Clinical conference I. Calvarium and cranial base in Apert's syndrome: an autopsy report.

A post-mortem examination of a 22-month-old boy with Apert's syndrome is reported. A linear coronal craniectomy performed at 11 months of age had fused completely in spite of the insertion of polyethylene film between the bony edges. Furthermore, progressive synostosis of other cranial sutures was observed. Histological examination of the cranial base showed that the sphenofrontal suture was fused and that the entire cerebral surface, including the sella turcica, was characterized by bone resorption. The sphenooccipital synchondrosis was extremely wide and abnormally shaped, and the chondral growth activity seemed diminished. It was concluded that the patient exhibited primary as well as secondary growth distrubances in the calvarium and cranial base. The premature synostosis of the coronal and sphenofrontal sutures and the anomaly of the sphenooccipital synchondrosis were interpreted as primary defects. The altered shape of the calvarium and the marked resorption and shortening of the cranial base were considered secondary changes. Furthermore, the investigation indicated that the conventional linear coronal craniectomy cannot be expected to improve the craniofacial growth pattern in patients with Apert syndrome. Alternative surgical procedures were suggested.