Ulnar Ray Dysgenesis With Postaxial Polydactyly And Renal Cystic Dysplasia

Although there are many syndromes of renal and/or genitorenal anomalies with radial ray dysostoses (Evans et al., 1992), the association of renal anomalies with ulnar ray dysgenesis has been found to occur mainly in 2 entities, the ulnar-mammary syndrome (181450) and Weyers ulnar ray/oligodactyly syndrome (602418). Kaplan and Bellah (1999) described brothers with variable expression of a possibly unique syndrome: an acrorenal syndrome with ulnar dysgenesis, oligodactyly, polydactyly, and dysplastic kidneys.