Background: Renovascular hypertension resulting from abdominal-aortic (ie mid-aortic) and renal artery stenosis may result in decreased kidney function. Data regarding the potential benefit of renal revascularization for the purpose of kidney function recovery in children is lacking.
Case description: A 3 months old male infant presented with atypical hemolytic uremic syndrome (aHUS) which responded to treatment with eculizumab . At 3 years of age, after negative genetic work up for aHUS, eculizumab was stopped, but he continued to have resistant HTN. At 6 years of age, a DMSA scan showed severe right kidney dysfunction, and a nephrectomy was performed, but there was no improvement in his BP status. He presented at 8 years of age with severely oliguric acute kidney injury (AKI) and malignant HTN. Angiography revealed inter-renal aortic stenosis and focal proximal occlusion of the left renal artery The left nephrogram was delayed, and perfused through collaterals, but was normal in size. DTPA scan showed very weak perfusion and parenchymal uptake of the kidney. He remained dialysis dependentwith refractory HTN despite 5 antihypertensives for 8 weeks, and was therefore referred to a multidisciplinary pediatric renovascular hypertension at an international high volume center of excellence for surgical revascularization, which was performed via patch aortoplasty and left renal artery reimplantation. This gradually led to increased urine output, termination of renal replacement therapy, returning to baseline eGFR and normalization of BP.
Conclusion: Surgical revascularization is a useful adjunctive therapeutic alternative for refractory hypertension and even severe kidney dysfunction in children.