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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: There is no defined preventive treatment protocol for persistent post-craniotomy headache. In several small case series and individual case reports onabotulinumtoxinA injected into the craniotomy scar has shown possible efficacy. What is lacking is long term follow-up and if focusing on the cranial suture lines along with the craniotomy scar can enhance improvement and provide more sustained benefit., Methods: Retrospective chart review with case series., Results: Four patients (three women, one man) with ICHD-3 defined persistent post craniotomy headache were treated using a novel onabotulinumtoxinA injection protocol. All the patients presented with continuous head pain of moderate to severe intensity. All had severe allodynia on the side of their craniotomy. All had significant reduction in quality of life. Our application of onabotulinumtoxinA involved injection into both the surgical scar and the transected/irritated cranial suture lines noted on neuroimaging and physical examination. With treatment all patients demonstrated significant benefit including a reduction in daily pain intensity (75%-100%), developing periods of pain freedom (2-7 days per week) and having a dramatic improvement in quality of life (close to 100% in all). The benefit was sustained for at least five years of follow-up., Conclusion: From our case series it appears that injection not only along the painful craniotomy scar but into the involved cranial suture lines provides positive efficacy and sustained improvement in patients with persistent post craniotomy headache., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Large-scale calvarial defects often coincide with cranial suture disruption, leading to impairments in calvarial defect restoration and skull development (the latter occurs in the developing cranium). However, the lack of a standardized model hinders progress in investigating suture-regenerative therapies and poses challenges for conducting comparative analyses across distinct studies. To address this issue, the current protocol describes the detailed modeling process of calvarial suture-bony composite defects in rats. The model was generated by drilling full-thickness rectangular holes measuring 4.5 mm × 2 mm across the coronal sutures. The rats were euthanized, and the cranium samples were harvested postoperatively at day 0, week 2, week 6, and week 12. µCT results from samples collected immediately post-surgery confirmed the successful establishment of the suture-bony composite defect, involving the removal of the coronal suture and the adjacent bone tissues. Data from the 6 th and 12 th postoperative weeks demonstrated a natural healing tendency for the defect to close. Histological staining further validated this trend by showing increased mineralized fibers and new bone at the defect center. These findings indicate progressive suture fusion over time following calvarial defects, underscoring the significance of therapeutic interventions for suture regeneration. We anticipate that this protocol will facilitate the development of suture-regenerative therapies, offering fresh insights into the functional restoration of calvarial defects and reducing adverse outcomes associated with suture loss.

Objective: As many as 5% of normocephalic children may have a prematurely fused sagittal suture, yet the clinical significance and best course of management of this finding remain unclear. Providers in the Synostosis Research Group were surveyed to create a multicenter consensus on an optimal treatment and monitoring algorithm for this condition., Methods: A four-round modified Delphi method was utilized. The first two rounds consisted of anonymous surveys distributed to 10 neurosurgeons and 9 plastic surgeons with expertise in craniosynostosis across 9 institutions, and presented 3 patients (aged 3 years, 2 years, and 2 months) with incidentally discovered fused sagittal sutures, normal cephalic indices, and no parietal dysmorphology. Surgeons were queried about their preferred term for this entity and how best to manage these patients. Results were synthesized to create a treatment algorithm. The third and fourth feedback rounds consisted of open discussion of the algorithm until no further concerns arose., Results: Most surgeons preferred the term "premature fusion of the sagittal suture" (93%). At the conclusion of the final round, all surgeons agreed to not operate on the 3- and 2-year-old patients unless symptoms of intracranial hypertension or papilledema were present. In contrast, 50% preferred to operate on the 2-month-old. However, all agreed to utilize shared decision-making, taking into account any concerns about future head shape and neurodevelopment. Panelists agreed that patients over 18 months of age without signs or symptoms suggesting elevated intracranial pressure (ICP) should not undergo surgical treatment., Conclusions: Through the Delphi method, a consensus regarding management of premature fusion of the sagittal suture was obtained from a panel of North American craniofacial surgeons. Without signs or symptoms of ICP elevation, surgery is not recommended in patients over 18 months of age. However, for children younger than 18 months, surgery should be discussed with caregivers using a shared decision-making process.

Summary: Premature fusion of the lambdoid suture is the most uncommon single-suture synostosis. It presents with a classic "windswept" appearance, a trapezoid-shaped head, and significant skull asymmetry notable for an ipsilateral mastoid bulge and contralateral frontal bossing. Because of the rarity of lambdoid synostosis, little is known about optimal techniques for its treatment. In particular, the proximity of the lambdoid suture to critical intracranial structures such as the superior sagittal and transverse sinuses represents a potential for significant intraoperative bleeding. Prior work has shown that parietal asymmetry persists after repair in these cases. In this article, the authors present a technique for the treatment of unilateral lambdoid craniosynostosis along with two representative cases. This calvarial vault remodeling technique requires the removal of both ipsilateral and contralateral parietal bones. These are moved across hemispheres and reinset on opposite sides to help correct the parietal asymmetry. Obliquely orientated barrel stave osteotomies are performed to provide a safe mechanism for correction of occipital flattening. The authors' early results show improvement in correction of volume asymmetry 1 year postoperatively relative to patients treated with prior calvarial vault remodeling techniques. The authors believe the technique presented here corrects the windswept appearance in patients with lambdoid craniosynostosis and also reduces the potential for complications. Further work will be necessary to confirm this technique's long-term efficacy in a larger cohort., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Premature fusion of craniofacial joints, i.e. sutures, is a major clinical condition. This condition affects children and often requires numerous invasive surgeries to correct. Minimally invasive external loading of the skull has shown some success in achieving therapeutic effects in a mouse model of this condition, promising a new non-invasive treatment approach. However, our fundamental understanding of the level of deformation that such loading has induced across the sutures, leading to the effects observed is severely limited, yet crucial for its scalability. We carried out a series of multiscale characterisations of the loading effects on normal and craniosynostotic mice, in a series of in vivo and ex vivo studies. This involved developing a custom loading setup as well as software for its control and a novel in situ CT strain estimation approach following the principles of digital volume correlation. Our findings highlight that this treatment may disrupt bone formation across the sutures through plastic deformation of the treated suture. The level of permanent deformations observed across the coronal suture after loading corresponded well with the apparent strain that was estimated. This work provides invaluable insight into the level of mechanical forces that may prevent early fusion of cranial joints during the minimally invasive treatment cycle and will help the clinical translation of the treatment approach to humans., (© 2023. The Author(s).)

This study investigated how the fusion states of the cranial base is related to the degree of increased intracranial pressure (ICP) in patients with Crouzon syndrome. This retrospective cohort study enrolled patients who were diagnosed with Crouzon syndrome between May 2007 and April 2022. We categorized the patients into three groups: A, B, and C, according to the severity of increased ICP and the number of cranial vault remodeling procedures for corrective operation. The preoperative fusion states of the cranial base sutures/synchondroses were examined using facial bone computed tomography and compared between groups. Overall, 22 patients were included in Groups A, B, and C, including 8, 7, and 7 patients, respectively. The preoperative average grades of the total cranial base suture/synchondrosis fusion appeared to significantly increase with severity, except for the frontoethmoidal suture, which showed the opposite tendency. In the subgroup analysis, frontosphenoidal, sphenoparietal, sphenosquamosal, parietomastoid, and occipitomastoid suture and petro-occipital synchondrosis were associated with earlier fusion in the more severe group. Premature closure of the cranial base sutures/synchodroses seems to be associated with increased ICP severity in patients with Crouzon syndrome. Precise evaluation of minor sutures/synchondroses at the first visit might help build subsequent operative plans and predict disease prognosis., Competing Interests: Declaration of competing interest The authors declare that there are no conflicts of interest related to the current study., (Copyright © 2024 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Background: Unilateral coronal synostosis (UCS) results in a surgically demanding deformation, as the deformity is asymmetric in the calvaria but also presents with facial scoliosis and orbital dystopia. Traditional cranioplasties correct the forehead but have little effect on the face and orbits. In this article, the authors describe a consecutive series of patients operated on for UCS with osteotomy of the fused suture combined with distraction osteogenesis., Methods: Fourteen patients (mean age, 8.0 months; range, 4.3 to 16.6 months) were included in this study. The authors measured and compared the orbital dystopia angle, anterior cranial fossa deviation, and anterior cranial fossa cant between preoperative computed tomography results and those at distractor removal., Results: Blood loss was 6.1 mL/kg (range, 2.0 to 15.2 mL/kg), and length of stay was 4.4 days (range, 3.0 to 6.0 days). The authors observed significant improvements in the median orbital dystopia angle from 9.8 degrees (95% CI, 7.0 to 12.6 degrees) to 1.1 degrees (95% CI, -1.5 to 3.7 degrees) ( P < 0.001), anterior cranial fossa deviation from 12.9 degrees (95% CI, 9.2 to 16.6 degrees) to 4.7 degrees (95% CI, 1.5 to 7.9 degrees) ( P < 0.001), and anterior cranial fossa cant from 2.5 degrees (95% CI, 1.5 to 3.5 degrees) to 1.7 degrees (95% CI, 0.0 to 3.4 degrees) ( P = 0.003)., Conclusions: Osteotomy combined with a distractor for UCS straightened the face and relieved orbital dystopia by affecting the nose angle relative to the orbits, correcting the deviation of the cranial base in the anterior fossa, and lowering the orbit on the affected side. Furthermore, this technique demonstrated a favorable morbidity profile with low perioperative bleeding and a short inpatient period, suggesting its potential to improve the surgical treatment of UCS., Clinical Question/level of Evidence: Therapeutic, IV., (Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Plastic Surgeons.)

Sagittal suture synostosis is one of the most common craniosynostoses and is often diagnosed by characteristic narrow and long skull shape, scaphocephaly. However, some patients with sagittal suture synostosis do not present with typical scaphocephaly, making early diagnosis difficult. In this study, five cases of characteristic skull deformity showing a narrowing of the cranium posterior to the coronal suture on computed tomography (CT) are presented. The three older children presented with papilledema and intellectual disability and a closed sagittal suture on CT. The two infant cases were diagnosed with the characteristic cranial deformities with aggravation of the deformity over time, but sagittal suture closure was not evident on CT. All patients underwent cranial remodeling surgery. In the two infant cases, the histopathological findings showed that the anterior part of the sagittal suture was firmly fused with fibrous tissue without bony fusion. These findings suggested that narrowing of the cranium posterior to the coronal suture might be due to functional fusion of the anterior portion of the sagittal suture prior to bony fusion. In an infant presenting with such a deformity that shows aggravation of the deformity over time, surgical treatment should be considered., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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.

Aim: To mathematically present the results of endoscopic assisted surgery in the treatment of metopic synostosis., Material and Methods: We present a follow-up of the stages of treatment of 43 patients with metopic synostosis who underwent surgery in a multicenter retrospective study between 2018 and 2021. We developed the formula D3/((D1+D2)/2) based on the theory of "The Angle of a Triangle Opposite The Longest Side is the Largest Angle". And we called this method "Metopic Angle Proportion (MAP)". Paired samples T-test was performed for statistical results. A p-value of < 0.05 was considered statistically significant., Results: According to the MAP method; measurements for frontal enlargement, mean pretreatment length D1: 34,57 mm, D2: 34,81 mm, D3: 60,46 mm, and the end of treatment as D1: 37,88 mm, D2: 38,19 mm, D3: 71,09 mm. We perfomed the formula D3/ [(D1+D2)/2]. While this rate was 1.74 before treatment, it increased to 1.87 after treatment. As a result of statistical analysis, it was also found to be significant (p≤0.05)., Conclusion: Endoscopy-assisted suturectomy can be applied in health centres where available, because it is safe, easy and cheaper.

Numerous classification systems of nonsyndromic sagittal craniosynostosis (NSC) are applied but none has gained a wide acceptance, since each classification is focused on distinct aspects. The aim of the study was to assess the accuracy of 4 classifications of NSC discussed in the literature by defining the associations among the classifications, individual features (sex, age, cranial index), and objective morphologic criteria (frontal bossing, retrocoronal constriction, sagittal ridge, and occipital bulleting). The study was conducted on anonymized thin-cut CT scans of 133 children with NSC 1 to 12 months old (mean age 5.42 mo). The type of cranial dysmorphology was assessed using 4 classification systems, focusing on skull shape, pattern of sagittal suture closure (Heuzé classification), deformation of skull vault (Sakamoto classification), and a single-dominant feature (David classification). Each patient was also independently investigated for the presence of morphologic criteria. A multivariate analysis was performed to explore the relations among the classifications and assess their accuracy. In the analyzed cohort sphenocephaly (38.3%), CFF type by Heuzé (30.8%), type I by Sakamoto (72.9%), and a central type by David (42.9%) were dominant findings. Regarding the morphologic criteria, frontal bossing was observed the most frequently (91.7%). The age of patients and cranial index differed significantly among the shapes of skull and David classifications ( P <0.001). The shape-based system showed the strongest correlation with other classifications and with measurable variables. Other classifications have much in common and some overlap, but none of them constitutes a standalone system to define all aspects of cranial dysmorphology in NSC., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Mutaz B. Habal, MD.)

Background: The prevalence of trigonocephaly has increased worldwide over the past 2 decades. Early identification and appropriate treatment are critical. The aim of this study is to evaluate the outcomes and the effect of metopic suture excision, perisutural frontal bone shave, and bilateral pericranial flap method on the shape of the forehead after surgical correction in infants with moderate trigonocephaly., Methods: The present study was performed as a cross-sectional study on 40 infants of 3 to 12 months old with trigonocephalus who underwent metopic suture excision and pericardial flap surgery in Mofid Pediatric Hospital from 2016 to 2022. The definitive diagnosis of patients' trigonocephaly was made based on clinical signs and computed tomography scan findings by a plastic surgeon., Results: Overall in 40 patients operated by this technique, 23 (57.5%) of cases were males, and 17 (42.5%) were females. The mean age of patients was 7.86 ± 2.22 months. Hospital stay was 2 to 4 days (mean: 3 d), intensive care unit admission was in 33 cases for 24 hours, and no intensive care unit admission for 7 cases. Blood was transfused during surgery for 25 patients, and 15 patients did not require blood transfusion use. Results were evaluated in 6 to 12 months after surgery by 3 independent plastic surgeons, with pre and postoperative photos. Satisfaction with the results of forehead shape was excellent for 60% of patients, good for 37.5%, and moderate for 2.5%. Only one female patient had a recurrence after the surgery., Conclusion: This study showed that the pericranial flap method after full metopic suture excision and frontal shave was very effective in the treatment of infants with moderate trigonocephaly., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

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., (© 2023. The Author(s).)

Purpose: To assess the role of FGFR2 mutations and sutural synostotic patterns on facial skeleton dysmorphology in children with syndromic craniosynostosis., Methods: Preoperative high-resolution CT images in 39 infants with syndromic craniosynostosis were evaluated. Patients were divided into infants with and without FGFR2 mutations; each group was split according to synostotic involvement of minor sutures/synchondroses: isolated or combined involvement of middle (MCF) and posterior cranial fossae (PCF). Quantitative analysis of the midface and mandible measures was performed. Each subgroup was compared with a group of age-matched healthy subjects., Results: Twenty-four patients with FGFR2 related syndromes were clustered in 3 subgroups: MCF + PCF (8 patients, 5.4 ± 1.75 months), MCF (8 patients, 3.62 ± 1.68 months), and PCF (8 patients, 2.75 ± 0.46 months). Fifteen no-FGFR2 patients were clustered in 2 subgroups: MCF + PCF (7 patients, 9.42 ± 0.78 months) and PCF (8 patients, 7.37 ± 2.92 months). Both FGFR2 and no-FGFR2 groups with involvement of minor sutures coursing in MCF showed more facial sutural synostoses. Children with minor suture/synchondrosis synostosis of MCF (MCF-PCF and MCF subgroups) showed altered position of glenoid fossa and mandibular inclination ([Formula: see text]), but children in the FGFR2 group had also reduced midfacial depth and maxillary length ([Formula: see text]). Children with minor suture/synchondrosis synostosis of PCF (PCF subgroups) had reduced posterior mandibular height, but those children in the FGFR2 group also showed reduced intergonion distance ([Formula: see text])., Conclusions: In children with syndromic craniosynostosis, both skull base and facial suture synostosis affect facial dysmorphology/hypoplasia. FGFR2 mutations may worsen facial hypoplasia both acting on bone development and causing an earlier premature closure of facial sutures., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

To present the outcomes and adverse events associated with the endoscopic-assisted, minimally invasive suturectomy in patients with multisuture synostosis. This retrospective cohort study included children < 65 days of age who underwent endoscopic-assisted suturectomy (EAS) for multisuture craniosynostosis at a single tertiary referral center from 2013 to 2021. The primary outcome was calvarial expansion, and the secondary outcome was adverse events. The pre- and post-operative 3-dimensional brain computed tomography (CT) scan was used to calculate the intracranial volume and cephalic index. During a period of 2 years, 10 infants (10-64 days) diagnosed with multisuture synostosis underwent single-stage EAS of every affected suture in our center. The coronal suture was the most prevalent involved suture among our cases. The mean age and weight of the patients were 39 ± 17.5 days and 4.39 ± 0.8 kg, respectively. The surgical procedure took 42 ± 17.4 min of time and caused 46 ± 25.4 mL of bleeding on average. Ninety percent of the operations were considered successful (n = 9) regarding calvarial expansion. There were two complications, one requiring an open vault surgery and one repairing a leptomeningeal cyst. In the eight patients who did not necessitate further interventions, the mean pre-operative intracranial volume was 643.3 ± 189.4 cm 3 . The follow-up results within the average of 38.9 months after surgery showed that as age increases, the intracranial volume also increased significantly (R: 0.6, P < 0.0001), which suggests continued skull growth in patients who underwent EAS. With the low rate of intra- or post-operative complications and promising results on revising the restricted skull sutures, EAS seems both a safe and effective therapeutic modality in patients with multisuture synostosis, especially if completed in the first months after birth., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Primary delayed onset craniosynostosis is defined as premature suture fusion that developed despite clear radiographic evidence of normal postnatal calvarial configuration and patent sutures earlier in life. It is rare in the literature and typically presents as secondary synostosis. In this brief clinical study, primary delayed onset craniosynostosis is described in its unique presentation at 4 years of age with a complex genetic history including ERF-related craniosynostosis syndrome and familial cerebral cavernous malformation syndrome. Although the delayed onset clinical course of ERF-related craniosynostosis syndrome has not been well described in the literature, our review suggests that it is distinctive to ERF-related craniosynostosis and should be considered when cases present without a history of trauma, when there is a positive family history, and particularly when cases present late onset; after 1 year of age.

Introduction: The squamosal suture (SQS) joins the temporal to the parietal bones bilaterally and is a poorly described site of craniosynostosis. SQS fusion is thought to occur as late as the fourth decade of life and beyond; however, we have incidentally noted its presence among our pediatric patients and hypothesize that it may occur earlier in life and more frequently than previously believed., Methods: A retrospective review of imaging performed on pediatric patients was completed to identify patients with SQS synostosis. This included a review of clinical notes as well as computed tomography (CT) images obtained by our craniofacial clinic. Relevant patient data and imaging were reviewed., Results: Forty-seven patients were identified with SQS synostosis, 21 were female (45%). Age at the time of radiographic diagnosis was 10.1 ± 8.4 years (range 17 days to 27 years). A majority of patients had bilateral SQS synostosis (57%), with a relatively even distribution of unilateral right (23%) versus left (19%). SQS was an isolated finding (no other suture involvement) in 15 patients (32%), all of whom were normocephalic and did not require surgical intervention. Thirty-two patients (68%) had concomitant craniosynostosis of other sutures, most commonly sagittal and coronal. Nine patients (19%) underwent surgery to correct cranial malformations-all these patients had multi-suture synostosis ( P  = 0.012). Twenty-seven patients (57%) had SQS synostosis diagnosed incidentally compared to 20 (43%) who were imaged with suspicion for synostosis. In those who were symptomatic, common findings included developmental delay, elevated intracranial pressure, hydrocephalus, seizures, and visual/hearing impairments. Ten patients (21%) were syndromic, the most frequent of which was Crouzon syndrome. No single pattern of calvarial malformation could be definitively described for SQS synostosis., Conclusion: Given that most isolated SQS synostosis cases were normocephalic, asymptomatic, and discovered incidentally, it is likely that there are many cases of unidentified SQS synostosis. The significance of SQS synostosis is currently unclear, and warrants further investigation into this phenomenon, its natural course, and its potential presence in the spectrum of normal development.

Background: Although craniosynostoses involving the major sutures have been well described, the frequency of isolated minor suture craniosynostoses is much lower. Squamosal craniosynostosis (SQS) is a rare form of cranial synostosis, and the paucity of literature has made the creation of a standardized treatment plan difficult. We present a systematic review of the literature on isolated SQS to identify disease characteristics that lead to a need for operative intervention and to delineate patterns in surgical management., Methods: A systematic literature review was performed using the electronic databases of PubMed, Scopus, and MEDLINE and the key words "squamosal AND craniosynostosis," "squamous AND craniosynostosis," "squamosal craniosynostosis, "squamosal suture craniosynostosis," and "isolated squamosal craniosynostosis." Only human studies that described presentation and management of SQS were included. A blinded, 2-reviewer analysis of the articles was performed. Data collected included patient and disease characteristics, imaging workup, and treatment specifics, which were analyzed by descriptive statistics., Results: A total of 19 studies examining 119 patients with SQS were reviewed, with 97 (82%) multisutural cases and 22 isolated cases (18%). Of the isolated cases, 6 (27%) required surgical craniosynostosis repair, of which 1 (17%) had unilateral sutural involvement and 5 (83%) had bilateral involvement. Of the patients with isolated SQS, 7 (32%) had a congenital syndrome and comprised 33% of patients who required surgical intervention. The nonsyndromic patients with isolated SQS who required surgery presented with a wide array of phenotypic findings; 3 patients underwent some form of cranial vault remodeling, whereas 1 patient underwent ventriculoperitoneal shunt only. Of the 4 nonsyndromic patients with isolated SQS who underwent surgical repair, half required operative intervention because of elevated intracranial pressure and the other half because of dysmorphic head shape., Conclusion: The findings of this updated systematic review suggest a trend toward surgical management in bilateral SQS versus unilateral SQS, and that patients with isolated SQS, previously considered to be a nonsurgical finding, should be carefully monitored, as there remains risk of increased intracranial pressure. Pooled systematic review data suggest isolated SQS has a 27% operative intervention rate, with the presence of coexisting syndromic diagnoses increasing that risk., Competing Interests: Conflicts of interest and sources of funding: The authors have no conflicts of interest or disclosures related to this article., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Jacobs syndrome is a rare trisomy (47, XYY) found in ~1 in 1000 male children associated with infertility, autism spectrum disorders, macrocephaly, hypertelorism, tall stature, and macroorchidism. Diagnosis is often delayed due to relatively subtle phenotypic changes. Craniosynostosis, a fusion of the cranial sutures, has been described in ~1 in 2000 live births, of which 25% are related to a diagnosed syndrome with the most common being Apert and Crouzon. Craniosynostosis does not have a known association with Jacobs syndrome and no prior cases have been reported. This case report seeks to describe the presentation and treatment of a patient with Jacobs syndrome and metopic craniosynostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Craniosynostosis is a developmental craniofacial defect in which one or more sutures of the skull fuse together prematurely. Uncorrected craniosynostosis may have serious complications including elevated intracranial pressure, developmental delay, and blindness. Proper diagnosis of craniosynostosis requires a physical examination of the head with assessment for symmetry and palpation of sutures for prominence. Often, if craniosynostosis is suspected, computed tomography (CT) imaging will be obtained. Recent literature has posited that this is unnecessary. This study aims to address whether physical examination alone is sufficient for the diagnosis and treatment planning of single suture craniosynostosis. Between 2015 and 2022, the Divisions of Pediatric Neurosurgery and Pediatric Plastic Surgery at UTHealth Houston evaluated 140 children under 36 months of age with suspected craniosynostosis by physical examination and subsequently ordered CT imaging for preoperative planning. Twenty-three patients received a clinical diagnosis of multi-sutural or syndromic craniosynostosis that was confirmed by CT. One hundred seventeen patients were diagnosed with single suture craniosynostosis on clinical examination and follow-up CT confirmed suture fusion in 109 (93.2%) patients and identified intracranial anomalies in 7 (6.0%) patients. These patients underwent surgical correction. Eight (6.8%) patients showed no evidence of craniosynostosis on CT imaging. Treatment for patients without fused sutures included molding helmets and observation alone. This evidence suggests that physical examination alone may be inadequate to accurately diagnose single suture synostosis, and surgery without preoperative CT evaluation could lead to unindicated procedures., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Very few clinical entities have undergone so many different treatment approaches over such a short period of time as craniosynostosis. Surgical treatments for this condition have ranged from simple linear craniectomies, accounting for the specific role of cranial sutures in assuring the normal growth of the skull, to more complex cranial vault reconstructions, based on the perceived role of the skull base in affecting the growth of the skull. While a great deal of evolution has occurred, there remains controversy regarding the ideal treatment including the best surgical technique, the optimal age for surgery, and the long-term morphological and neurodevelopmental outcomes. The evolution of the surgical management of craniosynostosis in the last 50 years has been affected by several factors. This includes the awareness of needing to operate on affected children during infancy to achieve the best results, the use of multistage operations, the availability of more sophisticated surgical tools, and improved perioperative care. In some forms of craniosynostosis, the operations can be carried out at a very young age with low morbidity, and with the postoperative use of a molding helmet, springs, or distractors, these operations prove to be as effective as traditional larger cranial reconstructions performed in older children. As a consequence, complex surgical operations have become progressively less utilized. A second relevant advance was the more recent advent of a molecular diagnosis, which allowed us to understand the pathogenesis of some associated malformations and neurodevelopmental issues that were observed in some children despite appropriate surgical treatment. Future research should focus on improving the analysis of longer-term outcomes and understanding the natural history of craniofacial conditions, including what issues persist despite optimal surgical correction. Progress in molecular investigations concerning the normal and pathological development of cranial sutures could be a further significant step in the management of craniosynostosis, possibly favoring a "medical" treatment in the near future. Artificial intelligence will likely have a role in establishing the diagnosis with less reliance on radiographic studies and in assisting with surgical planning. Overall, much progress has been made, but there remains much to do., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The purpose of this study was to characterize a novel type of calvarial thickening and provide objective measurements of skull thickness and calvarial suture morphology in patients with bronchopulmonary dysplasia., Methods: Infants with severe bronchopulmonary dysplasia who also had undergone computed tomography (CT) scans were identified from the neonatal chronic lung disease program database. Thickness analysis was performed using Materialise Mimics., Results: The chronic lung disease team treated 319 patients during the study interval of which, 58 patients (18.2%) had head CT available. Twenty-eight (48.3%) were found to have calvarial thickening. The rate of premature suture closure in the study population was 36.2% (21 of 58 patients), with 50.0% of affected cohort having evidence of premature suture closure on the first CT scan. Multivariate logistic regression identified 2 risk factors, requiring invasive ventilation at 6 months of age and fraction of inspired oxygen requirement at 6 months of age. Increased head circumference at birth protected against the development of calvarial thickening., Conclusions: We have described a novel subset of patients with chronic lung disease of prematurity who have calvarial thickening with remarkably high rates of premature closure of cranial sutures. The exact etiology of the association is unknown. In this patient population with radiographic evidence of premature suture closure, operative decision should be made after considering unequivocal evidence of elevated intracranial pressure or dysmorphology and balanced against the risk of the procedure., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Background: Primary craniosynostosis is a congenital craniofacial disorder in which cranial sutures prematurely close. Iatrogenic secondary stenosis is abnormal cranial suture closure caused by surgical manipulation of the suture. In contrast, idiopathic secondary stenosis develops in a suture that did not undergo surgical manipulation. The objective of this systematic review was to consolidate and characterize the incidence, classification, and management of idiopathic secondary stenosis in the literature., Methods: Literature from PubMed, Web Of Science, and EMBASE from 1970 to March 2022 was reviewed. The following information was extracted for individual patients: incidence of idiopathic secondary stenosis, index primary craniosynostosis, primary surgical correction, presenting signs of secondary stenosis, management, and further complications., Results: Seventeen articles detailing 1181 patients were included. Ninety-one developed idiopathic secondary stenosis (7.7%). Only 3 of these patients were syndromic. The most common index craniosynostosis was sagittal synostosis (83.5%). The most common suture undergoing idiopathic secondary stenosis was the coronal suture (91.2%). Patients presented at a median age of 24 months. The most common presenting sign was a radiologic finding (85.7%), although some patients presented with headache or head deformity. Only 2 patients, both syndromic, had complications following surgical correction of secondary stenosis., Conclusions: Idiopathic secondary stenosis is a rare, long-term complication following index surgical repair of craniosynostosis. It can occur following any surgical technique. It most commonly affects the coronal suture but can affect any of the sutures, including pansynostosis. Surgical correction is curative in nonsyndromic patients., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Objective: To analyze the clinical and genetic characteristics of an infant with craniosynostosis., Methods: An infant who was admitted to Wuhan Children's Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology in April 2021 due to widening of the lateral ventricles for over a month was selected as the study subject. Clinical data of the patient was collected. Peripheral blood samples were collected from the infant and her parents for chromosomal karyotyping and whole exome sequencing. Candidate variant was verified by Sanger sequencing and bioinformatic analysis. Relevant literature was retrieved from the PubMed, Wanfang and CNKI databases (up to December 2021) by using key words including ERF gene, craniosynostosis, ERF mutation, craniosynostosis and ERF-related craniosynostosis., Results: The infant, a 1-month-and-16-day-old female, was found to have sagittal synostosis by cranial X-ray radiography. Genetic testing revealed that she has harbored a heterozygous c.787C>T (p.Q263*) variant of the ERF gene, which was not found in either parent. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was predicted as pathogenic (PVS1+PS2+PM2&#95;Supporting). In total 63 relevant cases were retrieved from the database, and a total of 64 individuals were analyzed by genetic testing. Most of the cases were sporadic and males. Multiple cranial sutures (including at least two of the sagittal suture, coronal suture, lambdoid suture, and frontal suture) were involved in 45.45% of the cases, and those with sagittal suture closure only have accounted for 20.00%. The main clinical manifestations have included hypertelorism, exophthalmos, development delay, malar dysplasia, etc. Chiari type 1 malformation may present in some patients. Variants of the ERF gene have mainly included splicing and deletional variants, and there was a strong genetic heterogeneity among the infants and their pedigrees., Conclusion: The c.787C>T (p.Q263*) variant of the ERF gene probably underlay the craniosynostosis of this infant. Above finding has enriched the phenotype ~ genotype spectrum of the ERF gene.

Objective: Ridging along the metopic suture line can be a common cause of concern for parents and has been theorized to represent a mild form of trigonocephaly, a cranial deformity associated with risks of negative cosmetic outcomes, if not surgically corrected. Yet the literature contains sparse reports of long-term cosmetic results or expectations for infants with isolated metopic ridging compared with those with severe trigonocephaly, or even what objective metrics discriminate isolated metopic ridging from severe trigonocephaly. Therefore, the authors' goals for this study were to 1) quantify the degree of frontal deformity among patients with metopic ridge, metopic craniosynostosis, and normocephalic head shapes; and 2) document the natural history of frontal deformities in isolated metopic ridge patients in the 1st year of life., Methods: This was a retrospective cohort study of patients with normocephalic head shapes, metopic ridges, and metopic craniosynostoses who presented at < 1 year of age to the Connecticut Children's neurosurgery clinic from January 2019 to December 2021. Data were collected regarding demographics and photograph-based craniometrics., Results: A total of 212 normocephalic, 34 metopic ridge, and 29 metopic craniosynostosis patients were included. Both the normocephalic and metopic ridge groups had a significantly higher anterior arc angle (AAA) value compared with the metopic craniosynostosis group (p < 0.0001). The AAA did not differ significantly among normocephalic patients and those with ridging. Over the course of 1 year of follow-up, patients with metopic ridging demonstrated a slight decrease in AAA values, but overall remained within the same range as normocephalic patients., Conclusions: Photograph-based craniometrics suggest that metopic ridge patients with frontal bone angulations > 2.2 radians have a mild degree of frontal constriction that does not significantly worsen over the 1st year of life.

Background: The purpose of this study was to quantify change in cranial morphology in patients with nonsyndromic unilateral lambdoid craniosynostosis (ULC) from presentation (t0), after open posterior switch-cranioplasty (t1), and at 2-year follow-up (t2)., Methods: Volumetric, linear, and angular analysis were performed on computed tomographic scans at the three time points and against normal control subjects. Significance was set at P < 0.05., Results: Twenty-two patients were included. ULC cranial vault asymmetry index was higher than in control subjects before surgery (6.22 ± 3.55) but decreased after surgery (3.00 ± 2.53) to become comparable with the normal asymmetry range present in the controls. After surgery, both diagonals increased, but more on the fused side. In the 2 years after surgery, both diagonals in patients with ULC grew proportionately, but the fused diagonal remained slightly shorter than the patent side. Total cranial volume was higher in patients with ULC than in control subjects after surgery but became comparable at t2. Cranial base angulation improved by t2 but did not approach normal, and ear position remained unchanged. The facial twist was higher than in controls at t0 and t1 but was comparable at t2. Coronal asymmetry improved with surgery but remained undercorrected at t2, with the greatest residual asymmetry at opisthion., Conclusions: Open-switch cranioplasty normalizes cranial vault asymmetry index by increasing the fused cranial diagonal more than the patent side and is stable at 2 years. Skull base twist does not normalize, but facial twist approaches normal. Technique improvement should focus on residual coronal asymmetry present at opisthion., Clinical Question/level of Evidence: Therapeutic, IV., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Purpose: Craniosynostosis, which describes premature fusion of one or more cranial sutures, has been associated with a variety of neurocognitive deficits. We sought to explore the cognitive profiles of the various types of single-suture, non-syndromic craniosynostosis (NSC)., Methods: A retrospective review of children 6-18 years old with surgically corrected NSC who underwent neurocognitive testing (Weschler Abbreviated Scale of Intelligence, Beery-Buktenica Developmental Test of Visuomotor Integration) from the years 2014-2022 was conducted., Results: 204 patients completed neurocognitive testing (139 sagittal, 39 metopic, 22 unicoronal, 4 lambdoid suture). 110 (54%) of the cohort was male, and 150 (74%) were White. Mean IQ was 106.10±14.01 and mean age at surgery and testing were 9.0±12.2 months and 10.9±4.0 years, respectively. Sagittal synostosis was associated with higher scores than metopic synostosis, with significant differences in verbal IQ (109.42±15.76 vs 101.37±10.41), full-scale IQ (108.32±14.44 vs 100.05±11.76), visuomotor integration (101.62±13.64 vs 92.44±12.07), visual perception (103.81±12.42 vs 95.87±11.23), and motor coordination (90.45±15.60 vs 84.21±15.44). Sagittal synostosis was associated with significantly higher scores for visuomotor integration (101.62±13.64 vs 94.95±10.24) and visual perception (103.81±12.42 vs 94.82±12.75) than unicoronal synostosis., Conclusions: Compared to patients with sagittal synostosis, patients with metopic synostosis exhibited lower scores in verbal IQ, full-scale IQ, visuomotor integration, visual perception, and motor control after surgical correction. Despite surgical correction for premature metopic suture fusion, the effect on the adjacent frontal lobe and white matter connections to other regions of the brain may have a lasting functional impact. Patients with unicoronal synostosis exhibited lower visuomotor integration and visual perception scores., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: Nonsyndromic unilateral coronal craniosynostosis (UCS) is a rare congenital disorder that results from premature fusion of either coronal suture. The result is growth restriction across the suture, between the ipsilateral frontal and parietal bones, leading to bony dysmorphogenesis affecting the calvarium, orbit, and skull base. Prior studies have reported associations between UCS and visual abnormalities. The present study utilizes a novel geometric morphometric analysis to compare dimensions of orbital foramina on synostotic versus nonsynostotic sides in patients with UCS. Computed tomography head scans of pediatric UCS patients were converted into 3-dimensional mesh models. Anatomical borders of left and right orbital structures were plotted by a single trained team member. Dimensions between synostotic and nonsynostotic sides were measured and compared. Medical records were examined to determine prevalence of visual abnormalities in this patient cohort. Visual abnormalities were reported in 22 of the 27 UCS patients (77.8%). Astigmatism (66.7%), anisometropic amblyopia (44.4%), and motor nerve palsies (33.3%) represented the 3 most prevalent ophthalmologic abnormalities. Orbits on synostotic sides were 11.3% narrower ( P < 0.001) with 21.2% less volume ( P = 0.028) than orbits on nonsynostotic sides. However, average widths, circumferences, and areas were similar between synostotic and nonsynostotic sides upon comparison of supraorbital foramina, infraorbital foramina, optic foramina, and foramina ovalia. Therefore, previously proposed compression or distortion of vital neurovascular structures within bony orbital foramina does not seem to be a likely etiology of visual abnormalities in UCS patients. Future studies will examine the role of ocular and/or neuro-ophthalmologic pathology in this disease process., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Smaller operative exposures associated with suturectomy for craniosynostosis may result in difficulties visualizing the prematurely fused suture during surgery. The authors report cases of suturectomy for lambdoid and metopic craniosynostosis in which neuronavigation or frameless stereotaxy was used to assist with incision planning and intraoperative localization of the fused suture. In both cases, neuronavigation integrated easily and safely into established workflows and was associated with complete suture release. To our knowledge, this is the first report of applying this noninvasive technology, which does not require cranial pinning or rigid fixation, to suturectomy, and the authors demonstrate its use as an adjunct, especially for surgeons beginning in practice. Larger studies are needed to determine if neuronavigation in suturectomy is associated with a clinically significant reduction in blood loss or operative time or an increase in the rate of complete suturectomy., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Unilateral lambdoid synostosis is the rarest form of single-suture craniosynostosis. Although various surgical approaches have been described, cranial vault remodeling remains the predominant approach. To aid in surgical planning, preoperative virtual surgical modeling using a patient's presenting computed tomography scan can be used to increase reconstructive precision and to reduce operative time. Presented is a 7-month-old male with unilateral lambdoid synostosis who underwent medically modeled cranial vault reconstruction., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

The diagnosis of late-presentation sagittal suture craniosynostosis (SCS) can be challenging, especially in the setting of subtle physical exam findings. The clinical significance of clinocephaly-a retro-coronal concavity along the midvault-in this context remains unknown. The aim of this study is to evaluate the predictive value of clinocephaly in identifying late-presentation SCS., A retrospective chart review of all patients >1 year old presenting to the craniofacial clinic with a concern for SCS was performed. The presence or absence of SCS in the setting of clinocephaly was recorded following diagnostic imaging. Student's t test, Chi Square test, and multivariate logistic regression analysis were performed to determine predictors for SCS., 75 patients met inclusion criteria. 32 patients (42.7%, 6% female) were diagnosed with SCS. No difference in age between patients with and without SCS was detected. Stratification of patients by age (1-2, 2-4, and >4 years) revealed a higher rate of SCS in younger patients (P = 0.04). The cephalic index (C.I.) of those with sagittal synostosis was significantly smaller but within the normal range, indicating a more scaphocephalic shape (P = 0.003). Logistic regression analysis revealed that C.I. was a strong predictor for SCS (P = 0.003). Of those with SCS, a mix of complete and partial fusion of the sagittal suture was appreciated., This study found that 42.7% of patients with clinocephaly had SCS. C.I. was the only predictor for SCS and unique suture fusion patterns were identified in those with SCS. This study suggests that clinocephaly should be considered a core component of the exam and work-up for SCS. Future studies aimed at evaluating the positive predictive value of this exam finding and identifying risk factors associated with late-presentation SCS are underway.

Spring-assisted cranioplasty (SAC) for the treatment of craniosynostosis uses internal springs to produce dynamic changes in cranial shape over several months before its removal. The purpose of this study was to report the first Egyptian experiences with SAC in the treatment of children with sagittal synostosis and evaluate the preliminary outcome. A total of 17 consecutive patients with scaphocephaly underwent SAC with a midline osteotomy along the fused sagittal suture and insertion of 3 springs with bayonet-shaped ends across the opened suture. Operative time, blood transfusion requirements and length of ICU, total hospital stay, and complications graded according to Oxford protocol classification were recorded. Spring removal was performed once re-ossification of the cranial defect occurred. All patients successfully underwent SAC without significant complications. The mean age at surgery was 6.8 months. The mean time of the spring insertion surgery was 63 minutes (SD 9.7). Blood transfusion was needed in less than half of the patients (41.2%).The mean duration of hospital stay was 3.2 days. The mean timing of spring removal was 5.5 months (SD 0.4). The mean time of the second surgery (spring removal) was 22.8 minutes (SD 3.6). In conclusion, SAC can easily be incorporated into the treatment armamentarium of craniofacial surgeons. The technique offers a safe and minimally invasive option for the treatment of sagittal craniosynostosis with the benefit of limited dural undermining, minimal blood loss, operative time, anesthetic time, ICU stay, and hospital stay., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Background: Various surgical techniques for cranial reconstruction of patients with bicoronal synostosis have been suggested. The outcome is, however, still often suboptimal., Methods: In a 5-month-old child with apert syndrome, following a craniotomy incision, lambdoid suturotomy was done bilaterally. Two springs were implanted bilaterally over the lambdoid sutures. Cephalic index was obtained from three-dimensional computed tomography scans, and photographs were analyzed for aesthetic evaluation., Result: The preoperative calvarial shape was hyperbrachycephalic. The CI improved from 92 to 83 units. Duration of surgery was 1 h 45 min, blood loss was 30 mL, and total hospital stay was 3 days. No major complications were observed. Spring removal was done at 6 months postoperatively and frontoorbital advancement performed., Conclusion: Spring-assisted cranioplasty for bicoronal synostosis is a safe and elegant technique, is less invasive than many other cranioplasties, and results in marked improvement in the calvarial shape., Competing Interests: None

Introduction: After treating a child with familial sagittal craniosynostosis, clinocephaly, and bilateral parietomastoid/posterior squamosal suture fusion, the authors wondered if major-suture synostosis and clinocephaly were associated with abnormal fusion of minor lateral calvarial sutures., Methods: The authors reviewed all preoperative volume-rendered head computed tomography reconstructions performed for craniosynostosis at their institution from 2010 through 2014 and determined whether the sphenoparietal, squamosal, and parietomastoid sutures were open, partially fused, or fused. The authors determined whether any sutures were abnormally fused based upon a previous study from their center, in which abnormal fusion was defined as either 1 of 3 abnormal fusion patterns or abnormally-early fusion. The authors then determined the rate of abnormal fusion of these sutures and whether abnormal fusion was associated with (1) major-suture craniosynostosis, (2) type of craniosynostosis (sutures involved; single-suture versus multisuture; syndromic versus nonsyndromic), and (3) clinocephaly., Results: In 97 included children, minor lateral sutures were abnormally fused in 8, or 8.2%, which was significantly higher than in children without craniosynostosis from our earlier study. Abnormal minor lateral suture fusion was not associated with the type of single-suture synostosis or with multisuture synostosis but was associated with syndromic synostosis. Four of 8 children with abnormal minor lateral suture fusion had multisuture synostosis and 6 had syndromic synostosis. Lateral sutures were abnormally fused in 1 of 4 subjects with clinocephaly, which was not significant., Conclusion: Abnormal minor lateral calvarial suture fusion is significantly associated with major-suture craniosynostosis, especially syndromic synostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Objective: Vertex epidural hematoma (VEDH) is a relatively uncommon type of intracranial hematoma. Because of its unique location and the potential of massive intraoperative bleeding, diagnosis and surgical intervention of VEDH may be challenging., Materials and Methods: A retrospective analysis of 32 patients with VEDH was undertaken to investigate the prognostic factor and therapeutic strategy of VEDH. Special attention was paid to the relationship between fracture pattern, surgical method, intraoperative blood loss and outcome., Results: Patients treated surgically had a higher percentage of consciousness disturbance and a significantly larger size of VEDH compared with patients treated conservatively (p = 0.029 and p < 0.0001, respectively). Bleeding from the injured superior sagittal sinus (SSS) was noted in six of nine patients (67%) with a linear fracture parallel to the SSS. Only one patient (20%) with a linear fracture crossing the SSS had bleeding from the injured SSS. Five of eight patients (63%) with sagittal suture diastasis experienced bleeding from the SSS. All patients with massive blood loss and six of seven patients developing intraoperative shock had copious bleeding from the injured SSS. All patients with intraoperative massive bleeding and shock underwent traditional "simple craniotomy". No patients undergoing "strip craniotomy" experienced massive bleeding. Thrombocytopenia (p = 0.008), headache (p = 0.015), consciousness disturbance (p = 0.043), pupil reactivity (p = 0.010), GCS score (p < 0.0001) and the relationship between skull fracture and the SSS (p = 0.037) were significant prognostic factors., Conclusion: Our study demonstrated GCS score may be a significant prognostic factor in patients with VEDH. Bleeding from the injured SSS occurred frequently in VEDH patients with a linear skull fracture parallel to the SSS or sagittal suture diastasis and could cause devastating hemorrhage. When operating on such patients, the surgical team should prepare for the possibility of massive blood loss and intraoperative shock. Bilateral parasagittal craniotomies with preservation of a central bone strip containing the sagittal suture (strip craniotomy) to allow application of tack-up sutures from the dura to the bone strip may be more suitable for VEDH evacuation., Competing Interests: Conflicts of interest The authors report no conflicts of interest concerning the materials or methods used in this study or the findings specified in this paper., (Copyright © 2022. Published by Elsevier Ltd.)

Aim: To compare, and to analyze the effects of fronto-orbital band anterior displacement in the operation of premature closure of coronal suture in infants., Material and Methods: A total of 31 infants with premature closure of coronal suture were randomly divided into two groups; experimental group (n=16) and control group (n=15). In the experimental group, the skull model was reconstructed by an imaging examination and three-dimensional (3D) printing technique before the operation, and the fronto-orbital band was anteriorly displaced during the operation to guide the surgical treatment of craniosynostosis. In the control group, the skull model was reconstructed by an imaging examination and 3D printing technique before the operation, and the fronto-orbital band was not anteriorly displaced during the operation by the same operator. The surgical effects of the two groups were compared., Results: During the 12-month follow up after the operation, the cephalic index of short head deformity in the experimental group was 80.7 ± 1.1, while that in the control group was 89.3 ± 4.5. There was a significant difference between the two groups., Conclusion: Fronto-orbital band anterior displacement may guide the operation of craniosynostosis and significantly improve the effectiveness of surgical treatment of children with premature closure of coronal suture, which is worth popularizing in the clinical management of cases.

Background: Sagittal craniosynostosis may present with complete or partial fusion of the sagittal suture, but relationships between degree of sagittal suture fusion and head shape are currently poorly described. The aim of this study was to characterize sagittal suture fusion patterns and determine associations with head shape in a cohort of patients with nonsyndromic sagittal craniosynostosis., Methods: Patients with nonsyndromic sagittal craniosynostosis at a tertiary care center with available computed tomography imaging were included in this study. The anterior and posterior distances of sagittal suture patency were measured along 3-dimensional parietal bones. Degree of sagittal suture fusion was compared to head shape characteristics, including cephalic index (CI), frontal bossing, and occipital bulleting., Results: Ninety patients (69 male) were included in this retrospective study. The sagittal suture was on average 85.6±20.1% fused, and 45 (50.0%) patients demonstrated complete fusion of the sagittal suture. CI was associated with increased degree of fusion for the anterior one-half (ρ=0.26, P =0.033) and anterior one-third (ρ=0.30, P =0.012) of the sagittal suture. Complete fusion of the anterior one-third of the sagittal suture predicted higher CI (β=13.86, SE=6.99, z =-0.25, P =0.047). Total degree of sagittal suture fusion was not predictive of CI or head shape in any analysis., Conclusions: Decreased fusion of the anterior one-third of the sagittal suture, but not total suture, may paradoxically predict increased severity of scaphocephaly as quantified by CI in nonsyndromic sagittal craniosynostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Background: Midpalatal suture (MPS) repair in growing patients after RPE has been previously reported. However, differences between young and adult patients for timing and pattern of MPS repair after rapid maxillary expansion are expected. The aim of this study was to evaluate the midpalatal suture repair pattern after miniscrew-assisted rapid palatal expansion (MARPE) in adult patients., Materials and Methods: The study included 21 patients (six males, 15 females) successfully treated with MARPE with a mean initial age of 29.1 years of age (SD = 8.0; range = 20.1-45.1). MPS repair was evaluated using maxillary axial and coronal sections derived from CBCT exams taken 16 months after the expansion (SD = 5.9). Objective and subjective assessments of MPS repair were performed. Objective assessments were performed measuring MPS bone density at anterior, median and posterior region of hard palate. Pre-expansion and post-retention bone density changes were evaluated using paired t tests (p < 0.05). Midpalatal suture bone repair was scored 0 to 3 considering, respectively, the complete absence of bone repair in the MPS, the repair of less than 50% of the MPS, the repair of more than 50% of the MPS and the complete repair of the MPS. Intra- and interexaminer reliability evaluation were assessed using Kappa coefficient., Results: The objective evaluation showed a significant higher bone density at the pre-expansion stage in all palatal regions. The reliability of the subjective method was adequate with intra- and interexaminer agreements varying from 0.807 to 0.904. Scores 1, 2 and 3 were found in 19.05%, 38.09% and 42.86% of the sample, respectively. The most common region demonstrating absence of bone repair was the middle third. The anterior third of the midpalatal suture was repaired in all patients., Conclusions: A decreased bone density was observed after the retention period when compared to pre-expansion stage. Most adult patients demonstrated incomplete repair of the midpalatal suture 16 months after MARPE. However, adequate bone repair covering more than half of the hard palate extension was observed in 80.95% of the patients., (© 2022. The Author(s).)

Objective: Early suturectomy with a rigid endoscope followed by orthotic cranial helmet therapy is an accepted treatment option for single-suture craniosynostosis. To the authors' knowledge, flexible endoscope-assisted suture release (FEASR) has not been previously described. Presented herein is their experience with FEASR for the treatment of isolated sagittal craniosynostosis., Methods: A retrospective analysis of the health records of patients who had undergone FEASR between March 2018 and December 2020 was performed. Patients under the age of 6 months who had been diagnosed with isolated sagittal synostosis were considered eligible for FEASR. Exclusion criteria included syndromic synostosis or multiple-suture synostosis. The cephalic index, the primary measure of the cosmetic endpoint, was calculated at prespecified intervals: immediately preoperatively and 6 weeks and 12 months postoperatively. Parental satisfaction with the cosmetic outcome was determined throughout the clinical follow-up and documented according to a structured questionnaire for the first 12 months., Results: A total of 18 consecutive patients met the criteria for study inclusion. The mean patient age at the time of surgery was 3.4 months (range 2-6 months). All patients underwent a wide craniectomy with no need to convert to an open procedure. The mean craniectomy width was 3.61 cm. Estimated blood loss ranged from 5 to 30 ml. The mean operative time was 75 minutes. No intraoperative complications were observed. The average length of stay was 2.6 days. The mean cephalic index was 67.7 preoperatively, 77.1 at 6 weeks postoperatively, and 76.3 at 1 year postoperatively. The mean percentage change in the cephalic index from preoperatively to the 12-month follow-up was 10.44 (p < 0.001). The mean follow-up was 17 months (range 12-28 months). All parents were satisfied with the cosmetic outcome of the procedure. No patients developed symptoms of raised intracranial pressure (ICP) or needed invasive ICP monitoring during the follow-up period. No patients required reoperation., Conclusions: In this modest single-hospital series, the authors demonstrated the feasibility of FEASR in treating sagittal synostosis with favorable cosmetic outcomes. The morbidity profile and resource utilization of the procedure appear similar to those of procedures conducted via traditional rigid endoscopy.

Aim and Scope: The prevalence of increased intra-cranial pressure (ICP) in patients with scaphocephaly is controversial. Here, based on anthropological material, we aimed to determine whether adults with non-operated sagittal synostosis show indirect signs of increased ICP., Materials and Methods: Thirty-eight dry skulls (21 skulls with sagittal craniosynostosis and 17 controls) were selected from the collections of the National Museum of Natural History (Paris, France). All skulls registered as 'fused sagittal suture' or 'scaphocephaly' in the registry of the Museum were included. All had total fusion of the sagittal suture. Controls were selected within skulls of similar origin (France), without visible craniofacial anomalies. The 38 skulls were CT-scanned using a standard medical CT-scan with a protocol dedicated to dry bone imaging. Eight radiological signs associated with raised ICP were assessed: (1) calvaria and (2) skull base thinning, (3) dorsum sellae erosion, (4) sella turcica lengthening, (5) copper beaten skull, (6) suture diastasis, (7) persistent metopic suture, and (8) small frontal sinus. Scaphocephaly was assessed based on head circumference, cranial index, intra-cranial volume, fronto-nasal angle, and inter-zygomatic distance. Linear and non-linear logistic models were used to compare groups., Results: 19/21 skulls with sagittal synostosis were significantly scaphocephalic. None of the criteria for ICP were significantly different in skulls with scaphocephaly relative to controls. Nevertheless, 5 individual skulls with scaphocephaly had ≥ 3 signs in favor of a history of raised ICP. We do not report the significant prevalence of indirect signs of raised ICP in adults with scaphocephaly. These results do not allow ruling out a history of early raised ICP or of minor prolonged raised ICP. Even though our findings support the fact that scaphocephaly is not significantly associated with prolonged raised ICP, individual cases (5/21) with clear signs in favor of a history of brain compression indicate that scaphocephaly correction should be considered as a functional procedure until the production of clear evidence. Cognitive assessments of non-operated adult patients with scaphocephaly could contribute to tackle this recurring question in craniofacial surgery., Competing Interests: Declaration of interests 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 © 2022. Published by Elsevier Masson SAS.)

Abstract: This paper aims to describe a new technique of 1-piece frontoorbital osteotomy with a small supraorbital burr hole, which resulted in improved safety during osteotomy of the anterior skull base. An 11 × 4 mm burr hole was created at the center of the orbit above the superior orbital rim using an "eyebrow key-window approach." Extradural dissection from the nasion to the medial side of the sphenoid ridge was performed through this hole, and dissection of the lateral side of the sphenoid ridge was completed from the edge of the coronal osteotomy. Osteotomy along the zygomaticofrontal and sphenofrontal sutures were performed using an oscillating saw with secure protection of the dura. Between December 2019 and June 2020, 3 patients with unilateral coronal craniosynostosis underwent 1-piece frontoorbital advancement with the eyebrow key-window approach. The median age was 11 months (8-15 months), median operation time was 249 minutes (244-255 minutes), and the average blood transfusion was 10.8 ml/kg (0-18 ml/kg). No complications were observed, and the technique was associated with improved safety of osteotomy around the anterior skull base for 1-piece frontoorbital advancement., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Background: Craniosynostosis is a deformity of the skull that occurs as a result of early fusion of one or more cranial sutures and can be accompanied by neurological deficits. Craniosynostosis can be classified as syndromic or nonsyndromic according to the type of suture involved. Surgical treatment of craniosynostosis in infants basically involves loosening and opening the fused sutures to reduce intracranial pressure, allow the brain to grow, and also fix the skull shape. However, in such cases there is a risk of resynostosis after surgery. According to the literature, resynostosis rates vary between 0% and 70%. In this study, we aimed to evaluate the reoperation rate in craniosynostosis cases treated surgically in our clinic., Methods: A retrospective analysis of 70 nonsyndromic craniosynostosis cases treated surgically in the Neurosurgery Department of Bursa Uludağ University from 2005 to 2019 was performed. All patients had undergone total cranial vault remodeling surgically and had been followed up for at least a year., Results: The study group included 70 patients, comprising 40 (57.1%) male and 30 (42.9%) female patients. The mean age of the group was 10.9 ± 7.8 months (range 3-34 months). Out of 70 patients, repeat surgery due to resynostosis had been performed once in 5 (7.1%) patients and twice in 1 (1.4%) patient., Discussion: It should be kept in mind that resynostosis may occur in patients who have been operated for craniosynostosis. Patients should be examined cosmetically and if necessary, radiologically in the follow-up. Further studies based on larger sample size are recommended for more quantitative data and better results.

The prevalence of sagittal and lambdoid suture craniosynostosis differs considerably, as they are notably the most and least prevalent sutures involved in isolated suture craniosynostosis, respectively. The goals of reconstructing the cranial vault in both entities is the same: to release the fused suture, expand cranial volume, restore normal head shape and morphology, and allow for normal growth of the cranial vault. With regards to sagittal suture synostosis, opinions vary on whether reconstruction should focus on either the anterior or poster cranial vault. In contrast, the poster cranial vault is always targeted in lambdoid suture craniosynostosis., (Copyright © 2022 Elsevier Inc. All rights reserved.)

While many centers nowadays offer minimally invasive techniques for the treatment of single suture synostosis, surgical techniques and patient management vary significantly. We provide an overview of how scaphocephaly treated with endoscopic techniques is managed in the reported series and analyze the crucial steps that need to be dealt with during the management process. We performed a review of the published literature including all articles that examined sagittal-suture synostosis treated with endoscopic techniques as part of single- or multicenter studies. Fourteen studies reporting results of 885 patients were included. We identified 5 key steps in the management of patients. A total of 188 patients were female and 537 male (sex was only specified in 10 articles, for 725 included patients, respectively). Median age at surgery was between 2.6 and 3.9 months with a total range from 1.5 to 7.0 months. Preoperative diagnostics included clinical and ophthalmologic examinations as well as neuropsychological and genetic consultations if needed. In 5 publications, a CT scan was routinely performed. Several groups used anthropometric measurements, mostly the cephalic index. All groups analyzed equally recommended to perform endoscopically assisted craniosynostosis surgery with postoperative helmet therapy in children < 3 months of age, at least for non-syndromic cases. There exist significant variations in surgical techniques and patient management for children treated endoscopically for single suture sagittal synostosis. This heterogeneity constitutes a major problem in terms of comparability between different strategies., (© 2022. The Author(s).)

Abstract: Endoscopic suturectomy is a minimally invasive surgical treatment for single-suture craniosynostosis in children between 1 and 4 months of age. This study sought to characterize the role played by diagnostic imaging in facilitating early surgical management with endoscopic suturectomy. The authors also characterized the overall diagnostic utility of imaging in patients assessed for abnormal head shape at their institution, regardless of surgical status. A retrospective cohort of children diagnosed with singlesuture synostosis undergoing either primary endoscopic suturectomy or open calvarial reconstruction at the authors' institution from 1998 to 2018 was first reviewed. Of 132 surgical patients, 53 underwent endoscopic suturectomy and 79 underwent open repair. There was no difference in the proportion of endoscopic and open surgery patients imaged preoperatively before (24.5% versus 35.4%; P = 0.24) or after (28.3% versus 25.3%; P = 0.84) craniofacial assessment. Stratifying by historical epoch (1998-2010 versus 2011-2018), there was also no difference found between preoperative imaging rates (63.6% versus 56.4%; P = 0.35). In another cohort of 175 patients assessed for abnormal head shape, 26.9% were imaged to rule out craniosynostosis. Positive diagnostic imaging rates were recorded for suspected unicoronal (100%), metopic (87.5%), lambdoidal (75.0%), sagittal (63.5%), multisuture (50%), and bicoronal (0%) synostosis. The authors conclude that the use of diagnostic imaging at their institution has not increased despite higher utilization of endoscopic suturectomy and need for expedient identification of surgical candidates.However, their results suggest that imaging may play a greater diagnostic role for suspected bicoronal, sagittal, and multi-sutural synostosis among sutural subtypes of synostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Abstract: The posterior intraoccipital synchondrosis (PIOS) is a cartilaginous division separating the exoccipital and supraoccipital bones, allowing for flexibility of the cranial base at birth and which later ossifies in adolescence. The authors report a case of right PIOS synostosis that resembled right lambdoid synostosis, with left occipital bossing in a healthy, six-month-old female with an unremarkable birth history and no antecedent trauma. An initial referral was made from the pediatrician to oncology because of a concern over the presence and growth of a possible mass at the left occiput. Over 8 months, this mass grew and became more firm, accompanied by left occipital bossing and right occipital flattening. A computed tomography was obtained, which demonstrated the fusion of the PIOS, prompting a referral to plastic surgery. Cranial vault remodeling with switch cranioplasty was performed at age 14 months, complicated only by a superficial infection along the suture line. There are exceedingly few reports of PIOS synostosis, with occipital osteodiastasis related to birth trauma as the only prior explanation for this condition. With no birth trauma and delayed onset, our case likely represents idiopathic PIOS synostosis. With the many similarities in presentation, the surgical approaches commonly used for lambdoid synostosis, particularly switch cranioplasty, are suitable solutions to PIO synchondrosis with optimal functional and aesthetic outcomes., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Abstract: Scaphocephaly is the morphological consequence of premature sagittal suture fusion. Morphologic and morphometric studies on the cranial vault in scaphocephaly flourish in the literature. However, few studies are available on the cranial base in a scaphocephalic population, let alone the morphometry of its fossae. Therefore, this study aimed to analyze and compare the morphometry of the anterior, middle, and posterior cranial fossae (ACF, MCF, and PCF) in patients with scaphocephaly.The length and width of the ACF, MCF, and PCF were measured using fixed anatomical landmarks on the two-dimensional computed tomography scans of 24 consecutive patients diagnosed with isolated sagittal synostosis between 2014 and 2020, and 14 controls.A comparison of the results between patients with scaphocephaly and the controls showed that the ACF and PCF lengths increased significantly ( P = 0.041 and P = 0.018) in patients with scaphocephaly. No differences in the MCF lengths were observed ( P = 0.278; 0.774). When compared by the degree of severity, the ACF and PCF lengths were significantly increased ( analysis of variance [ANOVAI, P = 0.033; post-hoc, P = 0.013 and ANOVA, P = 0.015; post-hoc, P = 0.036) in scaphocephalic patients within the severe group as opposed to the control group.The morphometric data obtained indicate a preponderance of deformity in the ACF and PCF with elongation along the anteroposterior plane (lengths) in scaphocephalic patients. Minimal changes were observed in the transverse plane (widths) in scaphocephaly versus controls. This data could aid craniofacial surgeons in understanding the affectation of the cranial fossae and influencing the decision on the most suitable method of corrective modality., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

This paper describes a unique case of craniosynostosis in a female skull in which sagittal sutures were completely fused by adolescence. Despite sagittal synostosis, the skull was of normal shape and size. Regarding craniometric features, the synostotic normocephalic skull was markedly different than that of scaphocephalic skulls which typically result from premature obliteration of the sagittal suture.

Children with syndromic forms of craniosynostosis undergo a plethora of surgical interventions to resolve the clinical features caused by the premature fusion of cranial sutures. While surgical correction is reliable, the need for repeated rounds of invasive treatment puts a heavy burden on the child and their family. This study explores a non-surgical alternative using mechanical loading of the cranial joints to prevent or delay craniofacial phenotypes associated with Crouzon syndrome. We treated Crouzon syndrome mice before the onset of craniosynostosis by cyclical mechanical loading of cranial joints using a custom designed set-up. Cranial loading applied to the frontal bone partially restores normal skull morphology, significantly reducing the typical brachycephalic appearance. This is underpinned by the delayed closure of the coronal suture and of the intersphenoidal synchondrosis. This study provides a novel treatment alternative for syndromic craniosynostosis which has the potential to be an important step towards replacing, reducing or refining the surgical treatment of all craniosynostosis patients., (© 2022. The Author(s).)