Kuka Werimo Pascal, Nduati Paul Kareithi, Mwirigi Anne, Opio Christopher K
Department of Medicine, The Aga Khan University Hospital, Nairobi, Nairobi, Kenya.
Department of Imaging and Diagnostic Radiology, The Aga Khan University Hospital, Nairobi, Nairobi, Kenya.
SAGE Open Med Case Rep. 2023 Mar 21;11:2050313X231161190. doi: 10.1177/2050313X231161190. eCollection 2023.
Budd-Chiari syndrome is a rare disease characterized by the obstruction of hepatic venous outflow. Stepwise treatment options aimed to relieve obstruction and prevent complications of Budd-Chiari syndrome are medical therapy, interventional recanalization, and surgery. Aggressive interventions for complicated Budd-Chiari syndrome are placement of a transjugular intrahepatic portosystemic shunt, surgical shunting, or liver transplantation. Although literature suggests differences in the presentation and management between Europe and Asia, cases documenting successful use of stepwise management of Budd-Chiari syndrome in Sub-Saharan Africa are scarce. A 47-year-old male on treatment for chronic hepatitis B presented with abdominal pain and distension for 2 weeks and findings of gross ascites without stigmata of chronic liver disease. Laboratory investigations performed showed anemia, elevated transaminases, coagulopathy, and renal dysfunction. Abdominal ultrasound and computed tomography abdominal scan revealed filling defects in intrahepatic veins and inferior vena cava extending to bilateral renal and external iliac veins. Extensive workup for thrombophilia and myeloproliferative disorders was negative. The diagnosis was hepatic dysfunction secondary to inferior vena cava obstruction due to a thrombus in the setting of extensive inferior vena cava thrombosis, and heparin was initiated. However, due to lack of recanalization with anticoagulation, we performed aspiration thrombectomy, balloon angioplasty, and local thrombolysis. Transjugular intrahepatic portosystemic shunt procedure was subsequently done due to hepatic venous congestion and refractory ascites. He was discharged on oral anticoagulation. Imaging exams performed 4 months later showed patent inferior vena cava and transjugular intrahepatic portosystemic shunt, good flows in the portal vein and resolution of ascites.
布加综合征是一种罕见疾病,其特征为肝静脉流出道梗阻。旨在缓解梗阻并预防布加综合征并发症的逐步治疗方案包括药物治疗、介入再通和手术。针对复杂布加综合征的积极干预措施包括经颈静脉肝内门体分流术、手术分流或肝移植。尽管文献表明欧洲和亚洲在临床表现和治疗管理方面存在差异,但记录在撒哈拉以南非洲成功采用布加综合征逐步管理的病例却很稀少。一名正在接受慢性乙型肝炎治疗的47岁男性,出现腹痛和腹胀2周,检查发现有大量腹水,但无慢性肝病体征。实验室检查显示贫血、转氨酶升高、凝血功能障碍和肾功能不全。腹部超声和腹部计算机断层扫描显示肝内静脉和下腔静脉有充盈缺损,延伸至双侧肾静脉和髂外静脉。针对血栓形成倾向和骨髓增殖性疾病的广泛检查均为阴性。诊断为在广泛的下腔静脉血栓形成背景下,下腔静脉血栓导致下腔静脉梗阻继发肝功能障碍,并开始使用肝素。然而,由于抗凝治疗未能实现再通,我们进行了抽吸血栓切除术、球囊血管成形术和局部溶栓治疗。随后,由于肝静脉淤血和难治性腹水,进行了经颈静脉肝内门体分流术。患者出院时接受口服抗凝治疗。4个月后进行的影像学检查显示下腔静脉和经颈静脉肝内门体分流术通畅,门静脉血流良好,腹水消退。