Intravenous leiomyomatosis presenting as Budd-Chiari syndrome: a case report and literature review

被引:0
作者
Gan, Jingwen [1 ]
Ma, Xiao [1 ]
Shao, Jiang [2 ]
Wang, Jinhui [1 ]
Cao, Dongyan [1 ]
机构
[1] Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Obstet & Gynecol, Natl Clin Res Ctr Obstet & Gynecol Dis, Dongdan Campus,1 Shuaifuyuan Wangfujing Dongcheng, Beijing 100730, Peoples R China
[2] Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Vasc Surg, Beijing, Peoples R China
基金
中国博士后科学基金;
关键词
Intravenous leiomyomatosis; Budd-Chiari syndrome; Hepatic veins outflow obstruction; Abdominal distention; Lower extremity edema;
D O I
10.1186/s13023-025-03556-z
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
BackgroundBudd-Chiari syndrome (BCS) caused by intravenous leiomyomatosis (IVL) is rare. Further reports and thorough evaluation are needed to identify and manage this disease.MethodWe described the case of a 49-year-old lady, exhibiting features of BCS secondary to IVL, and reviewed three other previous cases of BCS caused by IVL.ResultsThe mean onset age of these four patients was 54.8 years. All but one (Patient No. 2) had a history of myoma, myomectomy, or hysterectomy. Abdominal pain, bloating or increasing abdominal circumference, and bilateral lower extremity edema were common symptoms. The establishment of clinical diagnoses of IVL and BCS mainly depends on clinical presentations and imaging, such as ultrasonography, computed tomography, and magnetic resonance imaging. Surgical intervention to alleviate the hepatic veins outflow obstruction is the most important treatment.ConclusionsBCS caused by IVL should be considered when the inferior vena cava and right atrium lesions were detected in a patient with characteristics of BCS and a history of uterine myoma or hysterectomy. Complete tumor resection is the only curative treatment and should be performed as soon as possible.
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页数:7
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