IMAGES IN CLINICAL MEDICINE

Phlegmasia Cerulea Dolens with Compartment Syndrome

Hsin-Ti Lai, and Shu-Hung Huang

N Engl J Med 2018; 378:658

DOI: 10.1056/NEJMicm1704996

An 81-year-old man with hypertension presented to the emergency department with pain and swelling of the left thigh and lower leg that had developed during the previous several hours. He had no history of recent surgery or trauma and no known personal or family history of clotting disorders. The left lower leg was tender, cold, and swollen (Panel A), and the left dorsalis pedis pulse was not palpable. Computed tomography performed after the administration of contrast material showed partial thrombosis of the inferior vena cava and complete thrombosis of the left common iliac, common femoral, superficial femoral, and deep femoral veins. Anticoagulation with unfractionated heparin was initiated, and emergency fasciotomy was performed to treat the acute compartment syndrome. The postoperative course was complicated by reperfusion injury with shock and acute kidney injury. The patient underwent percutaneous transluminal angioplasty and catheter-directed thrombolysis. Phlegmasia cerulea dolens is a rare syndrome caused by acute, extensive venous thrombosis. Prompt diagnosis is crucial for limb salvage and to minimize morbidity. The patient’s condition improved (Panel B), and he recovered renal function. After 25 days of hospitalization, he was discharged while receiving rivaroxaban. Subsequent evaluation that included age-appropriate cancer screening did not reveal the cause of this extensive clotting.

一名81岁男性高血压患者因左大腿及小腿疼痛肿胀数小时到急诊就诊。患者近期无手术或创伤病史，也没有已知凝血功能障碍的个人史或家族史。左小腿有压痛，皮温凉且肿胀（图A），左侧足背动脉未能触及脉搏。增强CT显示下腔静脉部分血栓形成，左侧髂总静脉、股总静脉、股浅静脉及股深静脉完全性血栓形成。开始使用普通肝素抗凝，并进行急诊筋膜切开术治疗急性骨筋膜室综合征。术后患者出现再灌注损伤、休克及急性肾损伤。患者接受经皮腔内血管成形术及导管引导溶栓治疗。股蓝肿是一种罕见的临床综合征，可见于急性广泛性静脉血栓形成。快速诊断对于挽救肢体并尽可能减少并发症至关重要。患者病情逐渐改善（图B），肾脏功能回复。住院25天后，患者出院时仍使用利伐沙班。后续评估（包括与年龄相关的肿瘤筛查）并未发现广泛血栓形成的病因。