Secondary Coronal Synostosis After Early Surgery for Sagittal Craniosynostosis: Implications for Cranial Growth.

ABSTRACT: Secondary Coronal Synostosis (SCS) in patients operated for non-syndromic Sagittal Craniosynostosis is a postoperative phenomenon with unclear implications. The aim of this study was to investigate whether SCS is a negative or a benign occurrence in the postoperative course. The authors hypothesized that SCS is related to reduced cranial growth and intracranial hypertension. Thirty-one patients operated for SC at an early age with the H-craniectomy technique were included in the study. Associations between SCS and cranial shape, growth, and signs of intracranial hypertension were analyzed. Intracranial volume distribution was assessed by measuring partial intracranial volumes defined by skull base landmarks. A total of 12/31 patients developed SCS during the postoperative course. The presence of SCS was associated with a higher prevalence of gyral impressions and a larger normalization of Cranial Index due to less growth in the anteroposterior plane. The SCS group had a smaller postoperative intracranial volume due to less posterior intracranial volume as well as less growth in head circumference. Whether this is a growth restriction caused by the SCS or a secondary effect of less primary brain growth remains to be determined. However, the correlation between SCS, less cranial growth and gyral impressions does imply that SCS should be taken into consideration during clinical follow-up as a potentially adverse event.

Orbital shape in intentional skull deformations and adult sagittal craniosynostoses.

Intentional cranial deformations are the result of external mechanical forces exerted on the skull vault that modify the morphology of various craniofacial structures such as the skull base, the orbits and the zygoma. In this controlled study, we investigated the 3D shape of the orbital inner mould and the orbital volume in various types of intentional deformations and in adult non-operated scaphocephaly - the most common type of craniosynostosis - using dedicated morphometric methods. CT scans were performed on 32 adult skulls with intentional deformations, 21 adult skull with scaphocephaly and 17 non-deformed adult skulls from the collections of the Muséum national d'Histoire naturelle in Paris, France. The intentional deformations group included six skulls with Toulouse deformations, eight skulls with circumferential deformations and 18 skulls with antero-posterior deformations. Mean shape models were generated based on a semi-automatic segmentation technique. Orbits were then aligned and compared qualitatively and quantitatively using colour-coded distance maps and by computing the mean absolute distance, the Hausdorff distance, and the Dice similarity coefficient. Orbital symmetry was assessed after mirroring, superimposition and Dice similarity coefficient computation. We showed that orbital shapes were significantly and symmetrically modified in intentional deformations and scaphocephaly compared with non-deformed control skulls. Antero-posterior and circumferential deformations demonstrated a similar and severe orbital deformation pattern resulting in significant smaller orbital volumes. Scaphocephaly and Toulouse deformations had similar deformation patterns but had no effect on orbital volumes. This study showed that intentional deformations and scaphocephaly significantly interact with orbital growth. Our approach was nevertheless not sufficient to identify specific modifications caused by the different types of skull deformations or by scaphocephaly.

Comparison of 2D radiography and a semi-automatic CT-based 3D method for measuring change in dorsal angulation over time in distal radius fractures.

OBJECTIVE: The aim of the present study was to compare the reliability and agreement between a computer tomography-based method (CT) and digitalised 2D radiographs (XR) when measuring change in dorsal angulation over time in distal radius fractures. MATERIALS AND METHODS: Radiographs from 33 distal radius fractures treated with external fixation were retrospectively analysed. All fractures had been examined using both XR and CT at six times over 6 months postoperatively. The changes in dorsal angulation between the first reference images and the following examinations in every patient were calculated from 133 follow-up measurements by two assessors and repeated at two different time points. The measurements were analysed using Bland-Altman plots, comparing intra- and inter-observer agreement within and between XR and CT. RESULTS: The mean differences in intra- and inter-observer measurements for XR, CT, and between XR and CT were close to zero, implying equal validity. The average intra- and inter-observer limits of agreement for XR, CT, and between XR and CT were ± 4.4°, ± 1.9° and ± 6.8° respectively. CONCLUSIONS: For scientific purpose, the reliability of XR seems unacceptably low when measuring changes in dorsal angulation in distal radius fractures, whereas the reliability for the semi-automatic CT-based method was higher and is therefore preferable when a more precise method is requested.

Three-Dimensional Eyeball and Orbit Volume Modification After LeFort III Midface Distraction.

The aim of our study was to evaluate orbital volume modification with LeFort III midface distraction in patients with craniosynostosis and its influence on eyeball volume and axial diameter modification. Orbital volume was assessed by the semiautomatic segmentation method based on deformable surface models and on 3-dimensional (3D) interaction with haptics. The eyeball volumes and diameters were automatically calculated after manual segmentation of computed tomographic scans with 3D slicer software. The mean, minimal, and maximal differences as well as the standard deviation and intraclass correlation coefficient (ICC) for intraobserver and interobserver measurements reliability were calculated. The Wilcoxon signed rank test was used to compare measured values before and after surgery. P < 0.05 was considered statistically significant. Intraobserver and interobserver ICC for haptic-aided semiautomatic orbital volume measurements were 0.98 and 0.99, respectively. The intraobserver and interobserver ICC values for manual segmentation of the eyeball volume were 0.87 and 0.86, respectively. The orbital volume increased significantly after surgery: 30.32% (mean, 5.96  mL) for the left orbit and 31.04% (mean, 6.31  mL) for the right orbit. The mean increase in eyeball volume was 12.3%. The mean increases in the eyeball axial dimensions were 7.3%, 9.3%, and 4.4% for the X-, Y-, and Z-axes, respectively. The Wilcoxon signed rank test showed that preoperative and postoperative eyeball volumes, as well as the diameters along the X- and Y-axes, were statistically significant. Midface distraction in patients with syndromic craniostenosis results in a significant increase (P < 0.05) in the orbit and eyeball volumes. The 2 methods (haptic-aided semiautomatic segmentation and manual 3D slicer segmentation) are reproducible techniques for orbit and eyeball volume measurements.

Springs Produce Favorable Morphological Outcomes Relative to H-craniectomy According to a Two-center Comparison of Matched Cases.

BACKGROUND: Sagittal synostosis is the most common type of premature suture closure, and many surgical techniques are used to correct scaphocephalic skull shape. Given the rarity of direct comparisons of different surgical techniques for correcting craniosynostosis, this study compared outcomes of craniotomy combined with springs and H-craniectomy for non-syndromic sagittal synostosis. METHODS: Comparisons were performed using available pre- and postoperative imaging and follow-up data from the two craniofacial national referral centers in Sweden, which perform two different surgical techniques: craniotomy combined with springs (Gothenburg) and H-craniectomy (Renier's technique; Uppsala). The study included 23 pairs of patients matched for sex, preoperative cephalic index (CI), and age. CI, total intracranial volume (ICV), and partial ICV were measured before surgery and at 3 years of age, with volume measurements compared against those of pre- and postoperative controls. Perioperative data included operation time, blood loss, volume of transfused blood, and length of hospital stay. RESULTS: Craniotomy combined with springs resulted in less bleeding and lower transfusion rates than H-craniectomy. Although the spring technique requires two operations, the mean total operation time was similar for both methods. Of the three complications that occurred in the group treated with springs, two were spring-related. Importantly, the compiled analysis of changes in CI and partial volume distribution revealed that craniotomy combined with springs resulted in superior morphological correction. CONCLUSIONS: The findings showed that craniotomy combined with springs normalized cranial morphology to a greater extent than H-craniectomy based on changes in CI and total and partial ICVs over time.

Persistent discrepancies in orbital morphology after surgical treatment of unicoronal craniosynostosis: a critical image-based analysis.

OBJECTIVE: Unicoronal craniosynostosis (UCS) is characterized by complex orbital deformity and is typically treated by asymmetrical fronto-orbital remodeling (FOR) during the 1st year of life. The aim of this study was to elucidate to what extent orbital morphology is corrected by surgical treatment. METHODS: The extent to which orbital morphology was corrected by surgical treatment was tested by analysis of differences in volume and shape between synostotic, nonsynostotic, and control orbits at two time points. In total, 147 orbits were analyzed from patient CT images obtained preoperatively (mean age 9.3 months), at follow-up (mean age 3.0 years), and in matched controls. Semiautomatic segmentation software was used to determine orbital volume. For analysis of orbital shape and asymmetry, geometrical models, signed distance maps, principal modes of variation, and three objective parameters (mean absolute distance, Hausdorff distance, and dice similarity coefficient) were generated by statistical shape modeling. RESULTS: Orbital volumes on both the synostotic and nonsynostotic sides were significantly smaller at follow-up than volumes in controls and significantly smaller both preoperatively and at follow-up than orbital volumes on the nonsynostotic side. Significant differences in shape were identified globally and locally, both preoperatively and at 3 years of age. Compared with controls, deviations were mostly found on the synostotic side at both time points. Asymmetry between synostotic and nonsynostotic sides was significantly decreased at follow-up, but not compared with the inherent asymmetry of controls. On a group level, the preoperative synostotic orbit was mainly expanded in the anterosuperior and anteroinferior regions and smallest on the temporal side. At follow-up, the mean synostotic orbit was still larger superiorly but also expanded in the anteroinferior temporal region. Overall, the morphology of nonsynostotic orbits was more similar to that of controls than to synostotic orbits. However, the individual variation in orbital shape was greatest for nonsynostotic orbits at follow-up. CONCLUSIONS: In this study, the authors presented what is, to their knowledge, the first objective automatic 3D bony evaluation of orbital shape in UCS, defining in greater detail than has been done previously how synostotic orbits differ from nonsynostotic and control orbits, and how orbital shape changes from 9.3 months of age preoperatively to 3 years of age at the postoperative follow-up. Despite surgical treatment, both local and global deviations in shape persist. These findings may have implications for future directions in the development of surgical treatment. Future studies connecting orbital morphology to ophthalmic disorders, aesthetics, and genetics could provide further insight to enable better outcomes in UCS.

Correcting Exorbitism by Monobloc Frontofacial Advancement in Crouzon-Pfeiffer Syndrome: An Age-Specific, Time-Related, Controlled Study.

BACKGROUND: In FGFR2 craniosynostosis, midfacial hypoplasia features oculo-orbital disproportion and symptomatic exorbitism. Clinical consequences may mandate surgery at a young age to prevent globe subluxation, corneal ulceration, and potential loss of vision. Monobloc osteotomy and distraction osteogenesis (monobloc distraction) seek to correct exorbitism. A report of the age-related impact of monobloc osteotomy and distraction osteogenesis on orbital volume, globe volume, and globe protrusion is presented. METHODS: Computed tomographic scan data from 28 Crouzon-Pfeiffer patients were assessed at preoperative, early postoperative, and 1-year follow-up time points. Orbital volumes, globe volumes, and globe protrusions were measured by manual and semiautomatic segmentation techniques, and these were compared to 40 age-matched controls. RESULTS: Crouzon-Pfeiffer syndrome orbital volumes are significantly small, and are significantly overexpanded by distraction to endpoints correcting symptomatic exorbitism. Globe volumes are significantly larger than controls under 5 years, do not independently correlate with globe protrusion, and are unaffected by surgery. Correlation between orbital volume expansion and reduction of globe protrusion is not significant. Age-related variations of postoperative growth potential occur to 1 year postoperatively. The Crouzon-Pfeiffer syndrome FGFR2 orbit exhibits early growth acceleration followed by premature growth arrest at 10 to 14 years. CONCLUSIONS: Orbital volume expansion by monobloc osteotomy and distraction osteogenesis is not the sole determinant of reduced globe protrusion. Mean volume relapse of the orbit at 1 year is insignificant across the series. Derived Crouzon-Pfeiffer growth curves suggest that "early functional monobloc" in infants occurs on a background of dynamic orbital growth, which remains programmed to a Crouzon-Pfeiffer FGFR2 phenotype and aligns with the incidence of delayed clinical regression and later secondary surgery. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Comparison analysis of orbital shape and volume in unilateral fractured orbits.

Facial fractures often result in changes of the orbital volume. These changes can be measured in three-dimensional (3D) computed tomography (CT) scans for preoperative planning and postoperative evaluation. The aim of this study was to analyze the orbital volume and shape before and after surgical treatment of unilateral orbital fractures using semi-automatic image segmentation and registration techniques. The orbital volume in 21 patients was assessed by a semi-automatic model-based segmentation method. The fractured orbit was compared relative to the contralateral orbit. The same procedure was performed for the postoperative evaluation. Two observers performed the segmentation procedure, and the inter- and intraobserver variability was evaluated. The interobserver variability (mean volume difference ± 1.96 SD) was -0.6 ± 1.0 ml in the first trial and 0.7 ± 0.8 ml in the second trial. The intra-observer variability was -0.2 ± 0.7 ml for the first observer and 1.1 ± 0.9 ml for the second observer. The average volume overlap (Dice similarity coefficient) between the fractured and contralateral side increased after surgery, while the mean and maximum surface distance decreased, indicating that the surgery contributed to a re-establishment of size and shape. In conclusion, our study shows that the semi-automatic segmentation method has precision for detecting volume differences down to 1.0 ml. The combination of semi-automatic segmentation and 3D shape analysis provides a powerful tool for planning and evaluating treatment of orbital fractures.

Orbital volume and shape in Treacher Collins syndrome.

Orbito-palpebral reconstruction is a challenge in Treacher Collins syndrome (TCS). This study investigates orbital phenotypes in TCS using cephalometry and orbital shape analysis. Eighteen TCS and 52 control patients were included in this study, using the Dr Warehouse database. Orbital cephalometry was based on 20 landmarks, 10 planes, 16 angles, and 22 distances. Orbits were segmented. Registration-based, age-specific mean models were generated using semi-automatic segmentation, and aligned and compared using color-coded distance maps - mean absolute distance (MAD), Hausdorff distance (HD), and Dice similarity coefficient (DSC). Symmetry was assessed by mirroring and DSC computing. Central orbital depth (COD) and medial orbital depth (MOD) allowed 100% of orbits to be classified. COD and lateral orbital depth (LOD) were different from the controls. Average MAD between TCS and controls was ≤1.5 mm, while for HD it was >1.5 mm, and for DSC <1. TCS orbits were more asymmetrical than controls, and orbital volumes were smaller when age was considered as a confounding factor, and had a trend for normalization with age. This report emphasizes the importance of combining different morphometric approaches in the phenotype characterization of non-trivial structures such as the orbit, and supports composite skeletal and soft-tissue strategies for the management of the peri-orbital region.

A new method for reconstructing brain morphology: applying the brain-neurocranial spatial relationship in an extant lungfish to a fossil endocast.

Lungfish first appeared in the geological record over 410 million years ago and are the closest living group of fish to the tetrapods. Palaeoneurological investigations into the group show that unlike numerous other fishes-but more similar to those in tetrapods-lungfish appear to have had a close fit between the brain and the cranial cavity that housed it. As such, researchers can use the endocast of fossil taxa (an internal cast of the cranial cavity) both as a source of morphological data but also to aid in developing functional and phylogenetic implications about the group. Using fossil endocast data from a three-dimensional-preserved Late Devonian lungfish from the Gogo Formation, Rhinodipterus, and the brain-neurocranial relationship in the extant Australian lungfish, Neoceratodus, we herein present the first virtually reconstructed brain of a fossil lungfish. Computed tomographic data and a newly developed 'brain-warping' method are used in conjunction with our own distance map software tool to both analyse and present the data. The brain reconstruction is adequate, but we envisage that its accuracy and wider application in other taxonomic groups will grow with increasing availability of tomographic datasets.

Fronto-facial advancement and bipartition in Crouzon-Pfeiffer and Apert syndromes: Impact of fronto-facial surgery upon orbital and airway parameters in FGFR2 syndromes.

A major concern in FGFR2 craniofaciosynostosis is oculo-orbital disproportion, such that orbital malformation provides poor accommodation and support for the orbital contents and peri-orbita, leading to insufficient eyelid closure, corneal exposure and eventually to functional visual impairment. Fronto-facial monobloc osteotomy followed by distraction osteogenesis aims to correct midfacial growth deficiencies in Crouzon-Pfeiffer syndrome patients. Fronto-facial bipartition osteotomy followed by distraction is a procedure of choice in Apert syndrome patients. These procedures modify the shape and volume of the orbit and tend to correct oculo-orbital disproportion. Little is known about the detailed 3D shape of the orbital phenotype in CPS and AS, and about how this is modified by fronto-facial surgery. Twenty-eight patients with CMS, 13 patients with AS and 40 control patients were included. CT scans were performed before and after fronto-facial surgery. Late post-operative scans were available for the Crouzon-Pfeiffer syndrome group. Orbital morphology was investigated using conventional three-dimensional cephalometry and shape analysis after mesh-based segmentation of the orbital contents. We characterized the 3D morphology of CPS and AS orbits and showed how orbital shape is modified by surgery. We showed that monobloc-distraction in CPS and bipartition-distraction in AS specifically address the morphological characteristics of the two syndromes.

Brain - Endocast Relationship in the Australian Lungfish, Neoceratodus forsteri, Elucidated from Tomographic Data (Sarcopterygii: Dipnoi).

Although the brains of the three extant lungfish genera have been previously described, the spatial relationship between the brain and the neurocranium has never before been fully described nor quantified. Through the application of virtual microtomography (µCT) and 3D rendering software, we describe aspects of the gross anatomy of the brain and labyrinth region in the Australian lungfish, Neoceratodus forsteri and compare this to previous accounts. Unexpected characters in this specimen include short olfactory peduncles connecting the olfactory bulbs to the telencephalon, and an oblong telencephalon. Furthermore, we illustrate the endocast (the mould of the internal space of the neurocranial cavity) of Neoceratodus, also describing and quantifying the brain-endocast relationship in a lungfish for the first time. Overall, the brain of the Australian lungfish closely matches the size and shape of the endocast cavity housing it, filling more than four fifths of the total volume. The forebrain and labyrinth regions of the brain correspond very well to the endocast morphology, while the midbrain and hindbrain do not fit so closely. Our results cast light on the gross neural and endocast anatomy in lungfishes, and are likely to have particular significance for palaeoneurologists studying fossil taxa.