The kleeblattschädel anomaly in Apert syndrome: intracranial anatomy, surgical correction, and subsequent cranial vault development.

Abstract

We present a case of Apert syndrome in which intracranial anomalies of the cranial base were localized to the lesser wings of the sphenoid and sphenoid ridge. The lesser wings of the sphenoid were displaced superiorly to follow the fused coronal sutures bilaterally, where they met at a single point on the skull vertex. Careful preoperative study of the intracranial anatomy in the kleeblattschädel anomaly led to a surgical plan for early correction of the anomaly. The present report indicates that an aggressive approach to the correction of the kleeblattschädel anomaly beginning early in infancy can result in normalization of the trilobar skull configuration. Although this approach can correct the kleeblattschädel anomaly, 3.5-year follow-up in this patient with Apert syndrome demonstrates progressive turricephaly despite repeated cranial vault remodeling. Although the trilobar skull configuration can be corrected through early surgical intervention, the long-term correction of progressive turricephaly in patients with Apert syndrome remains an unsolved problem.