Abstract：
Primary extra-hepatic portal vein obstruction (EHPVO) is a disease that develops ectopic varices due to portal hypertension and obstruction of the portal vein. Since bleeding from ectopic varices is life-threatening, the management of ectopic varices is important for patients with primary EHPVO. Here, we report a case of duodenal variceal bleeding in a patient with primary EHPVO. A 39-year-old man was diagnosed with F2-shaped duodenal varices (DV) due to primary EHPVO and was first treated with endoscopic variceal ligation for temporary hemostasis. We then performed angiography to understand the detailed hemodynamics and subsequently conducted endoscopic injection sclerotherapy (EIS) with a sclerosing agent containing N-butyl-2-cyanoacrylate for further hemostasis. After the treatment, dynamic computed tomography and endoscopic ultrasound revealed that the blood flow to the causative DV disappeared, although the DV itself remained. The patient was discharged without any re-bleeding or adverse events. Since treatment for DV due to primary EHPVO differs depending on hemodynamics (hepatofugal or hepatopetal blood flow), evaluating detailed hemodynamics for optimal treatment selection is crucial. Although EIS for this patient was not a radical treatment, it was effective in managing acute bleeding from the DV. This case will serve as a reference for successful treatment in future cases.
摘要：
原发性肝外门静脉阻塞（EHPVO）是一种由于门静脉高压和门静脉阻塞而导致异位静脉曲张的疾病。因为异位静脉曲张出血会危及生命,对于原发性EHPVO患者,治疗异位静脉曲张非常重要.这里,我们报告1例原发性EHPVO患者十二指肠静脉曲张破裂出血.一名39岁的男子因原发性EHPVO而被诊断为F2形十二指肠静脉曲张（DV），并首先接受内镜下静脉曲张结扎术治疗以进行临时止血。然后，我们进行了血管造影术以了解详细的血流动力学，随后用含有2-氰基丙烯酸正丁酯的硬化剂进行了内窥镜注射硬化疗法（EIS）以进一步止血。治疗后,动态计算机断层扫描和内窥镜超声显示，导致DV的血流消失，尽管DV本身仍然存在。患者出院，无任何再出血或不良事件。由于原发性EHPVO引起的DV的治疗因血液动力学（肝或肝瓣血流）而异，评估详细的血流动力学以选择最佳治疗方案至关重要.尽管对该患者的EIS并不是一种激进的治疗方法，它在治疗DV引起的急性出血方面是有效的。该病例将作为今后病例成功治疗的参考。
