Renal Dysplasia: Clinical Features I

Clinical Features - I: Multicystic renal dysplasia is the most common cause of an abdominal mass in children <1 year old. It is often recognized in utero by 20-24 weeks gestational age. Most cases of renal dysplasia are sporadic; however, a small percentage have a familial component or association with syndromic malformations. Renal dysplasia is unilateral in about 90% of cases. The contralateral kidney is normal or shows compensatory hypertrophy. From a clinico-pathologic perspective, renal dysplasia may be classified as unilateral or bilateral, obstructive or non-obstructive, syndromic or non-syndromic, and sporadic or hereditary.

This autopsy specimen of urinary system from a neonate shows bilateral multicystic renal dysplasia and bilateral hydronephrosis secondary to obstruction of uretero-pelvic junction. Both kidneys are replaced by misshapen masses of variably-sized, thin-walled cysts. (close up of the previous case)