Mid Aortic Syndrome as a Rare Pediatric Etiology for Secondary Hypertension, Case Report

Background: Renovascular disease is an important cause of secondary hypertension in children as fibromuscular dysplasia the main etiology, Mid aortic syndrome (MAS) is an isolated disease of the distal thoracic and abdominal aorta resulting in significant tubular narrowing with stenosis of the visceral and renal arteries.

Case Report: challenging case of a 14-year-old boy referred to our pediatric (ICU) because of severe headache, dysarthria and right central facial nerve palsy, Initial evaluation revealed hypertensive crisis.

The physical examination revealed Both the systolic and diastolic blood pressures were above the 95th percentile of age, heart murmurs and abdominal bruit auscultated, There was no significant pressure gradient between upper and lower extremities, no absent or weak limb pulses.

Brain Computed Tomography revealed right acute hemorrhagic CVA.

Further laboratory data revealed elevated plasma renin activity (392 mU/I), elevated serum aldosterone levels (2760 pmol/L) and a normal potassium level, Autoimmune panel was negative and inflammation markers wasn’t elevated.

Later evaluation by Computed Tomography Angiography (CTA) revealed

Finally, diagnosis of Mid Aortic Syndrome was made.

Treatment with several anti-hypertensive agents initiated, percutaneous transluminal renal angioplasty (PTRA) was performed, however minimal angiographic improvement achieved.

Due to persistent hypertension an Aorto-Mesenteric and Aorto-Renal Bypass was performed with better hypertension control.

In conclusion, clinical pediatric practitioners should keep MAS in mind when approaching children with renal vascular hypertension.