We report the use of preemptive dual-cannula venovenous extracorporeal membrane oxygenation (VV-ECMO) during orthotopic liver transplantation in a 21-year-old patient with Budd-Chiari syndrome (BCS) with diffuse hepatic vein thrombosis and inferior vena cava (IVC) narrowing. Progressive liver dysfunction and unsuccessful transjugular intervention led to urgent transplantation. Given the anticipated intraoperative cardiopulmonary instability and surgical complexity, VV-ECMO was initiated before IVC clamping. Classic hepatectomy with portocaval shunt was performed. Portal flow was restored without post-reperfusion syndrome. Protamine was not administered, and no ECMO-related complications occurred. This case highlights the role of preemptive ECMO as a safe strategy in high-risk liver transplantation because of the anticipated severe hemodynamic instability during hepatectomy and IVC clamping due to BCS with extensive hepatic vein thrombosis and IVC stenosis, providing continuous cardiopulmonary support.

Hepatic vascular disorders include portal hypertension in cirrhosis, Budd-Chiari syndrome (BCS), hepatic vascular malformations, and vascular complications after liver transplantation. These diseases depend on blood-flow direction, flow redistribution, and pressure transmission, and not only on gross vessel shape. Doppler ultrasound, computed tomography angiography (CTA), and digital subtraction angiography (DSA) are widely used in current clinical practice, but each has distinct limits for full hemodynamic assessment, and repeated follow-up can be difficult to implement effectively in daily clinical work. Four-dimensional flow magnetic resonance imaging (4D flow MRI) is a time-resolved three-dimensional (3D) phase-contrast MRI method with three-directional velocity encoding (VENC). It provides volumetric flow and multidirectional velocity data across the cardiac cycle, and it allows flexible retrospective plane placement and comprehensive network-level analysis. It can accurately measure flow volume, peak and mean velocity, retrograde fraction, fractional flow change, and shear-related metrics. It also supports pressure-gradient estimation when combined with physics-based modeling in selected clinical settings. Hepatic imaging still faces multiple major constraints, including VENC tradeoffs, respiratory motion, limited small-vessel resolution, and the need for unified standardized acquisition and analysis. Workflow limits from segmentation and plane placement can be greatly reduced by modern automated analysis. This review systematically summarizes technical foundations, processing steps, disease-focused applications, and practical barriers for clinical translation.

Budd-Chiari Syndrome (BCS), a rare vascular disorder of the liver characterized by obstruction of hepatic venous outflow, has an estimated prevalence of approximately 1 in 100,000 in the general population. Among its various etiologies, coagulation factor deficiencies are uncommon. This case, following Case Report (CARE) guidelines, describes a case of BCS associated with protein C deficiency resulting in a thrombus in the inferior vena cava. The patient was an unmarried Indian woman in her 40s who presented with progressive pedal edema and abdominal distension for 6 months. She was evaluated thoroughly, and other causes were excluded. Imaging showed classical features of BCS, including complete fibrotic occlusion of all three hepatic veins, intrahepatic venous and splenorenal collaterals, caudate lobe enlargement, and a 5.2 cm thrombus in the inferior vena cava. She was treated with interventional angioplasty along with anticoagulation and diuretic therapy. She is under regular follow-up and has been counseled for liver transplantation for liver cirrhosis. This case highlights the rare association of protein C deficiency with BCS and emphasizes the need for early recognition, multidisciplinary management, and supportive nursing care to improve outcomes and quality of life.

Unlike deceased-donor liver transplantation, living-donor liver transplantation (LDLT) for Budd-Chiari Syndrome (BCS) presents distinctive challenges in hepatic venous (HV)-outflow reconstruction because diseased HV-inferior vena cava (IVC) cannot be entirely replaced with healthy donor vessels. In the Asia-Pacific region, where LDLT predominates, BCS frequently involves the hepatic IVC, thus requiring innovative surgical strategies for HV-IVC reconstruction. Here we introduce a novel series of surgical technique-"intrapericardial approach (IPA)"-which reconstructs HV-outflow tract in the pericardium using only healthy vascular tissues, thereby minimizing post-transplant BCS recurrence. Detailed procedures are illustrated for four typical LDLT scenarios, classified by graft-type (left- or right-lobe) and by the need for IVC reconstruction (acute or chronic BCS). A representative surgical video is also provided. Of our 2089 consecutive liver transplants, 20 patients underwent LDLT for BCS: 10 with conventional IVC-preserving techniques (cavoplasty and/or patch-plasty) and 10 using IPA. Across all four clinical scenarios aforementioned, the key concept of IPA-creating new HV-outflow with only healthy vessels-was consistently applicable. IPA was associated with significantly lower post-transplant BCS recurrence (p = 0.04) and improved both relapse-free and overall recipient survival (p = 0.02 and 0.03, respectively) compared to conventional techniques, yielding 100% graft and patient survival through 10 years. This report also highlights key considerations and pitfalls for pre-, intra-, and post-transplant management in BCS-LDLT. In conclusion, IPA appears to be associated with reduced post-transplant BCS recurrence and favorable short- and long-term outcomes, offering a novel practical solution for challenging HV-IVC reconstruction in BCS-LDLT.

Budd-Chiari syndrome (BCS) is a clinical syndrome caused by stenosis or occlusion of the hepatic veins and/or the inferior vena cava (IVC) near their orifices. Endovascular therapy is a common therapeutic approach. However, the puncture procedure during angioplasty may damage adjacent arteries, compromising patient safety and affecting clinical outcomes. This report describes a case of a patient with BCS who suffered an inadvertent injury to the origin of the right common carotid artery (CCA) during IVC angioplasty under angiographic guidance. Through multidisciplinary consultation and emergency intervention, three covered stents were precisely placed at the bleeding site, achieving successful hemostasis. This case report aims to provide a guidance for the early clinical recognition and management of such intraprocedural complications.

Hepatobiliary emergencies include both traumatic and nontraumatic pathologies that can affect the gallbladder, bile ducts, and liver. Due to the complexity and urgency of these conditions, advanced imaging techniques are required for rapid, accurate diagnosis. Traumatic liver emergencies were historically treated surgically; however, with the implementation of contrast-enhanced computed tomography (CT), management options have evolved. Contrast-enhanced CT imaging can provide additional insight into the extent and severity of injuries, enabling clinicians to decide between operative and nonoperative management with greater confidence. Furthermore, nontraumatic biliary emergencies present diagnostic challenges due to the diversity in their clinical presentations. When biliary pathology is suspected, the use of ultrasound, CT, and magnetic resonance imaging (MRI) aids in differentiating between conditions such as acute cholecystitis, acalculous cholecystitis, gangrenous cholecystitis, and emphysematous cholecystitis, thereby directing management strategies. Moreover, liver pathologies such as liver abscesses, hemorrhagic liver lesions, and vascular disorders demand integrated imaging assessments. The combination of ultrasound, CT, and MRI facilitates the differentiation between pyogenic and parasitic abscesses, evaluation of spontaneous liver ruptures, and diagnosis of portal vein thrombosis and Budd-Chiari syndrome, guiding clinical interventions. In addition, biloma and cholangitis, while less frequent, require precise diagnosis. The use of multimodality imaging enables early detection and intervention while mitigating potential complications. In conclusion, this review highlights the key role of advanced imaging in hepatobiliary emergencies and provides a comprehensive overview to help clinicians navigate complex scenarios effectively.

Budd-Chiari syndrome (BCS) is a rare hepatic vascular disorder frequently associated with prothrombotic conditions such as myeloproliferative neoplasms. Pregnancy and assisted reproductive technologies further increase thrombotic and portal-hypertensive risk. We report a successful in vitro fertilization-conceived pregnancy in a 35-year-old woman with chronic BCS secondary to JAK2-positive polycythemia vera. The pregnancy was complicated by ovarian hyperstimulation syndrome and severe intrahepatic cholestasis of pregnancy. Progressive portal hypertension with worsening esophageal varices prompted planned preterm delivery at 34 weeks' gestation. A multidisciplinary team performed cesarean delivery under epidural anesthesia with prophylactic perioperative terlipressin administration and thromboprophylaxis. Maternal and neonatal outcomes were favorable. With careful multidisciplinary management, pregnancy in women with stable BCS is feasible.

Manganese (Mn) toxicity is a relatively uncommon neurotoxic condition characterized by neuropsychiatric symptoms and parkinsonism. We describe a 46-year-old woman with end-stage renal disease due to congenital obstructive uropathy, status post deceased-donor kidney transplantation (2021), who presented with one month of worsening tremors, gait instability, intermittent aphasia, and altered awareness. Brain magnetic resonance imaging demonstrated persistent but improved T1 hyperintensity of the globus pallidus compared to prior imaging, consistent with Mn deposition. Serum Mn was markedly elevated at 14.9 µg/L (reference range 0.5-1.2 µg/L). Chelation therapy with calcium disodium ethylenediaminetetraacetic acid was initiated but discontinued early due to decline in renal function. Despite early cessation, treatment resulted in biochemical improvement and partial clinical recovery. Kidney allograft biopsy, performed due to a rise in creatinine during the hospital course, demonstrated severe chronic changes without evidence of acute rejection. Thorough history taking and evaluation of environmental factors did not identify a clear source of Mn exposure. The patient's history was notable for Budd-Chiari syndrome with portal hypertension status post transjugular intrahepatic portosystemic shunt, which likely contributed to impaired Mn clearance. To our knowledge, Mn toxicity occurring in a functioning kidney transplant recipient has not previously been reported. This case underscores the diagnostic and therapeutic challenges of Mn toxicity.