drainage. Endovascular recanalization, balloon venoplasty, and stent placement successfully restored hepatic venous outflow, leading to improvement in hepatic congestion and complete resolution of symptoms. This case highlights the importance of advanced imaging in diagnosing chronic thrombotic occlusion and demonstrates that preserved hepatic venous drainage from chronic venous rerouting may allow successful IVC recanalization/stenting without the need for transjugular intrahepatic portosystemic shunt placement.

Liver vascular diseases include portal vein thrombosis (with or without cirrhosis), portal-sinusoidal vascular disease, Budd-Chiari syndrome, hepatic sinusoidal obstruction syndrome, non-obstructive sinusoidal dilatation and peliosis hepatis, visceral artery aneurysms, and hepatic arteriovenous fistulas. Importantly, with the exception of portal vein thrombosis in cirrhosis, the others are rare conditions. A substantial body of evidence has been published on the diagnosis, pharmacological treatment, and interventional therapy of liver vascular disorders since the European Association for the Study of the Liver issued the previous version of the clinical practice guidelines in 2016. These guidelines are based on a comprehensive review of relevant literature, providing recommendations for key clinical challenges and emphasizing implementation of individualized treatment by combining individual risk factors and clinical presentations. Thus, multidisciplinary collaborative management involving hepatologists, hematologists, pathologists, interventional radiologists, and surgeons is crucial in this field. The aim of this guideline is to provide guidance for the management of liver vascular diseases based on the best available evidence.

Behçet's disease (BD) is a chronic, relapsing multisystem inflammatory disorder classified as a variable-vessel vasculitis. Venous thrombosis is common, and Budd-Chiari syndrome (BCS) represents one of the most severe manifestations, whereas coronary artery involvement is rare and associated with poor prognosis. Concomitant presentation of BCS and coronary pseudoaneurysm has been rarely reported. A 23-year-old man with a history of BCS caused by thrombotic occlusion of the retrohepatic inferior vena cava (IVC), previously treated with balloon dilatation and anticoagulation, presented with acute chest pain and dyspnea. Transthoracic echocardiography revealed severe anterior wall motion abnormalities with a left ventricular ejection fraction of 25% and findings suggestive of a left anterior descending artery (LAD) pseudoaneurysm with suspected contained rupture, which were further supported by contrast-enhanced computed tomography. Coronary angiography demonstrated diffuse critical stenosis of the proximal LAD with pseudoaneurysm formation and severely impaired distal flow. Percutaneous coronary intervention with sequential stent implantation, including a covered stent, was performed to seal the pseudoaneurysm and restore coronary perfusion. Subsequent targeted history-taking revealed recurrent oral aphthous ulcers, scrotal ulceration, and lower-limb skin lesions, fulfilling the International Criteria for Behçet's Disease. Immunosuppressive therapy was initiated following revascularization, resulting in significant clinical and functional improvement during follow-up. In young patients presenting with myocardial infarction in the absence of conventional cardiovascular risk factors, particularly those with coexisting Budd-Chiari syndrome (BCS) or unexplained venous thrombosis, Behçet's disease (BD) should be strongly considered. Multimodality cardiovascular imaging is essential not only for establishing the diagnosis but also for guiding interventional strategies and subsequent immunosuppressive therapy in vascular Behçet's disease, and for prognostic assessment.

Vascular liver diseases are recognized risk factors for the development of hepatocellular carcinoma (HCC) in non-cirrhotic livers. While the association is well established in conditions like Budd-Chiari syndrome, which have defined surveillance strategies, data regarding HCC in non-cirrhotic portal fibrosis/idiopathic portal hypertension (NCPF/IPH) remain limited. We report a case of incidentally detected HCC in a 52-year-old woman with NCPF/IPH and preserved liver function. She initially presented with portal hypertension without hepatic decompensation and was diagnosed with NCPF/IPH based on histology and clinical criteria. Sixty-four months after her diagnosis, imaging revealed a large well-defined hepatic mass lesion, which showed arterial-phase hyperenhancement and porto-venous-phase washout with markedly elevated alpha-fetoprotein levels, consistent with HCC. Notably, the tumor developed in the absence of any prior hepatic decompensation and was detected incidentally during evaluation for portal vein thrombosis. This case highlights that HCC can occur in NCPF/IPH despite preserved liver function, the absence of severe portal hypertension, and prior nodules; it may also remain clinically silent. To our knowledge, this represents the first reported case of HCC in NCPF/IPH from India.

The reno-hemiazygos venous connection is a rare developmental variant that arises from the persistence of transient embryonic communications. A 75-year-old male underwent contrast-enhanced abdominal computed tomography (CT) for evaluation of abdominal pain. Imaging revealed a normal left renal vein (LRV) draining into the inferior vena cava (IVC) and an additional posterior connection from the LRV to the hemiazygos vein. No evidence of IVC obstruction or renal abnormality was present. Recognition of a reno-hemiazygos connection is important to prevent misinterpretation as a pathologic retroperitoneal vessel or lymphadenopathy, to appreciate its potential role as a collateral pathway in cases of IVC obstruction or Budd-Chiari syndrome, and to aid in preoperative planning to minimize the risk of vascular injury or bleeding during retroperitoneal or renal surgeries..

Background Coronavirus disease 2019 (COVID-19) has been associated with systemic effects that may influence thrombotic risk. While venous thromboembolism has been widely reported, national mortality trends for splanchnic thrombosis, including portal vein thrombosis, Budd-Chiari syndrome, mesenteric venous thrombosis, and mesenteric ischemia, remain poorly characterized. Methods The CDC WONDER Multiple Cause of Death database was queried for 2018-2023. Annual deaths, population counts, and crude mortality rates per 100,000 persons were extracted and stratified by year and sex. Mortality patterns during the pre-pandemic period (2018-2019) were compared with the pandemic/post-pandemic period (2020-2023) using descriptive trend analysis. Results A total of 32,738 deaths were attributed to splanchnic thrombosis during the study period. Annual deaths increased from 4,819 in 2018 to 5,986 in 2023. The pre-COVID-19 annual average was 4,931 deaths compared with 5,719 deaths during 2020-2023, representing a 16.0% relative increase. Crude mortality rates rose from 1.5 per 100,000 (2018-2019) to 1.7-1.8 per 100,000 (2021-2023). Increases were observed in both sexes. Conclusions Mortality from splanchnic thrombosis increased during the COVID-19 era. These findings demonstrate a temporal rise in mortality; however, the study design is descriptive and does not establish causality. Further research is needed to evaluate potential contributing factors and clinical implications.