Unicoronal Synostosis Research Articles

Visual Outcomes of Children With Craniosynostosis.

Craniosynostosis can impact the visual development of a child. Historically, children with craniosynostosis, particularly when associated with a syndrome, had a significant risk of vision loss. The authors aimed to study the incidence of ophthalmic pathology in a modern, multidisciplinary craniosynostosis practice. Children aged 7 to 13 years attending face-to-face ophthalmic craniofacial clinics between February 2020 and June 2021 were included in a retrospective case note review. Visual acuity, ocular alignment, optic nerve function, and retinal nerve fiber layer (RNFL) condition using optical coherence tomography (OCT) were recorded. Forty-three children (30 girls) were assessed at a median age of 10.3 years (7.8-13.1). Eleven children had unicoronal synostosis, 15 had single-suture synostosis not involving the coronal, 14 had multisuture synostosis involving the coronal, and 3 had multisuture synostosis not involving the coronal. Thirty-two out of 43 had craniofacial surgery. Sixty-seven percent required glasses. Forty-nine percent had strabismus, 11/43 (26%) had squint surgery, and 2/43 (5%) had tarsorrhaphy for corneal protection. Four out of 43 (9%) had papilloedema detected; however, at the final review, 15/68 (22%) eyes showed RNFL changes on OCT imaging, none of whom had optic atrophy. Two children did not meet UK driving standards due to refractive amblyopia; no children were registered as sight impaired. In this cohort, optic atrophy and visual loss due to exposure keratopathy were not seen. A high incidence of strabismus, glasses wear, and amblyopia is persistent. Binocular visual impairment was rare in this cohort: 95% met UK driving standards. Visual outcomes appear to be improving coinciding with improved craniofacial care alongside multidisciplinary team working.

The Evolution of Unicoronal Synostosis Correction: Long-Term Aesthetic Outcomes of Fronto-Orbital Distraction Versus Traditional Advancement.

This study compares the long-term aesthetic outcomes of patients with unicoronal synostosis (UCS) who underwent fronto-orbital distraction osteogenesis (FODO) versus traditional fronto-orbital advancement and remodeling (FOAR). Patients treated for nonsyndromic UCS from 2009 to 2023 were retrospectively reviewed. Perioperative and complication characteristics were compared between all patients who underwent FOAR, open FODO, and endoscopic-assisted FODO ("endo-FODO"). Aesthetic outcomes at greater than five years postoperatively were compared between patients who underwent FODO and a contemporaneous cohort of age-matched controls who underwent FOAR. Differences between pre- and postoperative periorbital symmetry ratios, canthal tilt symmetry, orbital dystopia angle (ODA), and frontal bossing angle were calculated, with positive differences representing postoperative improvement. Forty-one patients were treated at a median age of 9.7, 5.4, and 5.4 months in the FOAR, open FODO, and endo-FODO groups, respectively. Among 28 patients photographed at a median of 6.3 years postoperatively, the FODO cohort demonstrated greater margin-to-reflex distance 1 symmetry (6.5 [-9.3, 0.0] vs. -13.9 [-22.9, -11.5], p=0.010) and canthal tilt symmetry (0.9° [0.2°, 2.9°] vs. 3.3° [2.3°, 5.3°], p=0.004) postoperatively as well as ODA correction (5.4° [4.0°, 7.5°] vs. 3.0° [2.5°, 4.4°], p=0.027) compared to the FOAR cohort. Fewer patients in the FODO cohort exhibited temporal hollowing postoperatively compared to the FOAR cohort (14% vs. 71%, p=0.002). Compared to FOAR, FODO was associated with greater periorbital symmetry, greater orbital dystopia correction, and reduced temporal hollowing in the long-term. Follow-up to cranial maturity is needed to adequately compare the two techniques.

Spring-Assisted Cranioplasty in Unicoronal Synostosis: Analysis of Outcomes

Background: Spring-assisted cranioplasty and successful long-term outcomes for sagittal synostosis has been well described in recent literature. However, there is a paucity of data regarding the application of springs to unicoronal synostosis. Unicoronal synostosis (UCS) presents unique challenges in its causation of skull base scoliosis and orbital asymmetry. Many surgeons feel that the fronto-orbital advancement and vault remodeling is the most effective and reliable strategy to counteract these changes. Here we present our experience and outcomes with spring cranioplasty for unicoronal synostosis. Materials and methods: A retrospective review of non-syndromic unicoronal synostosis patients undergoing spring-assisted cranioplasty was performed. Preoperative and postoperative intracranial volume (ICV), orbital volume (OV), orbital height (OH), orbital width (OW), midface twist (MFT), and skull-base twist (SBT) were measured from DICOM CT data. Analysis was performed with Materialize software (Leuven, Belgium). Paired t-tests were performed using Excel. Results: Ten patients (5 females, 5 males) had springs placed at 100.1 days (avg). Average ICV: preop: 608.2 ± 101.4 cc, postop: 995.2 ± 166.1 cc. Differences in orbital volume and height in the preop affected orbit versus non-affected orbit was significant ( P = .01, P ≤ .001 respectively) whereas no significant difference between the two sides postoperatively was observed ( P = .19, P = .58 respectively). Average preop MFT was 81.1 ± 2° and postop was 87.6 ± 2.6° and preop SBT was 171.9 ± 2.7° and postop was 177.6 ± 2.6°; both showing significant improvement after spring cranioplasty (both P ≤ .001). Conclusion: Our experience and early outcomes with spring cranioplasty for UCS demonstrates that when used in younger patients (<5 months) cranial springs can be a safe and effective strategy to minimize skull base scoliosis, facial scoliosis, and orbital asymmetry, as well as increase intracranial volume. Although long-term data needs to be assessed, we are optimistic that early correction of cranial base scoliosis will translate to stable results as patients reach dentoskeletal maturity.

Understanding the heterogenicity of unicoronal synostosis – A morphometric analysis of cases compared to controls

BackgroundPreoperative severity of unicoronal synostosis varies greatly and involves the frontal bone, skull base and orbits. Degree of deformity affects long-term morphological and functional outcome after surgery. The aim of this study was to describe the morphological heterogenicity and investigate its relation to patient-specific factors. Materials and methodsIn this retrospective cohort study, non-syndromic unicoronal synostosis patients treated between 2006-2022 at Necker Hospital, France, or Uppsala University Hospital, Sweden, were included and matched to controls. Severity of skull base, orbital and posterior skull asymmetry, degree of anterior plagiocephaly and Harlequin deformity, lateralization, head circumference, age, timing of metopic fusion and fusion of peri-pterionic sutures were investigated. ResultsNinety-five patients and ninety-three controls were included. Skull base asymmetry was linearly related to orbital asymmetry (p < 0.001), correlated with earlier CT scans (p = 0.004), anterior (p < 0.001) and posterior (p = 0.03) plagiocephaly. Posterior plagiocephaly was more common in patients (31%) compared with controls (5%) (p < 0.001). A patent metopic suture above nine months of age was associated with severe Harlequin deformity (p = 0.04) and lower head circumference when fused (p = 0.03). Fronto-sphenoidal suture fusion was associated with later CT scans (p < 0.001) and less skull base asymmetry (p = 0.002). Spheno-parietal fusion was correlated with decreased skull base asymmetry. Right lateralization was more common in females. ConclusionHeterogenicity of unicoronal synostosis seems to be predominantly explained by variability in skull base morphology. Peri-pterionic fusions might limit deformity.

Alterations in Sphenoid Anatomy in Craniosynostosis: Implications for Fronto-Orbital Advancement.

Fronto-orbital advancement involves removal of the fronto-orbital bandeau. Visualization of the saw blade is lost as it passes through the fronto-orbital-sphenoid junction (FOSJ), placing the temporal lobe at risk of injury. We aim to provide a 3D analysis of the space surrounding this osteotomy to differentiate various types of craniosynostoses. Retrospective cohort. Institutional. Thirty patients with isolated unicoronal synostosis, nonsyndromic bicoronal synostosis, metopic synostosis, Apert syndrome, Crouzon syndrome, and Muenke syndrome. CT scans conducted between 2 months to 2 years of age were 3D reconstructed to compare craniometrics against normal controls. Craniometrics. The mean bone thickness of the FOSJ at the level of the supraorbital rim was significantly small for the Apert, unicoronal and bicoronal groups. The mean vertical height of the middle cranial fossa from the lesser sphenoid wing was significantly greater in the unicoronal group. The mean vertical height of the tip of the temporal lobe from the lateral sphenoid ridge was greater in the unicoronal, isolated bicoronal, and Apert groups. The mean corneal protrusion beyond the lateral orbital rim was significantly greater in the Apert and unicoronal groups. The mean horizontal depth of the orbit was smallest in the Apert group. The mean vertical distance between the dacryon and the foramen cecum, and the mean volume of temporal lobe beneath the sphenoid shelf were the largest in the Apert group. Patients with Apert syndrome have the most unfavorable morphology of the anterior and middle cranial fossae.

Speech and Language Development, Hearing, and Feeding in Patients With Genetically Confirmed Crouzon Syndrome With Acanthosis Nigricans: A 36-Year Longitudinal Retrospective Review of Patients at the Oxford Craniofacial Unit.

Crouzon syndrome with acanthosis nigricans (CAN) is caused by the specific mutation c.1172C>A (p.Ala391Glu) in the fibroblast growth factor receptor 3 gene, and has an estimated prevalence of 1:1,000,000 births. Most cases occur de novo; however, autosomal dominant inheritance may occur. The clinical presentation typically includes craniosynostosis, midface and maxillary hypoplasia, choanal atresia/stenosis, hydrocephalus, and intracranial hypertension. Patients develop acanthosis nigricans, a hyperkeratotic skin disorder. The authors present the first known study to investigate the speech, language, hearing, and feeding of patients with CAN. A retrospective case-note review of patients with a genetically confirmed diagnosis of CAN attending the Oxford Craniofacial Unit during a 36-year period (1987-2023) was undertaken. Participants were 6 patients with genetically-confirmed CAN (5 females, 1 male), all cases arose de novo. All patients had craniosynostosis (n = 5/6 multisuture synostosis, n = 1/6 left unicoronal synostosis). Hydrocephalus was managed through ventriculoperitoneal shunt in 67% (n = 4/6) of patients, and 67% (n = 4/6) had a Chiari 1 malformation. Patients had a complex, multifactorial feeding history complicated by choanal atresia/stenosis (100%; n = 6/6), and significant midface hypoplasia. All patients required airway management through tracheostomy (83%; n = 5/6); and/or continuous positive airway pressure (67%; n = 4/6). All patients underwent adenotonsillectomy (100%; n = 6/6). Initial failure to thrive, low weight, and/or height were seen in 100% (n = 6/6) patients; 80% (n = 4/5) had reflux; 100% (n = 6/6) had nasogastric, or percutaneous endoscopic gastrostomy based feeding during their treatment journey. All patients had hearing loss (100%; n = 6/6). Early communication difficulties were common: receptive language disorder (50%; n = 3/6); expressive language disorder (50%; n = 3/6); and speech sound disorder in 50% (n = 3/6)-necessitating the use of Makaton in 80% of patients (n = 3/5). Patients with CAN experience significant respiratory, neurological, and structural obstacles to hearing, speech, language, and feeding. The authors present a recommended pathway for management to support patients in these domains.

Comparing the Squamosal to Coronal Suture Relationship between Unicoronal and Bicoronal Synostosis

Comparing the Squamosal to Coronal Suture Relationship between Unicoronal and Bicoronal Synostosis

Image processing and machine learning for diagnosis and screening of craniosynostosis in children

Objectivecraniosynostosis (CSO) is a congenital disorder resulting from early closure of cranial sutures in newborns, while could cause significant cosmetic and neurodevelopmental problems. As a standard method, different craniometric indices are measured directly from child head or from their 3D CT scan of skull for diagnosis or in post-operative follow-up period. We propose a novel telehealth-compatible deep learning neural network-based method for identifying different craniometric indices in non-syndromic CSO patients 2D photographic data. Methods624 pre-operative and post-operative top-down cranial digital images of 145 craniosynostotic infants (59 sagittal, 55 metopic and 31 unicoronal synostosis) who had surgery at Mofid Children’s Hospital, Tehran, Iran were used in a deep learning neural network algorithm. Head boundary was defined by a faster region-based convolutional neural network (Faster R-CNN) and then different cranial indices (cranial index (CI), cranial vault asymmetry index (CVAI), anterior-posterior width ratio (APWR), anterior-midline width ratio (AMWR) and left–right height ratio (LRHR)) were calculated from segmented images. Accuracy, sensitivity and specificity were calculated for software versus specialist data association between cranial indices were evaluated with inter-class correlation coefficients. ResultsThe head border was segmented in the proposed images with accuracy of 88.67 ± 1.94 in comparison with standard hand made procedure with a sensitivity of 86.91 ± 3.75 and specificity of 88.60 ± 4.81. Among calculated cranial indices, significant decrease in CI value is most useful for diagnosis of sagittal synostosis (CIsagittal = 71.97 ± 4.33), significant increase in CVAI value and significant decrease in LRHR value is most appropriate for unicoronal suture synostosis diagnosis (CVAIunicoronal=6.79±3.80 and LRHRunicoronal = 0.91 ± 0.05) and significant decrease in APWR and AMWR values could be indicator of metopic synostosis (AMWRmetopic= 0.77 ± 0.04 and APWRmatopic = 0.83 ± 0.05). ConclusionDeep learning neural network algorithms could have high levels of capability in calculating cranial indices from routine 2D digital images of non-syndromic craniosynostotic children and act as a substitute for optical scanner or 3D CT-based craniometrics. This method could act as a corner stone for developing a software for a mobile platform that that would allow for screening by tele-medicine or in a primary care setting.

Correction of Unicoronal Synostosis With Springs: Two Patients With Improved Facial Symmetry.

Surgical correction of unicoronal synostosis (UCS) entails extensive cranioplasties which do not address facial scoliosis. This paper presents the first results with springs that motivated the shift from extensive cranioplasties to dynamic techniques for surgical correction of UCS. Two cases of UCS were operated with a linear osteotomy combined with springs. The deviation in facial symmetry (orbital dystopia angle) and skull base angles were measured on pre and postoperative computed tomography scans until 3 years of age. The facial scoliosis was corrected. At spring removal, the orbital dystopia angle had gone from a 9.2 to 13.2-degree deviation preoperatively to a 0.5 to 0.9-degree overcorrection compared with the ideal 0-degree deviation. Also, the skull base deviation improved. Linear osteotomy combined with springs corrects the facial scoliosis in UCS. These cases indicate that dynamic methods may be beneficial for improving the results of surgical correction of UCS.

Quantitative Detection and Follow-Up of Intracranial Hypertension in Craniosynostosis: An Optical Coherence Tomography Study.

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. 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. 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). 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. Diagnostic, III.

Automated three-dimensional analysis of facial asymmetry in patients with syndromic coronal synostosis: A retrospective study

Craniosynostosis, characterized by premature fusion of one or more cranial sutures, results in a distorted skull shape. Only three studies have assessed facial asymmetry manually in unicoronal synostosis patients. It is therefore important to understand how uni- and bicoronal synostosis affect facial asymmetry with a minimum risk of human bias.An automated algorithm was developed to quantify facial asymmetry from three-dimensional images, generating a mean facial asymmetry (MFA) value in millimeters to reflect the degree of asymmetry. The framework was applied to analyze postoperative 3D images of syndromic patients (N = 35) diagnosed with Muenke syndrome, Saethre-Chotzen syndrome, and TCF12-related craniosynostosis with respect to MFA values from a healthy control group (N = 89).Patients demonstrated substantially higher MFA values than controls: Muenke syndrome (unicoronal 1.74 ± 0.40 mm, bicoronal 0.77 ± 0.21 mm), Saethre-Chotzen syndrome (unicoronal 1.15 ± 0.20 mm, bicoronal 0.69 ± 0.16 mm), and TCF12-related craniosynostosis (unicoronal 1.40 ± 0.51 mm, bicoronal 0.66 ± 0.05 mm), compared with controls (0.49 ± 0.12 mm). Longitudinal analysis identified an increasing MFA trend in unicoronal synostosis patients.Our study revealed higher MFA in syndromic patients with uni- and bicoronal synostosis compared with controls, with the most pronounced MFA in Muenke syndrome patients with unilateral synostosis. Bicoronal synostosis patients demonstrated higher facial asymmetry than expected given the condition’s symmetrical presentation.

Improving Cranial Vault Remodeling for Unilateral Coronal Craniosynostosis-Introducing Automated Surgical Planning.

Cranial vault remodeling (CVR) for unicoronal synostosis is challenging due to the asymmetric nature of the deformity. Computer-automated surgical planning has demonstrated success in reducing the subjectivity of decision making in CVR in symmetric subtypes. This proof of concept study presents a novel method using Boolean functions and image registration to automatically suggest surgical steps in asymmetric craniosynostosis. The objective of this study is to introduce automated surgical planning into a CVR virtual workflow for an asymmetric craniosynostosis subtype. Virtual workflows were developed using Geomagic Freeform Plus software. Hausdorff distances and color maps were used to compare reconstruction models to the preoperative model and a control skull. Reconstruction models were rated as high or low performing based on similarity to the normal skull and the amount of advancement of the frontal bone (FB) and supra-orbital bar (SOB). Fifteen partially and fully automated workflow iterations were carried out. FB and SOB advancement ranged from 3.08 to 10.48mm, and -1.75 to 7.78mm, respectively. Regarding distance from a normal skull, models ranged from .85 to 5.49mm at the FB and 5.40 to 10.84mm at the SOB. An advancement of 8.43mm at the FB and 7.73mm at the SOB was achieved in the highest performing model, and it differed to a comparative normal skull by .02mm at the FB and .48mm at the SOB. This is the first known attempt at developing an automated virtual surgical workflow for CVR in asymmetric craniosynostosis. Key regions of interest were outlined using Boolean operations, and surgical steps were suggested using image registration. These techniques improved post-operative skull morphology.

Long-term neurocognitive outcomes in 204 single-suture craniosynostosis patients.

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). 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. 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. 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.

Cognitive performance in preschoolers with non-syndromic craniosynostosis undergoing surgery: A comparison with typically developing children

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.

3-Dimensional Morphometric Outcomes After Endoscopic Strip Craniectomy for Unicoronal Synostosis.

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

Management of Unicoronal and Metopic Synostoses: Minimally Invasive Approaches.

Early endoscopic-assisted correction of unicoronal and metopic synostosis is an excellent, safe, cost-effective, and highly effective option for affected patients. Although open calvarial remodeling has a place in the armamentarium of the craniofacial team, the skull base changes seen in endoscopic-assisted techniques are unparalleled. The procedures are associated with low morbidity and no mortality. There is minimal blood loss, decreased operating time, significantly reduced blood transfusion rates, decreased hospitalization length, decreased cost, and less pain and swelling. Early diagnosis and referral for surgical evaluation are critical to obtaining these results.

Establishing Standards of Care in Craniosynostosis: Results from a Survey of ERN CRANIO Member Institutions

European Reference Networks (ERNs) are networks involving hospitals with particular expertise in rare conditions. ERN-CRANIO focuses on rare disorders of the skull and face including craniosynostosis. We undertook a pilot study in the form of an electronic survey to understand current practice in craniosynostosis management across ERN-CRANIO, which at the time consisted of 29 member institutions across 11 countries. Most (19 of 29) units replied; however, some answered the survey only partially. The majority (87.5%) of units have specific management protocols. For single-suture cases, 8 of 15 units see >50 new cases per year, 4 of 15 see 21-50 cases, and 3 of 15 see fewer than 20 cases. Duration of follow-up ranges from age 10 years or less (3 of 6) to indefinite (2 of 6). A variety of surgical techniques are used. For sagittal synostosis, the endoscopic and helmet technique is the most common (5 of 14), and for metopic and unicoronal synostosis, preferred by 9 of 14 centers was the frontoorbital remodeling (bandeau) technique. For multisutural syndromic craniosynostosis, 2 of 16 centers see >20 new cases per year, 4 of 16 see 11-20 cases, 5 of 16 see 6-10 cases, and 4 of 16 see fewer than 5 cases. Most centers (12 of 15) either never discharge syndromic patients or follow until adulthood. Eleven of 14 units perform prophylactic vault expansion, while 3 of 14 wait for clinical indication. Nine of 13 units operate at 6-12 months. Again, a wide variety of techniques are used, most commonly frontal advancement (4 of 13) initially. This study provides a useful snapshot of current standards of care in craniosynostosis across the high-volume centers of the ERN. Going forward, these results can be used to direct more detailed analysis of current practice, which will then be useful for constructing a management guideline for patients presenting with both single-suture and multisutural craniosynostosis.

Comparison between different absorbable plating systems in craniofacial surgery-an original research

Aim: The purpose of this in vitro present research was to evaluate different absorbable plating systems in different craniofacial surgeries. Methodology: A sample of patients with single-suture nonsyndromic craniosynostosis treated using either Resorb-X or Delta bioresorbable plating systems were reviewed. Only patients with preoperative, immediate, and long-term 3-dimensional photographic images or computed tomography scans were included. A comparison of plating system outcomes was performed to determine the need for clinic and emergency room visits, imaging obtained, and incidence of subsequent surgical procedures due to complications. Results: Forty-six patients (24 Resorb-X and 22 Delta) underwent open repair with bioabsorbable plating for single suture craniosynostosis. The mean age at each imaging time point was similar between the 2 plating systems (P&gt;0.717). Deformity specific measures for sagittal (cranial index), metopic (inter fronto temporale), and unicoronal (frontal asymmetry) synostosis were equivalent between the systems at all time points (0.05&lt;P&lt;0.904). A single Delta patient developed bilateral scalp cellulitis and abscesses and subsequently required operative intervention and antibiotics. Conclusion: Bioabsorbable plating for craniosynostosis in children as well as craniofacial surgeries are effective and has low morbidity.

Examining the Role of Early Diagnostic Imaging for Craniosynostosis in the Era of Endoscopic Suturectomy: A Single Institution Experience.

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.

The use of a single-piece bone flap for cranial reshaping in anterior craniosynostosis patients: clinical experience and a description of a novel technique

BackgroundCraniosynostosis is known as premature closure of one or more of the cranial sutures. Anterior craniosynostosis involves anterior plagiocephaly and trigonocephaly. One of the issues in anterior craniosynostosis skull reshaping is maintaining an aesthetically pleasing forehead curve. Therefore, in this article, we demonstrate our novel technique to use a single-piece bone flap for cranial reshaping of the anterior mold in patients diagnosed with anterior craniosynostosis. A retrospective record review of patients who underwent single piece bone flap cranial reshaping for correction of unicoronal synostosis (UCS) and metopic synostosis (MS) at an Academic Institute in Riyadh, Saudi Arabia, between 2018 and 2020, was conducted.ResultsSix non-syndromic consecutive patients were included. Three of the patients had MS. The mean age at surgery was 11.16 months (range, 6–19 months). The average OR time was 315 min (range, 263–368 min). The average intraoperative blood loss was 225 ml (range, 100–400 ml). All patients had achieved acceptable functional and aesthetic results.ConclusionOur novel technique is an innovative and efficient reconstructive technique to simultaneously address MS and UCS and minimize intraoperative bleeding and surgery time. However, more studies with more cases are required.