Segmental multicystic dysplasia and ureteropelvic junction obstruction in a nonduplicated kidney

A 2-month-old child presented for evaluation of prenatal hydronephrosis. Imaging studies were consistent with a right duplex system with a dysplastic, nonfunctioning upper pole and lower pole ureteropelvic junction obstruction. We proceeded with removal of the upper pole and pyeloplasty and were surprised to find a single collecting system with a cystic, dysplastic upper pole segment and the absence of an upper pole pelvis or ureter. The rare diagnosis of a segmental multicystic dysplastic kidney with ipsilateral ureteropelvic junction obstruction was made. We present a review of the case and of previous literature on this topic.