バッド・キアリ症候群

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.