Frontonasal and Craniofrontonasal Dysplasia: Preoperative Quantitative Description of the Cranio-Orbito-Zygomatic Region Based on Computed and Conventional Tomography

Abstract

The unoperated cranio-orbito-zygomatic complex of 18 children (mean 4.7 years) with frontonasal dysplasia (FND) and 12 children (mean 1.1 years) with craniofrontonasal dysplasia (CFND) was quantified by 15 standard measurements performed on either computed tomography scans or facial tomograms. The results were compared with age-matched control values. In the FND group, the mean anterior interorbital and mid-interorbital distances were significantly increased at 148% and 118% of normal, and in the CFND patients, at 177% and 140% of normal. Excessive medial orbital wall protrusion (mean, 145% of normal in FND and 177% in CFND), shortened zygomatic arch lengths (mean, 94% of normal In FND and 91% in CFND), and reduced cephalic lengths (mean, 96% of normal in FND and 83% in CFND) were all observed. An expanded interzygomatic buttress distance was documented only in the CFND group, at 111% of normal. The clinical presentation of craniofacial deformities such as FND and CFND can be objectively described by a numerical analysis of the bony pathology.

Case of Complex Craniofacial Anomalies, Bilateral Nasal Proboscides, Palatal Pituitary, Upper Limbs Reduction, and Amnion Rupture Sequence: Disorganization Phenotype?

“…Hypertelorism, medial facial cleft, and agenesis of the corpus callosum are the features of the frontonasal dysplasia sequence [5,6]. However, in this condition the nose is usually present.…”