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静脉内平滑肌瘤病致肝静脉血流阻塞。

Obstruction of the Hepatic Venous Flow Caused by Intravenous Leiomyomatosis.

机构信息

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.

出版信息

Medicina (Kaunas). 2020 Dec 14;56(12):696. doi: 10.3390/medicina56120696.

Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/02df/7764919/d77aa07eb9f3/medicina-56-00696-g001.jpg

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