The Kleeblattschädel Phenomenon: Sign or Syndrome?

Abstract

To the Editor.—In a recent communication, Hall et al¹observed that a severe case of Crouzon's syndrome with a trilobed skull in an affected family might well have been interpreted as the Kleeblattschädel syndrome had the case arisen as a fresh mutation. They further noted that in some instances Crouzon's syndrome and the Kleeblattschädel syndrome might be one and the same disorder. They suggested that it would be more appropriate to name this cranial malformation the Kleeblattschädelanomalyrather than the Kleeblattschädel syndrome to avoid the implication of a specific etiology. The purpose of this letter is to support the ideas put forth by Hall et al. The Kleeblattschädel phenomenon is not always a disordersui generis, but a sign which may occur in a variety of disorders. The cloverleaf skull may occur (1) as an isolated anomaly or in association with (2) bony ankylosis of the limbs,