Pediatric Renal Artery Stenosis: A 19-Year Experience in Management and Outcomes at a Tertiary Pediatric Hospital

Abstract

Renal artery stenosis (RAS) is an uncommon cause of pediatric hypertension. Guidelines for workup and management have not been established. The most widely reported etiology of the pediatric renovascular disease has been fibromuscular dysplasia; 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. Reports for duplex ultrasound, computed tomography angiography, magnetic resonance imaging, and conventional angiography from an academic children's hospital between 2000 and 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. Data are summarized as count (n), geometric mean, median, or standard deviation as appropriate. Univariate differences between treatment cohorts were analyzed using Chi-squared tests for categorical variables. Nonparametric paired Wilcoxon signed-rank test and Mann-Whitney U-test were used for the analysis of paired ordinal or continuous data. A statistical analysis was performed with SPSS software (SPSS Inc., Chicago, IL) with significance defined at a P<0.05 level. Imaging for suspected RAS was performed on 984 children. Of the 38 patients with positive imaging for RAS, 60.5% were idiopathic, 31.5% (n=12) had concomitant congenital/systemic comorbidity, and 21.0% (n=8) had RAS and concomitant aortic pathology. Fibromuscular dysplasia only accounted for 13.2% (n=5) of patients. Regarding management, 34.2% (n=13) underwent invasive intervention, 23.7% (n=9) underwent endovascular intervention alone, and 10.5% (n=4) underwent endovascular plus surgical intervention. Conservative management was performed for 65.8% (n=25) of patients at a long-term follow-up (33.8months), 34.2% (n=13) requiring only lifestyle changes, and 31.6% (n=12) requiring only medical management. Pediatric RAS is a low-frequency disease and long-term outcomes have been under-reported. The incidence of associated aortic pathology in our intervention cohort appears higher than that was previously reported. A long-term follow-up demonstrated that up to 65.8% of patients could be managed successfully with conservative therapy.