Amanvermez Şenarslan Dilşad, Demren Halil, Tetik Ömer
Department of Cardiovascular Surgery, Manisa Celal Bayar University, Faculty of Medicine, Manisa, Türkiye.
Turk Gogus Kalp Damar Cerrahisi Derg. 2024 Dec 31;32(4 Suppl 2):122-123. doi: 10.5606/tgkdc.dergisi.2024.mep-24. eCollection 2024 Nov.
Middle aortic syndrome (MAS) is a segmental narrowing of the distal descending thoracic or abdominal aorta. This rare condition accounts for 0.5 to 2% of aortic coarctations. Middle aortic syndrome may be acquired, caused by Takayasu arteritis or giant cell arteritis, neurofibromatosis, fibromuscular dysplasia, retroperitoneal fibrosis, mucopolysaccharidosis, and the Williams syndrome, or it may be congenital. Stenosis is usually located at the suprarenal, interrenal, or infrarenal aorta. Concomitant stenoses in the renal (63%) and visceral (33%) arteries may be encountered. In this case report, we presented a 66-year-old male patient with MAS. The patient's primary complaint was claudication and rest pain in the last three months. The patient had hypertension for two years and a history of lumbar disc hernia operation. Thoracoabdominal computed tomography angiography revealed an enlarged ascending aorta, an aberrant right subclavian artery, and a narrowed segment with an hourglass appearance in the infrarenal aorta. The vessel diameter decreased to 9×10 mm at this level, and both iliac arteries were occluded distally. The patient underwent aortobifemoral bypass surgery with a 14/7 mm Dacron graft. In the postoperative period, all distal pulses were palpable, and leg pain resolved. The patient was discharged from the hospital on the sixth day without complications. This case report presented an adult MAS patient with rest pain in the legs related to occluded iliac arteries and aortic bifurcation. The coarctation in the infrarenal aorta was successfully treated by open surgery (aortobifemoral bypass). Children may benefit more from early diagnosis before the onset of severe hypertension. Open surgery is the primary treatment modality for tubular MAS. Usually bypass of the diseased segment or, less often, patch angioplasty is preferred. Bypass grafting of the stenosed renal and visceral arteries is performed when necessary. Endovascular therapy may provide less invasive treatment.
中段主动脉综合征（MAS）是降主动脉远端或腹主动脉的节段性狭窄。这种罕见疾病占主动脉缩窄的0.5%至2%。中段主动脉综合征可能是后天获得性的，由高安动脉炎或巨细胞动脉炎、神经纤维瘤病、纤维肌发育不良、腹膜后纤维化、黏多糖贮积症和威廉姆斯综合征引起，也可能是先天性的。狭窄通常位于肾上腺上、肾上腺间或肾上腺下主动脉。可能会出现肾动脉（63%）和内脏动脉（33%）的合并狭窄。在本病例报告中，我们介绍了一名66岁的中段主动脉综合征男性患者。患者的主要主诉是近三个月来的间歇性跛行和静息痛。患者有两年高血压病史，并有腰椎间盘突出症手术史。胸腹部计算机断层血管造影显示升主动脉增粗、右锁骨下动脉异常，以及肾上腺下主动脉有一段呈沙漏状的狭窄段。该水平血管直径降至9×10毫米，双侧髂动脉远端闭塞。患者接受了用14/7毫米涤纶移植物进行的主动脉双股动脉搭桥手术。术后，所有远端脉搏均可触及，腿痛缓解。患者于第六天无并发症出院。本病例报告介绍了一名成年中段主动脉综合征患者，其腿部静息痛与髂动脉闭塞和主动脉分叉有关。肾上腺下主动脉缩窄通过开放手术（主动脉双股动脉搭桥）成功治疗。儿童可能从严重高血压发作前的早期诊断中获益更多。开放手术是管状中段主动脉综合征的主要治疗方式。通常首选绕过病变节段，较少情况下首选补片血管成形术。必要时对狭窄的肾动脉和内脏动脉进行搭桥移植。血管内治疗可能提供侵入性较小的治疗。
