Beltagy Asmaa, Eshak Nouran, Abdelnabi Mahmoud Hassan, Almaghraby Abdallah, Magdy Samar, Shehata Hoda
Rheumatology Unit, Department of Internal Medicine, Faculty of Medicine, University of Alexandria, Alexandria, Egypt.
Cardiology and Angiology Unit, Department of Clinical and Experimental Internal Medicine, Medical Research Institute, University of Alexandria, Alexandria, Egypt.
Echocardiography. 2019 Aug;36(8):1590-1593. doi: 10.1111/echo.14428. Epub 2019 Jun 27.
Cogan's syndrome is a rare disorder characterized by the coexistence of ocular and audio-vestibular manifestations. Systemic manifestations are quite unusual with pan-vasculitis and cardiac involvement reported in the form of aortitis with aortic aneurysm, dissection, or extremely rare aortic valve perforation. Hereby, we report a case of a 56-year-old woman presented with ocular, audio-vestibular, and systemic manifestations with medium-sized vasculitis in the form of multiple splenic artery aneurysms, superior mesenteric artery thrombosis, and cardiovascular involvement in the form of aortic regurgitation due to noncoronary cusp perforation. To the best of our knowledge, this is the second case to report aortic perforation in the setting of Cogan's syndrome.
科根综合征是一种罕见的疾病,其特征是眼部和听觉前庭表现同时存在。全身表现非常少见,曾有报告称存在以主动脉炎伴主动脉瘤、夹层或极为罕见的主动脉瓣穿孔形式出现的全血管炎和心脏受累情况。在此,我们报告一例56岁女性病例,该患者出现眼部、听觉前庭及全身表现,伴有中型血管炎,表现为多发脾动脉瘤、肠系膜上动脉血栓形成,以及因非冠状动脉瓣叶穿孔导致主动脉反流的心血管受累情况。据我们所知,这是第二例报告在科根综合征背景下出现主动脉穿孔的病例。