Comparative three-dimensional analysis of CT-scans of the calvaria and cranial base in Apert and Crouzon syndromes

The purpose of this study is to describe and analyze Apert and Crouzon skulls from three-dimensional (3-D) reconstructions of CT-scans. 12 Apert patients and 19 with Crouzon syndrome were included in the study. The age range was 0 to 23 years. All CT-scannings were carried out according to the same protocol with a slice thickness of 2 or 4 mm and 3-D reconstructions of the craniofacial region included midsagittal and horizontal cuts. A number of qualitative characteristics of the calvaria and cranial base were recorded and the cranial base angle was measured on the 3-D models. Our results showed that Apert and Crouzon syndromes are very different in cranial development and their dysmorphology is highly age dependent. We suggest that cartilage abnormalities, especially in the anterior cranial base, play a primary role in cranial development in the Apert syndrome from very early intrauterine life. Several cranial anomalies observed postnatally, however, are caused by the resultant dysmorphic and compensatory growth and are probably compounded by early cranial deformation. The primary abnormality in Crouzon syndrome appears to be premature fusion of sutures and synchondroses. Based on the findings at birth and early infancy it would seem that such fusions occur relatively late in fetal life. The adult cranial form in Crouzon's patients is explainable by resultant dysmorphic and compensatory growth changes. Very early release of the coronal suture areas with advancement of the frontal bone is advocated in both syndromes but for somewhat different reasons. In Apert syndrome, the primary indication for such treatment is to reduce further dysmorphic growth changes in the calvaria and cranial base, whereas the risk for increased intracranial pressure is low considering the large midline calvarial defect found in early infancy. In contrast, newborns with Crouzon syndrome develop multiple suture synostoses and fused synchondroses, and early surgery is most often indicated to prevent or treat increased intracranial pressure.