Section Editor: Dharam M. Ramnani, MD

Virginia Urology, Richmond, VA, USA

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Clinical Features - II: The clinical picture of multicystic renal dysplasia is dictated by the degree of dysplasia and any associated urinary obstruction. When there is bilateral involvement, the neonate displays Potters sequence and succumbs to pulmonary hypoplasia within a few days of birth. With unilateral or segmental involvement, the infant may be asymptomatic at birth but may subsequently show failure to thrive, hypertension, and excessive thirst.

Almost 90% of cases have associated anomalies of the urinary system - obstruction of the uretero-pelvic junction causing hydronephrosis, ureteral atresia or stenosis, or urethral obstruction (usually posterior urethral valves). Other anomalies such as cardiac malformations may be present.

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