A Syndrome Characterized by Ectodermal Dysplasia, Polydactyly, Chondro-Dysplasia and Congenital Morbus Cordis: Report of Three Cases

Abstract

The association of the four following congenital abnormalities-ectodermal dysplasia affecting the hair, teeth and nails; polydactyly; chondro-dysplasia; and congenital morbus cordis-has been observed in two children in whom the clinical picture is so strikingly similar as to justify its description as a separate syndrome. Although many atypical cases of achondroplasia and chondro- osteo-dystrophy have been recorded with associated abnormalities, and there is an extensive literature relating to congenital ectodermal dysplasia, we have found no detailed description of the syndrome previously published. McIntosh (1933), however, in the tenth edition of ' Diseases of Infancy and Childhood ' includes illustrations and some clinical particulars of a child who is clearly an incomplete example of the same syndrome. He has kindly furnished a full report on this case, and it is included as case 3 in the present series ; thanks are also due to Dr. S. Levine, Professor of Pediatrics at the New York Hospital, for permission to include subsequent observations made on the same patient.