Pediatr Nephrol (2010) 25:1049–1056 DOI 10.1007/s00467-009-1320-9 EDUCATIONAL REVIEW Imaging in the evaluation of renovascular disease Kjell Tullus & Derek J. Roebuck & Clare A. McLaren & Stephen D. Marks Received: 21 May 2009 / Revised: 23 August 2009 / Accepted: 24 August 2009 / Published online: 24 October 2009 # IPNA 2009 Abstract Renovascular disease (RVD) is an important cause of hypertension in children, as it often is amenable to potentially curative treatment. Imaging aimed at finding RVD therefore needs to have high sensitivity so as not to miss important findings. Digital subtraction angiography is the gold standard investigation. Doppler ultrasonography, computed tomography (CT) angiography and magnetic resonance (MR) angiography can all be helpful, but none has, at present, high enough sensitivity to rule out RVD in a child with a suggestion of that diagnosis. Keywords Renovascular hypertension . Imaging . Ultrasound . Angiography . Magnetic resonance angiography (MRA) . Computed tomography angiography (CTA) . Technetium-99m dimercapto-succinic acid (DMSA) scintigraphy Introduction 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 K. Tullus (*) : S. D. Marks Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK e-mail: TulluK@gosh.nhs.uk D. J. Roebuck : C. A. McLaren Department of Radiology, Great Ormond Street Hospital for Children NHS Trust, London, UK (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 angiogra- phy, 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 fibromus- cular dysplasia (FMD) [5], although it is unusual for histological confirmation to be obtained and the diagnosis is usually made by exclusion. In some countries, Takaya- su’s disease is more common than is FMD [6]. Neurofi- bromatosis 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