ACROCEPHALY AND SCAPHOCEPHALY WITH SYMMETRICALLY DISTRIBUTED MALFORMATIONS OF THE EXTREMITIES

Abstract

That acrocephaly and syndactylism occur in combination has been generally appreciated since 1906, when the French pediatrician, Apert,¹published the report of eight cases discovered in the literature and of one case from personal experience. Inasmuch as all the subjects exhibited deformities of the head, hands and feet so similar as to seem almost identical, Apert believed that the combination constituted a clinical entity. Accordingly, he gave to it the name "acrocephalosyndactylism." Since the publication of Apert's paper, other cases have been reported. We have been able to collect from the literature eleven cases exactly like those in Apert's series, and four others presenting the same deformity of the head but much less extensive deformities of the extremities. In addition, we have found four cases in which there was a characteristic affection of the head with slight malformations of the extremities, probably of the same nature as the malformations