Canova C R, Zünd G, Valavanis A, Salomon F, a Wengen D, Hoffmann U
Abteilung für Angiologie, Klinik für Herz- und Gefässchirurgie, Universitätsspital Zürich.
Dtsch Med Wochenschr. 1997 Sep 26;122(39):1172-7. doi: 10.1055/s-2008-1047744.
A 32-year-old man developed acute right-sided sore throat, a thick voice and epistaxis. 13 years previously he had erythema nodosum, 12 and 10 year ago deep vein thrombosis. On admission he had Horner's syndrome, bulging of the gums and swelling of the paratracheal space. He had been on anticoagulation therapy with phenprocoumon since the leg vein thrombosis.
Computed tomography demonstrated a false aneurysm of the right internal carotid artery. 9 months later a right radial artery aneurysm occurred, which was treated surgically. An arterial cannula had previously been placed at this site for pressure monitoring. 2 months later a false aneurysm of the right femoral artery was diagnosed: it had previously been used for an intravascular intervention. As embolization to the popliteal artery occurred after sonographically controlled compression had failed and reduction of anticoagulation treatment, this aneurysm, too, was treated surgically.
DIAGNOSIS, TREATMENT AND COURSE: Because of the tendency to aneurysm formation after arterial puncture and the history of venous thromboses the diagnosis of Behçet's syndrome was considered, confirmed by subsequently elucidated history of recurrent oral aphthous ulcers and folliculitis. There were no other manifestations of the syndrome. Immunosuppressive treatment was instituted after another two aneurysm recurrences were noted in the right common femoral artery.
This case illustrates that the diagnosis of Behçet's syndrome may be made more difficult if its manifestation is primarily vascular with minimal mucocutaneous involvement. It should be considered in the differential diagnosis of recurrent arterial aneurysms in a young person.
一名32岁男性出现急性右侧咽痛、声音嘶哑和鼻出血。13年前他患过结节性红斑,12年前和10年前发生过深静脉血栓形成。入院时他有霍纳综合征、牙龈肿胀和气管旁间隙增宽。自腿部静脉血栓形成后,他一直在接受苯丙香豆素抗凝治疗。
计算机断层扫描显示右颈内动脉假性动脉瘤。9个月后出现右桡动脉动脉瘤,接受了手术治疗。此前曾在此部位放置动脉插管进行压力监测。2个月后诊断出右股动脉假性动脉瘤:该部位此前曾用于血管内介入治疗。由于在超声控制下压迫失败且抗凝治疗减量后出现了腘动脉栓塞,该动脉瘤也接受了手术治疗。
诊断、治疗与病程:由于动脉穿刺后有形成动脉瘤的倾向以及静脉血栓形成病史,考虑为白塞病综合征,随后明确的复发性口腔阿弗他溃疡和毛囊炎病史证实了这一诊断。该综合征无其他表现。在右股总动脉又出现两次动脉瘤复发后,开始进行免疫抑制治疗。
该病例表明,如果白塞病综合征主要表现为血管病变且黏膜皮肤受累轻微,其诊断可能会更困难。在对年轻人复发性动脉动脉瘤进行鉴别诊断时应考虑到该病。