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Purpose: Endoscopically assisted sagittal strip craniotomy with subsequent cranial orthosis is a frequently used surgical approach for non-syndromic sagittal synostosis. Originally, this technique involved a wide sagittal strip craniectomy with bilateral wedge osteotomies. More recent studies suggest omitting wedge osteotomies, achieving similar outcomes. The controversy surrounding wedge osteotomies and our efforts to refine our technique led us to create models and evaluate the mechanical impact of wedge osteotomies. Methods: We conducted a 3D-print study involving preoperative CT scans of non-syndromic scaphocephaly patients undergoing minimally invasive–assisted remodelation (MEAR) surgery. The sagittal strip collected during surgery underwent thickness measurement, along with a 3-point bending test. These results were used to determine printing parameters for accurately replicating the skull model. Model testing simulated gravitational forces during the postoperative course and assessed lateral expansion under various wedge osteotomy conditions. Results: The median sagittal strip thickness was 2.00 mm (range 1.35–3.46 mm) and significantly positively correlated (p = 0.037) with the median force (21.05 N) of the 3-point bending test. Model testing involving 40 models demonstrated that biparietal wedge osteotomies significantly reduced the force required for lateral bone shift, with a trend up to 5-cm-long cuts (p = 0.007). Additional cuts beyond this length or adding the occipital cut did not provide further significant advantage (p = 0.1643; p = 9.6381). Conclusion: Biparietal wedge osteotomies reduce the force needed for lateral expansion, provide circumstances for accelerated head shape correction, and potentially reduce the duration of cranial orthosis therapy. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study was to evaluate the surgical complications of patients treated for nonsyndromic sagittal craniosynostosis and the necessity for reoperations due to craniocerebral disproportion. Materials and methods: The patient cohort of this study consisted of patients (N = 82) who were treated in the Oulu University Hospital using the open vault cranioplasty with a modified H-technique between the years 2008 to 2022. There were 69 males (84.1%) and 13 females (15.9%). The mean age at the primary operation was 6.1 months. Mean follow-up time was 9.0 years. Results: There were no major complications related to the procedures. Two patients (2.4%) had a minor dural lesion. There were no postoperative wound infections. Of the 82 patients, seven patients with primary craniosynostosis (13.0%) developed symptomatic craniocerebral disproportion requiring reoperation to increase intracranial volume. In all these patients, invasive intracranial pressure (ICP) monitoring was performed prior to decision-making. In the majority of cases, the aesthetical outcome was considered good or excellent. Conclusion: The operative method used was feasible and safe. Thirteen percent of patients who were followed over 5 years required major surgery due to development of craniocerebral disproportion later in life. [ABSTRACT FROM AUTHOR]

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

Purpose: To provide additional information on optimal start times and therapeutic effectiveness based on treatment outcome of Japanese infants with positional plagio- and brachycephaly (PPB) receiving cranial molding helmet therapy (CMHT). Methods: In this retrospective cohort study, data from a 3D head scanning system was analyzed from 2173 Japanese infants who completed CMHT. Anterior and posterior symmetry ratio (ASR and PSR) and longitudinal to transverse diagonal ratios (LD/TDR) were calculated based on skull shape at helmet design and at completion of therapy. The outcomes were evaluated using the regression analysis and a predictive model using cranial parameters was developed. Results: The earlier the start of therapy, the greater the therapeutic effect on ASR, PSR, and LD/TDR (ASR, −0.134 percent points (ppt)/day; PSR, −0.086 ppt/day; and LD/TDR, −0.131 ppt/day). In the predictive model, in addition to starting age of the therapy, sex (male), the degree of deformity of the head (DoD) (moderate and severe), quadrant volume, PSR, and head circumference at the start of treatment also had a positive effect on changes in ASR, DoD (moderate and severe), ASR, LD/TDR and transverse diameter for PSR, sex (male), DoD (moderate), quadrant volume, PSR, and head circumference for LD/TDR. Conclusion: The starting age of therapy had a relatively smaller contribution to outcome effects. Applying the cranial parameter obtained at the start of treatment to the predictive model helps to predict the effect of CMHT and whether PPB can be treated with CMHT in infants of older age. [ABSTRACT FROM AUTHOR]

Scaphocephaly is the most common type of craniosynostosis and various surgical techniques are used for treatment. Due to late postoperative changes of the head shape, long-term outcome data is important for evaluating any new surgical technique. At our institution, minimally invasive strip craniectomy without regular helmet therapy is the standard treatment in scaphocephalic patients. Between October 2021 and February 2023, we retrospectively examined the skull shape of patients who underwent minimally invasive strip craniectomy for scaphocephaly using a 3D surface scan technique. The cephalic index (CI), the need for helmet therapy and additional cosmetic outcome parameters were investigated. We included 70 patients (72.5% male). The mean follow-up time was 46 (10–125) months and the mean CI was 75.7 (66.7–85.2). In 58 patients, the final cosmetic result was rated as "excellent/good" (mean CI: 76.3; 70.4–85.0), in 11 as "intermediate" (mean CI: 73.3; 66.7–77.6), and in one case as "unsatisfactory" (CI 69.3). The presence of a suboccipital protrusion was associated with a "less than good" outcome. The CI correlated significantly with the overall outcome, the presence of frontal bossing, and the interval between scan and surgery (age at scan). Minimally invasive strip craniectomy is an elegant and safe method to correct scaphocephaly. Our data show good cosmetic results in the long term even without regular postoperative helmet therapy. [ABSTRACT FROM AUTHOR]

Sagittal craniosynostosis, a rare but fascinating craniofacial anomaly, presents a unique challenge for both diagnosis and treatment. This condition involves premature fusion of the sagittal suture, which alters the normal growth pattern of the skull and can affect neurological development. Sagittal craniosynostosis is characterised by a pronounced head shape, often referred to as scaphocephaly. Asymmetry of the face and head, protrusion of the fontanel, and increased intracranial pressure are common clinical manifestations. Early recognition of these features is crucial for early intervention, and understanding the aetiology is, therefore, essential. Although the exact cause remains unclear, genetic factors are thought to play an important role. Mutations in genes such as FGFR2 and FGFR3, which disrupt the normal development of the skull, are suspected. Environmental factors and various insults during pregnancy can also contribute to the occurrence of the disease. An accurate diagnosis is crucial for treatment. Imaging studies such as ultrasound, computed tomography, magnetic resonance imaging, and three-dimensional reconstructions play a crucial role in visualising the prematurely fused sagittal suture. Clinicians also rely on a physical examination and medical history to confirm the diagnosis. Early detection allows for quick intervention and better treatment outcomes. The treatment of sagittal craniosynostosis requires a multidisciplinary approach that includes neurosurgery, craniofacial surgery, and paediatric care. Traditional treatment consists of an open reconstruction of the cranial vault, where the fused suture is surgically released to allow normal growth of the skull. However, advances in minimally invasive techniques, such as endoscopic strip craniectomy, are becoming increasingly popular due to their lower morbidity and shorter recovery times. This review aims to provide a comprehensive overview of sagittal craniosynostosis, highlighting the aetiology, clinical presentation, diagnostic methods, and current treatment options. [ABSTRACT FROM AUTHOR]

Purpose: The aim of the study was to compare the results of two surgical techniques for the treatment of isolated sagittal synostosis (ISS) by means of 3D stereophotogrammetry. One technique, the Renier's "H" technique (RHT) comprised a biparietal expansion, the other, the total vault remodeling (TVR) included also a frontal remodeling. Methods: The two groups of operated children were compared with a third control group of normocephalic children. The 3D scanning was performed in all children between 12 and 245 months of age. On each 3D image six measurements and indices have been made, with the aim of evaluating not only length and width of the head, but also the height. The cranial index (CI) was measured in a plane parallel to the nasion-tragus plane, at the intersection with the opisthocranion. Results: Each of the three groups (RHT, TVR, control group) included 28 children. The measurements that were influenced by the correction of the frontal bossing, namely the CI and the sagittal length, were closer to normocephaly after TVR than after RHT. Lesser or no statistical difference was documented in the measurements evaluating the biparietal aspect and the height of the vertex, indicating that the biparietal expansion is effective in both procedures. Conclusion: Based on our results TVR results in a better esthetical outcome, particularly in relation to the direct surgical remodeling of the frontal bossing. [ABSTRACT FROM AUTHOR]

Aim: To establish the prevalence of positional head deformations (PHDs) within a neonatal unit (NU) setting, and to evaluate the subsequent impact that PHDs have on NU graduates and their families. Methods: A prospective audit was conducted over a six‐week period within a tertiary NU (Brisbane, Australia). Eligible babies were measured weekly using a craniometer where presence, type and severity of PHD were determined. Univariate analysis was undertaken to establish differences in clinical characteristics between babies with, and without, the presence of PHD. A study‐specific survey was completed by a separate set of families returning for outpatient follow‐up services who represented similar clinical characteristics and risk factors for PHD. Results: Fifty‐three babies were eligible for inclusion in the audit. PHDs were identified in 66% (n = 35) of the cohort, the most common being scaphocephaly (52.8%, n = 28). Within that, 46% (n = 13) were classed as mild, 25% (n = 7) were moderate and 29% (n = 8) were severe. Moderate correlation (r = 0.55) was found between severity of scaphocephaly, and length of time spent in an isolette. Of the 10 (66% response from 15 families) surveys completed, 80% of respondents perceived that their child's PHD had impacted their life. Conclusions: Two‐thirds of babies developed a PHD during their neonatal admission. Most families surveyed perceived this condition to have an impact on their lives beyond the confines of the nursery. Further research is needed to identify preventative interventions to decrease the prevalence and severity of this common condition. [ABSTRACT FROM AUTHOR]

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Scaphocephaly remains the most prevalent form of isolated single-suture synostosis. In most Pediatric Neurosurgery Centers, the treatment consists of extensive calvarial reshaping operations which are performed in relatively late ages. We described a modified open technique of cranial vault reconstruction for scaphocephaly and we report the anesthetic approach and how it influences our results and enables the performance of this surgery at earlier ages. A total of 47 patients with an average age of 11 ± 2.72 months underwent to the same surgical technique. Blood transfusion during the surgical intervention was needed in 25 patients (53%), and 22 patients (46.8%) did not need blood transfusion. None of the 14 patients who used tranexamic acid (TXA) needed blood transfusions. We reported a statistically significant association between the use of TXA and blood transfusion (p

Purpose: 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 of cranial dysmorphology. The goal of this study was to depict the most common combinations of radiomorphologic characteristics of NSC and to separate groups where the patients were morphologically similar to one another and at the same time significantly different from others. Methods: The study was conducted on anonymized thin-cut CT scans of 131 children with NSC aged 1–12 months (mean age 5.42 months). The type of cranial dysmorphology was assessed using four criteria: skull shape, pattern of sagittal suture fusion, morphologic features and cerebrospinal fluid (CSF) spaces alterations. After assigning the categories, an unsupervised k-modes clustering algorithm was applied to identify distinct patients clusters representing radiomorphologic profiles determined by investigated characteristics. Results: Cluster analysis revealed three distinct radiomorphologic profiles including the most common combinations of features. The profiles were not influenced by sex nor age but were significantly determined by skull shape (V = 0.58, P < 0.0001), morphologic features (V = 0.50, P < 0.0001) and pattern of sagittal suture fusion (V = 0.47, P < 0.0001). CSF alterations did not significantly correlate with the profiles (P = 0.3585). Conclusion: NSC is a mosaic of radiologic and morphologic features. The internal diversity of NSC results in dissimilar groups of patients defined by unique combinations of radiomorphologic characteristics, from which the skull shape is the most differentiating factor. Radiomorphologic profiles support the idea of clinical trials targeted at more selective outcomes assessment. [ABSTRACT FROM AUTHOR]

Introduction: Sagittal craniosynostosis (SC) is associated with scaphocephaly, an elongated narrow head shape. Assessment of regional severity in the scaphocephalic head is limited by the use of serial computed tomographic (CT) imaging or complex computer programing. Three-dimensional measurements of cranial surface morphology provide a radiation-free alternative for assessing cranial shape. This study describes the creation of an occipital bulleting index (OBI), a novel tool using surface morphology to assess the regional severity in patients with SC. Methods: Surface imaging from CT scans or 3D photographs of 360 individuals with SC and 221 normocephalic individuals were compared to identify differences in morphology. Cartesian grids were created on each individual's surface mesh using equidistant axial and sagittal planes. Area under the curve (AUC) analyses were performed to identify trends in regional morphology and create measures capturing population differences. Results: The largest differences were located in the medial regions posteriorly. Using these population trends, a measure was created to maximize AUC. The OBI has an AUC of 0.72 with a sensitivity of 74% and a specificity of 61%. When the frontal bossing index is applied in tandem, the two have a sensitivity of 94.7% and a specificity of 93.1%. Correlation between the two scores in individuals with SC was found to be negligible with an intraclass correlation coefficient of 0.018. Severity was found to be independent of age under 24 months, sex, and imaging modality. Conclusions: This index creates a tool for differentiating control head shapes from those with SC and has the potential to allow for objective evaluation of the regional severity, outcomes of different surgical techniques, and tracking shape changes in individuals over time, without the need for radiation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Several craniofacial pathologies are present in an early adolescent from a cemetery (n = 22) associated with a Neolithic Funnel Beaker culture in Modřice, Czechia. The objective of the study was to document anomalies on the Modřice 3871 cranium, with an emphasis on investigating whether the mid-sagittal suture exhibited synostosis or bridging to account for the visible scaphocephaly. All available cranial and postcranial elements of the Modřice 3871 early adolescent were examined macroscopically. The cranial vault was subjected to radiography and compared to macroscopic views. Modřice 3871 is estimated to age to 12–14 years. Macroscopic examination and radiography support a diagnosis of mid-sagittal bridging rather than synostosis for this slender and anteroposteriorly elongated cranium. In comparison, the anterior sagittal, coronal and lambdoidal sutures are completely unfused. Craniofacial asymmetry, cribra orbitalia, porotic hyperostosis of the temporal and the presence of multiple wormian bones indicate additional maturational disruptions. The scaphocephaly observed in Modřice 3871 is not severe compared to modern clinical manifestations of craniosynostosis. Radiocarbon dated to 3,700–3,600 years BCE, Modřice 3871 presents one of the oldest recorded cases of scaphocephaly. The elongated vault is probably not the result of head-binding given the dearth of anthropogenic cranial reshaping in the Neolithic of Europe. This study adds to the growing recognition of prehistoric and historic craniofacial anomalies which will likely continue as additional human remains are excavated. In addition, the study increases the understanding of the lived experience of prehistoric individuals experiencing visible craniofacial pathologies such as cranial asymmetry and scaphocephaly.

Abstract Background Scaphocephaly is a craniofacial anomaly where the skull is disproportionately long and narrow. Premature closure of the sagittal suture is thought to be at the core of the etiopathology for scaphocephaly. Case presentation The skull is a well-preserved skull found in an antiquated graveyard at Parkand village Daregaz, Iran, with no visible signs of surgical manipulation. The craniofacial indices were measured. The maximum cranial length was 200 mm, and the measured length of nasion-bregma was 140mm, whereas most of the measurements were less than the normal scaphocephalic indices. Another morphological finding was the flattening of the frontal bone. Conclusions The cranial vault morphometry and morphology of the presented case fit the scalp. In addition to the characteristics of scaphocephaly, the presented skull was of a rare trait, frontal flattening, which has not been reported before.

Purpose: The aim of this case–control study was to investigate occlusal characteristics, received orthodontic treatment, oral health-related quality of life (OHRQoL), and satisfaction with dental esthetics in adults operated due to sagittal synostosis. Methods: The study group consisted of 40 adults (25 males, 15 females, mean age 27.4 years, range 18–41) who were operated due to isolated sagittal synostosis in childhood. The control group comprised 40 age and gender-matched adults. Occlusal characteristics were evaluated clinically during study visits. Information on the previous orthodontic treatment was collected from dental records. OHRQoL was measured using the 14-item Oral Health Impact Profile (OHIP-14), and satisfaction with dental esthetics was evaluated using a visual analogue scale. Results: No statistically significant differences were found between the patient group and the controls in malocclusion traits (overjet, overbite, molar relationships, crossbite, scissor bite), previous orthodontic treatment, pre-treatment malocclusion diagnoses, OHIP variables, or satisfaction with dental esthetics. However, there was a tendency toward increased overjet and overbite in scaphocephalic patients. Conclusion: It seems that adults with scaphocephaly operated in childhood do not differ from the average population in terms of occlusion, received orthodontic treatment, or oral health-related well-being. [ABSTRACT FROM AUTHOR]

Craniosynostosis is a rare condition of skull development, manifesting during fetal and early infant development, and is usually congenital. Craniosynostosis secondary to metabolic disorders, such as X‐linked hypophosphatemia (XLH), is less common and is typically diagnosed later than congenital craniosynostosis. XLH is a rare, progressive, and lifelong hereditary phosphate‐wasting disorder characterized by loss of function of the phosphate‐regulating endopeptidase homologue, X‐linked gene, which is associated with premature fusion of cranial sutures due to abnormal phosphate metabolism (hypophosphatemia) and altered bone mineralization or elevated levels of fibroblast growth factor 23. This targeted literature review of 38 articles seeks to provide an overview of craniosynostosis in individuals with XLH. The objectives of this review are to increase awareness of the prevalence, presentation, and diagnosis of craniosynostosis in XLH; examine the spectrum of craniosynostosis severity in XLH; discuss the management of craniosynostosis in those with XLH; recognize the complications for patients with XLH; and identify what is known about the burden of craniosynostosis for individuals with XLH. The presentation of craniosynostosis in individuals with XLH tends to manifest slightly later than congenital craniosynostosis and can vary in severity and appearance, making diagnosis difficult and resulting in inconsistent clinical outcomes. Consequently, craniosynostosis in patients with XLH is an underreported and potentially underrecognized condition. There have been no studies investigating the effects of craniosynostosis on the quality of life of people with XLH. Despite a growing awareness among researchers and experienced clinicians, there are still improvements to be made in general awareness and timely diagnosis of craniosynostosis in XLH. The XLH community would benefit from further study into the prevalence of craniosynostosis, the effect of XLH medical therapy on the development of craniosynostosis, and the effects of craniosynostosis on quality of life. © 2023 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research. [ABSTRACT FROM AUTHOR]

Sagittal craniosynostosis, a rare but fascinating craniofacial anomaly, presents a unique challenge for both diagnosis and treatment. This condition involves premature fusion of the sagittal suture, which alters the normal growth pattern of the skull and can affect neurological development. Sagittal craniosynostosis is characterised by a pronounced head shape, often referred to as scaphocephaly. Asymmetry of the face and head, protrusion of the fontanel, and increased intracranial pressure are common clinical manifestations. Early recognition of these features is crucial for early intervention, and understanding the aetiology is, therefore, essential. Although the exact cause remains unclear, genetic factors are thought to play an important role. Mutations in genes such as FGFR2 and FGFR3, which disrupt the normal development of the skull, are suspected. Environmental factors and various insults during pregnancy can also contribute to the occurrence of the disease. An accurate diagnosis is crucial for treatment. Imaging studies such as ultrasound, computed tomography, magnetic resonance imaging, and three-dimensional reconstructions play a crucial role in visualising the prematurely fused sagittal suture. Clinicians also rely on a physical examination and medical history to confirm the diagnosis. Early detection allows for quick intervention and better treatment outcomes. The treatment of sagittal craniosynostosis requires a multidisciplinary approach that includes neurosurgery, craniofacial surgery, and paediatric care. Traditional treatment consists of an open reconstruction of the cranial vault, where the fused suture is surgically released to allow normal growth of the skull. However, advances in minimally invasive techniques, such as endoscopic strip craniectomy, are becoming increasingly popular due to their lower morbidity and shorter recovery times. This review aims to provide a comprehensive overview of sagittal craniosynostosis, highlighting the aetiology, clinical presentation, diagnostic methods, and current treatment options.

ABSTRACT Craniosynostosis is a rare condition of skull development, manifesting during fetal and early infant development, and is usually congenital. Craniosynostosis secondary to metabolic disorders, such as X‐linked hypophosphatemia (XLH), is less common and is typically diagnosed later than congenital craniosynostosis. XLH is a rare, progressive, and lifelong hereditary phosphate‐wasting disorder characterized by loss of function of the phosphate‐regulating endopeptidase homologue, X‐linked gene, which is associated with premature fusion of cranial sutures due to abnormal phosphate metabolism (hypophosphatemia) and altered bone mineralization or elevated levels of fibroblast growth factor 23. This targeted literature review of 38 articles seeks to provide an overview of craniosynostosis in individuals with XLH. The objectives of this review are to increase awareness of the prevalence, presentation, and diagnosis of craniosynostosis in XLH; examine the spectrum of craniosynostosis severity in XLH; discuss the management of craniosynostosis in those with XLH; recognize the complications for patients with XLH; and identify what is known about the burden of craniosynostosis for individuals with XLH. The presentation of craniosynostosis in individuals with XLH tends to manifest slightly later than congenital craniosynostosis and can vary in severity and appearance, making diagnosis difficult and resulting in inconsistent clinical outcomes. Consequently, craniosynostosis in patients with XLH is an underreported and potentially underrecognized condition. There have been no studies investigating the effects of craniosynostosis on the quality of life of people with XLH. Despite a growing awareness among researchers and experienced clinicians, there are still improvements to be made in general awareness and timely diagnosis of craniosynostosis in XLH. The XLH community would benefit from further study into the prevalence of craniosynostosis, the effect of XLH medical therapy on the development of craniosynostosis, and the effects of craniosynostosis on quality of life. © 2023 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.

Several craniofacial pathologies are present in an early adolescent from a cemetery (n = 22) associated with a Neolithic Funnel Beaker culture in Modřice, Czechia. The objective of the study was to document anomalies on the Modřice 3871 cranium, with an emphasis on investigating whether the mid-sagittal suture exhibited synostosis or bridging to account for the visible scaphocephaly. All available cranial and postcranial elements of the Modřice 3871 early adolescent were examined macroscopically. The cranial vault was subjected to radiography and compared to macroscopic views. Modřice 3871 is estimated to age to 12–14 years. Macroscopic examination and radiography support a diagnosis of mid-sagittal bridging rather than synostosis for this slender and anteroposteriorly elongated cranium. In comparison, the anterior sagittal, coronal and lambdoidal sutures are completely unfused. Craniofacial asymmetry, cribra orbitalia, porotic hyperostosis of the temporal and the presence of multiple wormian bones indicate additional maturational disruptions. The scaphocephaly observed in Modřice 3871 is not severe compared to modern clinical manifestations of craniosynostosis. Radiocarbon dated to 3,700–3,600 years BCE, Modřice 3871 presents one of the oldest recorded cases of scaphocephaly. The elongated vault is probably not the result of head-binding given the dearth of anthropogenic cranial reshaping in the Neolithic of Europe. This study adds to the growing recognition of prehistoric and historic craniofacial anomalies which will likely continue as additional human remains are excavated. In addition, the study increases the understanding of the lived experience of prehistoric individuals experiencing visible craniofacial pathologies such as cranial asymmetry and scaphocephaly. [ABSTRACT FROM AUTHOR]

Introduction: Failure of ossification in the vertical direction between the two halves of the frontal bone is called a metopic suture; this suture is present in between the superciliary arch and tubers of the frontal bone so it is also called a median frontal suture. At birth, the frontal bone present is two half, in the 1st year they contact each other, and within the 7th to 8th year, they fused together. Sometimes, bones may not be fused completely and it becomes metopic fontanelle. Materials and Methods: The goal of the current study was to determine the prevalence of metopic suture in adult human skulls in Uttar Pradesh. One hundred and fifty macerated skulls of undetermined age and sex were used for this. The anatomical departments of Teerthankar Mahaveer Medical College and Research Center (Moradabad), King George Medical College (Lucknow), SRMS Institute of Medical Science (Bareilly), Govt. Medical College Kannauj, and Govt. Medical College Saharanpur provided these skulls. Results: This study has found the incidence of metopic sutures of about 12.90%. The two types of metopic sutures were found in this study, namely complete metopic suture 4.52% and incomplete metopic suture 8.38% in which found 0.64% "V" shape metopic suture. Conclusion: The morphological study of metopic sutures on adult crania is useful for anatomists, experts in forensic medicine, and neurologists for performing surgical procedures in this area.

Purpose: The foramen magnum (FM) presents various alterations in craniosynostoses, such as brachycephaly or Crouzon syndrome. However, to date, no study has been devoted to its morphology and morphometry in scaphocephaly, which is the most common of cranial deformities resulting from premature fusion of cranial sutures. Methods: We assessed the morphology and morphometry of FM using preoperative thin-cut CT scans of 107 children with non-syndromic sagittal craniosynostosis aged 1–12 months (mean age 5.38 months). A series of sagittal and transverse dimensions were taken and the FM area was calculated in each case. Obtained data were compared to the age-matched control group of 101 normocephalic children. Results: Dolichotrematous type of FM was dominant in the scaphocephaly group and observed in 63/107 cases (58.9%). The mean FM area in the scaphocephaly group was 519.64 mm2 and was significantly smaller compared to the control group (p = 0.0011). The transverse diameter and anterior sagittal diameter were also significantly smaller (p = 0.0112 and p = 0.0003, respectively). Conclusion: The area of FM in scaphocephaly is smaller compared to normal individuals. This is associated with a significant reduction of the width of FM in children with sagittal craniosynostosis. FM in scaphocephaly is larger than in other reported series of children with brachycephaly or Crouzon syndrome. [ABSTRACT FROM AUTHOR]

Orofaciodigital syndrome (OFD) is clinically heterogeneous and is characterized by abnormalities in the oral cavity, facial features, digits, and central nervous system. At least 18 subtypes of the condition have been described in the literature. OFD is caused by variants in several genes with overlapping phenotypes. We studied a consanguineous Pakistani family with two affected siblings with an atypical form of OFD type 4 (OFD4). In addition to the typical features of OFD4 that include limb defects and growth retardation, the siblings displayed rare features of scaphocephaly and seizures. Exome sequencing analysis revealed a novel homozygous splice site variant c.257‐1G>A in TCTN3 that segregated with disease. This homozygous splice site variant in TCTN3 is most likely the underlying cause of the atypical form of OFD4 observed in this family. Our results contribute to the phenotypic spectrum of TCTN3 associated ciliopathies and will facilitate better clinical diagnosis. [ABSTRACT FROM AUTHOR]

Background: Scaphocephaly is a craniofacial anomaly where the skull is disproportionately long and narrow. Premature closure of the sagittal suture is thought to be at the core of the etiopathology for scaphocephaly. Case presentation: The skull is a well-preserved skull found in an antiquated graveyard at Parkand village Daregaz, Iran, with no visible signs of surgical manipulation. The craniofacial indices were measured. The maximum cranial length was 200 mm, and the measured length of nasion-bregma was 140mm, whereas most of the measurements were less than the normal scaphocephalic indices. Another morphological finding was the flattening of the frontal bone. Conclusions: The cranial vault morphometry and morphology of the presented case fit the scalp. In addition to the characteristics of scaphocephaly, the presented skull was of a rare trait, frontal flattening, which has not been reported before. [ABSTRACT FROM AUTHOR]

Turricephaly is regarded as one of the most challenging cranial malformations to treat because increasing intracranial pressure can occur when cranial height is reduced. In typical medical and nursing situations, kids with turricephaly can be identified by thier osteogenesis imperfecta, ophthalmic proptosis, hydrocephalus, and certain facial characteristics such substantial temporal steamrolling, facial asymmetry hypoplasia, and micrognathia.Turricephaly is a rare and potentially fatal autoimmune disease. In the beginning, estimates of turricephaly from antiquity to the present are shown, and in the end, the findings of a personal investigation into the generally disturbed social behaviour and A 4 year male child admited in pediatric department in hospital the patient reported Head swelling, nausea, occipital area swelling, both eyes swelling, and irregular skull form. the primary therapeutic interventions was given to the patient and he was treated with syp zincovit 5 ml BD antibiotic intravenous fluids during hospitalization and the treatment was continued until my final day of care. [ABSTRACT FROM AUTHOR]

Craniosynostosis is a congenital craniofacial anomaly caused by the premature closure of one or more sutures. Scaphocephaly is the most common form. Endoscopic surgery is increasingly being used for the treatment of a single synostosis. We present our first case of endoscopy-assisted surgery for scaphocephaly in a three-month-old baby. Follow-up at one year was satisfactory, showing a good aesthetic result. The child did not require blood transfusion. Endoscopic surgery presents various advantages in treating scaphocephaly. It is necessary to educate our primary care physicians and improve their awareness about the importance of endoscopic surgeries in scaphocephaly., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Lafontaine et al.)

This contribution is part of a project concerning the creation of an artificial dataset comprising 3D head scans of craniosynostosis patients for a deep-learning-based classification. To conform to real data, both head and neck are required in the 3D scans. However, during patient recording, the neck is often covered by medical staff. Simply pasting an arbitrary neck leaves large gaps in the 3D mesh. We therefore use a publicly available statistical shape model (SSM) for neck reconstruction. However, most SSMs of the head are constructed using healthy subjects, so the full head reconstruction loses the craniosynostosis-specific head shape. We propose a method to recover the neck while keeping the pathological head shape intact. We propose a Laplace- Beltrami-based refinement step to deform the posterior mean shape of the full head model towards the pathological head. The artificial neck is created using the publicly available Liverpool-York-Model. We apply our method to construct artificial necks for head scans of 50 scaphocephaly patients. Our method reduces mean vertex correspondence error by approximately 1.3 mm compared to the ordinary posterior mean shape, preserves the pathological head shape, and creates a continuous transition between neck and head. The presented method showed good results for reconstructing a plausible neck to craniosynostosis patients. Easily generalized it might also be applicable to other pathological shapes.

We report a case of a patient with premature fusion of bilateral squamous sutures and perform a literature review of similar cases. We have noted in our case and in other reports that bilateral squamous synostosis does not lead to significant clinical effects such as deformed shape of the skull or raised intracranial pressure.

Introduction: Due to varying cranial morphologies, ventricular access in patients with craniosynostosis using conventional techniques is often a challenge. Although ventricular access may not be frequently required in scaphocephalic patients, it is vital that an ideal location of the access points be established for safe ventricular catheterization. This study aimed to document the morphometry of commonly used ventricular access points, i.e. Kocher's and Frazier's points, within a select South African scaphocephalic paediatric population. Methods: The craniometric dimensions of Kocher's and Frazier's points were measured relative to anatomical and craniometric landmarks on pre-operative computed tomography scans of 24 consecutive patients diagnosed with scaphocephaly between 2014 and 2020. Results were compared against age, sex, population group and the degree of severity of the deformity. Results: Kocher's point was located between 91.6 mm and 140.0 mm posterior to the nasion, and between 20.5 mm and 34.6 mm lateral to the midline. Statistically significant increases were reported in the mean distance posterior to nasion between patients in the 1-

• The "Peau d'ours" technique represents an alternative to treat Scaphocephaly (SC). We presented a total of 53 patients with SC enrolled between 2011 and 2016 in Mexico. • The advantages in this technique are health coronal and lambdoid suture preservation, and symmetrical parietal bone flap opening. • This technique is safe and simple to reproduce allowing a preservation of intellect, sight, and body image with a low incidence of secondary craniosynostosis. • This technique is ideal for patients older than 6 months, which represents a common clinical scenario in Mexico. Scaphocephaly (SC) is defined as an elongation of the anteroposterior axis of the skull resulting from the abnormal fusion of the sagittal suture. This study describes the "Peau d'ours" technique and results for correcting SC. We conducted a consecutive and retrospective analysis of patients treated from 2011 to 2016. We evaluated the gender, age, and surgical outcomes. A total of 53 patients were enrolled with a mean age of 19 months old. The advantages of this technique are healthy coronal and lambdoid suture preservation and symmetrical parietal bone flap opening. This technique is safe and simple to reproduce, allowing good surgical outcomes with a low incidence of secondary craniosynostosis. This technique is ideal for patients older than six months old. [ABSTRACT FROM AUTHOR]

Purpose: Scaphocephaly is the premature closure of the sagittal suture. The treatment strategies mainly focus on correcting the shape of the head, but there are very limited studies examining changes in brain structure. This study aimed to investigate shape differences in the shape of corpus callosum regarding the pre-treatment and post-treatment term at scaphocephaly patients. Methods: Cranium shape data were collected from the two-dimensional digital images. The generalized Procrustes analysis was used to obtain mean shapes in the pre- and postoperative phases. The shape deformation of the corpus callosum from the pre- to postoperative phases was evaluated using the thin plate spline method. Results: There is an enlargement of the splenium part of corpus callosum in the late group. In the early group, corpus callosum genu and body enlargement were observed in the postoperative period compared to the preoperative period, followed by a narrowing of the isthmus region. Conclusion: This study showed structural deformations in the corpus callosum in scaphocephaly patients using head shape with the landmark-based geometric morphometric method by taking into consideration the topographic distribution. An enlargement at the splenium part of corpus callosum exposes after the cranial vault expansion depending on time. [ABSTRACT FROM AUTHOR]

Craniosynostosis refers to the premature closure of calvarial sutures. Premature closure is subclassified into primary and secondary causes. The clinical manifestations of premature closure depend upon the involved suture and the resultant cranial dysmorphism. Common clinical disabilities include mental retardation, visual deficits, and oropharyngeal airway obstruction. Treatment is based on patient age and clinical severity. This article reviews the imaging manifestation of the most common forms of craniosynostosis with an emphasis on plain film and CT appearance of the disease. We also briefly review the appearance of common mimickers of suture pathology. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background and purpose: Morphological correction is one of the main aims of surgery for sagittal synostosis (SSO). Different surgical techniques have been developed; however, few studies have compared the different surgical protocols. The morphological outcome is poorly documented, because a consensual evaluation tool is lacking. Material and methods: We performed a prospective study of children operated for SSO in our institution. Children were operated whenever possible at 4 months for craniectomy; by default, children underwent cranioplasty at or after 9 months. The morphological outcome of all children was evaluated using traditional craniometry with head circumference (HC) and the cephalic index (CI), and with the Rotterdam scaphocephaly morphology score (RSMS), a total of semi-quantitative assessments of morphological hallmarks. Results: Craniectomy was significantly associated with a shorter operation time and hospital stay, and a better impact on HC and CI measurements, compared with cranioplasty. The RSMS was markedly improved after surgery in both groups; however, we found no significant difference in improvement between the two groups. Although the transfusion rate and the prevalence of developmental delay were lower in the craniectomy group, and reoperations for calvarial lacunae or complex craniosynostosis occurred only this group, these differences were not significant. Conclusions: Our results support early surgery with craniectomy whenever possible; however, cranioplasty at a later age is a very acceptable by-default indication. In addition to classical craniometry, morphological evaluation using the RSMS or a similar quantitative scale appears highly desirable for future studies [ABSTRACT FROM AUTHOR]

Background: Sensenbrenner syndrome, also known as cranioectodermal dysplasia (CED), is a genetically heterogeneous ciliopathy, characterized by dysmorphic features including dolichocephaly (with inconstant sagittal craniosynostosis), chronic kidney disease (CKD), hepatic fibrosis, retinitis pigmentosa, and brain abnormalities, with a partial clinical overlap with other ciliopathies. Patients and methods: A retrospective review of four children with Sensenbrenner syndrome treated at the Femme Mère Enfant University Hospital of Lyon from 2005 to 2020 was conducted. Results: Variants in WDR35 or WDR19 were found in all children. Two of them underwent surgery for a scaphocephaly in the first months of life. All patients developed CKD leading to end-stage renal disease during the first/second decades. Discussion: The diagnosis of scaphocephaly may precede the diagnosis of the underlying Sensenbrenner syndrome, thus highlighting the importance of a systematic multidisciplinary assessment and follow-up for craniosynostoses, in order to identify syndromic forms requiring specific management. In Sensenbrenner syndrome, patients' management should be coordinated by multidisciplinary teams of reference centers for rare diseases, with expertise in the management of craniofacial malformations as well as rare skeletal and renal disorders. Indeed, a prompt etiological diagnosis will result in an early diagnosis of multisystemic complications, notably renal involvement, thus improving global prognosis. [ABSTRACT FROM AUTHOR]

Purpose: Studies have examined the impact of fusion of the sagittal suture in the skull base while others have evaluated the growth of the skull base before and after surgery. This study aims to perform the anthropometric measures of the skull base in children with scaphocephaly to evaluate the influence of surgical repair in the remodeling of the skull base and anthropometric measures. Methods: Twenty-one children with diagnosis of scaphocephaly were operated between April 2007 and October 2008, and anthropometric measures at the skull base were performed before and after a year of surgery. The measures were the cranial index (CI), distance between the crista galli and tuberculum sellar (CG-TS), distance between the crista galli and the internal auditory meatus (CG-IAM), distance between the oval foramen (OF-OF), distance between the internal auditory meatus (IAM-IAM), the angle of the skull base (Â1), and the angle between the nasion, center of sella, and basion (Â2). Results: There was a normalization of the CI in all children, confirming an appropriate cranial remodeling. The CG-TS measure evaluated the anterior skull base, with proportional growth of 12.5%. The mediolateral growth was observed by the increase of OF-OF measures by 8.5% and IAM-IAM by 9.5%. The CG-TS measure grew by 7.2%. There was no statistical difference in the angles analyzed. Conclusion: Surgical treatment of scaphocephaly led to remodeling of the skull base, confirmed by the changes of anthropometric measures taken before and after a year of surgery. [ABSTRACT FROM AUTHOR]

Purpose: The aim is to implement Utrecht Cranial Shape Quantificator (UCSQ) for quantification of severity of scaphocephaly and compare UCSQ with the most used quantification method, cranial index (CI). Additionally, severity is linked to intracranial volume (ICV). Methods: Sinusoid curves of 21 pre-operative children (age < 2 years) with isolated scaphocephaly were created. Variables of UCSQ (width of skull and maximum occiput and forehead) were combined to determine severity. CI was calculated. Three raters performed visual scoring for clinical severity (rating of 6 items; total score of 12 represents most severe form). Pearson's correlation test was used for correlation between UCSQ and visual score and between both CIs. ICV was calculated using OsiriX. ICV was compared to normative values and correlated to severity. Results: Mean UCSQ was 22.00 (2.00–42.00). Mean traditional CI was 66.01 (57.36–78.58), and mean visual score was 9.1 (7–12). Correlations between both traditional CI and CI of UCSQ and overall visual scores were moderate and high (r = − 0.59; p = 0.005 vs. r = − 0.81; p < 0.000). Mean ICV was 910 mL (671–1303), and ICV varied from decreased to increased compared to normative values. Negligible correlation was found between ICV and UCSQ (r = 0.26; p > 0.05) and between ICV and CI and visual score (r = − 0.30; p > 0.05 and r = 0.17; p > 0.05, respectively). Conclusion: Our current advice is to use traditional CI in clinical practice; it is easy to use and minimally invasive. However, UCSQ is more precise and objective and captures whole skull shape. Therefore, UCSQ is preferable for research. Additionally, more severe scaphocephaly does not result in more deviant skull volumes. [ABSTRACT FROM AUTHOR]

Background: Craniosynostosis is the premature closure of cranial sutures and it continues to be a therapeutic challenge due to the diversity and complexity of the syndrome. Bibliometric analysis is a study of ranking citations and exploring the most impactful articles in a respective discipline. It also demonstrates the chronological trends of publications. Methods: In May 2020, we performed a title-specific search of the Scopus database using "craniosynostosis" as our query term without publication date restrictions. The top 100 articles in craniosynostosis were retrieved and analyzed. Results: The top 100 most-cited articles in craniosynostosis received a total 13,826 citations, and an average of 138 citations per paper. The publication dates ranged from 1920 to 2015, with a peak period of top publications between 1996 and 2005. The most common category is clinical, followed by neurogenetics. The top cited article received 540 citation counts and 19.29 citations per year. The USA was the most contributing country to the list. The Journal of Plastic and Reconstructive Surgery published the largest number of top cited articles. Neurosurgery as a specialty contributed to most articles in the list (27 articles). The institute who contributed the most was the Assistance Publique Hopitaux Paris. Conclusion: Bibliometric analysis in craniosynostosis revealed major trend changes of research over the years, with a focus on neurogenetics and the different types of surgical corrections. The current collection of highly cited publications may assist physicians in gaining a better understanding of the evidence-based approach in craniosynostosis. [ABSTRACT FROM AUTHOR]

Background: We sought to investigate associations between maternal/infant characteristics and isolated craniosynostosis as well as its subtypes sagittal, metopic, and coronal synostosis, and assess trends in the prevalence of these conditions. Methods: We identified cases in the Texas Birth Defects Registry from 1999 to 2014. We used Poisson regression to identify associations between maternal/infant characteristics and craniosynostosis. We used joinpoint regression and unadjusted Poisson regression to evaluate temporal trends. Finally, we computed adjusted Poisson models to evaluate whether temporal trends were evident after accounting for changes in the population distributions of maternal/infant characteristics over time. Results: Relative to all live births in the general population, cases were more frequently male or preterm. Mothers of cases were more frequently non‐Hispanic white and more frequently obese. Non‐Hispanic black or Hispanic maternal race/ethnicity was associated with a lower prevalence of all craniosynostosis subtypes. Previous live births were associated with sagittal synostosis; residence on the U.S.‐Mexico border was associated with sagittal and coronal synostosis. The prevalence of any isolated craniosynostosis increased (average annual percent change estimated from joinpoint regression [AAPC]: 2.9%), as did the prevalences of sagittal (AAPC: 3.3%) and metopic synostosis (AAPC: 5.4%). In crude Poisson models, the same temporal trends were observed, however these were attenuated after adjusting for maternal/infant characteristics. Conclusions: Prevalence of isolated craniosynostosis increased from 1999 to 2014. The largest AAPC was observed for metopic synostosis. Changes in the population distribution of associated maternal/infant characteristics may explain these trends. [ABSTRACT FROM AUTHOR]

Shunt-induced craniosynostosis is a rare complication of ventricular shunting for hydrocephalus in pediatric patients. Although the exact pathophysiology of this form of secondary craniosynostosis is not well understood, the current understanding is that persistent drainage of the ventricular shunt causes decreased dural tension, resulting in decreased expansile force on the cranium and premature sutural fusion. Due to the low incidence of this complication, there is no consensus on the ideal treatment for shunt-induced craniosynostosis. In recent years, distraction osteogenesis has been employed with greater frequency, as it is felt to counter the fundamental problem of decreased expansile force on the cranium. However, in a patient with a ventricular shunt, placement of external hardware in close proximity to the shunt could cause significant morbidity due to the increased risk of shunt infection. We present the management of a patient with shunt-induced craniosynostosis who continued to be shunt-dependent. We chose to use fully buried springs to create an expansile force on the cranium as an alternative to external distractors so as to mitigate the risk of infection. We demonstrate that spring-assisted distraction osteogenesis can be an effective treatment modality for patients with shunt-induced craniosynostosis. This method should be considered in patients with contraindications to external distraction devices, such as ongoing shunt dependency. [ABSTRACT FROM AUTHOR]

Sagittal craniosynostosis consists of premature fusion (ossification) of the sagittal suture during infancy, resulting in head deformity and brain growth restriction. Spring-assisted cranioplasty (SAC) entails skull incisions to free the fused suture and insertion of two springs (metallic distractors) to promote cranial reshaping. Although safe and effective, SAC outcomes remain uncertain. We aimed hereby to obtain and validate a skull material model for SAC outcome prediction. Computed tomography data relative to 18 patients were processed to simulate surgical cuts and spring location. A rescaling model for age matching was created using retrospective data and validated. Design of experiments was used to assess the effect of different material property parameters on the model output. Subsequent material optimization—using retrospective clinical spring measurements—was performed for nine patients. A population-derived material model was obtained and applied to the whole population. Results showed that bone Young's modulus and relaxation modulus had the largest effect on the model predictions: the use of the population-derived material model had a negligible effect on improving the prediction of on-table opening while significantly improved the prediction of spring kinematics at follow-up. The model was validated using on-table 3D scans for nine patients: the predicted head shape approximated within 2 mm the 3D scan model in 80% of the surface points, in 8 out of 9 patients. The accuracy and reliability of the developed computational model of SAC were increased using population data: this tool is now ready for prospective clinical application. [ABSTRACT FROM AUTHOR]

Background: In spite of literature data stating that children with single-suture craniosynostosis have an increased risk for neuropsychological deficits, no data are present clarifying the potential risk factors. Methods: All children with non-syndromic single-suture craniosynostosis operated on from January 2014 to January 2017 were enrolled. A comprehensive neurocognitive and neuro-ophthalmological evaluation was performed before surgery and 6 months after surgery. A further neurocognitive evaluation was performed 12 months after surgery. All children had a preoperative CT/MR study. Results: One hundred forty-two patients were enrolled; 87 are affected by sagittal craniosynostosis, 38 by trigonocephaly, and 17 by plagiocephaly. A global neurocognitive impairment was documented in 22/87 children with scaphocephaly, 5/38 children with trigonocephaly, and 6/17 children with anterior plagiocephaly. There was a significant relationship between results of the ophthalmological evaluation, global IQ, and CT findings at diagnosis (r = 0.296, p < 0.001; r =0.187, p 0.05). Though a significant recovery was documented after surgery, a persistence of eye coordination deficits was present at 6 months in 1 out of 3 children with abnormal preoperative exams. A significant correlation was found between pathological CT findings and persistence of below average neuro-ophthalmological and neurocognitive findings 6 months after surgery, as well as between CT findings and neurocognitive scores at the 1 year follow-up (r = 0.411; p < 0.01). Conclusion: The presence of neuroradiological abnormalities appears to be related to both ophthalmological and neurocognitive deficits at diagnosis. This relationship is maintained in spite of the surgical treatment in children who show the persistence of ophthalmological and neurocognitive deficits during the follow-up. [ABSTRACT FROM AUTHOR]

The occurrence of secondary synostosis of coronal sutures at distance from H-craniectomy surgery for scaphocephaly concerns about 10% of children. Intracranial hypertension in these children remains exceptional but generally requires a surgical reoperation. Two children aged 3 and 5- months- old had been operated for scaphocephaly by H-craniectomy in two different hospital centers. Their clinical follow-up described a partial persistence of dolichocephalic deformity and an impression of parietal stenosis. During their growth, chronic headaches appeared with a complaint expressed at the ages of 4 and 5 years. In both cases, ophthalmic examination revealed significant bilateral papillary edema without loss of visual acuity. The imaging assessment (CT-scan and MRI) showed the absence of Chiari malformation and venous abnormality. For both, there was a compression image of the parietal lobes in relation to the persistence of a temporoparietal synostosis. An osteogenic parietal distraction permitted a volumetric brain expansion consecutive to the skull and meninges remodeling in only 6 months, associated with a leap forward acquisition, a normalization of the ophthalmic examination, and a complete loss of headaches. In conclusion, this new approach could be used in the case of chronic intracranial hypertension consecutive to a secondary parietal synostosis after a scaphocephaly surgery. [ABSTRACT FROM AUTHOR]

Purpose: Among patients with isolated sagittal synostosis (ISS), the head shape varies considerably in relation to the severity of the abnormality. This study aimed to quantify skull base morphometry and intracranial volume to investigate their relationships with the severity of scaphocephaly. Methods: We studied 66 infants with ISS identifying three groups according to the morphological severity of cranial deformity (group I: mild deformity; group II: moderate deformity; group III: severe deformity), by combining two scaphocephaly severity indices as descriptors of the relation of three morphological measurements (length, width and height) We perform a quantitative analysis using high-resolution CT images calculating following parameters: cranial fossae dimensions, supratentorial (ICV) and infratentorial (PCFV) cranial volume, supratentorial (WBV) and infratentorial (PCFBV) brain volume, ICV/WBV, PCFV/PCFBV, supratentorial and infratentorial cerebrospinal fluid (CSF). Results: In all subgroups, anterior and middle skull base lengths were increased, while posterior hemifossae lengths were unchanged. In mild subgroup, ICV/WBV was significantly different and ICV, WBV and CSF supratentorial volume increased (p < 0.05). In moderate and severe subgroups, FCPV/FCPBV was significantly different and CSF infratentorial volume was reduced (p < 0.05); FCPBV was increased only in the severe subgroup (p < 0.05). Conclusion: This morpho-volumetric study provides new insights in understanding the compensatory changes occurring in infants at different stages of scaphocephaly severity. In particular, our study suggests that patients with severe deformity might have an earlier depletion of reserve mechanisms with a reduced compliance of the overall skull during encephalic growth and these patients might require early surgical cranial expansion. [ABSTRACT FROM AUTHOR]