Alex A. Kane

Anthropometric precision and accuracy of digital three-dimensional photogrammetry: comparing the Genex and 3dMD imaging systems with one another and with direct anthropometry.

A variety of commercially available three-dimensional (3D) surface imaging systems are currently in use by craniofacial specialists. Little is known, however, about how measurement data generated from alternative 3D systems compare, specifically in terms of accuracy and precision. The purpose of this study was to compare anthropometric measurements obtained by way of two different digital 3D photogrammetry systems (Genex and 3dMD) as well as direct anthropometry and to evaluate intraobserver precision across these three methods. On a sample of 18 mannequin heads, 12 linear distances were measured twice by each method. A two-factor repeated measures analysis of variance was used to test simultaneously for mean differences in precision across methods. Additional descriptive statistics (e.g., technical error of measurement [TEM]) were used to quantify measurement error magnitude. Statistically significant (P < 0.05) mean differences were observed across methods for nine anthropometric variables; however, the magnitude of these differences was consistently at the submillimeter level. No significant differences were noted for precision. Moreover, the magnitude of imprecision was determined to be very small, with TEM scores well under 1 mm, and intraclass correlation coefficients ranging from 0.98 to 1. Results indicate that overall mean differences across these three methods were small enough to be of little practical importance. In terms of intraobserver precision, all methods fared equally well. This study is the first attempt to simultaneously compare 3D surface imaging systems directly with one another and with traditional anthropometry. Results suggest that craniofacial surface data obtained by way of alternative 3D photogrammetric systems can be combined or compared statistically.

Speech, cognitive, and behavioral outcomes in nonsyndromic craniosynostosis

Background: The neuropsychological morbidity of nonsyndromic craniosynostosis is incompletely understood. The purpose of this study was to establish the prevalence of speech-language, cognitive, and behavioral abnormalities in this population and to stratify the findings on the basis of the affected suture and age of diagnosis with speech-language or psychological abnormalities. Methods: Charts of all patients with nonsyndromic craniosynostosis evaluated between 1978 and 2000 were reviewed, noting diagnoses of speech-language, cognitive, or behavioral abnormalities. Findings were statistically analyzed for variance with regard to affected suture and diagnosis of abnormalities. Results: Two hundred fourteen patients with nonsyndromic craniosynostosis had documented follow-up evaluations with an average age of 6 years 4 months at last visit. Speech, cognitive, and/or behavioral abnormalities were manifest in 49 percent of the patients with specific rates for each suture as follows: right unilateral coronal, 61 percent; bilateral coronal, 55 percent; multiple, 47 percent; metopic, 57 percent; left unilateral coronal, 52 percent; lambdoid, 44 percent; and sagittal, 39 percent. This prevalence of abnormalities was a statistically significant increase from the general population. Logistic regression demonstrated that as patient age increased, the percentage of abnormal diagnoses also increased. Conclusions: Nonsyndromic craniosynostosis is often associated with cognitive, speech, and/or behavioral abnormalities. The etiopathology of this association is unknown. Furthermore, the proportion of children diagnosed with cognitive and behavioral dysfunction increases with age. Therefore, longitudinal cognitive, behavioral, and speech assessment and treatment are integral to the care of these patients.

Isolated sagittal and coronal craniosynostosis associated with TWIST box mutations

Craniosynostosis, the premature fusion of one or more cranial sutures, affects 1 in 2,500 live births. Isolated single‐suture fusion is most prevalent, with sagittal synostosis occurring in 1/5,000 live births. The etiology of isolated (nonsyndromic) single‐suture craniosynostosis is largely unknown. In syndromic craniosynostosis, there is a highly nonrandom pattern of causative autosomal dominant mutations involving TWIST1 and fibroblast growth factor receptors (FGFRs). Prior to our study, there were no published TWIST1 mutations in the anti‐osteogenic C‐terminus, recently coined the TWIST Box, which binds and inhibits RUNX2 transactivation. RUNX2 is the principal master switch for osteogenesis. We performed mutational analysis on 164 infants with isolated, single‐suture craniosynostosis for mutations in TWIST1, the IgIIIa exon of FGFR1, the IgIIIa and IgIIIc exons of FGFR2, and the Pro250Arg site of FGFR3. We identified two patients with novel TWIST Box mutations: one with isolated sagittal synostosis and one with isolated coronal synostosis. Kress et al. [ 2006 ] reported a TWIST Box “nondisease‐causing polymorphism” in a patient with isolated sagittal synostosis. However, compelling evidence suggests that their and our sequence alterations are pathogenic: (1) a mouse with a mutation of the same residue as our sagittal synostosis patient developed sagittal synostosis, (2) mutation of the same residue precluded TWIST1 interaction with RUNX2, (3) each mutation involved nonconservative amino acid substitutions in highly conserved residues across species, and (4) control chromosomes lacked TWIST Box sequence alterations. We suggest that genetic testing of patients with isolated sagittal or coronal synostosis should include TWIST1 mutational analysis.

Helmet Versus Active Repositioning for Plagiocephaly: A Three-Dimensional Analysis

BACKGROUND AND PURPOSE: Orthotic helmets and active repositioning are the most common treatments for deformational plagiocephaly (DP). Existing evidence is not sufficient to objectively inform decisions between these options. A three-dimensional (3D), whole-head asymmetry analysis was used to rigorously compare outcomes of these 2 treatment methods. PATIENTS AND METHODS: Whole-head 3D surface scans of 70 infants with DP were captured before and after treatment by using stereophotogrammetric imaging technology. Helmeted (n = 35) and nonhelmeted/actively repositioned (n = 35) infants were matched for severity of initial deformity. Surfaces were spatially registered to a symmetric template, which was deformed to achieve detailed right-to-left point correspondence for every point on the head surface. A ratiometric asymmetry value was calculated for each point relative to its contralateral counterpart. Maximum and mean asymmetry values were determined. Change in mean and maximum asymmetry with treatment was the basis for group comparison. RESULTS: The helmeted group had a larger reduction than the repositioned group in both maximum (4.0% vs 2.5%; P = .02) and mean asymmetry (0.9% vs 0.5%; P = .02). The greatest difference was localized to the occipital region. CONCLUSIONS: Whole-head 3D asymmetry analysis is capable of rigorously quantifying the relative efficacy of the 2 common treatments of DP. Orthotic helmets provide statistically superior improvement in head symmetry compared with active repositioning immediately after therapy. Additional studies are needed to (1) establish the clinical significance of these quantitative differences in outcome, (2) define what constitutes pathologic head asymmetry, and (3) determine whether superiority of orthotic treatment lasts as the child matures.

Relationship between bone and muscles of mastication in hemifacial microsomia

&NA; The relationship between the bone and muscles of mastication in hemifacial microsomia was studied using three‐dimensional volumetric computed tomography scans and image processing techniques. High resolution head computed tomography scans were obtained from 31 patients with unilateral hemifacial microsomia and eight normal patients. Using three‐dimensional volume renderings of bone, mandibular deformities in patients with hemifacial microsomia were classified using the Pruzansky system. For each patient, specific craniofacial bones (temporal bone, maxilla mandible) and the muscles of mastication (masseter, temporalis and lateral and medial pterygoid) were segmented bilaterally from the image volume for independent display and volume measurement. Volumes were expressed as the ratio of the affected: unaffected sides. For the masseter and temporalis, the relationship between muscular hypoplasia and osseous hypoplasia in its origin and insertion was studied by plotting affected:unaffected bone volume as a function of affected:unaffected muscle volume for each muscle, bone of origin, bone of insertion triplet. The volumes of the pterygoid muscles were compared with hemimandibular volumes. The precision of object segmentations was examined by repetitive definition tasks, whereas the accuracy of volume measurement was tested by scanning custom‐made phantom objects and comparing digital to physical object volume measurements. Volume measurements performed using these techniques were both accurate and precise. In hemifacial microsomia, the extent of hypoplasia of specific muscles of mastication predicted the extent of dysplasia in their osseous origin and insertion. However, the reverse was not true. The extent of hypoplasia of the facial bones did not necessarily predict the extent of hypoplasia in the attached muscles of mastication. Pruzansky grade of the mandible described the degree of mandibular hypoplasia on the affected side, but was inconsistent in its prediction of volume decrease of the other facial bones.

Relationship of brain and skull in pre- and postoperative sagittal synostosis

Models of vertebrate skull evolution stress the coordinated developmental relationship between the skull and the brain that it houses. This study investigates the relationship between altered skull morphology and brain morphology in premature fusion of the cranial sagittal suture (isolated sagittal synostosis; ISS), a condition associated with dysmorphology of both neurocranium and brain. Although the skull displays a more normal shape following reconstructive cranial vault surgery, effects of this surgery on the brain have not been investigated. Landmark coordinate data were collected from three‐dimensional magnetic resonance imaging reconstructions of the brain in a sample of ISS patients and an age‐matched unaffected cohort. These data were analysed using Euclidean distance matrix analysis (EDMA). Results show that the brain in ISS is dysmorphic preoperatively, displaying a posteriorly directed neural expansion that does not ‘worsen’ with growth. Postoperatively, the brain in ISS displays a more globular shape overall as compared with the preoperative morphology, but differs from normal in its subcortical morphology. These results show that the ISS brain is altered following neurocranial surgery, but does not more closely approximate that of unaffected individuals. This suggests that although the brain is affected by manipulation of the skull, it retains a growth pattern that is, at least in part, independent of the skull.

Effect of distraction osteogenesis of the mandible on upper airway volume and resistance in children with micrognathia.

&NA; Children with craniofacial anomalies often have compromise of the upper airway, a condition with potential for morbidity and mortality. In children with microretrognathia, the diminutive size and retruded position of the mandible reduces the size of the oropharynx, thereby predisposing to glossoptosis and airway obstruction. Although several authors have reported successful use of mandibular distraction osteogenesis to alleviate this type of upper airway obstruction, the physiologic relationship between changes in mandibular shape, size, and position and upper airway dynamics remains undefined. The purpose of this study was to develop methodologies to quantitatively evaluate upper airway dynamics in children with micrognathia both before and after mandibular distraction osteogenesis. The patient population consisted of four children with micrognathia who had successfully undergone upper airway stabilization by bilateral mandibular distraction osteogenesis. The data used were digitally archived computed tomographic scan data from highresolution, thin‐slice head computed tomographic scans obtained before and after mandibular distraction. Upper airway evaluation was performed in two ways: static and dynamic. Static analysis consisted of computer quantification of predistraction and postdistraction mandibular and upper airway volumes using Analyze imaging software. Dynamic analysis consisted of fabrication of rigid stereolithographic hollow cast models of the upper airway produced from computed tomographic scan data. Models were used for characterization of upper airway resistance and flow patterns as related to respiration. After distraction osteogenesis, mandibular total volume increased 32, 32, 18, and 25 percent (mean, 27 percent) and upper airway volume increased by 20, 31, 23, and 71 percent (mean, 37 percent). A significant decrease in flow resistance, both inspiratory and expiratory, was observed in the patient with the greatest upper airway volume increase (71 percent) after distraction. After distraction, the inspiratory resistance was diminished by 51 percent and the expiratory resistance diminished by 85 percent. However, the three patients with more modest upper airway volume increases of 20 to 31 percent demonstrated no statistically significant change in flow resistance after distraction. Results of this study support the conclusion that distraction osteogenesis of the micrognathic mandible increases the volume of the upper airway, roughly paralleling the increase in mandibular volume. In the biomechanical airway model studied, upper airway volume expansion has been shown to be able to decrease the flow resistance over the length of the airway, presumably secondary to an increase in the average cross‐sectional area. The artificial rigidity of the stereolithographic “airway” compared with the elasticity of the human upper airway may account for the insensitivity of this model to smaller but clinically significant airway changes. (Plast. Reconstr. Surg. 109: 1809, 2002.)

Orbital Dysmorphology in Unilateral Coronal Synostosis

Unilateral coronal synostosis (UCS) produces overt craniofacial dysmorphology. UCS surgery in infancy aims to release the osseous restriction and normalize the fronto-orbital deformity. The quantitative effect of this surgery on the orbit and its contents is unknown. This study was conducted to quantify the preoperative orbital dysmorphology and its surgical outcome in patients with unilateral coronal synostosis. Twenty-eight UCS patients had preoperative three-dimensional computerized tomographic (CT) scans (at mean age 4.0 months), cranio-orbital reconstructive surgery (at 4.7 months), and postoperative scans (at 18.1 months). The CT data were analyzed using a computer workstation and AnalyzeTM biomedical imaging software. Four measurements were performed on both ipsilateral (same side as synostosis) and contralateral (opposite to synostosis) orbits of each scan: orbital index (OI, 100 x height/width of orbit), orbital cavity volume (OV), ocular globe volume (GV), and ventral globe Index (VGI, 100 x globe volume ventral to the anterior surface of orbital cavity/GV). The data were analyzed for statistical significance using Students ttest. Preoperatively, the OI was significantly greater on the ipsilateral than on the contralateral side (113.7 vs. 87.3). There was a significant improvement on both sides of the orbit postoperatively, with ipsilateral 99.1 and contralateral 92.1. However, the difference between both sides remained significant. The OV was smaller in the ipsilateral orbits both pre- and postoperatively, with ipse/contralateral ratios of 95.8 and 95.2, respectively. Importantly, the GV was consistently smaller in the ipsilateral orbits preoperatively, with an ipse/contralateral ratio of 93.3. The ratio increased to 97.1 postoperatively, a statistically significant change. In the ipsilateral orbits, the preoperative VGI was significantly greater. The VGI improved postoperatively. These data indicate that UCS affects the development of the osseous orbit as well as its soft-tissue contents. After cranio-orbital surgery, there is diminution of asymmetry of both the bony orbit and its soft-tissue contents. Partial normalization of orbital dysmorphology occurred during the first postoperative year. UCS surgery in infancy does not prevent growth of orbital hard or soft tissues, and it seems to permit normalization of previously impaired growth.

Endoscopically assisted versus open repair of sagittal craniosynostosis: the St. Louis Children's Hospital experience

OBJECT This study investigated the differences in effectiveness and morbidity between endoscopically assisted wide-vertex strip craniectomy with barrel-stave osteotomies and postoperative helmet therapy versus open calvarial vault reconstruction without helmet therapy for sagittal craniosynostosis. METHODS Between 2003 and 2010, the authors prospectively observed 89 children less than 12 months old who were surgically treated for a diagnosis of isolated sagittal synostosis. The endoscopic procedure was offered starting in 2006. The data associated with length of stay, blood loss, transfusion rates, operating times, and cephalic indices were reviewed. RESULTS There were 47 endoscopically treated patients with a mean age at surgery of 3.6 months and 42 patients with open-vault reconstruction whose mean age at surgery was 6.8 months. The mean follow-up time was 13 months for endoscopic versus 25 months for open procedures. The mean operating time for the endoscopic procedure was 88 minutes, versus 179 minutes for the open surgery. The mean blood loss was 29 ml for endoscopic versus 218 ml for open procedures. Three endoscopically treated cases (6.4%) underwent transfusion, whereas all patients with open procedures underwent transfusion, with a mean of 1.6 transfusions per patient. The mean length of stay was 1.2 days for endoscopic and 3.9 days for open procedures. Of endoscopically treated patients completing helmet therapy, the mean duration for helmet therapy was 8.7 months. The mean pre- and postoperative cephalic indices for endoscopic procedures were 68% and 76% at 13 months postoperatively, versus 68% and 77% at 25 months postoperatively for open surgery. CONCLUSIONS Endoscopically assisted strip craniectomy offers a safe and effective treatment for sagittal craniosynostosis that is comparable in outcome to calvarial vault reconstruction, with no increase in morbidity and a shorter length of stay.

The course of the inferior alveolar nerve in the normal human mandibular ramus and in patients presenting for cosmetic reduction of the mandibular angles.

This study was undertaken to quantify the path of the inferior alveolar nerve in the normal human mandible and in the mandibles of patients presenting for cosmetic reduction of the mandibular angles. The goals were: (1) to provide normative information that would assist the surgeon in avoiding injury to the nerve during surgery; (2) to characterize gender differences in the normal population; and (3) to compare the course of the nerve in the normal population to its course in a group of patients who presented with a complaint of “square face.” The study was based upon the computerized tomographic scans of 10 normal patients (six men, four women) and 8 patients (all women) complaining of “square face.” Using AnalyzePC 2.5 imaging software, the mandibles were segmented and the position of the nerve was recorded within its osseous canal in the mandibular ramus on each axial slice in which it was identifiable. Distances were calculated between the nerve and the anterior, posterior, lateral, and medial cortices. The positions of the lateral ramus prominence and the lowest point on the sigmoid notch were also recorded. The position of the mental foramen was recorded in relation to the nearest tooth, and the three-dimensional surface distances from the foramen to the alveolar bone, the inferior border of the mandible, and the mandibular symphysis were determined. The distances from the entrance of the nerve into the mandible to the lateral ramus prominence and the lowest point on the sigmoid notch were calculated. Summary statistics were obtained, comparing differences in gender. The nerve was identifiable in each ramus over a mean distance of 12.7 mm. On average, the lateral ramus prominence was 0.3 mm higher on the caudad-cephalad axis than the point at which the nerve entered the bone, whereas the location of the lowest point on the sigmoid notch was 16.6 mm above the nerve. The average distances from the nerve to the anterior, posterior, medial, and lateral cortices were 11.6, 12.1, 1.8, and 4.7 mm, respectively. Gender differences were significant for all of these except the medial cortex to nerve distance. On average, the mental foramen exited the body of the mandible immediately below the second premolar and the average surface distances from the foramen to the symphysis, the most cephalad alveolar bone, and the inferior border of the body were 30.9, 14.2, and 19.3 mm, respectively. With regard to the patients presenting for mandibular angle reduction, there were a few statistically significant but small scalar differences from normal controls.