Objective: Multicystic dysplastic kidney is a relatively common developmental abnormality in infants and children. Additional abnormalities like vesicoureteral reflux, ureteropelvic junction obstruction, and ureterovesical junction obstruction may accompany multicystic dysplastic kidney. In this article, we reviewed the pathogenesis, diagnosis, associated urinary tract anomalies and results of management of multicystic dysplastic kidney in the light of the literature.

Material and Method: We retrospectively assessed 20 children with unilateral multicystic dysplastic kidney between January 2005 and December 2009. Mean duration of follow-up was 35 ± 8.7 months. All children with multicystic dysplastic kidney underwent nephrectomy. Patient characteristics, the pathology findings, associated urinary tract anomalies and results of management were reviewed.

Results: Abdominal ultrasound, voiding cystourethrography and renal scintigraphy revealed vesicoureteral reflux in 3 (15%) children, ureteropelvic junction obstruction in 3 (15%) children, and a duplex system in the contralateral kidney in 1 (5%) child. Blood pressure values, serum urea, creatinine and urinalysis were within normal range in all children during follow-up.

Conclusion: A conservative approach to children with multicystic dysplastic kidney has been advocated, but surgical removal is also another management modality on the basis of risk of hypertension, mass effect, potential for malignant change, and cost of repeated ultrasound examination.