Multicystic Dysplastic Kidney Disease

Multicystic dysplastic kidney disease (MCDK) is a unique entity in the broad spectrum of cystic diseases of the kidney. Classical features include nonfunctioning renal parenchyma accompanied by multiple noncommunicating cysts. In the era of routine antenatal ultrasound scanning, MCDK is being diagnosed more frequently. For those cases not diagnosed prenatally, presenting symptoms usually include a palpable abdominal mass, pain, urinary tract infections, hematuria, and hypertension. The pathogenesis remains unclear and most likely relates to a form of renal/ureteric dysplasia. In the initial few years, MCDK can undergo partial or even complete involution. There is still significant controversy and differences of opinion in regard to the possibility of malignant transformation, and for this reason the management of MCDK is mostly conservative and dictated primarily by the coexisting abnormalities. Nephrectomy is only indicated in symptomatic cases or as a consequence of parental choice.