The supernumerary kidney is a rare anomaly characterized by the presence of an extra kidney. Obstructive cystic dysplasia occurs secondary to outflow tract obstruction. We present the case of a 10-year-old girl with recurrent urinary tract infection in whom evaluation revealed obstructive cystic dysplasia of the left kidney with ipsilateral vesicoureteric reflux and right supernumerary kidney. The history was significant for surgery for Ladd bands and spontaneous closure of an atrial septal defect. While ultrasound showed an empty left renal fossa, dimercaptosuccinic acid scan showed solitary right kidney with focal photopenic areas in the lower pole suggesting pyelonephritis. Contrast-enhanced computed tomography revealed right fused supernumerary kidney with focal nephronia and shrunken left kidney. The left ureter inserted ectopically into the urinary bladder; micturating cystourethrography confirmed grade 2 vesicoureteric reflux. The child was managed conservatively with antibiotics. Cystic renal dysplasia and supernumerary kidneys are a rare association that requires clinical and radiological monitoring. Screening of the contralateral functioning kidney and surgical correction of any anomalies is imperative. This case highlights the need for systematic evaluation for coexisting anomalies in patients with abnormal renal ultrasonography.
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