Galloway J R, Henderson J M
Department of Surgery, Emory University School of Medicine, Atlanta, Georgia 30322.
Am J Surg. 1990 Jul;160(1):122-7. doi: 10.1016/s0002-9610(05)80881-5.
Patients with extrahepatic portal vein thrombosis may present from infancy through adulthood with variceal bleeding. Physiologically, such patients differ from patients with cirrhosis and variceal bleeding in that they have a normal liver and maintain good portal perfusion through hepatopedal collaterals. Complete evaluation of these patients requires identification of the bleeding site by endoscopy, definition of the anatomy by angiography, and confirmation of a normal liver by biopsy examination. Causative factors, including hypercoagulable states, should be evaluated. Therapeutic options range from noninterventive, through ablative procedures, to shunt operations. The goal should be definitive control of bleeding and return to a normal lifestyle. Distal splenorenal shunt offers the best option if technically feasible, but if no shuntable veins are patent, ablative procedures and sclerotherapy may be required. A noninterventive, noninvestigational approach is inappropriate in patients who can be offered definitive therapy. Splenectomy for hypersplenism should not be done in these patients.
肝外门静脉血栓形成的患者从婴儿期到成年期都可能出现静脉曲张出血。从生理角度来看，这类患者与肝硬化和静脉曲张出血患者不同，他们肝脏正常，通过肝足侧支循环维持良好的门静脉灌注。对这些患者进行全面评估需要通过内镜检查确定出血部位，通过血管造影明确解剖结构，并通过活检检查确认肝脏正常。应评估包括高凝状态在内的致病因素。治疗选择范围从非介入性治疗到消融手术，再到分流手术。目标应该是彻底控制出血并恢复正常生活方式。如果技术可行，远端脾肾分流术是最佳选择，但如果没有可用于分流的通畅静脉，则可能需要进行消融手术和硬化疗法。对于可以接受确定性治疗的患者，采用非介入性、非研究性的方法是不合适的。这些患者不应因脾功能亢进而行脾切除术。
