Skull Volume Research Articles

Characterization of the craniofacial abnormalities of the homozygous G608G progeria mouse model

IntroductionHutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by premature aging, impacting multiple organ systems, including cardiovascular, musculoskeletal, and integumentary. Significant abnormalities in a transgenic mouse model (homozygous G608G mutation), specifically targeting the development of skull and facial bone indices through high-resolution CT scanning and cephalometric analysis.MethodsKey measurements include bone thickness, skull volume, and cranial suture integrity. Bone volume increased significantly in HGPS mice by 8 months of age compared to wildtype mice.ResultsCortical thickness showed a trend toward increased values in HGPS mice. Cranial metrics revealed distinct differences.DiscussionHGPS mice exhibited smaller internasal width, interzygomatic distance, and palatine length compared to WT mice over time.

Comprehensive comparative study ofChiari-like malformation inveterinary and human medicine.

This review aims toenrich our understanding ofChiari-like malformation (CLM) bycombining human and veterinary insights, and providing adetailed cross-species overview. CLM isadevelopmental abnormality characterised bycaudal displacement ofthe hindbrain into the foramen magnum due toan entire brain parenchymal shift caused byinsufficient skull volume. This malformation leads toaprogressive obstruction atthe craniocervical junction, which disrupts the normal cerebrospinal fluid flow, leading tosecondary syringomyelia. The clinical signs ofCLM and syringomyelia include phantom scratching, head tilt, head tremor, ataxia, tetraparesis, pain, muscle atrophy, and scoliosis ortorticollis. Magnetic resonance imaging remains the gold standard for diagnosing CLM, since itallows the visualisation ofabnormal findings such asthe caudal cerebellar herniation, caudal cerebellar compression from occipital dysplasia, and attenuated cerebrospinal fluid cisternae. Although various medical and surgical interventions, including foramen magnum decompression, can provide temporary symptomatic/clinical sign relief, current literature shows alack ofsustained long-term efficacy. Therefore, additional research isneeded toevaluate the long-term effects ofexisting treatment strategies and tocompare different techniques utilised inconjunction with foramen magnum decompression.

A computational modelling tool for prediction of head reshaping following endoscopic strip craniectomy and helmet therapy for the treatment of scaphocephaly

BackgroundEndoscopic strip craniectomy followed by helmet therapy (ESCH) is a minimally invasive approach for correcting sagittal craniosynostosis. The treatment involves a patient-specific helmet designed to facilitate lateral growth while constraining sagittal expansion. In this study, finite element modelling was used to predict post-treatment head reshaping, improving our comprehension of the necessary helmet therapy duration. MethodSix patients (aged 11 weeks to 9 months) who underwent ESCH at Connecticut Children's Hospital were enrolled in this study. Day-1 post-operative 3D scans were used to create skin, skull, and intracranial volume models. Patient-specific helmet models, incorporating areas for growth, were designed based on post-operative imaging. Brain growth was simulated through thermal expansion, and treatments were modelled according to post-operative Imaging available. Mechanical testing and finite element modelling were combined to determine patient-specific mechanical properties from bone samples collected from surgery.Validation compared simulated end-of-treatment skin surfaces with optical scans in terms of shape matching and cranial index estimation. ResultsComparison between the simulated post-treatment head shape and optical scans showed that on average 97.3 ± 2.1 % of surface data points were within a distance range of −3 to 3 mm. The cranial index was also accurately predicted (r = 0.91). ConclusionsIn conclusion, finite element models effectively predicted the ESCH cranial remodeling outcomes up to 8 months postoperatively. This computational tool offers valuable insights to guide and refine helmet treatment duration. This study also incorporated patient-specific material properties, enhancing the accuracy of the modeling approach.

Quantitative analysis of the skull in the Japanese wolf (Canis lupus hodophilax) using CT.

In this study using computed tomography (CT), the volumes of the internal cranial cavities, such as the braincase, frontal sinus and tympanic cavity, and the ratio of the volume of each cavity to the skull volume in Japanese wolves were quantified, and CT images of the frontal sinus were observed. The results were then compared with those of other wolf subspecies, including Akita, a dog breed, to clarify the characteristics of the internal cranial cavities in Japanese wolves. The present study revealed that the Japanese wolf had a relatively larger braincase volume and a relatively smaller frontal sinus volume than the wolf ssp. (a group of wild wolf subspecies except the Japanese wolf) and Akita. Moreover, the relative and absolute tympanic cavity volumes of the Japanese wolf and Akita were significantly smaller than those of the wolf ssp. In the CT image or macroscopic observations, the frontal sinuses of the wolf ssp. and Akita were relatively well developed to the caudal and dorsal directions, respectively, compared with that of the Japanese wolf, and the tympanic cavity of the wolf ssp. was more largely swelled ventrally and medially than that of other groups.

Relationship between edema and intracranial pressure following intracerebral hemorrhage in rat

Elevated intracranial pressure (ICP) is a potentially fatal consequence of intracerebral hemorrhage (ICH). As the mass of the hematoma and regional edema builds, ICP rises and becomes increasingly variable acutely after stroke. High ICP may worsen cellular injury and edema by impairing local tissue perfusion, fueling a cycle that may ultimately cause fatality through ischemia and brain herniation. Time spent above an ICP of 20 mmHg often predicts a greater risk of death and disability following ICH. Compensatory mechanisms combat rising ICP. Classically, these include cerebrospinal fluid volume loss and cerebrovascular autoregulation, such as a reduction in the volume of venous blood. Additional mechanisms such as brain tissue compliance and skull volume compensation may also contribute. Compensatory compliance mechanisms are limited, and they vary by age and many other factors. Animal models of ICH are widely used to assess these variables and to gauge putative therapeutics. Most often those studies rely upon simple measures of edema, which may not accurately predict ICP data. Thus, we analyzed our past studies characterizing ICP, edema, and tissue compliance responses to striatal ICH in rat, including the collagenase (C-ICH) and whole blood models (WB-ICH). We found that both ICH models raised ICP, with greater effects in the C-ICH model, which may thus better reflect clinical findings of concern. Importantly, measures of edema, such as in the damaged hemisphere, on their own are not predictive of average or peak ICP response within either model, unless assessing across a very wide range of injury severities, or when including non-stroke animals. We caution against using edema data as a surrogate measure of mass effect and ICP following ICH.

The Paediatric Vault Score (PVS)-A Novel Scoring Tool for Prioritisation of Surgical Management of Craniosynostosis Patients.

Craniosynostosis is the premature fusion of the skull sutures, resulting in abnormal skull shape and volume. Timely management is a priority in avoiding raised intracranial pressure which can result in blindness and neurodevelopmental delay. Due to the COVID-19 pandemic, theater access was reduced. A risk stratification scoring system was thus devised to score patients attending surgery and aid in prioritization according to surgical need. The authors present the Paediatric Vault Score (PVS), which can also be customized to each unit's individual protocols. Ten patients on the waiting list were randomly selected and their clinical information was summarized in uniform anonymized reports. Six craniofacial consultants were selected as assessors and given 1 week to independently rank the patients from 1 to 10. Each scorer's ranking was verified against the PVS template and concordance was analyzed using the Kendall tau correlation coefficient (KT). Three cycles of the scoring process were carried out. Improvements were made to the scoring tool following cycle 1. Cycle 1 revealed 2 clinicians to be concordant with the PVS system and 4 to be discordant. Cycle 2 revealed all 6 clinicians to be concordant, with a mean KT score of 0.61. The final cycle revealed all 6 clinicians to be concordant, with a mean KT score of 0.70. Four scorers increased their concordance once the scoring sheet was introduced. Kendall's correlation of concordance calculated the interrater reliability to be 0.81. The PVS is the first known vault scoring system to aid in risk stratification and waiting list prioritization.

Longitudinal MRI Evaluation of Brain Development in Fetuses with Congenital Diaphragmatic Hernia around the Time of Fetal Endotracheal Occlusion.

Congenital diaphragmatic hernia is associated with high mortality and morbidity, including evidence suggesting neurodevelopmental comorbidities after birth. The aim of this study was to document longitudinal changes in brain biometry and the cortical folding pattern in fetuses with congenital diaphragmatic hernia compared with healthy fetuses. This is a retrospective cohort study including fetuses with isolated congenital diaphragmatic hernia between January 2007 and May 2019, with at least 2 MR imaging examinations. For controls, we used images from fetuses who underwent MR imaging for an unrelated condition that did not compromise fetal brain development and fetuses from healthy pregnant women. Biometric measurements and 3D segmentations of brain structures were used as well as qualitative and quantitative grading of the supratentorial brain. Brain development was correlated with disease-severity markers. Forty-two fetuses were included, with a mean gestational age at first MR imaging of 28.0 (SD, 2.1) weeks and 33.2 (SD, 1.3) weeks at the second imaging. The mean gestational age in controls was 30.7 (SD, 4.2) weeks. At 28 weeks, fetuses with congenital diaphragmatic hernia had abnormal qualitative and quantitative maturation, more extra-axial fluid, and larger total skull volume. By 33 weeks, qualitative grading scores were still abnormal, but quantitative scoring was in the normal range. In contrast, the extra-axial fluid volume remained abnormal with increased ventricular volume. Normal brain parenchymal volumes were found. Brain development in fetuses with congenital diaphragmatic hernia around 28 weeks appears to be delayed. This feature is less prominent at 33 weeks. At this stage, there was also an increase in ventricular and extra-axial space volume.

Craniofacial characteristics in Crouzon's syndrome: A systematic review and meta-analysis.

The purpose of this meta-analysis was to strengthen the credibility of primary research results by combining open-source scientific material, namely a comparison of craniofacial features (Cfc) between Crouzon's syndrome (CS) patients and non-CS populations. All articles published up to October 7, 2021, were included in the search of PubMed, Google Scholar, Scopus, Medline, and Web of Science. The PRISMA guidelines were followed to conduct this study. PECO framework was applied in the following ways: Those who have CS are denoted by the letter P, those who have been diagnosed with CS via clinical or genetic means by the letter E, those who do not have CS by the letter C, and those who have a Cfc of CS by the letter O. Independent reviewers collected the data and ranked the publications based on their adherence to the Newcastle-Ottawa Quality Assessment Scale. A total of six case-control studies were reviewed for this meta-analysis. Due to the large variation in cephalometric measures, only those published in at least two previous studies were included. This analysis found that CS patients had a smaller skull and mandible volumes than those without CS.in terms of SNA° (MD = -2.33, p = <0.001, I2 = 83.6%) and ANB°(MD = -1.89, p = <0.005, I2 = 93.1%)), as well as ANS (MD = -1.87, p = 0.001, I2 = 96.5%)) and SN/PP (MD = -1.99, p = 0.036, I2 = 77.3%)). In comparison to the general population, people with CS tend to have shorter and flatter cranial bases, smaller orbital volumes, and cleft palates. They differ from the general population in having a shorter skull base and more V-shaped maxillary arches.

Transcranial Magnetic Stimulation: The Effect of Age and Other Factors on the Intensity of the Quadruple Butterfly Coil

Transcranial magnetic stimulation (TMS) is a non-invasive technique approved by the US Food and Drug Administration (FDA) to treat certain neurological disorders. When comparing TMS to techniques with similar desired outcomes, such as conventional brain surgery, TMS has far fewer risks due to its non-invasive nature. TMS works by inducing an electric field ( <inline-formula xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink"> <tex-math notation="LaTeX">$E$ </tex-math></inline-formula> -field) that polarizes or depolarizes brain tissues depending on treatment mode. The human brain reaches 90% of its adult volume at about age six and does not reach full maturity until approximately 25 years. The differences in the brain volume from adolescents to adults could alter the effectiveness of TMS, primarily because the coils have been designed for use on adult patients. Therefore, comparing the efficacy of TMS coils across age groups to determine whether the difference in brain volume is enough to warrant specific TMS treatment protocols for adolescents is of the essence in advancing TMS research. In this research, the authors compare the use of a novel TMS coil design, quadruple butterfly coil (QBC), across different age groups to test the effectiveness of the coil on various stages of brain development. Simulations were run using a finite element analysis software, Sim4Life. Besides age and brain (gray matter) volume, the authors also considered other factors such as the brain-scalp distance (BSD), cerebrospinal fluid (CSF) thickness, scalp thickness, skull thickness, and skull volume of the human models in the analysis. This study provides an understanding of the various factors contributing to the response from TMS. The maximum <inline-formula xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink"> <tex-math notation="LaTeX">$E$ </tex-math></inline-formula> -field intensity on the scalp ( <inline-formula xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink"> <tex-math notation="LaTeX">$E$ </tex-math></inline-formula> -Max scalp), maximum <inline-formula xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink"> <tex-math notation="LaTeX">$E$ </tex-math></inline-formula> -field intensity on the brain ( <inline-formula xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink"> <tex-math notation="LaTeX">$E$ </tex-math></inline-formula> -Max brain), and the proportion of the <inline-formula xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink"> <tex-math notation="LaTeX">$E$ </tex-math></inline-formula> -Max brain to <inline-formula xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink"> <tex-math notation="LaTeX">$E$ </tex-math></inline-formula> -Max scalp were parameters considered in this analysis.

Deletion of the Pyrophosphate Generating Enzyme ENPP1 Rescues Craniofacial Abnormalities in the TNAP-/- Mouse Model of Hypophosphatasia and Reveals FGF23 as a Marker of Phenotype Severity.

Hypophosphatasia is a rare heritable metabolic disorder caused by deficient Tissue Non-specific Alkaline Phosphatase (TNAP) enzyme activity. A principal function of TNAP is to hydrolyze the tissue mineralization inhibitor pyrophosphate. ENPP1 (Ectonucleotide Pyrophosphatase/Phosphodiesterase 1) is a primary enzymatic generator of pyrophosphate and prior results showed that elimination of ENPP1 rescued bone hypomineralization of skull, vertebral and long bones to different extents in TNAP null mice. Current TNAP enzyme replacement therapy alleviates skeletal, motor and cognitive defects but does not eliminate craniosynostosis in pediatric hypophosphatasia patients. To further understand mechanisms underlying craniosynostosis development in hypophosphatasia, here we sought to determine if craniofacial abnormalities including craniosynostosis and skull shape defects would be alleviated in TNAP null mice by genetic ablation of ENPP1. Results show that homozygous deletion of ENPP1 significantly diminishes the incidence of craniosynostosis and that skull shape abnormalities are rescued by hemi- or homozygous deletion of ENPP1 in TNAP null mice. Skull and long bone hypomineralization were also alleviated in TNAP−/−/ENPP1−/− compared to TNAP−/−/ENPP1+/+ mice, though loss of ENPP1 in combination with TNAP had different effects than loss of only TNAP on long bone trabeculae. Investigation of a relatively large cohort of mice revealed that the skeletal phenotypes of TNAP null mice were markedly variable. Because FGF23 circulating levels are known to be increased in ENPP1 null mice and because FGF23 influences bone, we measured serum intact FGF23 levels in the TNAP null mice and found that a subset of TNAP−/−/ENPP1+/+ mice exhibited markedly high serum FGF23. Serum FGF23 levels also correlated to mouse body measurements, the incidence of craniosynostosis, skull shape abnormalities and skull bone density and volume fraction. Together, our results demonstrate that balanced expression of TNAP and ENPP1 enzymes are essential for microstructure and mineralization of both skull and long bones, and for preventing craniosynostosis. The results also show that FGF23 rises in the TNAP−/− model of murine lethal hypophosphatasia. Future studies are required to determine if the rise in FGF23 is a cause, consequence, or marker of disease phenotype severity.

Reduction cranioplasty for hydrocephalic macrocephaly: clinical observations and literature review

Reduction cranioplasty for extreme hydrocephalic macrocephaly is performed for acquiring motor skills (head holding, sitting, moving), aesthetic correction and social adaptation of children. The authors analyze literature data and describe 2 patients with congenital hydrocephalic macrocephaly who underwent shunting surgery followed by reduction cranioplasty in early age using resorbable mini-plates. Reduction cranioplasty decreased skull volume, improved social adaptation of children and ensured favorable aesthetic outcome.

Calcium functional imaging with high-resolution CT in the inner ear

Although the otolith and otolith organs correlate with vertigo and instability, there is no method to investigate them without harmful procedures. We will create the technique for 3D microanatomical images of them, and investigate the in vivo internal state and metabolisms. The otolith and otolith organs images were reconstructed from a texture synthesis algorithm under the skull volume rendering algorithm using a cutting-plane method. The utricular macula was elongated pea-shaped. The saccular macula was almost bud-shaped. The changes in the amount of CaCO3 in the maculae and the endolymphatic sac showed various morphologies, reflecting the balance status of each subject. Both shapes and volumes were not always constant depending on time. In Meniere’s disease (MD), the saccular macula was larger and the utricular macula was smaller. In benign paroxysmal positional vertigo (BPPV), the otolith increased in the utricular macula but did not change much in the saccular macula. The saccule, utricle, and endolymphatic sac were not constantly shaped according to their conditions. These created 3D microanatomical images can allow detailed observations of changes in physiological and biological information. This imaging technique will contribute to our understanding of pathology and calcium metabolism in the in vivo vestibulum.

Automatic skull defect restoration and cranial implant generation for cranioplasty

A fast and fully automatic design of 3D printed patient-specific cranial implants is highly desired in cranioplasty - the process to restore a defect on the skull. We formulate skull defect restoration as a 3D volumetric shape completion task, where a partial skull volume is completed automatically. The difference between the completed skull and the partial skull is the restored defect; in other words, the implant that can be used in cranioplasty. To fulfill the task of volumetric shape completion, a fully data-driven approach is proposed. Supervised skull shape learning is performed on a database containing 167 high-resolution healthy skulls. In these skulls, synthetic defects are injected to create training and evaluation data pairs. We propose a patch-based training scheme tailored for dealing with high-resolution and spatially sparse data, which overcomes the disadvantages of conventional patch-based training methods in high-resolution volumetric shape completion tasks. In particular, the conventional patch-based training is applied to images of high resolution and proves to be effective in tasks such as segmentation. However, we demonstrate the limitations of conventional patch-based training for shape completion tasks, where the overall shape distribution of the target has to be learnt, since it cannot be captured efficiently by a sub-volume cropped from the target. Additionally, the standard dense implementation of a convolutional neural network tends to perform poorly on sparse data, such as the skull, which has a low voxel occupancy rate. Our proposed training scheme encourages a convolutional neural network to learn from the high-resolution and spatially sparse data. In our study, we show that our deep learning models, trained on healthy skulls with synthetic defects, can be transferred directly to craniotomy skulls with real defects of greater irregularity, and the results show promise for clinical use. Project page: https://github.com/Jianningli/MIA.

Postnatal cranial ontogeny and growth strategies in the black‐tailed gullLarus crassirostrisbreeding on Kabu Island, Aomori, Japan

AbstractAlthough cranial morphology of modern birds has been an important subject of evolutionary studies, detailed information on postnatal ontogeny of the avian skull remains very scarce. Herein, a wild population of the black‐tailed gull (Larus crassirostris) breeding on Kabu Island was examined to explore relationships between their growth strategies and ontogenetic changes in the cranial shape. By examining growth series covering a major part of the postnatal ontogenetic period, it was clarified that the typical form of the adult larid skull was produced through dynamic proportional changes among cranial structures after hatching. Cranial structures related to the oral capacity and deglutition (e.g., some elements of hyoid bones) attained the adult sizes before fledging, whereas other structures apparently reached the adult sizes well after leaving the nest. The palatal width exhibited positively allometric growth against the skull volume in chicks in the early nestling stage, followed by a period of negative allometry in the later growth stage. A nestling gull has to acquire swallowing ability by the time of fledging at the latest because this ability is apparently essential for a food intake without parental aid. In addition, because nestlings mainly feed on foodstuffs supplied by their parents, previous studies suggested the possibility that siblings within a nest compete for food resources. Under this scenario, early acquisition of swallowing abilities, as indicated by the allometric patterns of relevant cranial structures, may be an adaptive trait that enhances survival of nestlings.

Fixed posterior cranial vault expansion technique

Different techniques to reshape the posterior skull vault have been developed in the last decades, all sharing the same goals of increasing the skull volume, decreasing the intracranial pressure (ICP), correcting the cranial dysmorphy, and contributing to a better growth of the skull. Though over the last years most refinements in these techniques have focused on the use of hardware as distractors or springs, the fixed posterior vault expansion remains a valuable procedure for cranial remodeling. We describe in details the technique used for fixed posterior vault expansion in children that is applied at the French Referral center for Craniosynostosis of Lyon, France. The fixed posterior vault expansion increases the risk of perioperative complications due to the elevation of the posterior bone flap from the dura but allows an immediate decompression and correction of the shape, simplifying the postoperative course.

The Gendered Brain by Gina Rippon

Discussions on gender are a minefield of opinions, with gender too frequently the loser to agenda. This is not surprising, given the complexity of the discussion, but it is unhelpfully muddied by opinions which are not grounded in evidence and coloured by bias. Nevertheless, conversation which seeks to put evidence at its core helps to move the discussion onto a more useful level. Gina Rippon’s The Gendered Brain does just this, drawing on work in cognitive neuroscience and psychology to tackle the issues surrounding gender. Outside of the gender debate, neuroscience promises to push forward various stale philosophical discussions, and it is no different in this case.Surprisingly, Gina Rippon turns to the history of science for the source of many unfounded conclusions, deftly outlining how science has been guilty of serving a sexist social agenda. She describes how science research has pushed the idea of a male and a female brain, in the past often based on outlandish experiments, such as skull volume measurements using bird seed. Even recently, despite the advent of brain imaging techniques, conclusions have been declared regarding male–female differences which are often unsubstantiated. However, her point is not that there are necessarily no differences between male and female brains, but that the evidence accumulated so far is inconclusive. Certainly, the kinds of differences we hear sometimes, like women are inherently worse at spatial tasks, therefore are less equipped for a career in engineering, are not robustly supported and are woefully unfair: we exist in a rule-based environment and our brains are extremely adept at identifying these rules, however biased. It is perhaps lacking in discussion on sex differences elsewhere in the animal kingdom, as surely this is important when considering our own, but generally it is a well-balanced book, with fascinating information about our social brain, babies’ brains, and the issues around binary gender identity.A year on from publication, sociopolitical views are as polarized as ever, so it is crucial that we learn to identify our inherent biases and how they inform our world view. Reading books like The Gendered Brain, which is witty, accessible, and thought-provoking, is an excellent way to do this.

Intracranial Volume Measured and Correlated to Cephalic Index in Syndromic and Nonsyndromic Anterior Brachycephaly.

Premature fusion of both coronal sutures (anterior brachycephaly) alters skull shape and potentially affects intracranial volume (ICV). Currently little is known about preoperative ICV in anterior brachycephaly. Aim is to measure preoperative ICV and compare this with normative data. Additionally, ICV will be correlated to most used clinical method of quantification: cephalic index (CI). Preoperative patients with anterior brachycephaly (age, ≤12 months) were included and categorized by syndrome (when present). Computed tomography scans were used for ICV measurement by manual segmentation (OsiriX (Fondation OsiriX, Geneva, Switzerland)). Intracranial volume of each subgroup was compared with Lichtenberg normative cranial volume growth curves for controls. Cephalic index was calculated and correlated to ICV using Pearson correlation coefficient. Thirty-four patients with both syndromic and nonsyndromic anterior brachycephaly were included: 17 with Apert syndrome, 6 with Muenke syndrome, 5 with Saethre Chotzen syndrome, 3 with Crouzon, 1 with craniofrontonasal dysplasia, and 2 nonsyndromal. Mean age at preoperative computed tomography scan was 4 months (1-10 months). Mean ICV was 847.31 cm3 (473.91-1459.22 cm3). Nineteen of 34 patients had skull volumes between ±2 SD curves of Lichtenberg, none of the patients had an ICV smaller than -2 SD and 15 of 34 had an ICV larger than +2 SD. Mean ICV in Apert syndrome was 829.85 cm3 (473.91-1061.53 cm3), in Muenke syndrome 942.06 cm3 (768.02-1136.75 cm3), in Saethre Chotzen syndrome 779.72 cm3 (609.21-1002.95 cm3), in Crouzon syndrome 700.57 cm3 (652.31-784.32 cm3), in craniofrontonasal dysplasia 738.97 cm3, and in the nonsyndromal group 1154.64 cm3 (850.07-1459.22 cm3). Apert had a mean greater than +2SD above the mean, the other subgroups had a mean within normal ranges (±2 SD). Correlation between severity of brachycephaly and overall ICV was low (r = 0.42). Mean preoperative ICV in both syndromic and nonsyndromic anterior brachycephaly was 847.31 cm3. Intracranial volume in anterior brachycephaly is in 55.9% between normal ranges (±2 SD). In 44.1% ICV was greater than +2 SD, especially in Apert syndrome (11/16 Apert patients). None of the included patients had a deviant small ICV of less than -2 SD. Additionally, low correlation between ICV and CI (r = 0.42) was found and therefore CI is not suitable for estimating ICV in anterior brachycephaly.

Safety and Efficacy of Magnetic Resonance-Guided Focused Ultrasound Surgery With Autofocusing Echo Imaging.

ObjectiveMagnetic resonance-guided focused ultrasound surgery (MRgFUS) lesioning is a new treatment for brain disorders. However, the skull is a major barrier of ultrasound sonication in MRgFUS because it has an irregular surface and varies its size and shape among individuals. We recently developed the concept of skull density ratio (SDR) to select candidates for MRgFUS from among patients with essential tremor (ET). However, SDR is not the only factor contributing to successful MRgFUS lesioning treatment—refining the target through exact measurement of the ultrasonic echo in the transducer also improves treatment efficacy. In the present study, we carried out MRgFUS lesioning using an autofocusing echo imaging technique. We aimed to evaluate the safety and efficacy of this new approach, especially in patients with low SDR in whom previous focusing methods have failed.MethodsFrom December 2019 to March 2020, we recruited 10 patients with ET or Parkinson’s disease (PD) who had a low SDR. Two patients dropped out of the trial due to the screening failure of other medical diseases. In total, eight patients were included: six with ET who underwent MRgFUS thalamotomy and two with PD who underwent MRgFUS pallidotomy. The autofocusing echo imaging technique was used in all cases.ResultsThe mean SDR of the patients with ET was 0.34 (range: 0.29–0.39), while that of the patients with PD was 0.41 (range: 0.38–0.44). The mean skull volume of patients with ET was 280.57 cm3 (range: 227–319 cm3), while that of the patients with PD was 287.13 cm3 (range: 271–303 cm3). During MRgFUS, a mean of 15 sonications were performed, among which a mean of 5.63 used the autofocusing technique. The mean maximal temperature (Tmax) achieved was 55.88°C (range: 52–59°C), while the mean energy delivered was 34.75 kJ (range: 20–42 kJ) among all patients. No serious adverse events occurred during or after treatment. Tmax or sonication factors (skull volume, SDR, sonication number, autofocusing score, similarity score, energy range, and power) were not correlated with autofocusing technique (p > 0.05, autofocusing score showed a p-value of 0.071).ConclusionUsing autofocusing echo imaging lesioning, a safe and efficient MRgFUS treatment, is available even for patients with a low SDR. Therefore, the indications for MRgFUS lesioning could be expanded to include patients with ET who have an SDR < 0.4 and those with PD who have an SDR < 0.45.Clinical Trial Registrationclinicaltrials.gov, identifier: NCT03935581.

Head phantoms for bioelectromagnetic applications: a material study

BackgroundAssessments of source reconstruction procedures in electroencephalography and computations of transcranial electrical stimulation profiles require verification and validation with the help of ground truth configurations as implemented by physical head phantoms. For these phantoms, synthetic materials are needed, which are mechanically and electrochemically stable and possess conductivity values similar to the modeled human head tissues. Three-compartment head models comprise a scalp layer with a conductivity range of 0.137 S/m to 2.1 S/m, a skull layer with conductivity values between 0.066 S/m and 0.00275 S/m, and an intracranial volume with an often-used average conductivity value of 0.33 S/m. To establish a realistically shaped physical head phantom with a well-defined volume conduction configuration, we here characterize the electrical conductivity of synthetic materials for modeling head compartments. We analyzed agarose hydrogel, gypsum, and sodium chloride (NaCl) solution as surrogate materials for scalp, skull, and intracranial volume. We measured the impedance of all materials when immersed in NaCl solution using a four-electrode setup. The measured impedance values were used to calculate the electrical conductivity values of each material. Further, the conductivities in the longitudinal and transverse directions of reed sticks immersed in NaCl solution were measured to test their suitability for mimicking the anisotropic conductivity of white matter tracts.ResultsWe obtained conductivities of 0.314 S/m, 0.30 S/m, 0.311 S/m (2%, 3%, 4% agarose), 0.0425 S/m and 0.0017 S/m (gypsum with and without NaCl in the compound), and 0.332 S/m (0.17% NaCl solution). These values are within the range of the conductivity values used for EEG and TES modeling. The reed sticks showed anisotropic conductivity with a ratio of 1:2.8.ConclusionWe conclude that agarose, gypsum, and NaCl solution can serve as stable representations of the three main conductivity compartments of the head, i.e., scalp, skull, and intracranial volume. An anisotropic conductivity structure such as a fiber track in white matter can be modeled using tailored reed sticks inside a volume conductor.

The effects of oyster shell/alpha-calcium sulfate hemihydrate/platelet-rich plasma/bone mesenchymal stem cells bioengineering scaffold on rat critical-sized calvarial defects.

Engineering scaffolds combining natural biomineral and artificially synthesized material hold promising potential for bone tissue regeneration. We fabricated a bioengineering scaffold, oyster shell (OS) and alpha-calcium sulfate hemihydrate (α-CSH) as scaffold, platelet-rich plasma (PRP) as provider of growth factors and bone mesenchymal stem cells (BMSCs) as seed cells, and determined it could be applied as a new type of bone graft substitutes by rat calvarial defects repairing experiment in vitro and in vivo. SEM showed that the mean diameter of the pores was about 150 μm with a range of 50-200 μm, and scaffold's porosity was ~27.4% by Archimedes' Principle. In vitro, Scaffold + BMSCs + PRP group presented a higher ALP activity compared with other groups by ELISA (P < 0.05). But the expression of OC was not detectable on day 4 or 8. The MTT assay showed that the relative cell number of BMSCs+PRP group increased significantly (P < 0.05). In vivo, the smallest defect area of skull and highest volume of regenerated new bone were observed in Scaffold + PRP + BMSCs group by X-ray and Micro-CT analysis (P < 0.05). And the similar results also were observed in HE and Masson staining. The immunohistochemistry staining for osteogenic marker proteins ALP and OC showed that the most obvious positive staining was observed in Scaffold + PRP + BMSCs group (P < 0.05). The expression of inflammatory markers IL-6 and TNF-α was the lowest in control group (P < 0.05). In conclusion, a bioengineering scaffold based on OS, created by simply combining α-CSH and PRP and implanting with BMSCs, could be clinically useful and has marked advantages as a targeted, off-the-shelf, cell-loaded treatment option for the bone healing of critical-size calvarial defects.