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Midaortic syndrome and renovascular hypertension
Seminars in Pediatric Surgery ( IF 1.4 ) Pub Date : 2021-10-30 , DOI: 10.1016/j.sempedsurg.2021.151124
Jonathan M Durgin 1 , Brianna L Slatnick 1 , Khashayar Vakili 1 , Heung Bae Kim 1 , Alex G Cuenca 1
Affiliation  

Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta with or without the involvement of branch vessels. The majority of cases are thought to be idiopathic though MAS has been associated with a number of conditions including granulomatous vasculitis, neurofibromatosis-1 (NF-1), Alagille Syndrome, fibromuscular dysplasia (FMD), and Williams syndrome. Patients typically present with hypertension due to decreased renal perfusion. Less common presentations include renal insufficiency, heart failure, claudication, stroke, and abdominal pain. Imaging modalities help establish the diagnosis of MAS including duplex ultrasound, computed tomography angiography (CTA), magnetic resonance angiography (MRA), and angiography. Initial therapy focuses on medical management with antihypertensives prior to intervention. Invasive interventions are indicated when there is evidence of end organ damage or dysfunction such as decreased renal function, poorly growing kidneys, cerebrovascular accident, left ventricular hypertrophy or frank cardiac failure. Endovascular interventions may assist in diagnosis and may treat some lesions although reintervention rates are high. Most patients require some type of surgical intervention, and a variety of surgical options are available based on anatomic findings. Renal revascularization may be accomplished by renal artery bypass, autotransplantation, or renal artery reconstruction. Aortic lesions may be repaired using patch angioplasty or aortoaortic bypass. Mesenteric arteries do not typically require reconstruction as they are rarely symptomatic. More novel options include the use of tissue expanders to lengthen the aorta to allow for primary aortic reconstruction (TESLA) or the use of the meandering mesenteric artery as an autologous aortic bypass graft (MAGIC).



中文翻译:

中主动脉综合征和肾血管性高血压

中主动脉综合征 (MAS) 是一种罕见的疾病,其特征是腹主动脉狭窄,伴或不伴分支血管受累。大多数病例被认为是特发性的,尽管 MAS 与许多疾病有关,包括肉芽肿性血管炎、神经纤维瘤病-1 (NF-1)、阿拉吉勒综合征、纤维肌肉发育不良 (FMD) 和威廉姆斯综合征。患者通常因肾灌注减少而出现高血压。不太常见的表现包括肾功能不全、心力衰竭、跛行、中风和腹痛。成像方式有助于确定 MAS 的诊断,包括双工超声、计算机断层扫描血管造影 (CTA)、磁共振血管造影 (MRA) 和血管造影。初始治疗侧重于干预前的抗高血压药物治疗。当有终末器官损伤或功能障碍的证据时,如肾功能下降、肾脏生长不良、脑血管意外、左心室肥大或明显的心力衰竭,则需要进行侵入性干预。尽管再介入率很高,血管内介入治疗可能有助于诊断并治疗一些病变。大多数患者需要某种类型的手术干预,并且可以根据解剖学发现提供多种手术选择。肾血运重建可以通过肾动脉旁路、自体移植或肾动脉重建来完成。主动脉病变可以使用补片血管成形术或主动脉旁路术修复。肠系膜动脉通常不需要重建,因为它们很少有症状。

更新日期:2021-12-17
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