Department of Emergency medicine, College of Medicine, Yeungnam University, Daegu 42415, Korea.
Department of Emergency Medicine, Kyungpook National University School of Medicine, Daegu 41404, Korea.
Medicina (Kaunas). 2020 Dec 14;56(12):696. doi: 10.3390/medicina56120696.
Budd-Chiari syndrome (BCS) is a rare intrahepatic vascular disease that is characterized by a hepatic venous outflow obstruction. Intravenous leiomyomatosis (ILs) is a rare complication of a myoma. Here, we report a case of BCS that was caused by intracaval ILs. A woman presented to the emergency department (ED) with abdominal distension that had gradually progressed over a period of 3 years. Bedside ultrasonography and contrast-enhanced computed tomography (CECT) showed a large ascites and pelvic mass. The mass continued to the inferior vena cava and the right atrium. The intracaval mass was obstructing the left and middle hepatic veins. We established a tentative diagnosis of BCS caused by intracaval ILs and attempted surgical resection. Complete resection of the intracaval mass failed because of adhesion; however, she was discharged from the hospital without any postoperative complications. After 3 months, a pelvic ultrasonography showed a recurrence of a 4 × 3 cm pelvic mass. The mass size increased to 6 cm after 30 months. ILs can cause secondary BCS and can lead to life-threatening conditions. Owing to its extreme rarity, early detection in the ED is challenging. Bedside ultrasonography and CECT can enable the early recognition of BCS by ILs.
布加综合征(BCS)是一种罕见的肝内血管疾病,其特征为肝静脉流出道阻塞。静脉内平滑肌瘤病(ILs)是平滑肌瘤的罕见并发症。在此,我们报告一例由腔静脉内 ILs 引起的 BCS。一名女性因腹胀 3 年来逐渐加重而到急诊科就诊。床边超声和增强计算机断层扫描(CECT)显示大量腹水和盆腔肿块。肿块一直延伸到下腔静脉和右心房。腔静脉内肿块阻塞了左中肝静脉。我们初步诊断为腔静脉内 ILs 引起的 BCS,并尝试进行手术切除。由于粘连,未能完全切除腔静脉内肿块;但患者术后无并发症出院。3 个月后,盆腔超声显示盆腔肿块复发,大小为 4×3cm。30 个月后,肿块大小增至 6cm。ILs 可引起继发性 BCS,并可导致危及生命的情况。由于其极为罕见,ED 早期检测具有挑战性。床边超声和 CECT 可通过 ILs 早期识别 BCS。