Craniofacial morphology in the nevoid basal cell carcinoma syndrome

A roentgencephalometric analysis of eight males and four females with nevoid basal cell carcinoma syndrome (NBS) is presented. The patients were 14-54 years of age. Fourteen angular and 26 linear variables were measured and compared with control data. The main roentgencephalometric features were: proportionate increase of the size of the calvarium, protrusion of the frontal and parietal region, low position of the occipital region, increased interorbital distance, increased length of the mandible, mandibular retrognathia due to changes in shape and position of the mandible. The suggestion of a characteristic craniofacial morphology as a part of the syndrome was substantiated. Together with other skeletal aberrations the roentgencephalometric findings seemed to indicate that the syndrome is a dysostosis of generalized type.