Multicystic renal dysplasia is a common cause of childhood end-stage renal failure and is categorized under the entity known as congenital anomalies of kidney and urinary tract. With the increasing knowledge about their natural history, it is now known that most of the cases of unilateral multicystic dysplastic kidney undergo spontaneous involution; thus, most of the pediatric urologists now prefer conservative management with long-term nephrological and urological observation. However, the presence of recurrent urinary tract infection as in the present case report due to associated ipsilateral Grade V vesicoureteral reflux, a rare associated finding mandates nephrectomy.
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