Gastroenterology Clinic, University Hospital Sv Ivan Rilski, Sofia, Bulgaria.
Radiology Department, University Hospital Sv Ivan Rilski, Sofia, Bulgaria.
Medicine (Baltimore). 2023 Aug 11;102(32):e34549. doi: 10.1097/MD.0000000000034549.
Acute mesenteric ischemia due to superior mesenteric vein (SMV) thrombosis is a rare yet potentially life-threatening emergency. Our case report explores this condition in the context of a patient with liver cirrhosis due to Wilson disease. We specifically highlight the complex derangement of the coagulative balance in liver cirrhosis.
A 34-year-old female with Wilson disease-related cirrhosis presented with intractable abdominal pain, nausea, and vomiting that showed no response to antispasmodic medication.
A contrast-enhanced abdominal computed tomography scan and Doppler ultrasound confirmed an intraluminal filling defect in the SMV, leading to the diagnosis of SMV thrombosis.
Prompt anticoagulation, intravenous fluids, and an antibiotic were initiated. Surgical consultation recommended conservative therapy with close monitoring.
Over the following 2 days, the patient's condition improved considerably, with almost complete resolution of her symptoms. Genetic testing identified a 4G/4G homozygous genotype of the plasminogen activator inhibitor 1 gene, associated with a higher risk of thrombosis in the vessels of internal organs. After 2 months of sustained anticoagulant therapy, a follow-up contrast-enhanced computed tomography scan revealed near-complete recanalization of the SMV, and the patient remained symptom-free.
This case underscores the importance of early detection and treatment of acute mesenteric ischemia in patients with liver cirrhosis, as well as the potential role of genetic factors in thrombosis.
由肠系膜上静脉（SMV）血栓形成引起的急性肠系膜缺血是一种罕见但潜在危及生命的急症。我们的病例报告探讨了这种情况在威尔逊病相关肝硬化患者中的表现。我们特别强调了肝硬化患者凝血平衡的复杂紊乱。
一名 34 岁女性，患有威尔逊病相关肝硬化，出现无法缓解的腹痛、恶心和呕吐，对解痉药物治疗无反应。
腹部增强 CT 扫描和多普勒超声证实 SMV 腔内充盈缺损，确诊为 SMV 血栓形成。
立即开始抗凝、静脉补液和抗生素治疗。外科会诊建议采用保守治疗并密切监测。
在接下来的 2 天里，患者的病情明显改善，症状几乎完全缓解。基因检测发现纤溶酶原激活物抑制剂 1 基因的 4G/4G 纯合基因型，与内脏血管血栓形成风险增加相关。持续抗凝治疗 2 个月后，随访增强 CT 扫描显示 SMV 近完全再通，患者无症状。
该病例强调了早期发现和治疗肝硬化患者急性肠系膜缺血的重要性，以及遗传因素在血栓形成中的潜在作用。
