Budd–Chiari syndrome: Focus on surgical treatment

Hidaka, Masaaki; Eguchi, Susumu

doi:10.1111/hepr.12752

Cited by 18 publications

(11 citation statements)

References 63 publications

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“…Budd-Chiari syndrome (BCS) is defined as portal hypertension with hepatomegaly caused by hepatic venous outflow obstruction at the level of the major hepatic veins (HVs) or the retrohepatic segment of the inferior vena cava (IVC) ( 1 2 ). This rare disease has a high prevalence in less-developed regions and is more common in rural areas, where sanitation and medical facilities are insufficient and social conditions facilitate the spread of disease.…”

Section: Introductionmentioning

confidence: 99%

Magnetic Resonance Venography Findings of Obstructed Hepatic Veins and the Inferior Vena Cava in Patients with Budd-Chiari Syndrome

et al. 2018

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ObjectiveThis study aimed to illustrate the magnetic resonance venography (MRV) manifestations of obstructed hepatic veins (HVs), the inferior vena cava (IVC), and accessory hepatic veins (AHVs) in patients with Budd-Chiari syndrome (BCS) and to evaluate the visualization capacity of MRV in the diagnosis of BCS.Materials and MethodsFifty-two patients with chronic BCS were included in this study. All patients were examined via MRV performed with a 3T system following injections of gadolinium-diethylene triamine pentaacetic acid (Gd-DTPA) or Gd-ethoxibenzyl-DTPA. HV and IVC lesions were classified, and their characteristics were described. HV cord-like occlusions detected via MRV were compared using ultrasonography (US). Digital subtraction angiography (DSA) was performed as a contrast in the MRV detection of IVC lesions. The HVs draining collaterals, mainly AHVs, were carefully observed. HV lesions were classified as segmental stenosis, segmental occlusion, membranous stenosis, membranous occlusion, cord-like occlusion, or non-visualized. Except for patent IVCs, IVC lesions were classified as segmental occlusion, segmental stenosis, membranous occlusion, membranous stenosis, and hepatomegaly-induced stenosis.ResultsAll patients (52/52, 100%) showed HV lesions of different degrees. MRV was inferior to US in detecting cord-like occlusions (6 vs. 19, χ2 = 11.077, p < 0.001). Dilated AHVs, including 50 (50/52, 96.2%) caudate lobe veins and 37 (37/52, 71.2%) inferior HV and AHV lesions, were well-detected. There were no significant differences in detecting segmental lesions and thrombosis between MRV and DSA (χ2 = 0.000, p1 = 1.000, p2 = 1.000). The capacity of MRV to detect membranous lesions was inferior to that of DSA (7 vs. 15, χ2 = 6.125, p = 0.013).ConclusionIn patients with BCS, MRV can clearly display the lesions in HVs and the IVC, as well as in AHVs, and it has diagnostic and therapeutic value.

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Section: Introductionmentioning

confidence: 99%

Magnetic Resonance Venography Findings of Obstructed Hepatic Veins and the Inferior Vena Cava in Patients with Budd-Chiari Syndrome

et al. 2018

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“…BCS may be completely asymptomatic and diagnosed incidentally in up to 15% of patients. 19 Untreated acute or fulminant BCS is associated with a mortality risk of up to 60%. 20 A range of treatment options is available for BCS ranging from long-term anticoagulation through radiological interventions to shunt surgery and liver transplantation.…”

Section: Discussionmentioning

confidence: 99%

Living Donor Liver Transplantation for Budd-Chiari Syndrome: A Propensity Score-Matched Analysis

Gunasekaran¹,

Reddy²,

Rammohan³

et al. 2021

Preprint

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Introduction: There are unique technical and management challenges associated with living donor liver transplantation (LDLT) for Budd-Chiari Syndrome (BCS). The outcomes of LDLT for BCS in comparison to other indications remains unclear and warrants elucidation. Methods: Data of 24 BCS patients who underwent LDLT between January 2012 and June 2019 were analyzed. There were 20 adults and 4 children. The early and long-term outcomes of adult LDLT BCS patients were compared to a control group of LDLT patients for other indications and matched using propensity scoring methodology. Results: Primary BCS was observed in 22(91.7%) patients. Caval replacement was performed in 7(29.1%) patients. Early and late hepatic venous outflow tract (HVOT) complications were seen in 1(5.5%) and 3(16.7%) patients. Preoperative acute kidney injury was identified as a risk factor for mortality in the BCS cohort (p =0.013). On comparison, BCS recipients were younger with fewer comorbidities, more large volume ascites and higher rates of PVT. They also had longer cold ischemia time, increased blood loss and transfusion requirements, increased hospital stay, and higher late outflow complications. The 1-year and 3-year survivals were similar to non-BCS cohort (84.2% vs 94% and 70% vs 91.9%, respectively, log rank test p=0.09). Conclusion: LDLT is remains a good option for symptomatic BCS who have failed non-transplant interventions. The clinical and risk factor profile of BCS recipients is distinct from non-BCS recipients. By following an algorithmic management protocol, we show on propensity-score matched analysis that outcomes of LDLT for BCS are similar to non-BCS indications.

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“…Budd-Chiari syndrome is a hepatic outflow disorder occurring in the setting of narrowing or obstruction(usually from thrombosis) of the inferior vena cava or hepatic veins. It can occur in an acute, subacute or chronic manner and may be an indication for liver transplantation in select patients [18]. Grossly, the liver becomes enlarged and patients may experience right upper quadrant pain, ascites and liver failure.…”

Section: Budd-chiari Syndromementioning

confidence: 99%