Although multicystic kidney (MCK) is a common renal anomaly, the management of this entity remains controversial. Much of the controversy stems from a lack of long-term data on its natural history. Only recently have efforts been made to follow the MCK and accumulate long-term data on its natural history. The results of these efforts may have a significant impact on management.

Schwartz is credited with the first description of an MCK in a 7-month-old child. In addition to describing the kidney as having been replaced by multiple cysts in a “bunch of grapes” arrangement, he also reported the ureter to be atretic. Spence's classic article appeared in 1955, in which he further described the MCK, distinguishing it from other forms of renal cystic disease.

This chapter reviews the presentation of MCK and looks at the available long-term data in an attempt to arrive at a management protocol.

Presentation and diagnosis

The classic presentation of MCK was either a palpable mass in a newborn or infant or an incidental finding at autopsy. Pathak and Williams, in a series of 22 cases, reported that eight cases presented with an abdominal mass, seven had vomiting, and three had failure to thrive. With the advent of prenatal ultrasonography, many more asymptomatic lesions are being identified. A review of data accumulated by the National Multicystic Kidney Registry in the USA indicated that 72% of registered cases were discovered on prenatal ultrasound (J. Wacksman, personal communication).