Renal dysplasia with multisystem malformation--a study of 9 cases

Nine cases of renal dysplasia encountered in a review of necropsy material are described. All these cases had associated major and minor malformations of other systems. Three out of four cases identifying with 'prune belly' syndrome had lower urinary tract obstruction. Three cases fulfilled the criteria of Meckel's syndrome. Three of these six cases, in addition, showed overlapping features with other syndromes. Three cases of unilateral dysplasia with contralateral agenesis had associated anomalies not conforming to any definite combination. We suggest that the association of malformations of developmentally unrelated systems may be random, not sharing any feature except being incompatible with life.