Congenital unilateral renal tubular dysgenesis and severe neonatal hypertension

Severe arterial hypertension rarely occurs in the neonatal period but may have life-threatening consequences. It is most often caused by renal parenchymal or vascular abnormality, which, to be accurately diagnosed, may require a combination of imaging modalities. We report on a case of neonatal hypertension presenting as cardiac failure. Initial imaging suggested unilateral renal artery stenosis, but this was not corroborated by magnetic resonance angiography. Surgical nephrectomy was curative for the hypertension and also allowed diagnosis of renal tubular dysgenesis. Unilateral congenital tubular dysgenesis without renal infarction has not been previously reported. We speculate that the condition was secondary to a period of localised hypoperfusion during early foetal life.