Ochs A
Innere Abteilung, Loretto-Krankenhaus, Mercystraße 6-14, 79100, Freiburg.
Internist (Berl). 2011 Jul;52(7):795-6, 798-800, 802-3. doi: 10.1007/s00108-010-2795-y.
Acute hepatic vascular complications are rare. Acute portal vein thrombosis (PVT) and the Budd-Chiari syndrome (BSC) are the leading causes. Coagulopathy and local factors are present in up to 80% of cases. Diagnosis is established by colour-coded Doppler sonography, contrast-enhanced computed tomography or magnetic resonance imaging. Patients with acute PVT present with abdominal pain and disturbed intestinal motility. In the absence of cirrhosis anticoagulation with heparin is established followed by oral anticoagulation. In severe cases, surgical thrombectomy or transjugular thrombolysis with stent shunt may be necessary. Acute or fulminant BCS may require emergency liver transplantation or a transjugular intrahepatic portosystemic stent shunt, if patients present with acute liver failure. Milder cases receive anticoagulation for thrombolysis of occluded hepatic veins. Sinusoidal obstruction syndrome (SOS) is diagnosed after total body irradiation or chemotherapy, the term SOS replacing the former veno-occlusive disease. The treatment of congenital vascular malformations, complications in the setting of OLTX as well as patients with hepatic involvement of hereditary hemorrhagic telangiectasia requires significant expertise in a multidisciplinary approach.
急性肝血管并发症较为罕见。急性门静脉血栓形成(PVT)和布加综合征(BSC)是主要病因。高达80%的病例存在凝血功能障碍和局部因素。通过彩色多普勒超声、对比增强计算机断层扫描或磁共振成像进行诊断。急性PVT患者表现为腹痛和肠道蠕动紊乱。在无肝硬化的情况下,先使用肝素进行抗凝,随后口服抗凝药物。在严重病例中,可能需要进行手术取栓或经颈静脉溶栓并置入支架分流。如果急性或暴发性BSC患者出现急性肝衰竭,则可能需要紧急肝移植或经颈静脉肝内门体分流术。症状较轻的病例接受抗凝治疗以溶解闭塞的肝静脉血栓。窦性阻塞综合征(SOS)在全身照射或化疗后诊断,该术语取代了以前的静脉闭塞性疾病。先天性血管畸形的治疗、肝移植术中的并发症以及遗传性出血性毛细血管扩张症累及肝脏的患者的治疗需要多学科方法的专业知识。