PDF(Pubmed)
Abstract：
Budd-Chiari syndrome is one of the post-hepatic causes of portal hypertension and a potential obstruction causes liver fibrosis. In pregnancy, obstruction of hepatic veins could occur due to stenosis or thrombosis. Variceal bleeding is the most fatal complication in pregnancy with co-existing Budd-Chiari syndrome, with 29.4% incidence of abortion and 33.3% perinatal mortality. The aim of this case report was to present the management of non-cirrhotic variceal bleeding in pregnant women with Budd-Chiari syndrome in the early second trimester. We report a pregnant female at 13-14 weeks gestation presented to the hospital with profuse hematemesis. Doppler ultrasonography (USG) was utilized to confirm the diagnosis of Budd-Chiari syndrome-hepatic vein occlusion type in pregnancy. Abdominal USG revealed hepatomegaly with hepatic veins dilation, while endoscopy showed grade IV esophageal varices and grade IV gastric varices. Laboratory results indicated disseminated intravascular coagulation due to hemorrhage. The patient was given strict fluid resuscitation and three packed red cells transfusion to stabilize the hemodynamic. Bleeding was successfully managed by intravenous octreotide, tranexamic acid, and vitamin K. The case highlights that the management of non-cirrhotic variceal bleeding in pregnancy with Budd-Chiari syndrome requires a multidisciplinary approach and regular fetal monitoring to ensure optimal outcomes.
摘要：
Budd-Chiari综合征是门静脉高压的肝后原因之一，潜在的阻塞会导致肝纤维化。在怀孕期间,由于狭窄或血栓形成，可能会发生肝静脉阻塞。静脉曲张破裂出血是妊娠合并Budd-Chiari综合征最致命的并发症,流产发生率为29.4%，围产期死亡率为33.3%。本病例报告的目的是介绍妊娠中期早期Budd-Chiari综合征孕妇非肝硬化静脉曲张破裂出血的管理。我们报告了一名怀孕13-14周的孕妇，因大量呕血被送往医院。多普勒超声（USG）用于确认妊娠Budd-Chiari综合征-肝静脉阻塞型的诊断。腹部USG显示肝肿大伴肝静脉扩张，而内窥镜检查显示IV级食管静脉曲张和IV级胃静脉曲张。实验室结果提示出血导致弥散性血管内凝血。对患者进行了严格的液体复苏和三例红细胞输注，以稳定血流动力学。通过静脉注射奥曲肽成功治疗出血，氨甲环酸,和维生素K。该病例强调，妊娠合并Budd-Chiari综合征的非肝硬化静脉曲张破裂出血的管理需要多学科方法和定期胎儿监测，以确保最佳结局。
