Renal artery fibromuscular dysplasia: diagnostic challenges in a child presenting with hyponatremic hypertensive syndrome (HHS)

Abstract

A 2-year-9-month-old boy presented with renovascular hypertension caused by middle-segment stenosis of the right renal artery, and a severe hypertensive crisis, accompanied by hyponatremia, hypokalemia, and transient proteinuria. Fibromuscular dysplasia (FMD) is the most common cause of renovascular disease in children. However, the diagnosis of FMD is often delayed because findings on renal sonograms with Doppler are frequently missed. A high index of suspicion is required to further investigate a renovascular cause when persistent hypertension in a child cannot be otherwise explained. We present the challenges of reaching a diagnosis in a resource-limited setting and discuss how a multidisciplinary approach was key to successfully manage a young child with renovascular disease.