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Article Details
Clinical Image
Volume 3, Issue 3 (March Issue)

Mid-aortic Dysplastic Syndrome

João Paulo Rocha, Neuza Soares and Susana Ferreira*

Serviço de Medicina Interna, Centro Hospitalar Universitário São João, Porto, Portugal

*Corresponding author: Susana Ferreira, Serviço de Medicina Interna, Centro Hospitalar São João, Alameda Prof. Hernâni Monteiro, 4200-319 Porto, Portugal, E-mail: sumaia2@gmail.com

Received: February 14, 2021; Accepted: February 21, 2021; Published: March 26, 2021

Citation: João Paulo Rocha, Neuza Soares, Susana Ferreira, et al. Mid-aortic Dysplastic Syndrome. Clin Image Case Rep J. 2021; 3(3): 150.

Abstract

A 53-year-old female presented to the Internal Medicine clinic with a 2-year history of atypical chest pain and bilateral leg pain with nocturnal worsening. She had arterial hypertension diagnosed more than 10 years before and type 2 diabetes mellitus, with no end-organ damage documented, including coronary artery disease. Physical examination revealed a left periumbilical bruit and weak bilateral lower limb pulses. An abdominal computed tomography scan with contrast was performed for further evaluation, revealing a diffuse narrowing of infrarenal aortic artery (minimal diameter of 2 x 3 mm; arrow), stricture of the left common iliac artery and occlusion of the right common iliac artery with distal repermeabilization (*). Signs of collateral circulation were seen in the intervertebral arteries, and no occlusions of the renal arteries and superior and inferior mesenteric arteries were registered. She also underwent cervical doppler ultrasonography, documenting a sub-obliterative 80% stenosis of the proximal internal carotid artery with haemodynamic repercussion and significant intracranial collateralization to the right internal carotid vascular territory, through anterior and posterior communicating arteries. No elevation of acute phase reactants was detected. A diagnosis of mid-aortic dysplasia syndrome was made. This syndrome is a congenital disorder of unknown aetiology characterized by abdominal aortic stenosis and strictures of its main branches. Although it is rare, it represents a potentially curable cause of secondary arterial hypertension. During follow-up, no end-organ damage was identified with optimization of anti-hypertensive treatment and the patient was referred to Vascular Surgery clinics for further evaluation.