Multicystic dysplastic kidney: a retrospective study with clinicopathological correlation

Background: Multicystic dysplastic kidney (MCDK) is one of the most frequently observed congenital urinary tract abnormalities. Methods: Retrospective study of MCDK in nephrectomy specimens seen over a 20 year period at a tertiary care teaching hospital in South India. Results: MCDK was evident in 6 of the 230 nephrectomy specimens (2.6%) seen during the 20 year period of study. Their median age [interquartile range (IQR)] was 2.25 (1.075-7.750) years; there were 3 males. MCDK more frequently involved the right kidney (5/6); 4 cases presented with megaureter. Salient histopathological findings included cystically dilated spaces lined by flattened to cuboidal lining epithelium with intervening stroma showing entrapped, dilated, narrowed immature tubules with surrounding smooth muscle cuffing. Conclusions: Histopathological examination remains the mainstay in the diagnosis and helps in differentiating MCDK from other cystic lesions.