UNUSUAL CRANIAL ASPECTS OF THE APERT SYNDROME

Frank cloverleaf skull is found in approximately 4% of Apert-syndrome infants. However, the usual Apert skull and its cloverleaf form are sp ectral in nature. In all patients, the temporal bones are obliquely si tuated, and the degree to which this is so determines whether no, mild , moderate, or severe cloverleafing will occur. True encephalocele rar ely is found in the Apert syndrome, but pseudoencephalocele in the fro ntal region sometimes is confused with it. Because the Apert calvaria at birth is characterized by a widely gaping midline defect, the midfr ontal region of the brain is not covered by bone during early infancy. However, with time, closure of the midline defect occurs by coalescen ce of bony islands, thus covering the pseudoencephalic region. Also di scussed in this paper are (a) atypical calvarial development with pate ncy of the coronal sutures in one case, (b) size and position of the t emporal muscles, and (c) size and position of the superficial temporal fat pads.