Renal dysplasia: an autopsy study of associated congenital malformations.

ABSTRACT

A retrospective analysis of infant autopsies in the last 14 years was done with the aim of determining the frequency of associated malformations. Renal dysplasia comprised 36 of 594 autopsies done and was the commonest congenital urologic malformation (6.06% of all infant autopsies). Histologically all cases were characterised by hallmarks of disordered metanephric differentiation and organisation. Extrarenal malformations were seen in all (100%) of the cases of which other urologic anomalies were the commonest (28) followed by those of musculoskeletal (18), gastrointestinal (13), cardiovascular (10) and central nervous system (6). Syndromic association was seen in 5 (14%) cases. There were twenty two percent still births and 47% infants died perinatally. A thorough search for systemic malformations is mandatory in all cases of renal dysplasia as these may influence the survival significantly. Cystic renal dysplasia should be differentiated from other causes of cystic renal diseases.