Segmental multicystic dysplastic kidney in children: clinical presentation, imaging finding, management, and outcome

PurposeThe aim of the study was to assess the presentation, imaging findings, management, and outcome of segmental multicystic dysplastic kidney (MCDK) in children.Materials and MethodsSix patients with segmental MCDK were diagnosed and observed at our hospital. We reviewed the medical records to collect data on initial symptoms, results of imaging studies, associated urinary tract anomalies, operation, and outcome.ResultsThree patients had abnormal prenatal ultrasound and were diagnosed by further postnatal imaging; they were asymptomatic after birth. The other 3 children presented with a renal mass, recurrent urinary tract infection, or urinary incontinence. Five patients had associated ipsilateral or contralateral urinary tract abnormalities, including vesicoureteral reflux, ureterocele, duplex collecting system, ureteropelvic junction stenosis, and ectopic ureter. Lower tract reconstruction was performed in 3, and open biopsy was done in 1 child. None underwent partial or total nephrectomy, and all had involution of the cysts for a mean of 40 months.ConclusionsMost of the cases occur in the upper pole of a duplex kidney and often involute spontaneously without significant complication. The clinical presentation and imaging findings depend on the associated anomalies and complications. Although the latter may require surgery to resolve, segmental MCDK itself rarely requires resection.