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Introduction: Fronto-orbital remodelling for metopic synostosis is an extensive operation with substantial blood loss, particularly from emissary veins in the glabellar region. One possibility to reduce blood loss may be to stage dissection and cauterise anomalous emissary veins before dissecting in the subperiostal plane.  OBJECTIVE: The aim of the present study was to compare perioperative bleeding using a staged dissection in the glabellar region with the traditional subperiostal dissection technique during surgery for metopic synostosis., Methods: All consecutive patients operated for metopic synostosis with the new staged dissection technique (T2) were included. For comparison, the most recent equal number of cases operated with the traditional dissection technique (T1) were included. Age, sex, weight, surgical technique (spring or bone graft), perioperative blood loss, perioperative blood pressure, per- and postoperative blood transfusion, operation time and length of hospital stay were registered.  RESULTS: A total of 80 patients were included; 40 T1 and 40 T2, respectively. Perioperative blood loss was significantly reduced with the new staged dissection technique. Blood loss in absolute numbers was reduced from 160.0 (120-240) (median and (interquartile range)) ml to 150 (102.5-170.0) ml, p=0.028. Blood loss in relation to patient weight was reduced from 24.2 ml/kg (15.3-33.3) to 18.7 (16.6-23.1) ml/kg, p=0.024. As percentage of total blood volume, blood loss was reduced from 32.3 (20.3-32.3) % to 29.9 (18.4-30.8) %, p=0.024. No other variables differed significantly between the techniques.  CONCLUSION: In summary, staged dissection technique in the glabellar region, allowing control of the emissary veins, reduces perioperative blood loss during surgery for metopic synostosis.

Purpose: This study aims to investigate the anatomical features relating to nasofrontal and septum osteotomy during Le Fort Ⅲ osteotomy among patients with syndromic craniosynostosis, and to compare them with normal controls using computed tomographic cephalometry., Materials and Methods: A total of 63 subjects were included (syndromic craniosynostosis, n = 21; controls, n = 42). The craniosynostosis subjects were subdivided into group A (craniosynostosis patients without history of previous surgery, n = 8) and group B (craniosynostosis patients with history of previous surgery, n = 13). Cephalometric measurements based on computed tomographic images were analyzed., Results: The angle between the refencing plane (HP) of the anterior cranial base and the most inferior point of the sphenoid sinus via Nasion (∠HP-N-ISS) was 23.85 ± 3.10°, 21.15 ± 4.64° and 28.32 ± 2.92° for group A, group B and the control group, respectively. The angle between HP and the posterior nasal spine (PNS) via nasion (∠HP- N- PNS) was 42.63 ± 2.02°, 38.50 ± 4.84° and 47.68 ± 2.62° for group A, group B and the control group, respectively., Conclusion: This study characterized the surgically relevant anatomical features in nasofrontal osteotomy during Le Fort Ⅲ osteotomy. The safe range for osteotomy angle is significantly different between normal and syndromic craniosynostosis subjects. Awareness of this difference may prevent further complications., Competing Interests: Declaration of competing interest The authors declare no conflict of interests., (Copyright © 2024. Published by Elsevier Ltd.)

Purpose: Visual and motility disorders have been reported in cases of syndromic and non-syndromic craniosynostosis. However, to the best of our knowledge, no regional or local studies have addressed the prevalence of ocular manifestations in craniosynostosis among Middle Eastern populations. The purpose of this study was to describe the frequency and nature of ophthalmic abnormalities in Arab children with craniosynostosis and to evaluate these findings pre- and post-craniofacial surgery., Materials and Methods: A prospective cohort study of 31 children with craniosynostosis. Demographic information was gathered along with genetic results and ophthalmic evaluations, including cycloplegic refraction., Results: Age at presentation to the ophthalmology service ranged from 14 months to 8 years, with a median of 3 years. Sixteen patients were female (52%), and 24 patients had undergone a cycloplegic refraction. Of these, 20.8% had significant refractive errors; of those with refractive errors, 40% were myopic and 60% were hyperopic. Eight patients (25.8%) had papilledema, with significant improvement after craniofacial surgery. Five patients (16%) had visual acuity≤20/30 in at least one eye at baseline with improvements in follow-up visits., Conclusion: Craniosynostosis has a significant impact on visual function in the Arab population. Potentially correctable cases of visual impairment, such as those caused by amblyogenic refractive error and papilledema, are commonly encountered; therefore, timely referral and regular ophthalmic evaluation are recommended for these patients., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Purpose: This study aimed to analyze correlations among respiratory function, upper airway expansion, and the extent of midface advancement in syndromic craniosynostosis patients with obstructive sleep apnea., Materials and Methods: A retrospective study was conducted in 21 children with syndromic craniosynostosis who underwent Le Fort III osteotomy and distractive osteogenesis at the Department of Oral and Maxillofacial Surgery of Peking University International Hospital from October 2017 to December 2022. Computed tomography (CT) data of patients before surgery (T0), 3 months after surgery (T1), and 1 year after surgery (T2) were reviewed. Sleep apnea was evaluated using polysomnography at the corresponding postoperative times. Skeletal changes were evaluated by cephalometric measurements; airway morphology was evaluated by two-dimensional cross square and three-dimensional volume; and respiratory function was measured using the apnea-hypopnea index (AHI), mean oxygen saturation (SpO 2 ), minimum SpO 2 , and the 3% decline in the SpO 2 index. A paired t-test was used to evaluate changes before and after surgery. A P value of <0.05 was considered to indicate statistical significance. Pearson correlation analysis was used to determine correlations among the skeletal structure, airway morphology, and respiratory function., Results: Significant differences were noted between T0 and T1 in terms of cephalometry landmarks, airway volume, and cross-sectional area (P < 0.05) but not between T1 and T2 (P > 0.05). Similarly, significant differences were detected in AHI, average SpO 2 level, minimum SpO 2 level, and 3% oxygen hypoxia index between T0 and T1 but not between T1 and T2 (P > 0.05). The change in SN-PNS was significantly correlated with an improvement in AHI (P = 0.024) and 3% oxygen hypoxia index (P = 0.019), and the change in palatopharyngeal airway area(Ar B) was significantly correlated with an improvement in minimum SpO 2 (P = 0.018)., Conclusion: Le Fort III osteotomy and distraction are effective in enlarging the upper airway width and improving sleep apnea in syndromic craniosynostosis patients. Cephalometric changes in S-PNS and improvement in Ar B were correlated with long-term improvements in polysomnography outcomes., Competing Interests: Declaration of competing interest The authors declare that they have no conflicts of interest., (Copyright © 2024. Published by Elsevier Ltd.)

Craniosynostosis constitutes one of the most common congenital cranial malformations, affecting approximately 6/10,0000 live births. A genetic etiology has long been known for several forms of syndromic craniosynostosis, including pathogenic variants in TWIST1 and FGFR3 in children with Saethre-Chotzen and Muenke syndrome. Over the last decade, reports of genetic aberrations in TCF12 in children with craniosynostosis have emerged, in particular in cases with premature closure of the coronal suture(s). In this study, we, therefore, systematically reviewed the rapidly growing knowledge of TCF12-related coronal craniosynostosis, clearly illustrating its high degree of genotype and phenotype variability. With the two novel cases presented, at least 113 cases of TCF12-related coronal craniosynostosis have currently been reported. By pooling data from several prospectively collected undifferentiated craniosynostosis cohorts (n total  = 770), we estimate a prevalence of pathogenic TCF12 variants of at least 2%. Overall, pathogenic germline variants in TCF12 are relatively frequent in children with coronal craniosynostosis, accounting for ∼10-20% of TWIST1- and FGFR1/2/3-negative cases, with even higher rates for bicoronal and syndromic cases. Genetic counseling is recommended for all children with craniosynostosis, and involvement of the coronal suture(s) should precipitate TCF12 testing., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Favorable behavioral interactions are critical for academic and interpersonal success. An association between metopic synostosis and behavioral impairments has not been fully elucidated. Behavioral dysfunction in school-age children with surgically corrected metopic synostosis was evaluated using targeted testing to detect the most common behavioral abnormalities in this population., Methods: Parents of children 6 to 18 years of age with metopic synostosis completed the Conners Rating Scales, 3rd edition (Short Form) (Conners-3; attention-deficit/hyperactivity disorder), Social Responsiveness Scale, 2nd edition (SRS-2; autism spectrum disorder), Behavior Rating Inventory of Executive Function, 2nd edition (executive functioning), and Child's Behavioral Checklist (behavioral/emotional functioning). Children also completed neurocognitive testing. Multivariable regression was used to determine predictors of clinically significant behavioral impairments., Results: Sixty children were enrolled. Average age at surgery was 9.2 ± 7.9 months, with an average age at assessment of 10.3 ± 3.5 years. Nearly half of patients demonstrated symptoms associated with attention-deficit/hyperactivity disorder, demonstrated by reaching or exceeding borderline clinical levels for inattention and hyperactivity subscales of the Conners-3. Greater age at surgery was associated with worse executive function, measured by reaching or exceeding clinically significant levels of the executive function subscale of the Conners-3 ( P = 0.04) and subscales of the Behavior Rating Inventory of Executive Function, 2nd edition (Behavioral Regulator Index [ P = 0.05], Cognitive Regulatory Index [ P = 0.03], and Global Executive Composite [ P = 0.04])., Conclusions: Nearly half of patients with surgically corrected metopic synostosis reached borderline clinical scores for inattention and hyperactivity. Older age at surgery was associated with worse executive function. Prompt surgical correction of metopic synostosis may portend improved long-term emotional and behavioral function., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Introduction: Bibliometrics, a statistical method assessing the influence of scholarly works, was employed to analyze the evolution of endoscopic surgery for craniosynostosis., Methods: This comprehensive review followed PRISMA guidelines, sourcing data from PubMed, Embase, and Web of Science, focusing on clinical and surgical outcomes up to December 2023. We identified 1409 records, with 83 papers meeting inclusion criteria., Results: The majority of studies originated from the Americas (79%), predominantly from the United States (75.9%). The typical study design was retrospective cohort studies (62%), involving a total of 8,175 patients with median research duration of 8 years. Citation metrics indicated an average count of 38.9 per paper, with notable contributions from several key authors. A distinct increase in research was observed in recent years, particularly from 2011 onwards, peaking in 2022 and 2023. The top 10 most cited papers, largely emanating from the U.S., had a higher median patient number (103) compared to the overall median (53), suggesting their significant impact. These papers were primarily published in journals with higher impact factors and citation indicators. The most cited research was notably published in the Journal of Neurosurgery-Pediatrics., Conclusion: This analysis provides a comprehensive view of the field, highlighting the growing trend and clinical importance of endoscopic approaches in craniosynostosis, offering a valuable resource for future research and clinical practice., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Posterior cranial vault distraction osteogenesis (PCVDO) is a relatively new paradigm in the treatment of syndromic craniosynostosis, having first been introduced in 2009. PCVDO directly addresses the underdeveloped cranial vault and appears to allow for a larger increase in intracranial volume when compared to traditional techniques. Although reported as safe in the literature, critical appraisal is still required as PCVDO is a relatively uncommon procedure that may require greater numbers to detect true complication rates. The overall reported incidence of serious complications in PCVDO to date is low. This presentation highlights a rare case of sagittal sinus obstruction following posterior cranial vault distraction and raises questions as to the safest technical considerations when planning the operation., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Craniosynostosis (CRS) is a rare congenital cranial malformation in which 1 or more cranial or facial sutures are fused in utero or rapidly fused in early infancy. The cranial sutures separate the skull bone plates and enable rapid growth of the skull in the first 2 years of life, in which growth is largely dictated by growth of the brain. CRS is a rare disease that occurs in 1 in 2100 to 1 in 2500 births and may be either nonsyndromic (also referred to as isolated) or syndromic. In syndromic CRS, other birth defects are present next to the CRS. The distinction between nonsyndromic and syndromic manifestations is made on the basis of dysmorphologic evaluation and genetic evaluation. Owing to advances in genetic diagnostics, nonsyndromic patients are increasingly recognized as syndromic patients. CRS treatment is almost entirely surgical and is sometimes paired with postoperative helmet therapy for maintenance. Corrective procedures are complex, long, and associated with the risk of numerous complications, including heavy blood loss and its sequelae. Although surgery may restore a normal appearance, even in nonsyndromic patients, patients may experience persistent deficits in intellectual ability and cognitive function. The European Commission (EC) has prioritized rare diseases in recent horizon European research programs; indeed, collections or even individual samples may be extremely valuable for research., Methods and Results: Here, we present a study protocol in which the combined expertise of clinicians and researchers will be exploited to generate a biobank dedicated to CRS. The generation of the CRS biobank presented in this study will include the collection of different types of biological materials as well as advanced radiological images available to the scientific community., Conclusion: The activation of a CRS biobank will provide an opportunity to improve translational research on CRS and to share its benefits with the scientific community and patients and their families., (© 2024. The Author(s).)

Purpose: Anterior plagiocephaly ( AP), secondary to isolated frontosphenoidal synostosis (IFS), represents one of the rarest forms of craniosynostosis documented in medical literature. Therefore, there is a lack of comprehensive understanding regarding the long-term (> 2 years) postoperative outcomes of this minor suture synostosis., Methods: This study presents the long-term outcomes of two patients previously treated for IFS, detailing their clinical, imaging, and postoperative characteristics. Additionally, a systematic review was conducted following PRISMA criteria to summarize existing literature on the topic. The review specifically focuses on long-term aesthetic outcomes, reoperation rates, and complications following surgical intervention for IFS., Results: Four articles encompassing 12 patients were included in the systematic review. The review highlights clinical features, postoperative complications, reoperation rates, and long-term aesthetic outcomes. The mean age at surgery was 17.5 months (range 9.5-24 months), with an average age at diagnosis of 9.3 months (range 6-10 months). Fronto-orbital advancement was the predominant surgical approach described across all cases. The reoperation rate among patients was 16.6%, with only two cases reporting postoperative complications. In one of our cases, an intraoperative CT scan was utilized to illustrate real-time improvement in frontal bone alignment according to the surgical plan, which significantly contributed to positive long-term clinical outcomes observed during a follow-up period exceeding 24 months., Conclusion: IFS, is a rare minor suture synostosis, significantly affects the aesthetic appearance of the forehead and necessitates consideration in the management of AP cases. Surgical intervention has shown minimal reoperation rates and excellent long-term prognosis. These findings are reinforced by recent institutional data and a comprehensive systematic review of the current literature., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

This study combined qualitative and quantitative approaches to evaluate outcomes of isolated nonsyndromic unilateral lambdoid synostosis at the Children's Hospital of Philadelphia. Volumetric, linear, and angular analyses were performed on long-term postoperative and normal control scans. Preoperative and postoperative clinical photographs were evaluated for the presence of dysmorphic frontofacial features. Among 26 included patients, median age of surgery was 10 months and mean postoperative follow-up was 5.9 ± 5.7 years. Two (7.7%) patients underwent secondary cranial vault procedures. At most recent follow-up, 2 (7.7%) subjects reported intermittent headaches. Twenty (90.9%) of 22 patients were assigned Whitaker grade I. Among 9 subjects with long-term imaging at age 11.5 ± 5.3, posterior vault asymmetry, posterior fossa deflection angle, cranial base angle, and ear position asymmetry all remained greater than in the control group. Of subjects included in the frontofacial feature analysis (n = 10), 50% had resolution of all dysmorphic frontofacial features present preoperatively. Overall, most subjects who underwent PVR did not require revisional surgery and had good outcomes both aesthetically and functionally. Despite residual abnormalities in the cranial base and posterior vault, most had resolution of parietal bossing and facial scoliosis. Ear position asymmetry was the most common dysmorphic feature at long-term follow-up., (Copyright © 2024 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Background: In patients with craniosynostosis, the authors evaluated the diagnostic accuracy of fundoscopy and optical coherence tomography (OCT) to detect intracranial hypertension (ICH), the time course of retinal thickness after treatment of ICH, and the relationship between high hyperopia (HH) and fundoscopy/OCT scan findings., Methods: Patients with syndromic, multisuture, unicoronal, unilambdoid, or sagittal synostosis visiting the authors' national center were included in this longitudinal cohort study and formed a consecutive series. Retinal layers on OCT, OCT fundus images, and fundoscopy results were evaluated. ICH was scored according to presence of abnormal intracranial pressures, hydrocephalus, progressive cerebellar tonsillar herniation or fingerprinting, and growth arrest. Diagnostic accuracy of OCT, fundoscopy, and fundus image; the time course of retinal thickness after ICH; and interference of HH were analyzed using linear mixed models., Results: A total of 577 OCT scans in 307 patients were included. ICH was found in 7.2%. Combining total retinal thickness (TRT), OCT fundus imaging and fundoscopy resulted in a sensitivity of 76% and 81% specificity to detect signs of ICH. TRT was increased in patients who had had signs of ICH versus patients who had never had signs of ICH (β +44.9 µm in patients who had had ICH [95% CI, 9.0 to 80.8]; P = 0.01). TRT decreased to normal in the years after surgery (β -3.6 µm/yr [95% CI, -7.2 to -0.05]; P = 0.047). There were greater odds of having increased TRT in patients with HH (OR, 2.9 [95% CI, 1.1 to 7.6]; P = 0.03)., Conclusions: The correlation among TRT, OCT fundus image, fundoscopy, and particularly the combination of these measures with intracranial pressure surrogate markers is fair. Increased TRT in the presence of a clinical suspicion of ICH warrants further screening., Clinical Question/level of Evidence: Diagnostic, III., (Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Plastic Surgeons.)

Background: Soft tissue procedures are often crucial for normalizing the long-term facial appearance in patients with syndromic craniosynostosis, yet they are underrepresented in the literature and existing treatment algorithms. This study evaluated secondary soft tissue surgeries in relation to skeletal operations in patients with syndromic craniosynostosis., Methods: A retrospective review evaluated patients with syndromic craniosynostosis treated at a tertiary children's hospital from 2003 to 2023. Craniofacial skeletal and soft tissue procedures were assessed for timing and frequency. Skeletal surgeries included redo fronto-orbital advancements, monobloc advancement/distraction ± facial bipartition, and LeFort III or III/I advancement/distraction. Soft tissue surgeries encompassed oculoplastic procedures, scalp reconstruction, fat grafting, and septorhinoplasty., Results: Of 106 patients with syndromic craniosynostosis, 57 (57.8%) underwent ≥1 secondary skeletal operation, and 101 (95.3%) underwent ≥1 soft tissue procedures, averaging 3.7 ± 3.3 soft tissue procedures per patient. Patients who underwent secondary forehead advancement had significantly higher rates of subsequent lateral canthopexy (71.4% vs 25.6, P < 0.001), ptosis reconstruction (17.9% vs 7.7%, P = 0.025), and frontotemporal fat grafting (50.0% vs 15.4%, P < 0.001) than those who did not. Patients who underwent midface advancement surgery had significantly higher rates of subsequent lateral canthopexy (65.4% vs 11.1%, P < 0.001), medial canthopexy (7.7% vs 0.0%, P = 0.038), scalp reconstruction (36.5% vs 16.7%, P = 0.020), frontotemporal fat grafting (34.6% vs 14.8%, P = 0.018), malar fat grafting (11.5% vs 0.0%, P = 0.010), and septorhinoplasty (26.9% vs 1.9%, P < 0.001) than those who did not. Specifically, LeFort III or III/I advancement/distractions were significantly associated with higher rates of subsequent septorhinoplasties (33.3% vs 1.6%, P < 0.001). The mean follow-up was 10.2 ± 7.0 years., Conclusions: This study revealed an intricate interplay between skeletal and soft tissue surgery in patients with syndromic craniosynostosis. Exploring techniques to reduce the need for further corrective surgery and anticipating necessary secondary interventions may improve patient counseling and outcomes., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Dogs with a brachycephalic conformation often experience a collection of abnormalities related to their craniofacial conformation, which can lead to a variety of clinical signs such as stertor, exercise intolerance, respiratory distress, and gastrointestinal signs such as regurgitation, among others. This collection of abnormalities is termed brachycephalic obstructive airway syndrome (BOAS). With the rise in popularity of several brachycephalic breeds, veterinarians and veterinary surgery specialists are seeing these dogs with increasing frequency for surgical and medical treatment of these clinical signs, leading to an increased interest in developing surgical techniques for dogs with BOAS and evaluating objective methods of determining outcome after surgery. Advances in anesthetic management including standardized protocols and use of local nerve blocks to decrease opiate use may decrease postoperative complications. A variety of new or modified surgical techniques to manage hyperplastic soft palate and stenotic nares, among other BOAS components, have been developed and studied in recent years. Newer studies have also focused on risk factors for development of major complications in the postoperative period and on objective measurements that may help determine which patients will receive the most benefit from BOAS surgery. In this review, the newest studies focused on updates in anesthetic management, surgical techniques, and postoperative care will be discussed. Additionally, updated information on complication rates and outcomes for dogs undergoing surgical management of BOAS will be included., (© 2024 The Author(s). Veterinary Surgery published by Wiley Periodicals LLC on behalf of American College of Veterinary Surgeons.)

This study aimed to determine the cognitive profile of preschool children undergoing surgery to correct non-syndromic craniosynostosis, compare them with typically developing children, and analyze possible cognitive deficits in the most prevalent subtypes: sagittal and unicoronal. Thirty-one children aged 3 years to 5 years and 11 months with non-syndromic craniosynostosis (11 sagittal, 9 unicoronal, 4 metopic, 3 lambdoid, 4 multisutural) who underwent surgery were compared with thirty-one typically developing children. The Wechsler Preschool and Primary Scale of Intelligence-Third Edition (WPPSI-III) was used to assess cognitive function. Children with non-syndromic craniosynostosis scored below the typically developing children in the Verbal Intelligence Quotient (VIQ) and Full-Scale Intelligence Quotient (FISQ). When specific subtypes were compared, children with sagittal synostosis scored similarly to the typically developing children; in contrast, children with unicoronal synostosis had lower performance in the Processing Speed Quotient and FISQ. The proportion of participants scoring below one standard deviation on the VIQ, General Language Composite, and FISQ was greater in the non-syndromic craniosynostosis group. This study supports the finding that children with non-syndromic craniosynostosis, particularly those with unicoronal synostosis, have more cognitive difficulties than those with normal development. Assessing cognition at preschool age in children with non-syndromic craniosynostosis is important in order to detect difficulties before they become more apparent at school age.

The pathogenesis of craniosynostosis, characterized by the premature fusion of calvarial sutures, is multifaceted and often the result of an amalgamation of contributing factors. The current study seeks examine the possible contributors to craniosynostosis development and its surgical trends over time. A multicenter/national retrospective cohort study was conducted of patients who underwent surgical repair of craniosynostosis (n=11,279) between 2012 and 2021 identified in the American College of Surgeons National Surgical Quality Improvement Program Pediatric Data File. Main outcome measures included risk factors and trends relating to surgical repair of craniosynostosis. Nationwide reports of craniosynostosis in the NSQIP-P database have increased between 2012 and 2021 by 195%. The prevalence of craniosynostosis per overall cases has remained between 1.0% and 1.3%. There were predominantly more White male patients in the craniosynostosis cohort ( P <0.001). Craniosynostosis patients had significantly greater birth weights, gestational ages, and were less likely to be premature ( P <0.05). Linear regression demonstrated that operative time, anesthesia time, and length of stay significantly decreased over the study period ( P <0.001). This national data analysis highlights trends in craniosynostosis repair indicating potential improvements in safety and patient outcomes over time. While these findings offer insights for health care professionals, caution is warranted in extrapolating beyond the data's scope. Future research should focus on diverse patient populations, compare outcomes across institutions, and employ prospective study designs to enhance the evidence base for craniosynostosis management. These efforts will help refine diagnostic and treatment strategies, potentially leading to better outcomes for patients., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

This study aimed to provide an external validation of morphometric outcomes, including recently developed parameters - vertico-longitudinal index (VLI) and vertex-nasion-opisthocranion (VNO) angle - along with a proposed stratification system for nonsyndromic sagittal craniosynostosis (NSC). Thin-cut CT scans performed before and after total calvarial remodeling in 70 NSC children (mean preoperative age, 7.0 months; mean postoperative age 23.8 months) were evaluated. The parameters, including linear dimensions, morphometry-based indices, and cranial angles, were measured at the cranial vault and base. Each patient was also assigned a scaphocephaly severity score (SSS). The obtained data were compared with an age-matched control group of 80 normocephalic children. After surgery, all dimensions of the cranial vault increased, but did not normalize (p < 0.0001). Contrarily, some normalization was noted in the skull base, within the anterior and posterior cranial fossae. Postoperative results confirmed the diagnostic cut-off level for VNO at 50°; however, the proposed VLI cut-offs skewed the accuracy of SSS in the control group. To the best of our knowledge, this study was the first to discuss the value of an NSC stratification system in relation to underlying components. The SSS has a relevant background, but its accuracy would increase with a redefinition of normative VLI ranges., (Copyright © 2024 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Objective: Little is known about the response of the ventricular system to cranial vault surgery in patients with craniosynostosis. This study aims to evaluate the changes in the cerebral ventricular system in response to posterior vault distraction osteogenesis (PVDO) in patients with syndromic and nonsyndromic craniosynostosis., Methods: A single-institution retrospective review of all patients with craniosynostosis undergoing PVDO from 2000 to 2022 was completed. Patients were included for analysis if they had pre and postoperative cranial computed tomography scans. Ventricular volume (VV) and intracranial volume (ICV) were calculated using segmentation software., Results: Both patients with syndromic synostosis and nonsyndromic synostosis (NSS) experienced a significant increase in ICV after PVDO, but only patients with NSS experienced a significant VV change ( P = 0.004). After normalization by ICV, total, lateral, and third VV changes retained significance with percentage increases of 114%, 117%, and 89%, respectively ( P < 0.05 for all)., Conclusion: The differing results between cohorts reinforce the concept that the intracranial milieu is different between patients with syndromic synostosis and NSS. The results of the NSS cohort suggest that these patients may exist in a compensated state in which a reduction in cerebral blood flow and VV allows for the maintenance of parenchymal health to prevent the development of intracranial hypertension. Further studies may explore VV as a surrogate marker of ICP elevation, and the utility of cranial vault remodeling on nonsynostotic pathologies with cephalocranial disproportion., Competing Interests: J.A.T. is a co-founder of Ostiio LLC. J.W.S. has developed educational content for DePuy Synthes/Johnson & Johnson. The remaining authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

Cranial sutures are complex structures integrating mechanical forces with osteogenesis which are often affected in craniofacial syndromes. While premature fusion is frequently described, rare pathological widening of cranial sutures is a comparatively understudied phenomenon. This narrative review aims to bring to light the biologically variable underlying causes of widened sutures and persistent fontanelles leading to a common outcome. The authors herein present four syndromes, selected from a literature review, and their identified biological mechanisms in the context of altered suture physiology, exploring the roles of progenitor cell differentiation, extracellular matrix production, mineralization, and bone resorption. This article illustrates the gaps in understanding of complex craniofacial disorders, and the potential for further unification of genetics, cellular biology, and clinical pillars of health science research to improve treatment outcomes for patients., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

The elongated soft palate is an abnormality that characterizes most brachycephalic dogs and contributes to the brachycephalic obstructive airway syndrome (BOAS). Palatoplasty is routinely performed in brachycephalic dogs; several surgical techniques exist. The use of surgical instruments such as monopolar electrocoagulation, CO 2 or diode laser, bipolar vessel sealing device and harmonic shears has become routine to reduce the operating time, the intraoperative risk of bleeding and the postoperative oedema. This prospective study aimed to compare the histomorphological effect of a CO 2 laser and LigaSure device in palates of dogs undergoing palatoplasty. Twenty owned brachycephalic dogs were included, 10 palatoplasties were performed using CO 2 laser and 10 using LigaSure™ device. The dogs were positioned in sternal recumbency. A transoral approach was performed: the elongated soft palate was grasped with Allis forceps and brought rostrally, the palatoplasty was performed using the tonsillar crypts as anatomical landmarks. Surgical specimens were routinely fixed in 10 % formalin. Two sections perpendicular to the surgical margins were trimmed from each sample, paraffin-embedded and stained with hematoxylin and eosin (H&E). Tissue damage induced by the two types of surgical devices was graded (1-4, from minimal to severe) and the depth of thermal injury measured in μm on captured images (using an image analysis program - ImageJ). Mean values and standard deviations (SD) were calculated based on six measurements for each sample. The tissue damage was graded 3.7±0.48 in group LigaSure™ and 2.8±1 in group Laser. The mean depth of thermal injury was 874.94±184.92 μm in the LigaSure™ group and 451,76±137,86 μm in the Laser group. The comparison between the two groups showed significant lower grade and extension of thermal injury in the palate samples obtained with CO 2 laser (p<0.05). Additionally, there is a lack of literature that correlates the histological changes with the clinical outcomes of the different palatoplasty methods in brachycephalic dogs. By comparing histological changes and clinical outcomes, we aim to provide valuable insights for optimizing the surgical approach for palatoplasty in brachycephalic dogs, ultimately improving postoperative outcomes for these patients., Competing Interests: Declaration of Competing Interest None of the authors has any financial or personal relationships that could inappropriately influence or bias the content of the paper, (Copyright © 2024. Published by Elsevier Ltd.)

Background: Radiographic severity of metopic synostosis has been suggested as a predictor of long-term neurocognitive outcomes, and artificial intelligence (AI) has recently been used to quantify severity. Age at surgery is predictive of long-term neurocognition in sagittal synostosis but has not been adequately explored in metopic synostosis., Methods: Children ages 6 to 18 years with corrected metopic synostosis underwent testing of intelligence quotient, academic achievement, and visuomotor integration (VMI). Various manual measurements and AI-derived severity scores were determined. Scans were categorized as moderate or severe for head-to-head comparisons and multivariable linear regressions were used to assess the relationship of age at surgery and severity with neurocognitive outcomes., Results: A total of 41 patients with average age at testing of 10.8 ± 3.4 years were included. A total of 18 patients were in the severe group and 23 patients were in the moderate group, with average ages at surgery of 6.6 ± 2.7 and 10.6 ± 8.4 months, respectively ( P = 0.062). Greater AI-derived severity was significantly associated with lower reading comprehension ( P = 0.040 and 0.018) and reading composite scores ( P = 0.024 and P = 0.008). Older age at surgery was significantly associated with lower VMI scores ( P values ranging from 0.017 to 0.045) and reading composite scores ( P = 0.047 and 0.019)., Conclusions: This study suggests an association between greater AI-derived radiographic severity and lower reading ability in corrected metopic synostosis. Older age at surgery was independently associated with lower reading ability and VMI. Surgical correction may mitigate neurodevelopmental differences based on severity that have been observed preoperatively., Clinical Question/level of Evidence: Risk, II., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Background: This study evaluated spring-assisted posterior vault expansion (SA-PVE) in children aged > 2 years with craniosynostosis and signs of high intracranial pressure (ICP)., Methods: We retrospectively analysed all consecutive patients aged > 2 years and operated with SA-PVE between 2018 and 2020 at the Craniofacial Center at Sahlgrenska University Hospital, Sweden. During the procedure, a circumferent occipital bone flap extending below the torcula was created and remained attached to the dura. Intracranial volumes (ICVs) were calculated from computed tomography (CT) images, and demographic data and information regarding symptoms and signs of high ICP were collected., Results: The study included eight patients [Crouzon/Pfeiffer (n = 4), multiple craniosynostosis (n = 3), and secondary synostosis (n = 1)]. Median age at SA-PVE was 3.8 years (range: 2.5-12.8 years), and springs were removed after a median of 5.5 months (range: 2.3-8.3 months). The median operating time was 164 min (range: 102-221 min), and estimated blood loss was 4.5 mL/kg body weight (range: 1.4-59.1 mL/kg body weight), with 50% of patients receiving a blood transfusion. The median increase in ICV was 206 cm3 (range: 122-344 cm3) representing an 18.7% increase (range: 7.9-24.1%; p = 0.01). We observed no major perioperative complications, and symptoms related to high ICP were improved or absent at clinical follow-up., Conclusion: These results demonstrated that SA-PVE involving creation of a large occipital bone flap including the torcula as a safe and effective surgical treatment in children aged >2 years with craniosynostosis and elevated ICP.

Purpose: Children with surgically corrected nonsyndromic craniosynostosis have been previously found to have neurocognitive and behavioral difficulties. Children with metopic synostosis have been described to have more difficulties than children with sagittal synostosis. This study aims to characterize the behavioral differences between children with metopic and sagittal synostosis., Methods: Children with metopic and sagittal synostosis were recruited at school age. Parents completed four separated behavioral assessments: Conners-3 (evaluation of ADHD), Social Responsiveness Scale-2 (SRS-2: evaluation of autism), Behavior Rating Inventory of Executive Function-2 (BRIEF-2: evaluation of executive function), and Child Behavior Checklist (CBCL: evaluation of overall behavioral problems). Children underwent intelligence quotient (IQ) testing using the Wechsler Abbreviated Scale of Intelligence (WASI-II)., Results: There were 91 children (45 with metopic and 46 with sagittal synostosis). More children with metopic synostosis reported requiring supportive services (57.7% vs 34.7%, p = 0.02) and more reached or exceeded borderline clinical levels of two executive function subscales of the BRIEF-2 (emotion regulation index: 33.3% vs 17.4%, p = 0.05; global executive composite: 33.3% vs 17.4%, p = 0.05). Children with sagittal synostosis had higher scores on the rule-breaking and externalizing problem subscales of the CBCL. Increasing age at surgery was associated with worse executive function scores., Conclusions: A relationship between suture subtype and behavioral outcomes exists at school age. More children with metopic synostosis required social services indicating more overall difficulties. Children with metopic synostosis have more specific problems with executive function, while children with sagittal synostosis had more difficulties with externalizing behaviors., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The aim of this study was to compare a traditional fronto-orbital remodeling and advancement (FORA) with the dynamic cranioplasty for trigonocephaly (DCT). The authors analyzed patients who underwent surgery for trigonocephaly. Perioperative data were compared. Parents were asked to use a visual analog scale to evaluate the pre- and postoperative distance between the eyes, the forehead shape, and the global appearance of the face. A panel of observers was asked to grade pre- and postoperative photographs using a similar visual analog scale. Pre- and postoperative anthropometric data were collected and analyzed in a subset of the study population aged 9 years or older. The total sample size was 51 patients (DCT n = 39; FORA n = 12). Durations of surgery and anesthesia were shorter in the DCT group (115 vs 194 min, p = 0.001; 226 vs 289 min, p = 0.001). Patients in the DCT group received similar preoperative ratings to those in the FORA group, but significantly higher postoperative ratings by parents for all three questions. There were no significant differences in postoperative ratings by the panel or postoperative anthropometric data. DCT is safe and effective. It is preferred over FORA because it is associated with shorter durations of surgery and anesthesia, while providing higher degrees of parental satisfaction and similar aesthetic and anthropometric outcomes., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare in relation to the content of this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objective: To assess whether adding metoclopramide to a protocol of maropitant and pantoprazole would reduce incidence of ptyalism, vomiting and regurgitation in brachycephalic dogs undergoing thoracolumbar spinal surgery., Study Design: Randomized blinded controlled trial., Animals: A total of 43 brachycephalic dogs undergoing thoracolumbar spinal surgery., Methods: In addition to a standardized anaesthetic regimen, dogs were randomized to be administered either a 2 mg kg -1 day -1 metoclopramide constant rate infusion (CRI) or a saline solution at an equivalent infusion rate, started after anaesthetic induction and discontinued 5 hours after tracheal extubation. The presence of vomiting, regurgitation and pytalism, and short form of the Glasgow Composite Pain Scale pain scores were assessed by a blinded observer hourly for 4 hours, starting 1 hour postextubation., Results: Regurgitation occurred in six dogs postoperatively; three dogs were in the placebo group and three in the metoclopramide group. The odds ratio (OR) of regurgitation after surgery did not differ between groups [OR: 0.76, 95% confidence interval (CI): 0.13-4.3, p = 0.76]. The odds of observing ptyalism at 3 and 4 hours was approximately 15 times less than 1 hour postoperatively (both OR: 15.4, 95% CI: 1.8-130.7, p = 0.012) and did not differ based on the addition of metoclopramide (OR: 0.73, 95% CI: 0.07-8.0, p = 0.79). The odds of observing pain did not change over time and did not differ based on the addition of metoclopramide (OR: 0.71, 95% CI: 0.12-4.2, p = 0.71). Vomiting did not occur during the study (0.0%, 95% CI: 0.0-8.2%). No adverse effects were observed during the study period in either group., Conclusions and Clinical Relevance: The addition of a metoclopramide CRI to maropitant and pantoprazole did not result in a significant reduction in ptyalism or regurgitation in brachycephalic dogs undergoing thoracolumbar spinal surgery., (Copyright © 2024 Association of Veterinary Anaesthetists and American College of Veterinary Anesthesia and Analgesia. Published by Elsevier Ltd. All rights reserved.)

Background: Le Fort III distraction for syndromic craniosynostosis is performed using internal or external devices. The authors compared the results of both devices., Methods: The authors retrospectively evaluated 60 patients with syndromic craniosynostosis treated with Le Fort III distraction (internal or external device) between 2001 and 2021. The authors verified demographic data, surgery-related data, and complications using medical records. For each of the 2 devices, they compared the various factors associated with the device to each other., Results: For the external device, 32 patients with syndromic craniosynostosis were included. The mean age at surgery was 11.7 years, and the mean elongation length was 20.0 mm. Class III occlusion reoccurred in 11 patients and was significantly associated with age at surgery. Seven complications (device problems and others) were noted. Cranial pin slippage was significantly related to the elongation length. For the internal device, 28 patients with syndromic craniosynostosis were included. The mean age at surgery was 10.4 years, and the mean elongation length was 18.7 mm. There were 15 complications, including device problems, zygomaticomaxillary fractures, and infections. Elongation length was significantly related to these complications. Class III occlusion reoccurred in 9 patients and was significantly related to age at surgery., Conclusions: This study found that complications are significantly more likely to occur in internal devices than in external devices, especially device infection. The authors' findings identified several factors that may assist surgeons in selecting between external and internal devices. The relationship between the amount of extension and device-related problems found in this study will be beneficial for solving these problems., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Purpose: To investigate whether patients with craniosynostosis exhibit higher rates of nasolacrimal duct obstruction (NLDO) and to explore potential risk factors., Methods: Retrospective review including all craniosynostosis patients treated at both the Divisions of Ophthalmology and Plastic, Reconstructive, and Oral Surgery at The Children's Hospital of Philadelphia between 2009 and 2020 was conducted. Synostosis characteristics, lacrimal disorders, and genetic data were collected. Main outcome measures were the rate of NLDO and associations with anatomical and syndromic/genetic risk factors., Results: The total of 767 participants had a mean age of 2.8 ± 3.8 years, 465 (60.6%) were males, 485 (63.2%) had no syndromic association; 631 (82.3%) had one major suture involved, 128 (17%) had involvement of 2 to 4 major sutures, and 429 (55.9%) underwent craniofacial surgery. Forty-eight (6.2%) patients had NLDO, which more prevalent in the genetic/syndromic group (11.0% vs. 3.5%, respectively, p < 0.001), with the highest prevalence observed in patients with Apert syndrome (n = 4, 30.8%). The genetic variants most associated with NLDO were EFNB1 (n = 1, 100%) and FGFR2 (n = 6, 19.4%). There was no association between NLDO and the number or types of sutures involved or a history of craniofacial surgery., Conclusions: Nasolacrimal duct obstruction is more common in patients with craniosynostosis compared to the general population. Having a putative syndrome or a putative genetic variant and female sex were risk factors for NLDO. Ophthalmic evaluations for all craniosynostosis patients and careful assessments of any symptoms of tearing are recommended., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Background: Flat head syndrome (FHS) sometimes occurs when a baby maintains the same head position during the first several months of life, causing a skull deformity. FHS usually improves with time and natural growth, although some show aggravation against conservative treatment. We reviewed pathologically proved early closure of skull suture that may be seen secondary to FHS., Methods: The clinical and radiologic findings of the patients who showed progressive skull deformity resembling FHS were retrospectively reviewed. All the patients underwent surgical treatment and pathologic specimens were obtained., Results: The detected patients included two 5-month-old infants and one 1-year-old child. The former were conservatively treated without any obvious premature suture closure on computed tomography (CT), and later developed progressive tower-like skull deformities. The infants were diagnosed with possible premature fusion of lambda site and underwent removal around lambda depression (LD). The latter showed evident sagittal suture closure on CT with digital markings, and was diagnosed with increased intracranial pressure and underwent cranioplasty of posterior expansion. Histopathologic specimens obtained from the patients' resected sutures showed irregularly narrowed suture structure with ossification and fibrous tissue proliferation within them, supporting the diagnosis of premature closure of the sagittal sutures. Their postoperative courses were uneventful, and their skull deformities subsequently improved., Conclusions: Conservative therapy-resistant progressive occipital skull deformity with LD may be a sign of early suture closure, even if CT does not show obvious suture closure. The findings are helpful for early diagnosis and might lead to minimal invasive surgery if needed., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Early surgical management of syndromic craniosynostosis varies widely between centers, with patients typically undergoing multiple intracranial procedures through childhood. This study evaluates the long-term impact of early posterior vault distraction osteogenesis (PVDO) versus conventional treatment paradigms on the number and timing of subsequent craniofacial procedures., Methods: We retrospectively analyzed the longitudinal operative patterns of patients with syndromic craniosynostosis treated from 2000 to 2023 with greater than five years of follow-up. Outcomes of patients who underwent early PVDO and conventional vault reconstruction were compared., Results: Fifty-five patients met the inclusion criteria (30 PVDO and 25 conventional). Age at initial vault surgery was similar between the PVDO and conventional cohorts (7.6 vs. 8.8 months), as were baseline craniometrics (p > 0.05). Multiple fronto-orbital advancement (FOA) procedures were performed in only 1/30 (3%) PVDO-treated patient versus 12/25 (48%) conventionally-treated patients (p < 0.001). Twelve (40%) PVDO-treated patients did not undergo FOA at all. Among patients with Apert and Crouzon syndromes, fewer PVDO-treated patients required FOA prior to midface surgery (33% vs. 92%, p = 0.004) or repeat FOA (6% vs. 50%, p = 0.005) compared to conventionally-treated patients. Among patients with Muenke and Saethre-Chotzen syndromes, a similar proportion of patients required FOA in the PVDO and conventional cohorts (91% vs. 100%, p = 0.353), though no PVDO-treated patients required repeat FOA (0% vs. 44%, p = 0.018)., Conclusions: Early PVDO is associated with a reduction in the high burden of both FOA and, critically, revisionary frontal procedures which are commonly seen in conventionally-treated patients with syndromic craniosynostosis., (© 2024. The Author(s).)

Background: Surgical correction of unicoronal craniosynostosis (UCS) is highly complex due to its asymmetric appearance. Although fronto-orbital advancement (FOA) is a versatile technique for craniosynostosis correction, harmonization of the orbital bandeau in UCS is difficult to predict. This study evaluates the biomechanics of the orbital bandeau using different patterns and varying characteristics of inner cortical bone layer osteotomies in a finite element (FE) analysis., Method: An FE model was created using the computed tomography (CT) scan of a 6.5-month-old male infant with a right-sided UCS. The unaffected side of the orbital bandeau was virtually mirrored, and anatomical correction of the orbital bandeau was simulated. Different combinations of osteotomy patterns, numbers, depths, and widths were examined (n = 48) and compared to an uncut model., Results: Reaction forces and maximum stress values differed significantly (p < 0.01) among osteotomy patterns and between each osteotomy characteristic. Regardless of the osteotomy pattern, higher numbers of osteotomies significantly (p < 0.05) correlated with reductions in reaction force and maximum stress. An X-shaped configuration with three osteotomies deep and wide to the bone was biomechanically the most favorable model., Conclusion: Inner cortical bone layer osteotomy might be an effective modification to the conventional FOA approach in terms of predictable shaping of the orbital bandeau., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

To determine the skeletal changes after midface surgery in patients with syndromic craniosynostosis who underwent Le Fort III (LFIII), monobloc (MB), or facial bipartition (FB). This was a retrospective study including 75 patients: 33 treated by LFIII, 29 by MB, and 13 by FB. Twenty-five had a diagnosis of Apert, 39 Crouzon, and 11 craniofrontonasal syndrome. A three-dimensional mesh was created from the preoperative scan and registered to the postoperative scan to visualise the advancement. LFIII at age 7-12 years effectuated a higher mean advancement in the maxillary (15.5 mm) and zygomatic (7.6 mm) regions when compared to ≥13 years (10.2 mm and 5.5 mm). After MB, mean advancement of the fronto-orbital region was higher at <7 years (16.4 mm), and similarly lower at ages 7-12 (13.8 mm) and ≥13 (12.5 mm). The mean preoperative inter-dacryon distance (34.4 ± 4.4 mm) was reduced by 8.7 ± 4.2 mm after FB without distraction (n = 10). More advancement was seen when midface surgery was performed at a younger age, due to more severe cases and a desire for overcorrection. The highest mean advancement was observed in the fronto-orbital region. Antero-inferior rotational movement was seen after all three techniques., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Purpose: Sagittal synostosis is the most common isolated craniosynostosis. Surgical treatment of this synostosis has been extensively described in the global literature, with promising outcomes when it is performed in the first 12 months of life. However, in some cases, patients older than 12 months arrive at the craniofacial center with this synostosis. A comprehensive study on efficacy and perioperative outcomes has yet to be fully explored in this population. This systematic review and meta-analysis aimed to assess the available evidence of surgical outcomes for the treatment of sagittal synostosis among older patients to analyze the efficacy and safety of synostosis surgery in this unique population., Methods: PubMed, Embase, and Scopus were searched for studies published from inception to March 2024 reporting surgical outcomes of synostosis surgery in older patients (> 12 months) with isolated sagittal synostosis. The main outcome was the reoperation rate, with secondary endpoints including transfusion rates, aesthetic outcomes, and surgical complications., Results: Nine studies were included in the final analysis. The pooled proportion of the reoperation rate was 1%. The rate of excellent aesthetic results was 95%. The need for transfusion associated with the procedures was 86%, and finally, surgical complications attained a pooled ratio of 2%, indicating minimal morbidity associated with the surgical repair., Conclusion: Sagittal synostosis surgery is a safe and effective procedure to perform in older patients; this meta-analysis suggests that open surgery confers a significant rate of excellent aesthetic results with a low reoperation rate and minimal complications associated with the intervention. Future research with direct comparisons among different techniques will validate the findings of this study, which will all contribute to the rigor of synostosis management., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study aims to evaluate the effectiveness of the Piezosurgery® device in endoscopic-assisted correction of trigonocephaly. Trigonocephaly is a type of craniosynostosis characterized by a triangular-shaped forehead due to the premature fusion of the metopic suture. Traditional open cranial vault reconstruction, although common, is invasive and poses risks. The study explores a less invasive alternative using ultrasonic microvibrations for bone cutting, potentially reducing soft tissue damage and improving surgical outcomes., Methods: The Piezosurgery® device was employed in endoscopic trigonocephaly correction surgeries performed on patients under 4 months old at the French Referral Center for Craniosynostosis in Lyon. The technique involves making a small skin incision and performing osteotomies from the anterior fontanel to the glabella. A rigid 0° endoscope provides visibility, and the Piezosurgery® device enables precise bone cutting while preserving the dura mater. Post-surgery, patients were discharged within 3 days and required to wear a remodeling helmet for 6-8 months., Results: The use of Piezosurgery® device allowed precise osteotomies with minimal soft tissue damage. No dura mater injuries occurred in the patient series. The procedure was efficient, with an average duration of 80 min, and blood loss was minimal, reducing the need for blood transfusions. The endoscopic approach facilitated shorter surgical times and reduced postoperative infection risks. Enhanced visibility during surgery, due to cavitation effects, improved the accuracy of bone cuts. The technique demonstrated promising safety and esthetic outcomes, although it incurred higher costs compared to traditional methods., Conclusion: Piezosurgery® device provides a safe and effective method for minimally invasive endoscopic correction of trigonocephaly. The device's ability to selectively cut bone while preserving soft tissues offers significant advantages, despite longer surgical times and higher costs. This technique represents a viable alternative to traditional open surgery, promoting better clinical outcomes and reduced recovery times., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: Craniosynostosis is characterized by the premature fusion of one or more cranial sutures, which can lead to abnormal skull shape and restricted skull growth. Although most cases are present in isolation, some are associated with genetic syndromes, such as Pfeiffer, Muenke, Couzon, Apert, and others, which increases the complexity of care. Today, a spectrum of surgical options to treat craniosynostosis are available and range from traditional open cranial vault remodeling to newer and less invasive suturectomy-based techniques. Which procedure is offered to a patient depends on not only the specific synostosis pattern but also factors such as patient age, the need for additional procedures or interventions, and evidence of elevated intracranial pressure. Thorough consultations with families to discuss achievable goals for cosmesis and function are essential in providing optimal care to each impacted child., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Cranial vault remodelling for craniosynostosis aims to increase intracranial volume to facilitate brain growth, avoid the development of raised intracranial pressure and address cosmesis. The extent of vault expansion is predominantly limited by scalp closure and reconstruction technique. Virtual surgical planning tools have been developed to predict post-operative changes and guide expansion. We present a validation study of a novel 'Dura-based Automated Vault Expansion-Remodeling' (DAVE-R) model to guide pre-operative planning for fronto-orbital advancement and remodelling (FOAR)., Methods: Patients with trigonocephaly who underwent FOAR with pre- and post-operative imaging from 2018 to 2020 were identified from a prospectively maintained database. Post-operative scans, normative atlas and whole brain parcellation were registered to the pre-operative images to quantify the change in intracranial volume and morphology (utilising measurement of fronto-orbital advancement and bifrontozygomatic distance) compared to that predicted by the DAVE-R model., Results: Ten patients were included. The DAVE-R model predicted bifrontozygomatic distances of 92.0 + / - 5.14 mm (mean + /SD), which closely matched the post-operative results of 92.7 + / - 6.02 mm (mean + / - SD); (t(d.f. 9) = -0.306, p = 0.77). The fronto-orbital advancement predicted by the DAVE-R method was 11.5 + / - 1.96 mm (mean + / - SD) which was significantly greater than 8.6 + / - 2.94 mm (mean ± SD); (t(d.f. 9) = 3.137, p = 0.01) achieved post-operatively., Conclusions: We demonstrate that the DAVE-R model provides an objective means of extracting realistic surgical goals in patients undergoing FOAR for trigonocephaly that closely correlates with post-operative outcomes. The normative dural model warrants further study and validation for other forms of craniosynostosis correction., Competing Interests: Declarations. Conflict of interest: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Endoscopically assisted sagittal strip craniotomy with subsequent cranial orthosis is a frequently used surgical approach for non-syndromic sagittal synostosis. Originally, this technique involved a wide sagittal strip craniectomy with bilateral wedge osteotomies. More recent studies suggest omitting wedge osteotomies, achieving similar outcomes. The controversy surrounding wedge osteotomies and our efforts to refine our technique led us to create models and evaluate the mechanical impact of wedge osteotomies., Methods: We conducted a 3D-print study involving preoperative CT scans of non-syndromic scaphocephaly patients undergoing minimally invasive-assisted remodelation (MEAR) surgery. The sagittal strip collected during surgery underwent thickness measurement, along with a 3-point bending test. These results were used to determine printing parameters for accurately replicating the skull model. Model testing simulated gravitational forces during the postoperative course and assessed lateral expansion under various wedge osteotomy conditions., Results: The median sagittal strip thickness was 2.00 mm (range 1.35-3.46 mm) and significantly positively correlated (p = 0.037) with the median force (21.05 N) of the 3-point bending test. Model testing involving 40 models demonstrated that biparietal wedge osteotomies significantly reduced the force required for lateral bone shift, with a trend up to 5-cm-long cuts (p = 0.007). Additional cuts beyond this length or adding the occipital cut did not provide further significant advantage (p = 0.1643; p = 9.6381)., Conclusion: Biparietal wedge osteotomies reduce the force needed for lateral expansion, provide circumstances for accelerated head shape correction, and potentially reduce the duration of cranial orthosis therapy., Competing Interests: Declarations. Ethics approval: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The study was approved by the Ethics Committee of the University Hospital Motol and 2nd Faculty of Medicine, Charles University in Prague (No. EK- 362/24). Competing interests: This project was supported by the Charles University Grant Agency (GAUK No. 32122)., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: The purpose of this study was to evaluate the surgical complications of patients treated for nonsyndromic sagittal craniosynostosis and the necessity for reoperations due to craniocerebral disproportion., Materials and Methods: The patient cohort of this study consisted of patients (N = 82) who were treated in the Oulu University Hospital using the open vault cranioplasty with a modified H-technique between the years 2008 to 2022. There were 69 males (84.1%) and 13 females (15.9%). The mean age at the primary operation was 6.1 months. Mean follow-up time was 9.0 years., Results: There were no major complications related to the procedures. Two patients (2.4%) had a minor dural lesion. There were no postoperative wound infections. Of the 82 patients, seven patients with primary craniosynostosis (13.0%) developed symptomatic craniocerebral disproportion requiring reoperation to increase intracranial volume. In all these patients, invasive intracranial pressure (ICP) monitoring was performed prior to decision-making. In the majority of cases, the aesthetical outcome was considered good or excellent., Conclusion: The operative method used was feasible and safe. Thirteen percent of patients who were followed over 5 years required major surgery due to development of craniocerebral disproportion later in life., Competing Interests: Declarations. Conflict of interest: There are no conflicts of interest. Ethics approval: Not applicable. Consent to participate: Not applicable. Consent to publication: Not applicable. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

Purpose: Treatment of subjects with refractory idiopathic intracranial hypertension (IIH) or shunted hydrocephalus with chronic shunt complications is challenging. What is the role for cranial vault expansion, particularly utilizing posterior vault distraction osteogenesis (PVDO), in these cases? This study assesses medium-term efficacy of cranial vault expansion in this unique patient population., Methods: A retrospective review was conducted of patients who underwent cranial vault expansion from 2008 to 2023 at the Children's Hospital of Philadelphia. Subjects who did not have a diagnosis of primary craniosynostosis were included in the study. Demographic information, medical history, and perioperative details were collected from medical records. Primary outcomes were the rate of CSF diversion procedures and resolution of presenting signs and symptoms. Secondary outcomes were perioperative and 90-day complications and reoperation requirement., Results: Among 13 included subjects, nine (69.2%) patients had a primary diagnosis of shunted hydrocephalus and 4 (30.8%) patients had IIH. Twelve (92.3%) subjects underwent posterior vault distraction osteogenesis (PVDO) and one (7.7%) underwent posterior vault remodeling (PVR). All 4 patients with IIH demonstrated symptomatic improvement following PVDO, including resolution of headaches, vomiting, and/or papilledema. Among 9 patients with shunted hydrocephalus, CSF diversion requirement decreased from 2.7 ± 1.6 procedures per year preoperatively to 1.2 ± 1.8 per year following cranial vault expansion (p = 0.030). The mean postoperative follow-up was 4.1 ± 2.1 years and four (30.8%) patients experienced complications within 90 days of surgery, including infection (n = 2), CSF leak (n = 1), and elevated ICP requiring lumbar puncture (n = 1). Four (30.8%) patients underwent repeat cranial vault expansion for recurrence of ICP-related symptoms. At most recent follow-up, 7 of 9 patients with shunted hydrocephalus demonstrated symptomatic improvement., Conclusion: Cranial vault expansion reduced intracranial hypertension-related symptomology as well as the rate of CSF diversion-related procedures in patients with refractory IIH and shunted hydrocephalus without craniosynostosis, and should be considered in those who have significant shunt morbidity., Competing Interests: Declarations. Competing interests: The authors declare no competing interests. Conflict of interest: The authors have no relevant financial or non-financial interest to disclose. No funding was received to assist with the preparation of this manuscript., (© 2024. The Author(s).)

Purpose: Trigonocephaly is the most common craniosynostosis involving orbits. Although some degree of agreement has been reached regarding surgical timing and indications for treatment, there is no consensus regarding the ideal operative technique to guarantee an optimal morphological outcome. The purpose of this study is to describe both strategies and to compare morphological outcomes by means of morphological surface analysis obtained from three-dimensional (3D) stereophotogrammetry, with two different techniques., Methods: We retrospectively investigated 43 patients with metopic synostosis surgically treated between 2004 and 2020. Two different techniques were applied, addressed as technique A and B. Ten patients undergone postoperative 3d stereophotogrammetry were enrolled, and cephalometric measurements were taken and compared to a cohort of unaffected patients matched by age and gender., Results: Comparison of the groups demonstrated a hypercorrection of the metopic angle of the second technique, associated with a slightly lower correction of the interfrontoparietal diameter. The metopic angle showed to be significantly undercorrected with the first method., Conclusions: Alternated barrel staving technique appears to be a quick and satisfactory method in cranial remodelling for metopic synostosis. It guarantees an optimal aesthetic result in the first years after surgery., Competing Interests: Declarations. Ethics approval: This study has been approved by the local Ethical Committee CEAVEC. Conflict of interest: The authors did not receive any financial support for this study., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Children with metopic synostosis have been found to have more neurocognitive and behavioral difficulties. The variables that may affect future neurodevelopmental outcomes, including presenting morphologic severity, have not been fully studied. In the largest study to date, we aimed to assess what portends worse neurocognitive and behavioral outcomes at school age., Methods: Children 6-18 years old with surgically corrected metopic nonsyndromic craniosynostosis underwent neurocognitive testing. Parents completed behavior rating surveys about their child: Conners-3 (ADHD), Social Responsiveness Scale-2 (autism spectrum disorder), Behavior Rating Inventory of Executive Function-2 (BRIEF-2: executive function), and Child's Behavior Checklist (overall behavior). The endocranial bifrontal angle (EBA), adjusted EBA (aEBA), frontal angle (FA), and AI-derived metopic severity score (MSS) were determined on pre-operative CT images. Multivariate linear regressions were used to evaluate the association of age at surgery and severity., Results: There were 87 children who underwent neurocognitive testing (average age 10.9 ± 3.3 years) of whom 67 also completed behavioral assessments. Greater phenotypical severity of metopic synostosis (lower FA, aEBA, and EBA) was associated with worse scores on the subscales of the BRIEF-2 (executive function) and executive subscale of the Conners-3. Increasing age at surgery was associated with worse executive function subscale scores of the Conners-3 when controlling for each severity measurement and sociodemographic risk., Conclusion: Children with greater phenotypic severity of metopic synostosis have worse executive function at school age. The majority of children with metopic synostosis have signs of ADHD. Later surgeries (greater than 12 months) may impact executive functioning, regardless of the degree of severity. Future research should aim at identifying the direct structural changes to the brain., Competing Interests: Declarations. Ethics approval: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The study was approved by the Yale University Institutional Review Board. (HIC# 0804003650). Consent to participate: All participants’ informed assent and informed consent were obtained by legal guardians. Consent for publication: Not applicable. Competing interests: The authors did not receive support from any organization for the submitted work. Disclaimer: Dr. Alperovich receives funding from CTSA Grant Number KL2 TR001862 from the National Center for Advancing Translational Science (NCATS), a component of the National Institutes of Health (NIH) and consults for Johnson & Johnson and LifeNet Health. No funding was received for this work. The manuscript contents are solely the responsibility of the authors and do not necessarily represent the official view of NIH., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

To determine the effect of midface surgery on soft tissue changes and their relationship to hard tissue changes in patients with syndromic craniosynostosis. A retrospective analysis of patients who had undergone Le Fort III (LFIII), monobloc (MB), or facial bipartition (FB) was conducted. A 3D soft tissue mesh was generated from the preoperative scan and registered to the postoperative scan, after which the advancement was visualised. A total of 68 patients were included: 28 had undergone LFIII, 27 MB, and 13 FB. The included diagnoses were Apert (n = 23), Crouzon (n = 34), and craniofrontonasal syndrome (n = 11). After LFIII, most soft tissue advancement was seen around subnasale and pronasale (mean 15.1 ± 5.9 mm and 14.7 ± 5.7 mm, at age 7-12 years). After MB, a greater hard tissue than soft tissue advancement was seen for most landmarks, showing a high positive correlation. In patients undergoing FB without distraction (n = 10), mean preoperative inter-canthal distance was 48.9 mm, this reduced by 6.9 mm postoperatively. This study provides a comprehensive overview of the outcomes after midface surgery using 3D quantification for a better understanding of the soft tissue changes and their relationship to hard tissue changes., Competing Interests: Competing interests None., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

An esophageal bronchus is a subtype of congenital bronchopulmonary foregut malformations in which a lobar bronchus arises directly from the esophagus, creating a communication between the esophagus and lung tissue. Early diagnosis is crucial to prevent worsening pulmonary sequelae but is challenging due to the rarity of the anomaly and nonspecific respiratory symptoms. We present a child whose esophageal bronchus was identified incidentally during preanesthetic assessment for craniosynostosis repair and discuss the role an anesthesiologist can play in identifying and managing this diagnosis., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 International Anesthesia Research Society.)

Objective: The objective of this study was to investigate the longitudinal changes in cranial growth following fronto-orbital advancement (FOA) surgery in patients with unilateral and bilateral coronal craniosynostosis., Methods: This retrospective review analyzed head circumference (HC) and CT data during preoperative (T0), immediate postoperative (T1), and final follow-up (T2) visits in 40 patients (23 female, 17 male) who underwent FOA using either the open approach or distraction osteogenesis (DO) between 1987 and 2018. The mean follow-up period was 90.62 months. The z-scores of HC, CT-based intracranial volume, anteroposterior diameter (APD), biparietal diameter (BPD), and cranial height (CH) were calculated using sex- and age-specific standards. Logistic regression analysis was performed., Results: While the z-scores of HC, intracranial volume, and BPD remained within the normal range, the z-scores of APD fluctuated between -2 and -1, and the z-scores of CH were > 2, indicating a substantial elevation compared with norms from T0 to T2. Age at surgery significantly influenced the z-scores of HC, BPD, and CH at T2 (all p < 0.05). Delayed surgical timing was correlated with increased BPD and CH z-scores from T1 to T2 (p = 0.007 and 0.019, respectively). The DO for FOA resulted in elevated HC z-scores at T2 and increased APD from T0 to T1, followed by a significant APD relapse from T1 to T2., Conclusions: These findings suggest that delayed surgical timing may support better cranial growth, as indicated by increased HC at long-term follow-up. However, delayed timing is also associated with worsening abnormally elevated CH. Despite the immediate APD expansion and long-term HC increase with DO, potential relapse warrants caution. While intentional overcorrection of APD is recommended, careful consideration of surgical timing and planning is essential.

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 nonsyndromic sagittal synostosis., Methods: Comparisons were performed using available preoperative and postoperative imaging and follow-up data from the 2 craniofacial national referral centers in Sweden, which perform 2 different surgical techniques: craniotomy combined with springs and H-craniectomy (the Renier technique). The study included 23 pairs of patients matched for sex, preoperative cephalic index, and age. Cephalic index, total intracranial volume (ICV), and partial ICV were measured before surgery and at 3 years of age, with volume measurements compared against those of preoperative 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 2 operations, the mean total operation time was similar for the methods. Of the 3 complications that occurred in the group treated with springs, 2 were spring-related. The compiled analysis of changes in cephalic index and partial volume distribution revealed that craniotomy combined with springs resulted in superior morphologic correction., Conclusion: The findings showed that craniotomy combined with springs normalized cranial morphology to a greater extent than H-craniectomy based on changes in cephalic index and total and partial ICVs over time., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Purpose: To evaluate the refractive outcome and strabismus at 5 years of age, in children operated for various types of non-syndromic craniosynostosis, and further analyse the refractive and strabismic development over time., Methods: Eighty-nine children, who had undergone operations for non-syndromic craniosynostosis, were examined at 5 years of age. These children also underwent ophthalmological examination preoperatively and up to 1 year after the operation. An age-matched control group including 32 healthy children was also recruited. Strabismus and eye motility were registered. Refraction was measured in cycloplegia., Results: There was a difference regarding the refractive outcome between the different types of craniosynostosis. Higher values of hypermetropia were found in the metopic craniosynostosis group on both eyes. In the unicoronal craniosynostosis group, high values of hypermetropia and a higher degree of astigmatism were found on the side contralateral to the craniosynostosis. Strabismus was found in 11/88 children of whom 10/11 had unicoronal craniosynostosis. A vertical deviation on the side ipsilateral to the fused suture was highly prevalent (6/10 cases). Ophthalmological dysfunctions were rare in children operated for sagittal craniosynostosis., Conclusion: Ocular manifestations such as strabismus, astigmatism and anisometropia were highly prevalent in children operated for unilateral coronal craniosynostosis. Children operated for metopic craniosynostosis had higher rates of hypermetropia. The screening and follow-up protocols need to be tailored with regard to the type of craniosynostosis., (© 2023 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Marshall-Smith Syndrome (MSS) is a rare progressive developmental disorder that severely impairs a patient's intellectual development and physical health. The only known cause for MSS is a mutation in the nuclear factor 1 X (NFIX) gene. This mutation affects neuronal development and protein transcription. Historically, most patients with MSS do not survive beyond 3 years of age. Reports of ocular findings are limited. We report a case of a 9-year-old MSS patient with progressive craniosynostosis, elevated intracranial pressure, and catastrophic ocular complications. A comprehensive PubMed literature search from 2018 to August 2022 updating a previous review of older literature produced 72 articles relating to MSS, which are reviewed., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)