MR imaging in Drash syndrome

Drash syndrome is a disorder of the genitourinary ridge differentiation and includes abnormal genital development, nephropathy, and a predisposition to develop Wilms tumor. In three patients with Drash syndrome, magnetic resonance was correlated with ultrasound, CT, and surgical findings. Two patients with glomerulonephritis had enlarged kidneys, loss of corticomedullary differentiation, and overall decrease in signal intensity on T1-weighted sequences. Two large Wilms tumors were identified, but a small 1 cm nephroblastoma was not recognized. Anomalies associated with ambiguous genitalia, such as absent uterus, were also correctly diagnosed.