Nevoid Basal cell carcinoma syndrome: a cephalometric study of patients and controls.

Abstract Craniofacial morphology of patients with nevoid basal cell carcinoma syndrome (NBCCS) has sometimes been reported at clinical examination, but any investigation has described it on the basis of cephalometric measurements. The purpose of this study was to conduct a cephalometric analysis of patients with NBCCS and to compare measurements with non-NBCCS subjects of similar ages, to elucidate if there is any relationship between NBCCS and craniofacial morphology. The study population consisted of 14 adult patients (9 men and 5 women), ranging in age from 18.2 to 56.8 years, with the diagnosis of NBCCS, with good-quality lateral cephalometric radiographs, and 14 adult healthy patients matched for age and sex to the NBCCS group. Cephalometric measurements were carried out on radiographs, and measurements of angles and distances were performed. Statistical differences between NBCCS subjects and controls were observed. Data analysis displayed that the measurements of the anterior cranial base (P ≤ 0.0043), mandibular length (P ≤ 0.0168), and maxillary length (P ≤ 0.0284), posterior facial height (P ≤ 0.0406), and of mandibular angle (P ≤ 0.0026), facial axis angle (P ≤ 0.0402), lower facial height angles (P ≤ 0.0135), and of interincisal angulation (P ≤ 0.0148) were higher in NBCCS subjects in respect to controls. On the contrary, the facial convexity (P ≤ 0.0189) and the mandibular arc angle (P ≤ 0.0378) were reduced in NBCCS subjects. According to these findings, NBCCS patients presented a sagittal lengthening of the anterior cranial base and maxilla and a vertically and horizontally overdeveloped mandible, together with the features of a long-face syndrome with a large gonial angle.