Dahi Firouza, Keese Michael, Thalhammer Axel, Schmitz-Rixen Thomas, Gkremoutis Asimakis
Department of Vascular and Endovascular Surgery, University Hospital Frankfurt am Main, Frankfurt, Germany.
Vascular Surgery, University Hospital Mannheim, Mannheim, Germany.
Ann Vasc Surg. 2019 Oct;60:474.e7-474.e10. doi: 10.1016/j.avsg.2018.11.018. Epub 2019 Feb 11.
Hughes-Stovin syndrome is a disorder characterized by deep vein thrombosis and pulmonary artery aneurysms with potentially life-threatening complications. The case of a 22-year-old Moroccan male patient, presenting with signs of sepsis of unclear etiology, is presented here. Computed tomography (CT) scan revealed thrombosis of the inferior vena cava up to the hepatic veins, thrombosis of both common iliac veins and a thrombus in the right atrium. Primarily suspecting septic thrombosis, surgical thrombectomy was performed. The patient recovered uneventfully and was discharged with oral anticoagulants. Three weeks later, he was admitted again with acute shortness of breath. A new CT scan showed bilateral pulmonary embolism and multiple pulmonary artery aneurysms. Hughes-Stovin syndrome was diagnosed, and high-dose heparin and an immunosuppressant (prednisolon) were administered. Two weeks later, the patient presented again with massive epistaxis and hemoptysis. A CT scan showed diffuse parenchymal bleeding. After prophylactic intubation and conservative treatment, he recovered rapidly and was again discharged uneventfully. Under immunosuppressants, a rapid reduction in the diameter of the pulmonary aneurysms was observed and the patient remained symptom-free during follow-up.
休斯 - 斯托文综合征是一种以深静脉血栓形成和肺动脉瘤为特征的疾病，可能伴有危及生命的并发症。本文介绍了一名22岁摩洛哥男性患者的病例，该患者出现病因不明的败血症症状。计算机断层扫描（CT）显示下腔静脉直至肝静脉血栓形成、双侧髂总静脉血栓形成以及右心房血栓。最初怀疑为感染性血栓形成，遂进行了手术取栓术。患者顺利康复并口服抗凝剂出院。三周后，他因急性呼吸急促再次入院。新的CT扫描显示双侧肺栓塞和多个肺动脉瘤。诊断为休斯 - 斯托文综合征，并给予大剂量肝素和免疫抑制剂（泼尼松龙）治疗。两周后，患者再次出现大量鼻出血和咯血。CT扫描显示弥漫性实质出血。经过预防性插管和保守治疗，他迅速康复并再次顺利出院。在免疫抑制剂治疗下，观察到肺动脉瘤直径迅速缩小，患者在随访期间无症状。
