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

Abstract Background Budd-Chiari Syndrome (BCS) caused by Intravenous leiomyomatosis (IVL) is a rare and uncommon. Further reports and thorough evaluation are needed to identify and manage this disease. Method We described the case of a 49-year-old lady, exhibiting features of Budd-Chiari syndrome secondary to intravenous leiomyomatosis, and reviewed other three previous cases of BCS caused by IVL. Results The 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 birth, and bilateral lower extremity edema were common symptoms. The establishment of clinical diagnoses of IVL and BCS mainly depends on clinical presentations and imaging like ultrasonography, CT (with vascular reconstruction), and magnetic resonance imaging (MRI). Surgical intervention to alleviate the hepatic veins outflow obstruction is the most important treatment. Conclusions BCS caused by IVL should be considered when inferior vena cava (IVC) and right atrium (RA) lesions occur in a patient with characteristics of BCS and a history of uterine myoma or hysterectomy. Complete tumor resection with TAHBSO is the only curative treatment and should be performed as soon as possible.