I. Medizinische Klinik und Poliklinik, Universitätsmedizin der Johannes Gutenberg-Universität Mainz, Germany.
Klinik und Poliklinik für Diagnostische und Interventionelle Radiologie, Universitätsmedizin der Johannes Gutenberg-Universität Mainz, Germany.
Z Gastroenterol. 2021 Jan;59(1):43-49. doi: 10.1055/a-1330-9827. Epub 2021 Jan 11.
Non-cirrhotic portal vein thrombosis (PVT) in patients with antiphospholipid syndrome (APS) is a rare complication, and the management has to be determined individually based on the extent and severity of the presentation. We report on a 37-year-old male patient with non-cirrhotic chronic PVT related to a severe thrombophilia, comprising APS, antithrombin-, factor V- and factor X-deficiency. Three years after the initial diagnosis of non-cirrhotic PVT, the patient presented with severe hemorrhagic shock related to acute bleeding from esophageal varices, requiring an emergency transjugular intrahepatic portosystemic stent shunt (TIPSS). TIPSS was revised after a recurrent bleeding episode due to insufficient reduction of the portal pressure. Additionally, embolization of the dilated V. coronaria ventriculi led to the regression of esophageal varices but resulted simultaneously in a left-sided portal hypertension (LSPH) with development of stomach wall and perisplenic varices. After a third episode of acute esophageal varices bleeding, a surgical distal splenorenal shunt (Warren shunt) was performed to reduce the LSPH. Despite anticoagulation with low molecular weight heparin and antithrombin substitution, endoluminal thrombosis led to a complete Warren shunt occlusion, aggravating the severe splenomegaly and pancytopenia. Finally, a partial spleen embolization (PSE) was performed. In the postinterventional course, leukocyte and platelet counts increased rapidly and the patient showed no further bleeding episodes. Overall, this complex course demonstrates the need for individual assessment of multimodal treatment options in non-cirrhotic portal hypertension. This young patient required triple modality porto-systemic pressure reduction (TIPSS, Warren shunt, PSE) and involved finely balanced anticoagulation and bleeding control.
非肝硬化性门静脉血栓形成（PVT）在抗磷脂综合征（APS）患者中较为罕见，其管理必须根据表现的程度和严重程度进行个体化确定。我们报告了一例 37 岁男性患者，患有与严重血栓形成倾向相关的非肝硬化性慢性 PVT，包括 APS、抗凝血酶、因子 V 和因子 X 缺乏症。在最初诊断为非肝硬化性 PVT 三年后，患者因食管静脉曲张急性出血出现严重失血性休克，需要紧急经颈静脉肝内门体分流术（TIPSS）。由于门静脉压力降低不足，TIPSS 在再次发生出血事件后进行了修订。此外，扩张的冠状静脉的栓塞导致食管静脉曲张消退，但同时导致左侧门静脉高压（LSPH），胃壁和脾周静脉曲张形成。在第三次急性食管静脉曲张出血后，进行了外科远端脾肾分流术（Warren 分流术）以降低 LSPH。尽管使用低分子肝素和抗凝血酶替代物进行抗凝，但腔内血栓形成导致完全 Warren 分流闭塞，加重了严重的脾肿大和全血细胞减少症。最终，进行了部分脾脏栓塞术（PSE）。在介入治疗后，白细胞和血小板计数迅速增加，患者未再发生出血事件。总体而言，这种复杂的病程表明需要对非肝硬化性门静脉高压的多种治疗选择进行个体化评估。这位年轻患者需要三重模式的门体系统降压（TIPSS、Warren 分流术、PSE），并需要精细平衡抗凝和出血控制。
