Renal artery stenosis in association with congenital anomalies of the kidney and urinary tract

Objectives:

To describe 8 cases of renal artery stenosis (RAS) in children with congenital anomalies of the renal tract.

Methods:

We conducted a retrospective chart review of 78 children with RAS who were followed up at Great Ormond Street Hospital, London, United Kingdom between 2003 and 2012. We used an interventional radiology database to identify all patients who had RAS confirmed by digital subtraction angiography and examined all cases of congenital anomaly of the renal tract that had been diagnosed during childhood.

Results:

We documented the following renal anomalies: multicystic dysplastic kidney (n=2), renal hypoplasia (n=1), congenital solitary kidney with hydronephrosis (n=1), and unilateral vesicoureteric reflux with poorly functioning kidneys (n=2). The anomaly was unknown in 2 cases. Seven children had unilateral nephrectomy at a median age of 2.5 years (range, 0.4-10 years) for various urological abnormalities. All children were confirmed to have RAS after presentation with hypertension at a median age of 10 (3.5-16.2) years. Angioplasty was performed in 7 children, of which 6 achieved control of their blood pressure on reduced medications.

Conclusion:

We highlight the association between RAS and other renal anomalies, which indicates that they could share a common genetic background.Renovascular disease (RVD) is an important cause of severe hypertension in children.1 It is caused by impairment of blood flow to a part or all one or both kidneys as a result of narrowing of renal arteries.2 It has several different etiologies, but the most common is fibromuscular dysplasia (FMD), in Western countries, and Takayasu arteritis (TA) in the developing world.1 The underlying diagnosis of children with RVH is not always well defined as some children experience renal artery stenosis (RAS) as part of a genetic syndrome, such as neurofibromatosis types 1 or Williams syndrome.1 It could also occur secondary to other conditions such as tumor surgery, radiation therapy, or rarely following neonatal umbilical artery catheterization.1 There is no known relationship between congenital abnormalities in the renal tract (CAKUT) and RVD. However, RVD has previously been reported in a few subjects in association with renal anomalies such as solitary kidneys, multicystic dysplastic kidney, and polycystic disease.3-13 In this study, we report a series of 8 patients with RAS after being diagnosed earlier in life with CAKUT.