背景:急性肠系膜缺血(AMI)是指肠道灌注不足的突然发作,也可能是内脏静脉阻塞所致。肠门静脉系统(PMVS)是蛋白S缺乏症患者血栓形成的不寻常部位,其阻塞是AMI的罕见原因。本报告的目的是说明在采用开放式腹部(OA)方法治疗的大面积小肠梗塞的情况下,一种成功的策略。
方法:一名64岁女性因急性腹痛就诊于急诊科,直肠出血,腹泻和呕吐。对比增强计算机断层扫描(CECT)显示小肠缺血和所有PMVS分支完全闭塞。采用OA方法进行手术,并立即开始抗凝治疗。进一步检查发现孤立的蛋白S缺乏和萎缩性胃炎病史。出院时开始使用华法林预防血栓,在一年的随访期间没有记录到血栓事件的复发。
结论:与蛋白S缺乏相关的PMVS血栓形成是一种罕见的疾病,可迅速导致急腹症。CECT是黄金标准,因为它能检测内脏血栓形成及其可能的并发症,比如肠缺血.如果是手术,计划的二次手术是评估肠活力和可能的缺血进展的最佳策略.
结论:OA管理在肠缺血切除的情况下发挥着重要作用。在不常见部位血栓形成的患者应进一步检查血栓前状态。
BACKGROUND: Acute mesenteric ischemia (AMI) refers to the sudden onset of intestinal hypoperfusion that can also result from splanchnic venous occlusion. The
portomesenteric venous system (PMVS) is an unusual site of thrombosis in patients with protein S deficiency and its obstruction is a rare cause of AMI. Aim of this report is to illustrate a successful strategy in a
case of massive small bowel infarction managed with an open abdomen (OA) approach.
METHODS: A 64 year-old woman presented to the emergency department with acute abdominal pain, rectal bleeding, diarrhea and vomiting. Contrast-enhanced computed tomography (CECT) showed small bowel ischemia and the complete occlusion of all the PMVS branches. Surgery was performed with an OA approach and anticoagulation was immediately begun. Further workup revealed isolated protein S deficiency and history of atrophic gastritis. Thromboprophylaxis with warfarin was started on discharge and no recurrence of thrombotic events was recorded during the one-year follow-up.
CONCLUSIONS: PMVS thrombosis related to protein S deficiency is a rare condition that can rapidly lead to an acute abdomen. CECT is the gold standard, because it detects splanchnic thrombosis and its possible complications, like bowel ischemia. In
case of surgery, a planned second-look operation is the best strategy to assess bowel viability and possible ischemic progression.
CONCLUSIONS: OA management plays a fundamental role in
case of resection for bowel ischemia. Patients with thrombosis at an uncommon site should be further investigated for prothrombotic states.