Mid-aortic syndrome: a case report and review of the literature

Abstract

Renovascular disease is an important cause of secondary hypertension in children. In contrary to the adult patients whose major cause of renal artery stenosis (RAS) is atherosclerosis, fibromuscular dysplasia is responsible for the renovascular hypertension in most children. Mid-aortic syndrome (MAS) is a rare abnormality referring to an isolated disease of the distal thoracic and abdominal aorta resulting in significant tubular narrowing with stenosis of the visceral and renal arteries. It is usually diagnosed in young adults, but may present in childhood as a challenging problem. Patients with MAS are often first detected due to refractory hypertension. Other later presentations include intermittent claudication, congestive heart failure, renal insufficiency and symptoms of hypertensive associated end-organ damage. We report a case of a 16-year-old patient with MAS who suffered from malignant arterial hypertension. A percutaneous transluminal renal angioplasty (PTRA) was first performed, however the stenosis proved resistant to dilatation and only resulted in a minimal angiographic improvement. Due to persistent hypertension, an aortorenal bypass using the saphenous vein was performed. We also reviewed the literature on PTRA and the surgical management of RAS in MAS patients, in which PTRA often carries poor results.