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Objective Several severity metrics have been developed for metopic craniosynostosis, including a recent machine learning-derived algorithm. This study assessed the diagnostic concordance between machine learning and previously published severity indices. Design Preoperative computed tomography (CT) scans of patients who underwent surgical correction of metopic craniosynostosis were quantitatively analyzed for severity. Each scan was manually measured to derive manual severity scores and also received a scaled metopic severity score (MSS) assigned by the machine learning algorithm. Regression analysis was used to correlate manually captured measurements to MSS. ROC analysis was performed for each severity metric and were compared to how accurately they distinguished cases of metopic synostosis from controls. Results In total, 194 CT scans were analyzed, 167 with metopic synostosis and 27 controls. The mean scaled MSS for the patients with metopic was 6.18 ± 2.53 compared to 0.60 ± 1.25 for controls. Multivariable regression analyses yielded an R-square of 0.66, with significant manual measurements of endocranial bifrontal angle (EBA) (P = 0.023), posterior angle of the anterior cranial fossa (p Conclusions The machine learning algorithm offers an objective assessment of morphologic severity that provides a reliable composite impression of severity. The generated score is comparable to other severity indices in ability to distinguish cases of metopic synostosis from controls.

Metopic craniosynostosis is the second most frequent type of craniosynostosis. When the phenotypic presentation has been deemed severe the treatment is surgical in nature and is performed in infancy with fronto-orbital advancement and cranial vault remodeling. At the time of this writing, there is no consensus regarding an objective evaluation system for severity, diagnostic criteria, or indications for surgery. This study aims to review the anthropometric cranial measurements and the relative diagnostic criteria/classification of severity/surgical indications proposed so far for this skull malformation, and to investigate if there is any scientific support for their utility.

OBJECTIVE The objective of this study was to propose a new skull outline–based method to objectively quantify complex 3D skull shapes and frontal and supraorbital retrusion in metopic craniosynostosis using 3D photogrammetry. METHODS A standard section from 3D photogrammetry, which represents the trigonocephalic shape, was used in this study. From the midpoint of the area of this section, half diagonals were calculated to the skull outline at 5° increments in the anterior half of the head. These half diagonals were used to create a sinusoidal curve, and the area under the sinusoidal curve (AUC) was used to represent the mathematical expression of the trigonocephalic head shape. The AUC from 0° to 180° (90° from the midline to each side) was calculated and is referred to as AUC0→180. The AUC from 60° to 120° (30° from the midline to each side) was also calculated and is referred to as AUC60→120. A total of 24 patients who underwent endoscopic strip craniectomy and 13 age- and sex-matched controls were included in the study. The AUC values obtained in patients at different time points and controls were analyzed. RESULTS The mean preoperative AUC60→120 and AUC0→180 in the patients were significantly lower than those in control individuals. The increase in both AUC60→120 and AUC0→180 values is statistically significant at the discontinuation of helmet therapy and at final follow-up. Receiver operating characteristic curve analysis indicated that AUC60→120 is a more accurate classifier than AUC0→180. CONCLUSIONS The proposed method objectively quantifies complex head shape and frontal retrusion in patients with metopic craniosynostosis and provides a quantitative measure for follow-up after surgical treatment. It avoids ionizing radiation exposure.

Background Minimal invasive suturectomy is one of the many surgical approaches to treat isolated single suture craniosynostosis. This approach can be technically challenging in metopic craniosynostosis given the narrow corridor and steep angle of the forehead. New instruments such as the Piezosurgery device (Mectron) have the potential to improve the ability to safely perform minimal invasive surgery in metopic craniosynostosis. Objective To demonstrate the safety and efficacy of Piezosurgery technology in minimal invasive suturectomy for nonsyndromic metopic suture craniosynostosis and to describe our technique. Methods A retrospective chart review was performed of all the single metopic suturectomies performed at our single institution from March 2018 to November 2019. Pre-, intra-, and postoperative data were collected to assess the safety of Piezosurgery. Results The cohort consisted of 12 patients with an average of 95.25 d old and an average weight of 6.2 kg. A total of 91.7% were male, and 91.7% were Caucasian. There were no intraoperative or postoperative Piezosurgery device-related complications in the entire cohort. Conclusion The use of the Piezosurgery instrument was safe in this cohort of minimal invasive metopic suturectomy. This device has greatly increased the ease of this procedure in our hands.

Frontoorbital advancement (FOA) was mainstay treatment of coronal and metopic craniosynostosis. There are several techniques and materials such as absorpable plate, surgical suture fixation, and osteogenesis distraction. Each techniques and materials has advantages to clinical outcome. This study describe our experience in FOA using surgical suture in our Institution.

Surgical correction of metopic craniosynostosis typically involves open cranial vault remodeling. Accurate translation of the virtual surgical plan into the operating room is challenging due to the lack of tools for intraoperative analysis of the surgical outcome. This study aimed to evaluate the feasibility of using a hand-held 3D photography device for intraoperative evaluation and guidance during cranial vault surgical reconstruction. A hand-held structured light scanner was used for intraoperative 3D photography during five craniosynostosis surgeries, obtaining 3D models of skin and bone surfaces before and after the remodeling. The accuracy of this device for 3D modeling and morphology quantification was evaluated using preoperative computed tomography imaging as gold-standard. In addition, the time required for intraoperative 3D photograph acquisition was measured. The average error of intraoperative 3D photography was 0.30 mm. Moreover, the interfrontal angle and the transverse forehead width were accurately measured in the 3D photographs with an average error of 0.72 degrees and 0.62 mm. Surgeon’s feedback indicates that this technology can be integrated into the surgical workflow without substantially increasing surgical time. Hand-held 3D photography is an accurate technique for objective quantification of intraoperative cranial vault morphology and guidance during metopic craniosynostosis surgical reconstruction. This noninvasive technique does not substantially increase surgical time and does not require exposure to ionizing radiation, presenting a valuable alternative to computed tomography imaging. The proposed methodology can be integrated into the surgical workflow to assist during cranial vault remodeling and ensure optimal surgical outcomes.

Metopic craniosynostosis is a condition in which the metopic suture is prematurely fused. Trigonocephaly and hypotelorism are the major abnormal findings associated with synostosis. Fronto-orbital advancement with cranial remodelling procedure is the widely practised method for correction of the deformities. Previously, a few studies have shown a low incidence of secondary raised intracranial pressure after the primary surgery. Thus, we reviewed our database to investigate the outcomes of metopic craniosynostosis treatment between 1999 and 2020 in Cleft and Craniofacial South Australia. One hundred twelve patients (77 males and 35 females) with metopic synostosis were operated. The mean age of primary surgery was 11.1 months (range 2.8-131.7 months). Ten patients (9%) who had secondary raised intracranial pressure underwent secondary surgery. Among those, the mean age of primary and secondary surgery were 5.4 and 57.1 months, respectively. Syndromic patients were significantly revealed to have a higher incidence of secondary raised intracranial pressure. There were three patients (2.7%) who had other secondary procedures (hardware removal and aesthetic contouring procedures). This cohort identified a higher incidence of secondary raised intracranial pressure requiring secondary surgery than previous studies. Syndromic patients were significantly related to secondary raised intracranial pressure. The patients who had raised intracranial pressure tended to have primary correction at younger age. Long-term multidisciplinary follow-up is mandatory. The demand for secondary surgery for metalwork complications or cosmetic contouring is uncommon.

To assess intermediate-term (> 3 years) outcomes of endoscopic strip craniectomy with postoperative helmet therapy (ESC + HT) for the treatment of infants with severe trigonocephaly. This retrospective study examined cranial morphology of consecutive patients with severe trigonocephaly treated with minimally invasive ESC + HT. Preoperative and follow-up clinical parameters were collected from patient charts. Interfrontal divergence angle (IFDA), a validated and accurate measure of forehead narrowing, was measured on preoperative CT scans and on preoperative and postoperative 2D photographs. Seven patients (4 male, 3 female) were included with a mean age at surgery of 2.76 months (range 1.8 to 4.1 months) and mean clinical follow-up of 3.71 years (photographic follow-up 2.73 years). The mean operative time was 91.4 min, with a mean estimated blood loss (EBL) of 57.1 ml and mean hospital length of stay of 1.14 days. IFDA improved from 118.8° to 135.9° (p < 0.01), with the mean final measurement falling within normal limits. The head circumference percentile was not significantly changed in follow-up. There was a statistically significant improvement in the inner-to-outer canthal distance ratio (p = 0.01) in follow-up, showing an improvement in hypotelorism. There were no dural tears, CSF leaks, infections, or other significant surgical morbidities, and there were no serious complications related to the use of helmet therapy. All patients achieved excellent aesthetic results judged by photographic comparison. This study demonstrated that patients treated with ESC + HT for metopic craniosynostosis showed measurable and significant improvement in forehead shape. This technique is a safe and effective alternative to more invasive surgical interventions.

BACKGROUND Reports of neurodevelopmental delays in adolescents with metopic craniosynostosis have ranged from 15 to 61 percent. Previously, event-related potentials have correlated preoperative radiographic severity with language deficiencies in infancy. This study sought to characterize neurocognitive testing at cranial maturity and correlate outcomes to preoperative radiographic severity. METHODS Patients diagnosed with metopic craniosynostosis who underwent surgical correction in infancy completed a neurodevelopmental battery evaluating age-normalized intelligence quotient, academic achievement, and visuomotor integration. Data were stratified by preoperative endocranial bifrontal angle (moderate, >124 degrees; severe

BACKGROUND The impact of metopic craniosynostosis on intracranial volume (ICV) and ICV growth is unclear. In addition, the relationship between head circumference (HC) and ICV in these patients is not previously described. METHODS A retrospective review of 72 patients with metopic craniosynostosis was performed. The ICVs were calculated from manually segmented preoperative computed tomography scans. Magnetic resonance imaging data for 270 healthy children were available. The ICVs were calculated in FreeSurfer.First, a growth curve for metopic patients was generated and a logarithmic best-fit curve was calculated. Second, the impact of metopic craniosynostosis on ICV relative to healthy controls was assessed using multivariate linear regression. Third, the growth curves for metopic patients and healthy children were compared.Pearson's correlation was used to measure the association between HC and ICV. RESULTS Mean metopic ICV was significantly lower than normal ICV within the first 3 to 6 months (674.9 versus 813.2 cm; P = 0.002), 6 to 9 months (646.6 versus 903.9 cm; P = 0.005), and 9 to 12 months of life (848.0 versus 956.6 cm; P = 0.038). There was no difference in ICV after 12 months of age (P = 0.916).The ICV growth in patients with metopic craniosynostosis is defined by a significantly different growth curve than in normal children (P = 0.005).The ICV and HC were highly correlated across a broad range of ICVs and patient age (r = 0.98, P

BACKGROUND Metopic craniosynostosis is traditionally repaired with fronto-orbital advancement (FOA) or, alternatively, limited short scar strip craniectomy (LSSSC) followed by helmet therapy. There is controversy among surgeons regarding resultant head shape outcomes between the 2 methods. This study aims to assess how surgeons perceive the postoperative aesthetic results of the 2 metopic craniosynostosis repair methods. METHODS A retrospective analysis was performed on 13 (n = 6 LSSSC; n = 7 FOA) patients who presented for surgical correction of isolated metopic craniosynostosis via either LSSSC (followed by helmet therapy) or FOA. Clinical photographs at 1 year postop were shown to 10 craniofacial surgeons who rated the aesthetic outcomes on a Likert scale of 1 (poor) to 5 (excellent) and guessed which surgical method was performed. RESULTS Mean age at the time of the procedure was younger in LSSSC than FOA (3.1 ± 1.0 versus 17.5 ± 8.5 months; P

Background:. The surgical correction of metopic craniosynostosis usually relies on the subjective judgment of surgeons to determine the configuration of the cranial bone fragments and the degree of overcorrection. This study evaluates the effectiveness of a new approach for automatic planning of fronto-orbital advancement based on statistical shape models and including overcorrection. Methods:. This study presents a planning software to automatically estimate osteotomies in the fronto-orbital region and calculate the optimal configuration of the bone fragments required to achieve an optimal postoperative shape. The optimal cranial shape is obtained using a statistical head shape model built from 201 healthy subjects (age 23 ± 20 months; 89 girls). Automatic virtual plans were computed for nine patients (age 10.68 ± 1.73 months; four girls) with different degrees of overcorrection, and compared with manual plans designed by experienced surgeons. Results:. Postoperative cranial shapes generated by automatic interventional plans present accurate matching with normative morphology and enable to reduce the malformations in the fronto-orbital region by 82.01 ± 6.07%. The system took on average 19.22 seconds to provide the automatic plan, and allows for personalized levels of overcorrection. The automatic plans with an overcorrection of 7 mm in minimal frontal breadth provided the closest match (no significant difference) to the manual plans. Conclusions:. The automatic software technology effectively achieves correct cranial morphometrics and volumetrics with respect to normative cranial shapes. The automatic approach has the potential to reduce the duration of preoperative planning, reduce inter-surgeon variability, and provide consistent surgical outcomes.

SUMMARY Metopic craniosynostosis results in trigonocephaly, characterized by a triangular forehead with metopic ridge, bitemporal pinching, and narrow-set eyes with recessed lateral orbital rims. Surgical management is controversial but may include open expansion and vault remodeling, to enhance neurocognitive and aesthetic outcomes. This article and video vignette depict the diagnosis and treatment of metopic craniosynostosis, demonstrating the senior author's open approach, three-dimensional virtual surgical planning, and technical steps.

Combined sagittal and metopic craniosynostosis is an uncommon condition that may result in a wide spectrum of cranial morphologies. This occurs as a result of the compound effects of both prematurely fused sutures. The authors present an incidence of sagittal craniosynostosis with a specific altered head morphology, and delayed diagnosis that the authors believe occurred due to structural changes associated with concurrent metopic suture synostosis This patient underscores the limitations of relying on classic diagnostic patterns and highlights the critical role of noninvasive imaging in the diagnosis of craniosynostosis.

Attention problems are common in patients with craniosynostosis. Craniosynostosis is a rare condition, studies face challenges of selection bias, small sample sizes, and wide age ranges. Th...

Surgery is the first treatment option for patients with metopic craniosynostosis. Fronto-orbital advancement is the preferred method for correction of isolated trigonocephaly, but it is hard to understand whether surgery has been successful mainly in an early period. We aim to investigate the shape differences in the head shapes of trigonocephaly patients compared between preoperative and postoperative term. Cranial shape data were collected from the two-dimensional digital images. The Generalized Procrustes analysis was used to obtain mean shapes of the preoperative and postoperative term. The shape deformation of the frontal calvarium from preoperative to the postoperative term was evaluated using the thin-plate spline (TPS) method. There was significant cranial shape difference between preoperative and postoperative term. The high-level deformations for preoperative to postoperative term determined seen in TPS graphic. Highest deformation was observed at the bifrontal dimension especially at nasion and posterior edge of the forehead. In this study, we showed that the shape difference and structural deformation of the calvarium were correlated with the metopic craniosynostosis. The present study also shows that preoperative and postoperative head shapes of patients with trigonocephaly can be compared using the landmark-based geometrical morphometric method by taking into consideration the topographic distribution.

OBJECTIVEThe objective of this study was to present the authors’ 19-year experience treating metopic craniosynostosis by using an endoscopy-assisted technique and postoperative cranial orthotic therapy. The authors also aimed to provide a comprehensive, comparative statistical analysis of minimally invasive surgery (MIS) versus open surgery in reports previously published in the literature (through 2014) regarding only patients with metopic synostosis.METHODSA total of 141 patients with single-suture metopic nonsyndromic craniosynostosis sutures were treated between 1998 and 2017 by endoscopically resecting the synostosed bone followed by postoperative custom cranial orthosis use. All data used in the case series were collected prospectively and stored in a secure database. A comprehensive literature review was performed that included all previous case series reporting common surgical performance measures. A statistical comparison of traditional open methods versus MIS techniques was performed with regard to age, length of hospital stay (LOS), surgical time, estimated blood loss (EBL), and transfusion rate.RESULTSThe mean age at the time of surgery in the current series was 4.1 months. The mean EBL was 33 ml (range 5–250 ml). One patient underwent an intraoperative blood transfusion and 5 underwent postoperative blood transfusion for a total transfusion rate of 4.3%. The mean operating time was 56 minutes. Ninety-eight percent of patients were discharged on the 1st postoperative day. The median size of the removed synostosed bone was 0.6 cm × 10 cm. The primary goal of achieving correction of the forehead deformity was obtained in 94% of the patients. One hundred eight patients presented with hypotelorism (76.6%). Those with a minimum 1-year follow-up achieved 99% correction (n = 97). Six patients younger than 1 year had not achieved correction at the time of follow-up (6%). There were no intra- or postoperative deaths. One patient had a temporary contact dermatitis to the helmet materials and 2 patients developed pseudomeningoceles, which were successfully treated with a lumbar drain and/or spinal tap. No patient required nor underwent a second surgical procedure. Regarding the previously published literature through 2014, the reported EBL in patients who underwent MIS versus traditional open methods was 54.7 ml versus 224 ml, respectively. The reported average age for patients undergoing MIS versus traditional open methods was 3.8 months versus 11.5 months. The average LOS for patients undergoing MIS versus traditional open methods was 1.7 days versus 3.7 days. The average reported surgical time for those undergoing MIS versus traditional open methods was 66.7 minutes versus 223.7 minutes. The transfusion rate for patients undergoing MIS versus traditional open methods was 22% versus 77%. All of the above differences demonstrated statistical significance.CONCLUSIONSThe authors’ team has safely and effectively performed 141 metopic craniosynostosis corrections over the past 19 years, with excellent outcomes. Literature review comparing metrics such as LOS, EBL, operating time, and transfusion rate demonstrates a statistically significant improvement in all commonly reported measurements. MIS techniques are safe and effective and should be offered to parents and patients as an option at craniofacial centers treating this condition.

Objective The aim of this study was to discuss the results of craniometric measurements and surgical treatments in patients operated for isolated trigonocephaly (TC) in light of the current literature. Methods A total of 18 cases who underwent surgery for isolated TC were included in the study. Age, gender, family history, follow-up time, complications, duration of surgery, surgical blood loss, and amount of blood replacement in these patients were recorded. Craniometric measurements such as metopic angle (MA), cephalic index (CI), interparietal distance (IPD), intercoronal distance (ICD), and their ratio to each other were evaluated according to pre-and postoperative parameters. Photographs of the patients were taken before and after the operation. The Whitaker classification and Kampf "aesthetic outcome staging" were used in the evaluations. Results The mean MA values after the operation increased to reach above 147 degrees in all cases. The average CI did not change. ICD measurement averages increased significantly. The average IPD/ICD ratio decreased due to the increase in ICD and the enlargement of the anterior fossa after the operation. According to the Whitaker classification and Kampf “aesthetic outcome staging” scale, 17 of our cases were at stage I, rated as perfect, and one was at stage II, rated as good. Conclusion Surgery performed at the appropriate time for TC yields cosmetically satisfactory results. Since potential neurological and cognitive morbidities occur mostly in school-age patients, long-term follow-up of the cases is required. Performing craniometric measurements enables patients to be evaluated with objective and measurable numerical data.

Background Previous research has shown that patients with metopic craniosynostosis have significantly reduced intracranial volumes (ICVs) compared to normal healthy children. Furthermore, the metopic index (ratio of midfrontozygomatic diameter to maximal cranial width) has been described as an anthropometric cranial index for patients with metopic craniosynostosis. We aimed to determine whether patients with isolated metopic ridge have significantly different ICVs or metopic indices than normal children and patients with metopic craniosynostosis. Methods A retrospective chart review of all patients with a diagnosis of a metopic ridge or metopic craniosynostosis was performed from 2000 to 2015 at Rady Children's Hospital. Patients were grouped based on computed tomographic scans consistent with metopic craniosynostosis versus metopic ridge. Results Data were available for 15 metopic ridge patients, 74 metopic craniosynostosis patients, and 213 normal patients. Mean metopic ridge ICV was greater than mean metopic craniosynostosis ICV at 4 to 6 months and 7 to 12 months. Controlling for age and sex, the difference in ICV associated with metopic ridging was 197.484 cm3 and 137.770 cm3 at 4 to 6 and 7 to 12 months, respectively. Similarly, mean metopic index was significantly greater in metopic ridge patients compared to mean metopic craniosynostosis at 4 to 6 months and at 7 to 12 months. Conclusions Our study provides volumetric and anthropometric data to support the hypothesis that isolated metopic ridge is an intermediate phenotype between metopic craniosynostosis and normal cranial anatomy. We hope that characterizing the spectrum of disease involving premature closure of the metopic suture with regard to ICV and metopic index will aid physicians in their management of patients with isolated metopic ridge.

Surgical options for metopic craniosynostosis include the traditional open approach or a minimally invasive approach that typically involves an endoscopy-assisted strip craniectomy. The minimally invasive approach has been associated with less blood loss and operative time, a lower transfusion rate, and a shorter length of stay. Additionally, it is more cost-effective than open reconstruction, despite the need for a postoperative cranial orthosis and multiple follow-up visits. The authors describe a variation of the minimally invasive approach using a lighted retractor to perform a strip craniectomy of the metopic suture in a 2-month-old patient with metopic craniosynostosis. The video can be found here: https://vimeo.com/511237503.

Aim:Metopic craniosynostosis (MCS), with its trigonocephalic head shape, is often treated with either limited incision strip craniectomy (LISC) followed by helmet orthotic treatment, or open cranial vault reconstruction techniques (OCVR). There is controversy regarding resultant shape outcomes among craniofacial surgeons. Those adverse to LISC claim normal head shape is never attained, while proponents believe there is gradual correction to an equivalent outcome. This study aims to quantitate, over time, the three-dimensional (3D) head shapes in patients who have undergone LISC or OCVR intervention for MCS.Methods:Sixty-three 3D images of 26 patients with MCS were analyzed retrospectively. Head shape analyses were performed at: (1) preoperative, (2) 1-month postoperative, (3) 10 to 14 months postoperative (1 year), and (4) 2 years postoperative. Composite 3D head shapes of patients were compared at each time point. Two-dimensional (2D) standardized cross sections of the forehead were also compared.Results:Composite head shapes for both groups were nested, to allow visual comparison as the child’s forehead grows and expands. The difference between LISC and OCVR 2D cross sections was calculated; 108.26 mm preoperatively, 127.18 mm after 1-month postoperative, 51.05 mm after 10 to 14 months postoperative, and 27.03 mm after 2 years postoperative.Conclusions:This study found excellent head shape outcomes for both the LISC and OCVR techniques at 2 years of age. It also corroborates the slow and progressive improvement in head shape with the LISC technique. This study highlights the advantages of 3D photography for measurement of contour outcomes, utilizing both 2D vector and 3D whole head analytical techniques.

Background Children who undergo bi-fronto-orbital advancement (BFOA) frequently develop a contour deformity on the temporal and supra-orbital region, with an incidence reported as high as 55% and 75%, respectively. Up to 20% of patients may require correction. Hydroxyapatite cement (HAC) is a good alternative to autogenous tissue. The available literature on its use focusses on the reconstruction of bone defects, but little has been published on its efficacy and safety as an onlay graft over intact cranium. Objectives To describe our institution's experience with HAC in the pediatric population. Methods Retrospective chart review from 1998 to 2018 on all patients from the Craniofacial Unit at the Sydney Children's Hospital who had either coronal or metopic craniosynostosis and underwent BFOA and later in life required cranioplasty with HAC for contour repair. Findings We have performed 166 BFOA and nineteen secondary cranioplasties for contour repair using onlay HAC. The mean age at the time of operation was 14 years. Bi-coronal craniosynostosis was most frequently associated with secondary cranioplasty and 37% had an associated syndrome. The mean volume of HAC used was 37 mL. There was only 1 patient who had a complication (5.3%) and required partial removal of allograft. The mean length of admission was 2 days. Mean follow up time of 22.4 months. Conclusions HAC represents a safe option when used correctly, with low rates of complication and satisfactory cosmetic outcomes.

The aim of this study was to identify quantitative tools to classify the severity of trigonocephaly to guide surgical management and predict outcome. METHODS We reviewed high-resolution computed tomography images of 59 patients with metopic synostosis. We assessed the craniofacial sutural pattern as well as interfrontal and metopic angles, and we related the frontal angulation degree with the sutural pattern, the surgical management, and clinical outcome. RESULTS We identified 3 groups according to the severity of trigonocephaly. No difference was found between the sutural pattern of nasion complex and severity, whereas the closure of zygomatic maxillary sutures increased with the severity degree (P < 0.05). The operative management was related to the severity degree (P < 0.001) and to the reduced age (P = 0.009). CONCLUSIONS Interfrontal and metopic angles are complementary measurements to evaluate with high accuracy the degree of frontal angulation. In preoperative assessment, they may guide surgery decision in particular when the choice is not straightforward.

The standard for diagnosing metopic craniosynostosis (CS) utilizes computed tomography (CT) imaging and physical exam, but there is no standardized method for determining disease severity. Previous studies using interfrontal angles have evaluated differences in specific skull landmarks; however, these measurements are difficult to readily ascertain in clinical practice and fail to assess the complete skull contour. This pilot project employs machine learning algorithms to combine statistical shape information with expert ratings to generate a novel objective method of measuring the severity of metopic CS.Expert ratings of normal and metopic skull CT images were collected. Skull-shape analysis was conducted using ShapeWorks software. Machine-learning was used to combine the expert ratings with our shape analysis model to predict the severity of metopic CS using CT images. Our model was then compared to the gold standard using interfrontal angles.Seventeen metopic skull CT images of patients 5 to 15 months old were assigned a severity by 18 craniofacial surgeons, and 65 nonaffected controls were included with a 0 severity. Our model accurately correlated the level of skull deformity with severity (P

Resume Contexte La scaphocephalie augmente la frequence de certaines modifications du profil cognitif et de l’humeur, de facon encore non elucidee. Objectif Notre objectif est de decrire le retentissement de la scaphocephalie sur le profil neuropsychologique et plus particulierement sur les fonctions executives. Patients et methodes Un groupe experimental de 19 enfants de 5 ans a 14 ans operes pour une scaphocephalie a ete compare a un groupe controle de 10 enfants operes pour une trigonocephalie avec les tests de QI, des tests d’attention et des echelles de l’humeur. Un groupe de 6 enfants de 2 a 4 ans operes pour une scaphocephalie et un groupe de 6 enfants porteurs de scaphocephalie non operes sont egalement decrits. Resultats Pour le groupe experimental comme pour le groupe controle, les QI sont inchanges, tandis que les deficits d’attention et de troubles anxieux sont plus frequents que dans la population de reference. Les profils cognitifs etaient differents selon le groupe. Les resultats suggerent une plus grande frequence de deficits du controle inhibiteur dans le traitement visuel pour le groupe scaphocephalie, a la difference d’une plus grande frequence de deficits de memoire de travail auditivo-verbale pour le groupe trigonocephalie. Des profils comparables sont egalement observes dans les groupes d’enfants avec scaphocephalie plus jeunes ou non operes. Conclusions De nombreux enfants presentant une scaphocephalie doivent composer tout au long de leur developpement avec un profil neuropsychologique specifique. L’ensemble suggere l’interet de proposer a ces enfants et a leur famille un suivi specifique dans des centres de reference.

Craniofacial dysmorphology varies significantly along a wide spectrum of severity in metopic cranial synostosis (MCS). This study aimed to quantify craniofacial changes, in MCS, to investigate their relationships with the severity of trigonocephaly. By combining the metopic ridge and interfrontal angles, we identified three groups of trigonocephaly severity (mild group n.14, moderate group n.19, severe group n.18). We perform a quantitative analysis using high-resolution CT images evaluating (1) cranial fossae dimensions; (2) vault indices and ratios: interparietal/ intercoronal (IPD/ICD), interparietal/intertemporal (IPD/ITD), cephalic index, vertico-longitudinal index; (3) orbito-facial distances (midfacial depth, maxillary height, upper facial index, orbital distances, globe protrusions), maxilla and orbital volumes; (4) supratentorial (ICV) and infratentorial (PCFV) cranial volumes and supratentorial (WBV) and infratentorial (PCFBV) brain volumes. In all groups, middle skull base lengths and upper midface index were increased. In moderate and severe groups: anterior hemifossa lengths were reduced, IPD/ICD and vertico-longitudinal index were changed; midfacial depth, anterior, mild, and lateral interorbital distances were reduced; globe protrusions were increased. The comparison between moderate and severe groups showed an increase of both globe protrusions and IPD/ICD. Among all groups, ICV and WBV were reduced in the severe group. This morpho-volumetric study provides new insights in understanding the craniofacial changes occurring in infants at different severity of trigonocephaly. The increase of globe protrusions and the reduction of supratentorial volumes found in the severe group reflect the severity of trigonocephaly; these findings might have a clinical and surgical relevance.

Objective: To evaluate 3-dimensional (3-D) photogrammetry as a tool for assessing the postoperative head shape of patients who had undergone cranial vault remodeling for metopic synostosis. Design: We prospectively analyzed images of patients with metopic craniosynostosis who had undergone anterior cranial vault remodeling and age-matched controls. To ensure standardized facial orientation, each 3-D image was positioned to “best fit” the preoperative face by aligning 6 soft tissue landmarks. Forehead measurements were taken from a standardized position behind the surface of the face to landmarks placed in a ray configuration across the forehead. Setting: Academic teaching hospital. Patients, Participants: Thirteen pediatric patients with metopic craniosynostosis who had undergone anterior cranial vault remodeling and age-matched controls. Interventions: Images were taken preoperatively, immediately postoperatively, and over 1-year postoperatively. Main Outcome Measures: Forehead contours preoperatively and postoperatively, with statistics performed using a multivariate analysis of variance shape analysis. Results: Mean postoperative follow-up was 1.8 (0.6) years. The average distance from the origin to forehead landmarks was 55.1 (3.4) mm preoperatively, 59.3 (0.7) mm immediate postoperatively, 59.1 (1.0) mm 1-year postoperatively, and 59.4 (0.6) mm in controls. Postoperative metopic forehead contours varied significantly from preoperative contours ( P < .01), while there was no statistical difference between the 2 postoperative time points ( P = .70). One-year postoperative patients were not significantly different from their age-matched controls ( P > .99). Conclusions: Preoperative metopic forehead contours varied significantly from postoperative contours. Cranial reconstructions approximated the foreheads of normal controls, and reconstructions were stable at more than 1-year follow-up.

Cranial dysmorphology observed in patients with metopic craniosynostosis varies along a spectrum of severity including varying degrees of metopic ridging, bitemporal narrowing, and trigonocephaly. Management has been based upon the subjective clinical impression of presence and severity of trigonocephaly. Severity of cranial dysmorphology does not predict the occurrence or severity of associated abnormal neurodevelopment, as children with mild-to-moderate trigonocephaly may also experience developmental delays. The authors sought to determine the relationship between mild-to-moderate trigonocephaly and anterior cranial volume using a noninvasive laser shape digitizer (STARscanner) in patients with abnormal head shape.An IRB-approved retrospective review of a prospectively maintained database and medical records was performed. Two hundred three patients less than 1 year of age with abnormal head shape were categorized as having a metopic ridge with mild-to-moderate trigonocephaly, metopic ridge without trigonocephaly, or no ridge. Measurements of cranial volume, circumference, and symmetry were calculated by the STARscanner, which quantifies three-dimensional shape of the cranial surface. Measures were analyzed using a series of analyses of variance and post-hoc Tukey honest significant difference.The authors results showed ACV was significantly reduced in patients with mild-to-moderate trigonocephaly compared with those without metopic ridge (P = 0.009), and trended toward significance compared with those with a ridge but without trigonocephaly (P = 0.072). The ratio of anterior-to-posterior cranial volume was significantly reduced in those with mild-to-moderate trigonocephaly compared with those without metopic ridge (P = 0.036).In conclusion, patients with milder anterior cranial deformities demonstrated an association between a metopic ridge with mild-to-moderate trigonocephaly and reduced anterior cranial volume.

Endoscopic approaches for craniosynostosis are a growing field in pediatric neurosurgery. In metopic synostosis, previous reports for complete fronto-orbital remodeling have proposed an intervention with multiple incisions (bregmatic, tarsal, and preauricular) to open frontonasal and frontoethmoidal synostotic sutures, and orbital roof. We propose a technique to complete all these osteotomies with a unique incision anterior to the bregmatic fontanel under 3D endoscopic vision, and review possible complications, limits, and pitfalls. Under endoscopic assistance, a complete fronto-orbital remodeling could be completed with a unique incision without mayor drawbacks.

PURPOSE Treatment of metopic craniosynostosis is performed by either fronto-orbital advancement (FOA) or endoscopic-assisted techniques. Interfrontal angle (IFA) is a validated measure of trigonocephaly, but requires a computed tomography scan. The most common direct measure to assess surgical outcome in patients with trigonocephaly is frontal width (ft-ft). The aim of this study is to determine if frontal width correlates with IFA and successful surgical correction 1 year after treatment. A review of current morphologic assessment techniques is also provided. METHODS Three-dimensional computed tomography scans (preoperative and 1 year postoperative) of patients who underwent FOA (n = 13) or endoscopic (n = 13) treatment of metopic craniosynostosis were reviewed. Age-matched scans of unaffected patients served as controls. Frontal width was measured by a straight line between the bilateral frontotemporal points. Measurements were performed by 2 experienced observers and compared to IFA. RESULTS Mean frontal width at preoperative scan for endoscopic and open patients was 55 ± 0.6 and 64 ± 0.7 mm, respectively (Z-score 1.6 and -3.7). Mean frontal width at postoperative scan for endoscopic and open patients was 80 ± 0.4 and 81 ± 0.7 mm (Z-score 0.0 for both groups). Frontal width for endoscopic correction significantly correlated with IFA (r = 0.536, P = 0.005), as well as for the open patients (r = 0.704, P

BACKGROUND In recent years, metopic synostosis has represented up to 25 percent of all nonsyndromic craniosynostosis cases, manifesting with varying degrees of trigonocephaly. It is unknown whether the degree of severity of anterior dysmorphology is proportionate to neurologic dysfunction. This knowledge is essential to guide future surgical treatment recommendations. Given the reported correlation of abnormal auditory processing with future language dysfunction and poorer academic performance in craniosynostosis patients, the present study aims to evaluate auditory processing in preoperative patients with moderate and severe metopic synostosis, and in control infants, to discern the neurologic risk carried by different severities of trigonocephaly. METHODS Thirty-eight infants underwent evaluation of auditory processing: nine patients with severe metopic synostosis, seven with moderate metopic synostosis, and 22 controls. Brain activity was recorded by electroencephalography while the participants listened to language-based stimuli. Electroencephalographic data were analyzed to extract event-related potentials evoked by the speech sounds. RESULTS Severe metopic synostosis patients demonstrated significantly attenuated event-related potential responses in the left frontal scalp region overlying the left frontal lobe compared with controls (p < 0.05). The moderate metopic synostosis patients did not show significantly different language processing compared to the control infants or the severe metopic synostosis patients in the frontal scalp region. CONCLUSIONS The results of this study suggest that untreated severe metopic synostosis is associated with reduced language response in the frontal cortex. Less severe (moderate) forms are indeterminate, and mild forms do not show calculable irregularity by the event-related potential method of analysis. CLINICAL QUESTION/LEVEL OF EVIDENCE Risk, II.