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BACKGROUND: Plagiocephaly is defined as an asymmetrical distortion of the skull, resulting in an oblique trapezoid or parallelogram head shape. Deformational plagiocephaly (DP) is caused by forces acting on one side of the back of the head, distorting normal skull symmetry. OBJECTIVE: The aims of this systematic review and meta-analysis were to critically assess the evidence for nonobstetric risk factors for DP and to make evidence-based recommendations for reducing the prevalence of DP. METHODS: The selection criterion was studies reporting risk factors for DP. Case reviews, case series, expert opinions, and systematic reviews were excluded. PubMed and Web of Science were searched from August 21, 2010, to August 21, 2022. Publication bias was assessed using funnel plots. Meta-analyses were presented using forest plots. RESULTS: A total of 19 studies (cohort studies: n=13, 68%; case-control studies: n=5, 26%; and cross-sectional studies: n=1, 5%) with a total of 14,808 participants were included. Of the 43 investigated potential nonobstetric factors, 16 (37%) were associated with DP. Of these 16 factors, 12 (75%) had odds ratios (ORs) with 95% CIs not crossing 1: insufficient vitamin D intake (OR 7.15, 95% CI 3.77-13.54), head position preference (OR 4.75, 95% CI 3.36-6.73), bottle-only feeding (OR 4.65, 95% CI 2.70-8.00), reduced tummy time (OR 3.51, 95% CI 1.71-7.21), sleeping position (OR 3.12, 95% CI 2.21-4.39), fewer motor milestones reached by the age of 6 months (OR 2.56, 95% CI 1.66-3.96), obesity (OR 2.45, 95% CI 1.02-5.90), maternal education level (OR 1.66, 95% CI 1.17-2.37), male sex (OR 1.51, 95% CI 1.07-2.12), formula feeding (OR 1.51, 95% CI 1.00-2.27), head circumference (OR 1.22, 95% CI 1.06-1.40), and mechanical ventilation (OR 1.10, 95% CI 1.00-1.14). No evidence of publication bias was detected. CONCLUSIONS: This study provides a comprehensive assessment of the nonobstetric factors associated with DP and presents 11 evidence-based recommendations for reducing its prevalence. The primary limitation is that only publication bias was assessed. TRIAL REGISTRATION: PROSPERO CRD42020204979; https://www.crd.york.ac.uk/prospero/display_record.php? ID=CRD42020204979.

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Apert (AS), Crouzon (CS), Muenke (MS), Pfeiffer (PS), and Saethre Chotzen (SCS) are among the most frequently diagnosed syndromic craniosynostoses. The aims of this study were (1) to train an innovative model using artificial intelligence (AI)-based methods on two-dimensional facial frontal, lateral, and external ear photographs to assist diagnosis for syndromic craniosynostoses vs controls, and (2) to screen for genotype/phenotype correlations in AS, CS, and PS. We included retrospectively and prospectively, from 1979 to 2023, all frontal and lateral pictures of patients genetically diagnosed with AS, CS, MS, PS and SCS syndromes. After a deep learning-based preprocessing, we extracted geometric and textural features and used XGboost (eXtreme Gradient Boosting) to classify patients. The model was tested on an independent international validation set of genetically confirmed patients and non-syndromic controls. Between 1979 and 2023, we included 2228 frontal and lateral facial photographs corresponding to 541 patients. In all, 70.2% [0.593-0.797] (p < 0.001) of patients in the validation set were correctly diagnosed. Genotypes linked to a splice donor site of FGFR2 in Crouzon-Pfeiffer syndrome (CPS) caused a milder phenotype in CPS. Here we report a new method for the automatic detection of syndromic craniosynostoses using AI.

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BACKGROUND: Advancements in artificial intelligence and the development of shape models that quantify normal head shape and facial morphology provide frameworks by which the outcomes of craniofacial surgery can be compared. In this work, we will demonstrate the use of the Swap Disentangled Variational Autoencoder (SD-VAE) to objectively assess changes following midfacial surgery. MATERIALS AND METHODS: Our model is trained on a dataset of 1405 3D meshes of healthy and syndromic patients which was augmented using a technique based on spectral interpolation. Patients with a diagnosis of Apert and Crouzon syndrome who had undergone sub- or trans-cranial midfacial procedures utilising rigid external distraction were then interpreted using this model as the point of comparison. RESULTS: A total of 56 patients met our inclusion criteria, 20 with Apert and 36 with Crouzon syndrome. By using linear discriminant analysis to project the high-dimensional vectors derived by SD-VAE onto a 2D space, the shape properties of Apert and Crouzon syndrome can be visualised in relation to the healthy population. In this way, we are able to show how surgery elicits global shape changes in each patient. To assess the regional movements achieved during surgery, we use a novel metric derived from the Malahanobis distance to quantify movements through the latent space. CONCLUSION: Objective outcome evaluation, which encourages in-depth analysis and enhances decision making, is essential for the progression of surgical practice. We have demonstrated how artificial intelligence has the ability to improve our understanding of surgery and its effect on craniofacial morphology.

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BACKGROUND: Endoscopic strip craniectomy followed by helmet therapy (ESCH) is a minimally invasive approach for correcting sagittal craniosynostosis. The treatment involves a patient-specific helmet designed to facilitate lateral growth while constraining sagittal expansion. In this study, finite element modelling was used to predict post-treatment head reshaping, improving our comprehension of the necessary helmet therapy duration. METHOD: Six patients (aged 11 weeks to 9 months) who underwent ESCH at Connecticut Children's Hospital were enrolled in this study. Day-1 post-operative 3D scans were used to create skin, skull, and intracranial volume models. Patient-specific helmet models, incorporating areas for growth, were designed based on post-operative imaging. Brain growth was simulated through thermal expansion, and treatments were modelled according to post-operative Imaging available. Mechanical testing and finite element modelling were combined to determine patient-specific mechanical properties from bone samples collected from surgery. Validation compared simulated end-of-treatment skin surfaces with optical scans in terms of shape matching and cranial index estimation. RESULTS: Comparison between the simulated post-treatment head shape and optical scans showed that on average 97.3 ± 2.1 % of surface data points were within a distance range of -3 to 3 mm. The cranial index was also accurately predicted (r = 0.91). CONCLUSIONS: In conclusion, finite element models effectively predicted the ESCH cranial remodeling outcomes up to 8 months postoperatively. This computational tool offers valuable insights to guide and refine helmet treatment duration. This study also incorporated patient-specific material properties, enhancing the accuracy of the modeling approach.

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OBJECTIVE: Outcomes of surgical repair of trigonocephaly are well reported in the literature, but there is a paucity of information on the natural history of unoperated children. The authors evaluated a group of unoperated children with metopic synostosis to describe the natural change in head shape over time. METHODS: A database was screened for scans of children with unoperated trigonocephaly (2010-2021). Multisuture cases and those with a metopic ridge were excluded. Three-dimensional surface scans (3D stereophotogrammetry/CT) were used for morphological analysis. Nine previously published parameters were used: frontal angle (FA30°), anteroposterior (AP) volume ratio (APVR), AP area ratio (APAR), AP width ratios 1 and 2 (APWR1 and APWR2), and 4 AP diagonal ratios (30° right APDR [rAPDR30], 30° left APDR [lAPDR30], 60° right APDR [rAPDR60], and 60° left APDR [lAPDR60]). RESULTS: Ninety-seven scans were identified from a cohort of 316 patients with a single metopic suture, in which the male-to-female ratio was 2.7:1. Ages at the time of the scan ranged from 9 days to 11 years and were stratified into 4 groups: group 1, < 6 months; group 2, 6-12 months; group 3, 1-3 years; and group 4, > 3 years. Significant improvements were detected in 5 parameters (APVR, APAR, APWR1, rAPDR30, and lAPDR30) over time, whereas no significant differences were found in FA30, APWR2, rAPDR60, and lAPDR60 between age groups. CONCLUSIONS: Forehead shape (surface area and volume), as well as narrowing and anterolateral contour at the frontal points, differed significantly over time without surgery. However, forehead angulation, narrowing, and anterolateral contour at temporal points did not show significant differences. This knowledge will aid in surgical and parental decision-making.

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Spring-assisted posterior vault expansion has been adopted at the London Great Ormond Street Hospital for Children to treat raised intracranial pressure in patients affected by syndromic craniosynostosis, a congenital calvarial anomaly causing the premature fusion of skull sutures. This procedure involves elastic distractors used to dynamically reshape the skull and increase the intracranial volume (ICV). In this study, we developed and validated a patient-specific model able to predict the ICV increase and carried out a parametric study to investigate the effect of surgical parameters on that final volume. Pre- and post-operative computed tomography data relative to 18 patients were processed to extract simplified patient-specific skull shape, replicate surgical cuts, and simulate spring expansion. A parametric study was performed to quantify each parameter's impact on the surgical outcome: for each patient, the osteotomy location was varied in a pre-defined range; local sensitivity of the predicted ICV to each parameter was analysed and compared. Results showed that the finite element model performed well in terms of post-operative ICV prediction and allowed for parametric optimization of surgical cuts. The study indicates how to optimize the ICV increase according to the type of procedure and provides indication on the most robust surgical strategy.

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BACKGROUND: The four different local therapy strategies used for head and neck rhabdomyosarcoma (HNRMS) include proton therapy (PT), photon therapy (RT), surgery with radiotherapy (Paris-method), and surgery with brachytherapy (AMORE). Local control and survival is comparable; however, the impact of these different treatments on facial deformation is still poorly understood. This study aims to quantify facial deformation and investigates the differences in facial deformation between treatment modalities. METHODS: Across four European and North American institutions, HNRMS survivors treated between 1990 and 2017, more than 2 years post treatment, had a 3D photograph taken. Using dense surface modeling, we computed facial signatures for each survivor to show facial deformation relative to 35 age-sex-ethnicity-matched controls. Additionally, we computed individual facial asymmetry. FINDINGS: A total of 173 HNRMS survivors were included, survivors showed significantly reduced facial growth (p < .001) compared to healthy controls. Partitioned by tumor site, there was reduced facial growth in survivors with nonparameningeal primaries (p = .002), and parameningeal primaries (p ≤.001), but not for orbital primaries (p = .080) All patients were significantly more asymmetric than healthy controls, independent of treatment modality (p ≤ .001). There was significantly more facial deformation in orbital patients when comparing RT to AMORE (p = .046). In survivors with a parameningeal tumor, there was significantly less facial deformation in PT when compared to RT (p = .009) and Paris-method (p = .007). INTERPRETATION: When selecting optimal treatment, musculoskeletal facial outcomes are an expected difference between treatment options. These anticipated differences are currently based on clinicians' bias, expertise, and experience. These data supplement clinician judgment with an objective analysis highlighting the impact of patient age and tumor site between existing treatment options.

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LEVEL OF EVIDENCE: III.

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BACKGROUND: Endoscopic suturectomy with postoperative helmeting (ESCH) has emerged as a successful treatment for craniosynostosis, initially in North America. We report early outcomes from the first cohort of ESCH patients treated in the United Kingdom (UK). METHODS: Retrospective cohort study with electronic chart review. RESULTS: 18 consecutive patients from the first ESCH procedure in UK (May 2017) until January 2020 identified. 12 male and 6 female infantsd, with a mean age of 4.6 months (range 2.5-7.8 months) and weight of 6.8 kg (range 4.8-9.8 kg). Diagnoses were metopic (n = 8), unicoronal (n = 7), sagittal (n = 2) and multi-sutural (n = 1) synostoses. Median incision length was 3 cm (range 2-10 cm). 16/18 received no blood products, with 2 (both metopics) requiring transfusion (1 donor exposure). Mean operative time (including anaesthesia) was 96 min (range 40-127 min). Median length of hospital stay was 1 night. 1 surgical complication (superficial infection). All patients are currently undergoing helmet orthosis therapy. So far, no patients have required revisional or squint surgery. CONCLUSION: Early experience from the first UK cohort of ESCH suggests that this is a safe and well tolerated technique with low morbidity, transfusion and short hospital stay. Long-term results in terms of shape, cosmetic and developmental outcome are awaited.

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BACKGROUND: Endoscopic strip craniectomy with postoperative helmeting (ESCH) for unicoronal synostosis has shown to be a less morbid procedure when compared with fronto-orbital remodeling (FOR). We aim in this pilot study to report objective methods and quantitative morphologic outcomes of endoscopically treated unicoronal synostosis using 3-dimensional surface scans. METHODS: Our electronic records were reviewed for ophthalmological, neurodevelopmental outcomes, and helmet-related complications. For morphologic outcomes, the following parameters were used: Cranial Index, Cranial Vault Asymmetry Index, Anterior Symmetry Ratio (ASR), and Root Mean Square between the normal and synostotic sides of the head. Three-dimensional stereophotogrammetry scans were evaluated at 3 time points preoperative, 6 months post-op, and at the end of the treatment, which was compared with age-matched scans of normal controls and FOR patients. Nonparametric tests were used for statistical analysis. RESULTS: None of the ESCH cases developed strabismus, major neurodevelopmental delay, or helmet complications. All morphologic parameters improved significantly at 6 months post-op except for the Cranial Vault Asymmetry Index. The ASR was the only parameter to change significantly between 6 months post-op and final scans. At end of helmet treatment, ASR and Root Mean Square differed significantly between the ESCH and both FOR and control groups. CONCLUSIONS: Endoscopic strip craniectomy with postoperative helmeting for single unicoronal synostosis had excellent clinical outcomes. Most of the improvement in head morphology occurred in the first 6 months of treatment. Despite the normalization of the overall head shape, there was residual asymmetry in the frontal and temporal regions of the head.

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OBJECTIVE: Sagittal craniosynostosis (SC) is the most commonly encountered form of craniosynostosis. Despite its relative frequency, there remains significant heterogeneity in both operative management and follow-up between centers and a relative paucity of long-term outcome data in the literature. At the authors' institution, families of children presenting with SC are offered the following options: 1) conservative management with ophthalmic surveillance, 2) minimally invasive surgery at < 6 months of age (spring-assisted cranioplasty [SAC]) or 3) calvarial vault remodeling at any age (CVR). The authors reviewed outcomes for all children presenting with SC during a 5-year period, regardless of the treatment received. METHODS: Consecutive children born between January 1, 2008, and December 31, 2012, presenting with SC were identified, and detailed chart reviews were undertaken. Demographic, surgical, perioperative, head shape, scar, and neurodevelopmental (behavioral, education, speech, and language) data were analyzed. The cohort was divided by type of surgery (none, SAC, or CVR) and by age at surgery (early, defined as ≤ 6 months; or late, defined as > 6 months) for comparison purposes. RESULTS: A total of 167 children were identified, 129 boys and 38 girls, with a median age at presentation of 5.0 (range 0.4-135) months. Three families opted for conservative management. Of the 164 children who underwent surgery, 83 underwent SAC, 76 underwent CVR, and 5 underwent a "hybrid" procedure (CVR with springs). At a median age of 7.0 (range 0.5-12.3) years, there was no significant difference in concerns regarding head shape, scar, or neurodevelopmental outcomes between the early and late intervention groups over all procedures performed, or between the early or late SAC and CVR cohorts. There were more head shape concerns in the SAC group than in the CVR group overall (25.7% vs 11.8%, respectively; p = 0.026), although most of these concerns were minor and did not require revision. CONCLUSIONS: In this cohort, regardless of operative intervention and timing of intervention, infants achieved similar neurodevelopmental outcomes. Minimally invasive surgery (SAC) appears to result in less complete correction of head shape than CVR, but this may be balanced by advantages in reduced operative time, hospitalization, and blood loss. SAC was equal to CVR in neuropsychological outcomes.

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OBJECTIVE: Endoscopic strip craniectomy with postoperative molding helmet therapy (ESC-H) and spring-assisted cranioplasty (SAC) are commonly used minimally invasive techniques for correction of nonsyndromic sagittal craniosynostosis, but it is unclear which, if either, is superior. Therefore, the authors undertook a systematic review to compare ESC-H with SAC for the surgical management of nonsyndromic single-suture sagittal craniosynostosis. METHODS: Studies were identified through a systematic and comprehensive search of four databases (Embase, MEDLINE, and two databases in the Cochrane Library). Databases were searched from inception until February 19, 2021. Pediatric patients undergoing either ESC-H or SAC for the management of nonsyndromic single-suture sagittal craniosynostosis were included. Systematic reviews and meta-analyses, single-patient case reports, mixed cohorts of nonsyndromic and syndromic patients, mixed cohorts of different craniosynostosis types, and studies in which no outcomes of interest were reported were excluded. Outcomes of interest included reoperations, blood transfusion, complications, postoperative intensive care unit (ICU) admission, operative time, estimated blood loss, length of hospital stay, and cephalic index. Pooled summary cohort characteristics were calculated for each outcome of interest. Methodological quality was assessed using the Newcastle-Ottawa Scale. The study was reported in accordance with the 2020 PRISMA statement. RESULTS: Twenty-two studies were eligible for inclusion in the review, including 1094 patients, of whom 605 (55.3%) underwent ESC-H and 489 (44.7%) underwent SAC for nonsyndromic sagittal craniosynostosis. There was no difference between the pooled estimates of the ESC-H and SAC groups for operative time, length of stay, estimated blood loss, and cephalic index. There was no difference between the groups for reoperation rate and complication rate. However, ESC-H was associated with a higher blood transfusion rate and higher postoperative ICU admission. CONCLUSIONS: The available literature does not demonstrate superiority of either ESC-H or SAC, and outcomes are broadly similar for the treatment of nonsyndromic sagittal craniosynostosis. However, the evidence is limited by single-center retrospective studies with low methodological quality. There is a need for international multicenter randomized controlled trials comparing both techniques to gain definitive and generalizable data.

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OBJECTIVE: Endoscopic strip craniectomy with postoperative helmet orthosis therapy (ESCH) has emerged as a less invasive alternative to fronto-orbital remodeling for correction of trigonocephaly. However, there is no standardized objective method for monitoring morphological changes following ESCH. Such a method should be reproducible and avoid the use of ionizing radiation and general anesthesia for diagnostic imaging. The authors analyzed a number of metrics measured using 3D stereophotogrammetry (3DSPG) following ESCH, an imaging alternative that is free of ionizing radiation and can be performed on awake children. METHODS: 3DSPG images obtained at two time points (perisurgical and 1-year follow-up [FU]) of children with metopic synostosis who had undergone ESCH were analyzed and compared to 3DSPG images of age-matched control children without craniofacial anomalies. In total, 9 parameters were measured, the frontal angle and anteroposterior volume in addition to 7 novel parameters: anteroposterior area ratio, anteroposterior width ratios 1 and 2, and right and left anteroposterior diagonal ratios 30 and 60. RESULTS: Six eligible patients were identified in the operated group, and 15 children were in the control group. All 9 parameters differed significantly between perisurgical and age-matched controls, as well as from perisurgical to FU scans. Comparison of FU scans of metopic synostosis patients who underwent surgery to scans of age-matched controls without metopic synostosis revealed that all parameters were statistically identical, with the exception of the right anteroposterior diagonal ratio 30, which was not fully corrected in the treated patients. The left anterior part of the head showed the most change in surface area maps. CONCLUSIONS: In this pilot study, ESCH showed satisfactory results at 1 year, with improvements in all measured parameters compared to perisurgical results and normalization of 8 of 9 parameters compared to an age-matched control group. The results indicate that these parameters may be useful for craniofacial units for monitoring changes in head shape after ESCH for trigonocephaly and that 3DSPG, which avoids the use of anesthesia and ionizing radiation, is a satisfactory monitoring method.

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Temporal indentations are the most impacting craniofacial complication after coronal flap dissection. It is mainly due to a temporal fat pad or temporalis muscle dissection. Because of the great improvements achieved recently in CAD-CAM-aided surgery and the possibility of performing accurate pre-surgical virtual planning, it is now possible to correct it with a customised virtual approach. Furthermore, advancements in material science have allowed surgeons to rely on biocompatible materials like PEEK (showing a low complication and recurrence rate) for the manufacturing of patient-specific implants. We hereby describe our experience on a case of secondary and corrective surgery after a fronto-orbital remodelling, in which we used PEEK implants designed by CAD and optimized by finite element modelling.

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Limited information is available on the effect of sagittal craniosynostosis (CS) on morphological and material properties of the parietal bone. Understanding these properties would not only provide an insight into bone response to surgical procedures but also improve the accuracy of computational models simulating these surgeries. The aim of the present study was to characterise the mechanical and microstructural properties of the cortical table and diploe in parietal bone of patients affected by sagittal CS. Twelve samples were collected from pediatric patients (11 males, and 1 female; age 5.2 ± 1.3 months) surgically treated for sagittal CS. Samples were imaged using micro-computed tomography (micro-CT); and mechanical properties were extracted by means of micro-CT based finite element modelling (micro-FE) of three-point bending test, calibrated using sample-specific experimental data. Reference point indentation (RPI) was used to validate the micro-FE output. Bone samples were classified based on their macrostructure as unilaminar or trilaminar (sandwich) structure. The elastic moduli obtained using RPI and micro-FE approaches for cortical tables (ERPI 3973.33 ± 268.45 MPa and Emicro-FE 3438.11 ± 387.38 MPa) in the sandwich structure and diploe (ERPI1958.17 ± 563.79 MPa and Emicro-FE 1960.66 ± 492.44 MPa) in unilaminar samples were in strong agreement (r = 0.86, p < .01). We found that the elastic modulus of cortical tables and diploe were correlated with bone mineral density. Changes in the microstructure and mechanical properties of bone specimens were found to be irrespective of patients' age. Although younger patients are reported to benefit more from surgical intervention as skull is more malleable, understanding the material properties is critical to better predict the surgical outcome in patients <1 year old since age-related changes were minimal.

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PURPOSE: Children affected by premature fusion of the cranial sutures due to craniosynostosis can present with raised intracranial pressure and (turri)brachycephalic head shapes that require surgical treatment. Spring-assisted posterior vault expansion (SA-PVE) is the surgical technique of choice at Great Ormond Street Hospital for Children (GOSH), London, UK. This study aims to report the SA-PVE clinical experience of GOSH to date. METHODS: A retrospective review was carried out including all SA-PVE cases performed at GOSH between 2008 and 2020. Demographic and clinical data were recorded including genetic diagnosis, craniofacial surgical history, surgical indication and assessment, age at time of surgery (spring insertion and removal), operative time, in-patient stay, blood transfusion requirements, additional/secondary (cranio)facial procedures, and complications. RESULTS: Between 2008 and 2020, 200 SA-PVEs were undertaken in 184 patients (61% male). The study population consisted of patients affected by syndromic (65%) and non-syndromic disorders. Concerns regarding raised intracranial pressure were the surgical driver in 75% of the cases, with the remainder operated for shape correction. Median age for SA-PVE was 19 months (range, 2-131). Average operative time for first SA-PVE was 150 min and 87 for spring removal. Median in-patient stay was 3 nights, and 88 patients received a mean of 204.4 ml of blood transfusion at time of spring insertion. A single SA-PVE sufficed in 156 patients (85%) to date (26 springs still in situ at time of this analysis); 16 patients underwent repeat SA-PVE, whilst 12 underwent rigid redo. A second SA-PVE was needed in significantly more cases when the first SA-PVE was performed before age 1 year. Complications occurred in 26 patients with a total of 32 events, including one death. Forty-one patients underwent fronto-orbital remodelling at spring removal and 22 required additional cranio(maxillo)facial procedures. CONCLUSIONS: Spring-assisted posterior vault expansion is a safe, efficient, and effective procedure based on our 12-year experience. Those that are treated early in life might require a repeat SA-PVE. Long-term follow-up is recommended as some would require additional craniomaxillofacial correction later in life.

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ABSTRACT: Patients with Apert syndrome experience midfacial hypoplasia, hypertelorism, and downslanting palpebral fissures which can be corrected by midfacial bipartition distraction with rigid external distraction device. Quantitative studies typically focus on quantifying rigid advancement and rotation postdistraction, but intrinsic shape changes of bone and soft tissue remain unknown. This study presents a method to quantify these changes. Pre- and post-operative computed tomography scans from patients with Apert syndrome undergoing midfacial bipartition distraction with rigid external distraction device were collected. Digital Imaging and Communications in Medicine files were converted to three-dimensional bone and soft tissue reconstructions. Postoperative reconstructions were aligned on the preoperative maxilla, followed by nonrigid iterative closest point transformation to determine local shape changes. Anatomical point-to-point displacements were calculated and visualized using a heatmap and arrow map. Nine patients were included.Zygomatic arches and frontal bone demonstrated the largest changes. Mid-lateral to supra-orbital rim showed an upward, inward motion. Mean bone displacements ranged from 3.3 to 12.8 mm. Soft tissue displacements were relatively smaller, with greatest changes at the lateral canthi. Midfacial bipartition distraction with rigid external distraction device results in upward, inward rotation of the orbits, upward rotation of the zygomatic arch, and relative posterior motion of the frontal bone. Local movements were successfully quantified using a novel method, which can be applied to other surgical techniques/syndromes.

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BACKGROUND: Spring-Assisted Posterior Vault Expansion has been adopted at Great Ormond Street Hospital for Children, London, UK to treat raised intracranial pressure in patients affected by syndromic craniosynostosis, a congenital calvarial anomaly which causes premature fusion of skull sutures. This procedure aims at normalising head shape and augmenting intracranial volume by means of metallic springs which expand the back portion of the skull. The aim of this study is to create and validate a 3D numerical model able to predict the outcome of spring cranioplasty in patients affected by syndromic craniosynostosis, suitable for clinical adoption for preoperative surgical planning. METHODS: Retrospective spring expansion measurements retrieved from x-ray images of 50 patients were used to tune the skull viscoelastic properties for syndromic cases. Pre-operative computed tomography (CT) data relative to 14 patients were processed to extract patient-specific skull shape, replicate surgical cuts and simulate spring insertion. For each patient, the predicted finite element post-operative skull shape model was compared with the respective post-operative 3D CT data. FINDINGS: The comparison of the sagittal and transverse cross-sections of the simulated end-of-expansion calvaria and the post-operative skull shapes extracted from CT images showed a good shape matching for the whole population. The finite element model compared well in terms of post-operative intracranial volume prediction (R2 = 0.92, p < 0.0001). INTERPRETATION: These preliminary results show that Finite Element Modelling has great potential for outcome prediction of spring assisted posterior vault expansion. Further optimisation will make it suitable for clinical deployment.

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Monobloc and bipartition advancement by external distraction plays a major role in the treatment of syndromic craniosynostosis. They can reverse the associated facial deformity and play a role in the management of ocular exposure, intracranial hypertension, and upper airway obstruction. Facial bipartition distraction corrects the intrinsic facial deformities of Apert syndrome. Both procedures are associated with relatively high complication rates principally related to ascending infection and persistent cerebrospinal fluid leaks. Modern perioperative management has resulted in a significant decline in complications. External distractors allow fine tuning of distraction vectors and improve outcome but are less well tolerated than internal distractors.

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INTRODUCTION: Children with Apert syndrome have hypertelorism and midfacial hypoplasia, which can be treated with facial bipartition (FB), often aided by rigid external distraction. The technique involves a midline osteotomy that lateralizes the maxillary segments, resulting in posterior cross-bites and midline diastema. Varying degrees of spontaneous realignment of the dental arches occurs postoperatively. This study aims to quantify these movements and assess whether they occur as part of a wider skeletal relapse or as dental compensation. METHODS: Patients who underwent FB and had high quality computed tomography scans at the preoperative stage, immediately postsurgery, and later postoperatively were reviewed. DICOM files were converted to three-dimensional bone meshes and anatomical point-to-point displacements were quantified using nonrigid iterative closest point registration. Displacements were visualized using arrow maps, thereby providing an overview of the movements of the facial skeleton and dentition. RESULTS: Five patients with Apert syndrome were included. In all cases, the arrow maps demonstrated initial significant anterior movement of the frontofacial segment coupled with medial rotation of the orbits and transverse divergence of the maxillary arches. The bony position following initial surgery was shown to be largely stable, with primary dentoalveolar relapse correcting the dental alignment. CONCLUSIONS: This study showed that spontaneous dental compensation occurs following FB without compromising the surgical result. It may be appropriate to delay active orthodontic for 6-months postoperatively until completion of this early compensatory phase.

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