Fourteen reproducible cranio-orbito-zygomatic measurements taken from 26 standard axial computed tomographic (CT) scans of unoperated individuals with symmetric forms of Treacher Collins syndrome (TCS) were compared to age-matched controls. The interorbital measurements of the TCS patients were at the mean when compared to their cohort group (medial and lateral orbital wall separation), while the zygomatic measurements were significantly less than normal confirming the extent of malar hypoplasia. The congenitally deficient lateral aspect of the orbits in TCS patients was confirmed by the greater than normal values measured for globe protrusion and medial orbital wall protrusion in conjunction with the diminished lateral orbital wall lengths, all of which use the lateral orbital rim as a reference point. The abnormal shape of the anterior cranial vault in patients with TCS was documented as a diminished intercoronal distance (width) and decreased cephalic length when compared to normal age-matched controls.
Fourteen reproducible cranio-orbito-zygomatic measurements taken from 26 standard axial computed tomographic (CT) scans of unoperated individuals with symmetric forms of Treacher Collins syndrome (TCS) were compared to age-matched controls. The interorbital measurements of the TCS patients were at the mean when compared to their cohort group (medial and lateral orbital wall separation), while the zygomatic measurements were significantly less than normal confirming the extent of malar hypoplasia. The congenitally deficient lateral aspect of the orbits in TCS patients was confirmed by the greater than normal values measured for globe protrusion and medial orbital wall protrusion in conjunction with the diminished lateral orbital wall lengths, all of which use the lateral orbital rim as a reference point. The abnormal shape of the anterior cranial vault in patients with TCS was documented as a diminished intercoronal distance (width) and decreased cephalic length when compared to normal age-matched controls.
The unoperated crano-orbito-zygomatic complex of 18 children (mean 4.7 years) with frontonasal dysplasia (FND) and 12 children (mean 1.1 years) with crainofrontonasal 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 11% 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.
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.