Otoyama Yumi, Kimura Masamichi, Nishikawa Koji, Imamura Jun, Kimura Kiminori
Department of Hepatology, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, Japan.
Clin J Gastroenterol. 2025 Apr 24. doi: 10.1007/s12328-025-02135-w.
Budd-Chiari syndrome is a rare and potentially fatal disorder characterized by obstruction or narrowing of the hepatic veins and inferior vena cava. The acute form of the disease often leads to rapid liver failure. In Asia, membranous obstruction of the hepatic portion of the inferior vena cava and localized stenosis at the hepatic venous origin are more common, while in Western countries, hepatic vein obstruction due to underlying thrombotic conditions is more prevalent. Treatment strategies vary and depend on disease severity and location. Anticoagulation therapy is the standard treatment in Western countries but is less commonly used in Japan where catheter-based interventions, such as recanalization, balloon dilation, and stenting, are more frequently employed. We report the case of a 51-year-old man diagnosed with Budd-Chiari syndrome after the treatment of esophageal varices. He initially responded well to nafamostat and balloon dilation, maintaining stability for 8 years, but later developed acute complications, including venous thrombosis and liver failure, despite previously stable follow-up. The patient succumbed to his condition despite aggressive treatment, highlighting the importance of early detection and intervention. This case underscores the need for clearer guidelines on anticoagulation therapy in Japan and emphasizes early management to improve prognosis.
布加综合征是一种罕见且可能致命的疾病,其特征为肝静脉和下腔静脉梗阻或狭窄。该病的急性形式常导致快速肝衰竭。在亚洲,下腔静脉肝段的膜性梗阻和肝静脉起始部的局限性狭窄更为常见,而在西方国家,由潜在血栓形成情况导致的肝静脉梗阻更为普遍。治疗策略各不相同,取决于疾病的严重程度和部位。抗凝治疗在西方国家是标准治疗方法,但在日本较少使用,在日本,基于导管的干预措施,如再通、球囊扩张和支架置入,使用更为频繁。我们报告一例51岁男性病例,该患者在治疗食管静脉曲张后被诊断为布加综合征。他最初对那法莫司他和球囊扩张反应良好,保持稳定8年,但后来尽管之前随访稳定,仍出现了急性并发症,包括静脉血栓形成和肝衰竭。尽管进行了积极治疗,患者仍死于该病,凸显了早期检测和干预的重要性。该病例强调了日本需要更明确的抗凝治疗指南,并强调早期管理以改善预后。