Anomalous venous collaterals in Apert and Crouzon syndromes and their relationship to ventricle size and increased intracranial pressure.

Objective: The exact association between the frequently present anomalous intracranial venous vasculature, emissary collaterals, ventriculomegaly, and increased intracranial pressure (ICP) in children with Apert and Crouzon syndromes remains an enigma. This study aimed to evaluate the association between the aberrant venous system and ventricle size and increased ICP, and to assess the development of the venous structures over time.

Methods: This retrospective cohort study included all patients with Apert or Crouzon syndrome with available CT venography (CTV) scans of the brain.

Forehead shape analysis following surgical and conservative treatment in metopic synostosis: a 3D photogrammetry analysis.

Background: The aim of this study is to describe and compare head shape in surgically and conservatively treated patients using 3D photogrammetry.

Methods: A retrospective review (2017-2020) of consecutive patients with isolated metopic synostosis based on 3D photogrammetry was conducted at the age of 4 years old. Images were aligned using a healthy age-matched template, and mean head shapes were reconstructed to evaluate shape development.

Quantifying dysmorphologies of the neurocranium using artificial neural networks.

Background: Craniosynostosis, a congenital condition characterized by the premature fusion of cranial sutures, necessitates objective methods for evaluating cranial morphology to enhance patient treatment. Current subjective assessments often lead to inconsistent outcomes. This study introduces a novel, quantitative approach to classify craniosynostosis and measure its severity.

Child-Patient Perspective on Results After Correction of Sagittal Synostosis and the Difference Between Child-Patient and Parent's Perspectives.

Objective: This study assesses the level of child-patient satisfaction with the surgical result after scaphocephaly correction and the difference between child-patient and parents' perspectives.

Methods: A questionnaire was sent out to children between 6 and 18 years old with isolated sagittal synostosis, who had undergone either a frontobiparietal remodeling or extended strip craniotomy, and separately to their parents.

Results: The questionnaire was completed by 96 patients, 81.

3D Analysis of the Cranial and Facial Shape in Craniosynostosis Patients: A Systematic Review.

With increasing interest in 3D photogrammetry, diverse methods have been developed for craniofacial shape analysis in craniosynostosis patients. This review provides an overview of these methods and offers recommendations for future studies. A systematic literature search was used to identify publications on 3D photogrammetry analyses in craniosynostosis patients until August 2023.

A European Multicenter Outcome Study of Perioperative Airway Management Policies following Midface Surgery in Syndromic Craniosynostosis.

Background: Perioperative airway management following midface advancements in children with Apert and Crouzon-Pfeiffer syndromes can be challenging, and protocols often differ. This study examined airway management following midface advancements and postoperative respiratory complications.

Methods: A multicenter, retrospective cohort study was performed to obtain information about the timing of extubation, perioperative airway management, and respiratory complications after monobloc or Le Fort III procedures.

Health-related Quality of Life in Children and Adolescents With Sagittal Synostosis.

Background: This study evaluated the health-related quality of life (HR-QoL) in patients with sagittal synostosis (SS), and the influence of frequent headaches and surgical techniques on the HR-QoL.

Method: Patients with SS aged 8 to 18 years were invited to participate between June 2016 and February 2017. The Child Health Questionnaire was used to assess the HR-QoL.

Potential higher risk of tethered spinal cord in children after prenatal surgery for myelomeningocele: A systematic review and meta-analysis.

Introduction: We performed a systematic review and meta-analysis on the incidence of secondary tethered spinal cord (TSC) between prenatal and postnatal closure in patients with MMC. The objectives was to understand the incidence of secondary TSC after prenatal surgery for MMC compared to postnatal surgery for MMC.

Material And Methods: On May 4, 2023, a systematic search was conducted in Medline, Embase, and the Cochrane Library to gather relevant data.

Meaningful outcomes for children and their caregivers attending a paediatric brain centre.

Aim: To identify meaningful outcomes of children and their caregivers attending a paediatric brain centre.

Method: We compiled a long list of outcomes of health and functioning of children with brain-related disorders such as cerebral palsy, spina bifida, (genetic) neurodevelopmental disorders, and acquired brain injury. We incorporated three perspectives: patients, health care professionals, and published outcome sets.

Evaluation of AR visualization approaches for catheter insertion into the ventricle cavity.

Augmented reality (AR) has shown potential in computer-aided surgery. It allows for the visualization of hidden anatomical structures as well as assists in navigating and locating surgical instruments at the surgical site. Various modalities (devices and/or visualizations) have been used in the literature, but few studies investigated the adequacy/superiority of one modality over the other.

Trapped fourth ventricle: to stent, shunt, or fenestrate-a systematic review and individual patient data meta-analysis.

Trapped or isolated fourth ventricle (TFV) is a rare but critical neurosurgical condition, mostly occurring in pediatric patients, caused by a blockage of the in- and outlets of the fourth ventricle. The purpose of this study is to review all available data on the treatment options of TFV and to compare their safety, efficacy, and durability. MEDLINE, Embase, and Google Scholar were searched from inception to September 13, 2022, for prospective or retrospective cohorts, case-control studies, case series or case reports, reporting detailing outcomes of TFV patients, treated with an endoscopic-, microsurgical-, shunt placement,- or hybrid approaches to TFV.

Newborns with myelomeningocele: their health-related quality of life and daily functioning 10 years later.

Objective: Ten years ago, the authors reported on the outcome of their study investigating the degree of discomfort and pain in newborns with myelomeningocele (MMC), using the parameters of unbearable and hopeless suffering. In the current study, they investigated the quality of life, daily functioning, pain and fatigue, ability to communicate, and number of surgeries in the same cohort of patients. They subdivided their study population into severe (Lorber) and less severe (non-Lorber) cases and compared these cases with a healthy population (non-MMC group) and with each other.

The use of OCT to detect signs of intracranial hypertension in patients with sagittal suture synostosis: Reference values and correlations.

Purpose: To obtain pediatric normative reference values and determine whether optical coherence tomography (OCT) corresponds better with clinical signs of intracranial hypertension (ICH) compared to the traditional screening method fundoscopy in a large cohort of one type of single suture craniosynostosis.

Methods: Control subjects without optic nerve diseases and isolated sagittal synostosis patients aged 3-10 years who underwent fundoscopy and OCT were included in this prospective cohort study. Normative reference values were obtained through bootstrap analysis.

Augmented reality navigation for minimally invasive craniosynostosis surgery: a phantom study.

Purpose: In minimally invasive spring-assisted craniectomy, surgeons plan the surgery by manually locating the cranial sutures. However, this approach is prone to error. Augmented reality (AR) could be used to visualize the cranial sutures and assist in the surgery planning.

A Diffusion Tensor Imaging Analysis of Frontal Lobe White Matter Microstructure in Trigonocephaly Patients.

Background: Children with trigonocephaly are at risk for neurodevelopmental disorders. The aim of this study is to investigate white matter properties of the frontal lobes in young, unoperated patients with metopic synostosis as compared to healthy controls using diffusion tension imaging (DTI).

Methods: Preoperative DTI data sets of 46 patients with trigonocephaly with a median age of 0.

Cerebral Blood Flow of the Frontal Lobe in Untreated Children with Trigonocephaly versus Healthy Controls: An Arterial Spin Labeling Study.

Background: Craniofacial surgery is the standard treatment for children with moderate to severe trigonocephaly. The added value of surgery to release restriction of the frontal lobes is unproven, however. In this study, the authors aim to address the hypothesis that the frontal lobe perfusion is not restricted in trigonocephaly patients by investigating cerebral blood flow.

The Course and Interaction of Ventriculomegaly and Cerebellar Tonsillar Herniation in Crouzon Syndrome over Time.

Unlabelled: Children with Crouzon syndrome have a higher incidence of cerebellar tonsillar herniation (TH) and ventriculomegaly than the general population, or children with other craniosynostosis syndromes.

Objective: This retrospective cohort study aimed to determine how ventriculomegaly and TH develop and progress over time, and determine associations between ventriculomegaly and TH in Crouzon patients, treated according to our center's protocol.

Methods: Fronto-occipital horn ratio (FOHR) and TH were determined over time using brain-imaging.

Does the association between abnormal anatomy of the skull base and cerebellar tonsillar position also exist in syndromic craniosynostosis?

Purpose: Cerebellar tonsillar herniation (TH) occurs frequently in syndromic craniosynostosis; however, the exact pathogenesis is unknown. This study evaluates the association between skull base deformities and TH in syndromic craniosynostosis.

Methods: Retrospective study MRI study comparing syndromic craniosynostosis to controls.

Quantified Retinal Morphology and Its Association With Papilledema and Visual Acuity in Syndromic and Complex Craniosynostosis: An Optical Coherence Tomography Study.

Purpose: This study is the first to evaluate the prevalence of retinal thinning and the correlation with papilledema and visual acuity (VA) in a large population with craniosynostosis.

Design: Prospective clinical cohort study.

Methods: All and complex patients syndromic and complex with craniosynostosis who visited the only national referral center between 2018 and 2020 were included.

Subgroup and subtype-specific outcomes in adult medulloblastoma.

Medulloblastoma, a common pediatric malignant central nervous system tumour, represent a small proportion of brain tumours in adults. Previously it has been shown that in adults, Sonic Hedgehog (SHH)-activated tumours predominate, with Wingless-type (WNT) and Group 4 being less common, but molecular risk stratification remains a challenge. We performed an integrated analysis consisting of genome-wide methylation profiling, copy number profiling, somatic nucleotide variants and correlation of clinical variables across a cohort of 191 adult medulloblastoma cases identified through the Medulloblastoma Advanced Genomics International Consortium.

Clinical signs, interventions, and treatment course of three different treatment protocols in patients with Crouzon syndrome with acanthosis nigricans.

Objective: Crouzon syndrome with acanthosis nigricans (CAN) is a rare and clinically complex subtype of Crouzon syndrome. At three craniofacial centers, this multicenter study was undertaken to assess clinical signs in relation to the required interventions and treatment course in patients with CAN.

Methods: A retrospective cohort study of CAN was performed to obtain information about the clinical treatment course of these patients.

Cerebral cortex maldevelopment in syndromic craniosynostosis.

Aim: To assess the relationship of surface area of the cerebral cortex to intracranial volume (ICV) in syndromic craniosynostosis.

Method: Records of 140 patients (64 males, 76 females; mean age 8y 6mo [SD 5y 6mo], range 1y 2mo-24y 2mo) with syndromic craniosynostosis were reviewed to include clinical and imaging data. Two hundred and three total magnetic resonance imaging (MRI) scans were evaluated in this study (148 patients with fibroblast growth factor receptor [FGFR], 19 patients with TWIST1, and 36 controls).