Daldoul S
Service de chirurgie générale A, hôpital Charles-Nicolle, Tunis faculty of medicine, university of Tunis El-Manar, boulevard 9-Avril-1938, 1006 Tunis, Tunisie.
J Mal Vasc. 2014 Jul;39(4):274-7. doi: 10.1016/j.jmv.2014.04.007. Epub 2014 Jun 3.
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare hematologic disorder that can exceptionally be complicated by splanchnic thrombosis and intestinal necrosis. The discovery of multiple and dispersed distal ischemia of the small bowel is a real problem because the therapeutic approach depends on the range and the number of the segments to resect and also on the risk of recurrence of new peri-operative ischemic lesions. We report the case of a patient suffering from PNH, operated with the diagnosis of mesenteric infarction. We discovered multiple distal ischemic lesions of the gut extending from the first duodenum to the penultimate loop without perforation. Resection was then ruled out and curative anticoagulation was initiated. Outcome was favorable with restitution ad integrum of the digestive lesions without progression to secondary stenosis. Discovery of distal ischemic lesions without perforation in patients with PNH does not necessarily require resection. Curative anticoagulation can avoid surgery that may be insufficient.
阵发性夜间血红蛋白尿（PNH）是一种罕见的血液系统疾病，极少数情况下会并发内脏血栓形成和肠坏死。发现小肠多处散在的远端缺血是一个实际问题，因为治疗方法取决于需要切除的肠段范围和数量，也取决于围手术期新的缺血性病变复发的风险。我们报告一例患有PNH的患者，诊断为肠系膜梗死并接受了手术。我们发现肠道多处远端缺血性病变，从十二指肠第一部延伸至倒数第二肠袢，无穿孔。于是排除了切除术，并开始进行根治性抗凝治疗。结果良好，消化性病变完全恢复，未进展为继发性狭窄。PNH患者发现无穿孔的远端缺血性病变不一定需要进行切除术。根治性抗凝治疗可以避免可能不充分的手术。
