Postaxial polydactyly, ulnar ray dysgenesis, and renal cystic dysplasia insibs

We describe two brothers with variable expression of a unique syndrome. One sib has postaxial polydactyly of the right hand and feet, two digits on th e left hand (a thumb and first digit), bilateral ulnar ray dysgenesis, ectr odactyly of one hand, and ultrasonic evidence of cystic kidneys. His brothe r has postaxial polydactyly and small kidneys. The parents and a third sib are normal. They do not have the Pallister ulnar-mammary syndrome but may h ave an unusual form of the Weyers oligodactyly syndrome. This appears to be the first report of an acro-renal syndrome with ulnar dysgenesis, oligodac tyly, polydactyly, and dysplastic kidneys. Am. J. Med. Genet. 87:426-429, 1 999. (C) 1999 Wiley-Liss, Inc.