Diagnosis and Management of Budd-Chiari Syndrome: An Update in 2023

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: closed (31 May 2023) | Viewed by 11131

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Medicina Interna 1, Azienda di Rilievo Nazionale ad Alta Specializzazione Civico - Di Cristina - Benfratelli, Piazzale Leotta 4, 90100 Palermo, Italy
Interests: liver; hepatology; portal vein; hepatitis
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Dear Colleagues,

Budd–Chiari syndrome (BCS) is characterized by significant clinical and pathophysiological aspects that seem to allow a sharp differentiation between a variant in the West from one in the East. However, both diagnostic features and treatment of BCS should be managed by experienced clinicians, who are familiar with BCS. In fact, difficulties in diagnosis are frequent and there are different possible therapeutic approaches available. The former issue regards the need of agreed standardized criteria for BCS diagnosis. The latter concerns a diagnostic approach to focal liver lesions in BCS. Moreover, the timing of treatment for BCS has been a hotly debated issue. In particular, the so-called step-wise strategy for the management of BCS, generally suggested by guidelines, albeit not supported by strong evidence, has been challenged by some recently published experiences, suggesting that early interventional treatment could improve the outcome.

The aim of this Special Issue is to focus on the main issues and controversies centred around both the diagnosis and management of BCS.

Prof. Dr. Andrea Mancuso
Guest Editor

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Published Papers (5 papers)

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Editorial

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6 pages, 245 KiB  
Editorial
Budd–Chiari Syndrome Management: Controversies and Open Issues
by Andrea Mancuso
Diagnostics 2022, 12(11), 2670; https://doi.org/10.3390/diagnostics12112670 - 3 Nov 2022
Cited by 2 | Viewed by 1461
Abstract
Budd–Chiari Syndrome (BCS) is due to thrombosis of hepatic veins (HVs), inferior vena cava (IVC) or both, leading to impaired hepatic venous outflow [...] Full article
(This article belongs to the Special Issue Diagnosis and Management of Budd-Chiari Syndrome: An Update in 2023)

Review

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8 pages, 2476 KiB  
Review
Histopathology of Budd–Chiari Syndrome
by Alberto Quaglia
Diagnostics 2023, 13(15), 2487; https://doi.org/10.3390/diagnostics13152487 - 26 Jul 2023
Viewed by 1693
Abstract
The histopathological changes in Budd–Chiari syndrome (BCS) overlap with those of sinusoidal obstruction syndrome (SOS) and of cardiac or pericardiac disorders resulting in right cardiac failure. These conditions, however, are different on both clinical and pathological grounds and need to be differentiated from [...] Read more.
The histopathological changes in Budd–Chiari syndrome (BCS) overlap with those of sinusoidal obstruction syndrome (SOS) and of cardiac or pericardiac disorders resulting in right cardiac failure. These conditions, however, are different on both clinical and pathological grounds and need to be differentiated from BCS. This review is centred on the three main aspects of BCS in diagnostic liver histopathology: (1) general histopathology of BCS; (2) implications for liver biopsy interpretation; and (3) BCS in the liver allograft. The histological features of BCS form a complex spectrum which is shaped differently in each individual case according to the topographical distribution and chronological evolution of the obliterative insult, its upstream effect of the hepatic vascularisation and the consequent parenchymal injury, scarring and remodelling. Sampling variation limits the use of liver biopsy for prognostication in patients with BCS. Full article
(This article belongs to the Special Issue Diagnosis and Management of Budd-Chiari Syndrome: An Update in 2023)
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18 pages, 14755 KiB  
Review
Focal Liver Lesions in Budd-Chiari Syndrome: Spectrum of Imaging Findings
by Francesco Rizzetto, Davide Rutanni, Luca Alessandro Carbonaro and Angelo Vanzulli
Diagnostics 2023, 13(14), 2346; https://doi.org/10.3390/diagnostics13142346 - 12 Jul 2023
Cited by 1 | Viewed by 1478
Abstract
Budd–Chiari syndrome (BCS) is a rare clinical entity characterized by hepatic venous outflow obstruction, resulting in liver congestion and subsequent chronic parenchymal damage. This condition often leads to the development of focal liver lesions, including benign focal nodular hyperplasia-like regenerative nodules, hepatocellular carcinoma, [...] Read more.
Budd–Chiari syndrome (BCS) is a rare clinical entity characterized by hepatic venous outflow obstruction, resulting in liver congestion and subsequent chronic parenchymal damage. This condition often leads to the development of focal liver lesions, including benign focal nodular hyperplasia-like regenerative nodules, hepatocellular carcinoma, and perfusion-related pseudo-lesions. Computed tomography, ultrasound, and magnetic resonance are the commonly employed imaging modalities for the follow-up of BCS patients and for the detection and characterization of new-onset lesions. The accurate differentiation between benign and malignant nodules is crucial for optimal patient management and treatment planning. However, it can be challenging due to the variable and overlapping characteristics observed. This review aims to provide a comprehensive overview of the imaging features and differential diagnosis of focal liver lesions in BCS, emphasizing the key findings and discussing the challenges associated with their interpretation, with the purpose of facilitating the subsequent clinical decision-making. Full article
(This article belongs to the Special Issue Diagnosis and Management of Budd-Chiari Syndrome: An Update in 2023)
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15 pages, 6583 KiB  
Review
Budd-Chiari Syndrome Imaging Diagnosis: State of the Art and Future Perspectives
by Giorgia Porrello, Giuseppe Mamone and Roberto Miraglia
Diagnostics 2023, 13(13), 2256; https://doi.org/10.3390/diagnostics13132256 - 3 Jul 2023
Cited by 2 | Viewed by 3542
Abstract
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. [...] Read more.
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. Full article
(This article belongs to the Special Issue Diagnosis and Management of Budd-Chiari Syndrome: An Update in 2023)
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15 pages, 3964 KiB  
Review
Interventional Treatment of Budd–Chiari Syndrome
by Martin Rössle
Diagnostics 2023, 13(8), 1458; https://doi.org/10.3390/diagnostics13081458 - 18 Apr 2023
Cited by 3 | Viewed by 2148
Abstract
Medical treatment is regarded as the primary course of action in patients with Budd–Chiari syndrome (BCS). Its efficacy, however, is limited, and most patients require interventional treatment during follow-up. Short-segment stenosis or the occlusion (the so-called web) of hepatic veins or the inferior [...] Read more.
Medical treatment is regarded as the primary course of action in patients with Budd–Chiari syndrome (BCS). Its efficacy, however, is limited, and most patients require interventional treatment during follow-up. Short-segment stenosis or the occlusion (the so-called web) of hepatic veins or the inferior vena cava are frequent in Asian countries. An angioplasty with or without stent implantation is the treatment of choice to restore hepatic and splanchnic blood flow. The long-segment thrombotic occlusion of hepatic veins, common in Western countries, is more severe and may require a portocaval shunting procedure to relieve hepatic and splanchnic congestion. Since it was first proposed in a publication in 1993, the transjugular intrahepatic portosystemic shunt (TIPS) has gained more and more attention, and in fact it has been so successful that previously utilized surgical shunts are only used for few patients for whom it does not work. Both interventional treatment options can be performed successfully in about 95% of patients even after the complete obliteration of the hepatic veins. The long-term patency of the TIPS, a considerable problem in its early years, has been improved with PTFE-covered stents. The complication rates of these interventions are low and the survival rate is excellent with five- and ten-year survival rates of 90% and 80%, respectively. Present treatment guidelines recommend a step-up approach indicating interventional treatment after the failure of medical treatment. However, this widely accepted algorithm has several points of contention, and early interventional treatment is proposed instead. Full article
(This article belongs to the Special Issue Diagnosis and Management of Budd-Chiari Syndrome: An Update in 2023)
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