Segmental multicystic kidney and ipsilateral duplication anomalies.

Abstract

We characterize segmental multicystic dysplasia, in which there is a duplex collecting system with multicystic changes in the upper pole. Three neonates with an abdominal mass and/or prenatal diagnosis of a multicystic kidney were evaluated. Postnatal ultrasound showed a classic multicystic kidney and small ipsilateral orthotopic ureterocele in all cases. All patients had ipsilateral grade V lower pole reflux and a renal scan showed functioning lower pole systems. One patient underwent bilateral ureteroneocystostomy and another underwent upper pole nephrectomy. There has been reduction in the size of the segmental multicystic kidney in 2 patients and decrease in reflux grade in 2 (followup 2 to 5 years). Newborns with an apparent multicystic kidney should undergo a voiding cystourethrogram and renal scan to confirm the diagnosis. Segmental multicystic kidney can be followed nonoperatively in most cases because the cysts tend to involute.