Renovascular disease (RVD) causes 10% of hypertension in children [
1,
2] and is important to be diagnosed, as it may be amenable to treatment [
3]. The best investigation for evaluation of RVD is digital subtraction angiography (DSA), which is an invasive procedure with associated risks and is not available in all children’s hospitals. Therefore, it must be used with discretion for children. As a result, other modalities of investigating RVD have evolved over the past few years, including magnetic resonance angiography, computed tomography angiography, isotope studies and duplex ultrasonography (US).
The aetiopathogenesis and course of RVD is different between children and adults. The dominating diagnosis in adults is atherosclerosis, mostly affecting the main renal arteries [
4]. The diagnostic spectrum is different in children, with the most common reported diagnosis being fibromuscular dysplasia (FMD) [
5], although it is unusual for histological confirmation to be obtained and the diagnosis is usually made by exclusion. In some countries, Takayasu’s disease is more common than is FMD [
6]. Neurofibromatosis type 1 is another common cause of paediatric RVD. The vascular disease in children is often widespread, often with bilateral involvement of the renal arteries and their branches. In a study at our institution we found that, in 33 patients, 16 (48%) had bilateral renal artery stenosis (RAS) and 15 (45%) had intra-renal disease [
7]. Ten (30%) patients had both bilateral RAS and intra-renal disease. There was mid-aortic involvement in eight (24%) of these patients, cerebral vascular involvement in seven (21%), and mesenteric vessel involvement in ten (30%). Other parts of the vasculature are also often involved [
7]. A study of 21 children (24 stenotic lesions) without co-morbid conditions showed less widespread disease but often involvement of branch arteries: main arteries (six), second-order arteries (12), third-order arteries (three) and accessory arteries (three) [
8]. This poses greater demands on the imaging investigations used.
The burden of lifelong antihypertensive treatment and the risks of poorly controlled blood pressure are considerable. As renovascular disease is potentially amenable to treatment with various endovascular and surgical techniques, any non-invasive imaging test must have a very high sensitivity, or potentially treatable children will be missed [
9,
10]
In this review we attempted to define the role of these investigations in children with hypertension. However, as there is scanty evidence in the literature to date of using alternative investigations to diagnose RVD in children, we relied on studies of adults and clinical experience in children.