L'ischémie intestinale au cours de l'hémoglobinurie paroxystique nocturne : l'embarras du choix

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.