Pediatric Renal Artery Stenosis: A 19 Year Experience in Management And Outcomes At A Tertiary Pediatric Hospital

Introduction and objectives: Renal artery stenosis (RAS) is an uncommon cause of pediatric hypertension. Guidelines for the work up and management have not been established. The most widely reported etiology of pediatric renovascular disease has been fibromuscular dysplasia (FMD); however, other etiologies including middle aortic syndrome (MAS) and vasculitides have been described. We reviewed cases of radiologically identified pediatric RAS and describe etiologies, management, and long term clinical outcomes in our patients. Method(s): Reports for duplex ultrasound, computed tomography angiography, magnetic resonance imaging, and conventional angiography from an academic children's hospital between 2000-2019 were evaluated. Positive reports for RAS were confirmed by a vascular surgeon and a radiologist. Demographics, indications for evaluation, management and long term clinical outcomes were documented. Result(s): Imaging for suspected RAS was performed on 984 children. 42 patients [Female: 25 (60%); Male: 17 (40%); Mean age: 9.7 years (range 0.04-17)] were found to have evidence of RAS based on screening imaging (Duplex 93%; MRA/CTA 7%); 4 had normal findings on repeat exam. Of the 38 patients with positive imaging, 34% (n=13) underwent intervention. Of those requiring intervention, 58% (n=7) had RAS and concomitant aortic pathology [4 MAS; 2 thoracoabdominal vasculitis; 1 aortic thrombus]. Only 16% (n=2) had FMD, 16% (n=2) with neurofibromatosis and 16% (n=2) with unspecified RAS. None of the conservatively managed patients with positive RAS imaging (66%) required interventions during the mean follow up of 33.8 months. Conclusion(s): Pediatric RAS is a low frequency disease and long-term outcomes have been underreported. The incidence of associated aortic pathology in our intervention cohort appears higher than previously reported. Long term follow up of the conservative management cohort demonstrated that up to 66% of patients could be managed successfully without therapy.Copyright © 2019