Spring-assisted cranioplasty is an established technique for the correction of isolated sagittal craniosynostosis, yet data on long-term clinical durability and patient-perceived outcomes remain limited. The purpose of this study was to evaluate long-term surgical, aesthetic, and patient-reported outcomes following spring-assisted cranioplasty, with a particular focus on the need for secondary cranial surgery and concordance between surgeon- and parent-reported assessments. A single-centre cohort study was performed, including consecutive patients with isolated, nonsyndromic sagittal synostosis treated with spring-assisted cranioplasty between April 2010 and September 2015. Patients were followed within a standardized multidisciplinary craniofacial pathway extending into late childhood and adolescence. Long-term outcomes were assessed using reoperation rates, Kaplan–Meier reoperation-free survival analysis, surgeon-reported Whitaker classification, and structured parent-reported satisfaction measures. Ninety-one of the original 100 patients had complete long-term follow-up and were included, with a mean follow-up duration of 10.0 years (range 5–15 years). Nine patients (9.9

OBJECTIVE:The aim of this study was to evaluate whether indirect revascularization is associated with a reduction in systemic blood pressure (BP) over time in pediatric patients with moyamoya. METHODS:This is a retrospective cohort study of pediatric patients with moyamoya who were treated at a single institution between June 2019 and January 2025. BP measurements were collected preoperatively (preadmission clinic evaluation) and postoperatively (outpatient follow-up evaluation at ≥ 3 months). BP was categorized as hypertensive or normotensive based on percentile thresholds adjusted for age, sex, and height, with measurements at the 95th and 99th percentiles indicating hypertension. The chi-square test was used to assess changes in hypertensive classification, and the paired t-test was used to compare mean absolute BP values at the two time points. RESULTS:Overall, 63 children (39 female, mean age 11 years) with moyamoya who underwent indirect revascularization surgery were included in this analysis. The mean follow-up duration was 20.8 months. Preoperatively, 21 patients (33.9%) were classified as hypertensive for systolic BP and 9 (14.5%) for diastolic BP. These measurements were acquired preoperatively at a mean of 3.5 months (range 1 day to 11 months). Postoperatively, the proportion of hypertensive patients decreased to 10 (16.1%) and 3 (4.8%) for systolic BP and diastolic BP (p = 0.02 and 0.06, respectively). There was no significant change between the preoperative and postoperative mean arterial pressure measurements (77.3 mm Hg vs 76.2 mm Hg, respectively; p = 0.52). CONCLUSIONS:Indirect revascularization surgery in pediatric patients with moyamoya was associated with a reduction in hypertension over time. This might reflect the delayed physiological effect of indirect revascularization, in which neovascularization and improved cerebral perfusion typically develop 3-6 months after surgery. Understanding BP changes following surgery might help avoid unnecessary use of antihypertensive drugs, which carry risk of precipitating hypotension and thus stroke.

PURPOSE:Spring-assisted cranioplasty is an established technique for the correction of isolated sagittal craniosynostosis, yet data on long-term clinical durability and patient-perceived outcomes remain limited. The purpose of this study was to evaluate long-term surgical, aesthetic, and patient-reported outcomes following spring-assisted cranioplasty, with a particular focus on the need for secondary cranial surgery and concordance between surgeon- and parent-reported assessments. METHODS:A single-centre cohort study was performed, including consecutive patients with isolated, nonsyndromic sagittal synostosis treated with spring-assisted cranioplasty between April 2010 and September 2015. Patients were followed within a standardized multidisciplinary craniofacial pathway extending into late childhood and adolescence. Long-term outcomes were assessed using reoperation rates, Kaplan-Meier reoperation-free survival analysis, surgeon-reported Whitaker classification, and structured parent-reported satisfaction measures. RESULTS:Ninety-one of the original 100 patients had complete long-term follow-up and were included, with a mean follow-up duration of 10.0 years (range 5-15 years). Nine patients (9.9%) required secondary cranial surgery during follow-up, including four for raised intracranial pressure and five for aesthetic indications. Reoperation-free survival was 90.1% at 10 years, with the median survival not reached. Surgeon-reported outcomes were excellent, with 87.9% classified as Whitaker Class I. Parent-reported satisfaction was high, with 82.4% completely satisfied and unwilling to consider further surgery. CONCLUSION:Spring-assisted cranioplasty for isolated sagittal synostosis provides durable long-term functional and aesthetic outcomes, with a low incidence of secondary cranial surgery and high patient and parent satisfaction when combined with structured multidisciplinary follow-up.

BACKGROUND AND OBJECTIVE:Endoscopic strip craniectomy is a minimally invasive surgical technique offered to infants for craniosynostosis repair. We examine our institution's experience with infants undergoing this surgery with respect to perioperative physiological parameters, transfusion rates, complications, and length of hospital stay. METHODS:We performed an observational retrospective review of all infants undergoing endoscopic strip craniectomy at Great Ormond Street Hospital, UK from 2019 to 2024. Data were collected via the digital health record system EPIC (Epic Systems Corporation [2023], USA) and analyzed in Microsoft Excel. RESULTS:One hundred and eleven patients were included in the study undergoing single or multicranial suture repair: metopic (n = 67), unicoronal (n = 27), sagittal (n = 9), frontosphenoidal (n = 2), bicoronal (n = 4), and multisuture (n = 2). We present a mean age of 4.4 months (±1.05 SD), weight 6.95 kg (±1.05 SD), male (n = 66) population predominance, and ASA score from 1 to 3. Surgical procedure time was 73 min (±23 SD) across all sutures, with multisuture repair requiring a longer operative time of 96 min (±15 SD). The overall red cell transfusion rate was 1 in 5 children, with a higher incidence in those undergoing metopic suture repair (18/67, 26%). Mean preoperative and postoperative hemoglobin in the single suture repair group was 114 g/L (±11 g/L SD) and 87 g/L (±13 g/L SD) resulting in a mean reduction in hemoglobin of 26 g/L (±15 g/L SD). Mean preoperative and postoperative hemoglobin in the bilateral or multisuture repair group was 118 g/L (±7.17 g/L SD) and 85.5 g/L (±14.29 g/L SD) resulting in a mean reduction in hemoglobin of 35 g/L (±15 g/L SD). One hundred and six infants (95%) were discharged on Day 1 postoperatively, and no children required high dependency care. Complications reported were inadvertent extubation on positioning (n = 2), laryngospasm (n = 1), and a minor transfusion reaction (n = 1). CONCLUSION:Endoscopic strip craniectomy is a well-established minimally invasive surgical technique. Anesthesia for this procedure is typically performed in young infants who may be at greater risk of perioperative anesthetic complications and clinically significant blood loss and blood transfusion. We report a > 20% transfusion rate in our infant cohort mostly with metopic repairs.

center dot OBJECTIVE: To evaluate optic nerve head (ONH) morphology in children with craniosynostosis versus healthy controls. center dot DESIGN: Single-center, prospective cohort study. center dot METHODS: Handheld optical coherence tomography (OCT) was performed in 110 eyes of 58 children (aged 0-13 years) with craniosynostosis. Inclusion criteria were as follows: normal intracranial pressure on invasive overnight monitoring, or clinically stable intracranial pressure. The latter was defined as stable VA within 1 logMAR line and no papilledema on fundoscopy for at least 4 months following OCT, and normal/stable visual evoked potentials. Control data for 218 eyes of 218 children were obtained from a published normative dataset. The main outcome measures were disc width, cup width, rim width, and retinal nerve layer thickness (nasal and temporal). Outcome measures were compared using three-way linear mixed model regression analysis (fibroblast growth factor receptor [FGFR] 1/2-associated craniosynostosis, non-FGFR 1/2-associated craniosynostosis, and controls). center dot RESULTS: Out of 63 eligible children with craniosynostosis, handheld OCT imaging was successful in 110 eyes of 58 children (92%). Of these, 22 (38%) were female. Median subject age at OCT examination was 53 months (range: 2-157; IQR: 39-73). Twelve children (21%) had FGFR1/2-associated syndromes (Crouzon, n = 6; Apert, n = 4; Pfeiffer, n = 2). Control data were available for 218 eyes of 218 healthy children. 122 controls (56%) were female. Median control age at OCT examination was 20 months (range: 0-163; IQR: 6-59). When comparing ONH morphology in craniosynostosis (n = 58) versus controls (n = 218), disc width was 6% greater (P = .001), temporal cup width was 13% smaller (P = .027), rim width was 16% greater (P < .001) and temporal retinal nerve fiber layer was 11% smaller (P = .027). When comparing FGFR1/2-associated syndromes (Crouzon, Apert, and Pfeiffer syndromes, n = 12) to the rest of the craniosynostosis group (n = 46), disc width was 10% smaller (P = .014) and temporal cup width was 38% smaller (P = .044). center dot CONCLUSIONS: This cohort demonstrated morphological differences of the ONH in craniosynostosis, most markedly in Crouzon, Apert, and Pfeiffer syndromes. These findings could help improve ophthalmological monitoring and surgical decision-making in children with craniosynostosis. Further work on longitudinal ONH changes in syndromic and nonsyndromic craniosynostosis would be valuable. (c) 2024 The Author(s). Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/ )

To evaluate outcomes of three posterior vault expansion (PVE) techniques in children with craniosynostosis and confirmed raised intracranial pressure (ICP) and propose a practical framework to guide surgical technique selection based on patient-specific characteristics. We retrospectively analyzed 116 pediatric patients who underwent their first PVE for confirmed raised ICP between January 2018 and January 2024 at a tertiary craniofacial center. Patients underwent one of three surgical techniques: static remodeling (PVE-S), spring-assisted posterior vault expansion-classic (SAPVE-C), or spring-assisted posterior vault expansion-vertical vector (SAPVE-VV). Outcomes included functional improvement (fundoscopy, VEPs, symptoms, Chiari I), volumetric change in intracranial volume (ICV), operative time, transfusion rates, complications, and reoperation-free survival. Analyses were stratified by age and syndromic diagnosis. All techniques significantly improved functional markers, with complete resolution of papilledema and normalized VEPs in over 80

AIM:To study the relationship between brain and cerebrovascular imaging and neurocognitive metrics in children with moyamoya. METHOD:This was a retrospective observational study of 34 children with moyamoya. Intellectual function was assessed using the Wechsler scales. Neuroimaging variables included the Suzuki stage, ivy sign score, paediatric moyamoya MRI score (PMMS), and a novel angiogram score. RESULTS:Intellectual function was significantly below average (mean IQ = 86.6). Ivy sign and PMMS were negatively associated with all indices of intellectual function (r = -0.32 to 0.5, p < 0.05). The angiogram score was negatively correlated with non-verbal reasoning, full-scale IQ (FSIQ), and working memory (r = -0.32 to -0.4, p < 0.05). Children with posterior circulation involvement had significantly lower mean IQ scores in the mild-moderate impairment range compared to the average range for children without. The posterior cerebral artery (PCA) ivy sign score and PMMS together significantly explain 35% of the variance in FSIQ (p < 0.001), and accurately classified children with moyamoya who had weak cognitive ability (FSIQ <85), with area under the curve of 0.76 (p = 0.01) and 0.735 (p = 0.02) respectively. INTERPRETATION:This study reliably identified a relationship between simple, routine neuroradiology sequences and neuropsychological outcomes in paediatric moyamoya. PMMS and/or PCA ivy sign score can be used to identify those children most at risk of cognitive impairment.

This is a retrospective study investigating intracranial pressure monitoring (ICPM) as a tool for managing children with craniosynostosis. We report on our experience of chronic (≥24 hour) ICP measurement in patients with CS and related conditions, with the aim of statistically assessing the relationship between ICP and derived variables to clinically relevant patient characteristics, such as clinical/ophthalmological symptoms/signs, diagnosis, syndrome and surgical management. It is a single-centre study following 32 patients who underwent ICP monitoring over 24–72 h. We collected a consecutive series of children (aged < 18 years) who underwent invasive ICPM between 1st Jan 2019 and 31st December 2021 from our institution’s electronic surgical database. All patients had either craniosynostosis or foramen magnum pathology. We excluded children with non-CS diagnoses, except those with non-CS-related foramen magnum stenosis (FMS) who were used as comparative controls. Statistical analysis included the Kruskal–Wallis test to compare median ICP results between craniosynostosis and foramen magnum pathology, surgery vs non-surgical management, and differences in syndromic subgroups. A Spearman correlation was used to determine the relation of ICPM results to presentation symptoms. Results showed that patients who underwent surgery had a higher ICPM than those that did not. They also showed that patients with CS had higher ICP when compared to foramen magnum pathologies. Results also showed differences in pulse altitude (PA) in different subtypes of CS, although not statistically significant, and a mild correlation of PA with certain presenting symptoms of CS deterioration. In conclusion, our study shows that ICP and PA are safe and useful tools that can differentiate between craniosynostosis and similar pathology, as well as correlate to symptomatology. Furthermore, the data in this study can help us understand what a 'well' ICP is, in patients with various CS conditions, which differs from a "signature" raised ICP in patients close to decompensation. ICP can therefore be a valuable tool in surgical decision making, in conjunction with clinical correlation.

To discuss the role of surgical revascularization (SR) in inoperable deep-seated brain arteriovenous malformation (bAVM) to treat ischaemic symptoms owing to cerebral hypoperfusion from vascular steal in paediatric patients with vascular steal phenomenon. We discuss the management of two children with high-grade deep-seated bAVMs who underwent indirect SR with encephalo-duro-arterio-myo-synangiosis (EDAMS) and superficial temporal artery (STA) pial synangiosis. Long-term clinical and radiological follow-up were presented. Both children aged 11 and 14 years with deep-seated basal ganglia/thalamic bAVMs (Spetzler Martin 5, Lawton-Young Supplemented Score 8 who presented with ischaemic-related symptoms (progressive motor deficit). There were no ischaemic lesions evidenced on the MRI. EDAMS with STA pial synangiosis was performed with marked improvement of the progressive and fluctuating symptoms. At 2-year follow-up, angiogram showed satisfactory revascularization from the STA graft to the hypoxemic peri-Rolandic territories. Both patients presented stabilization of clinical symptoms up to 4-year follow-up after IR. There was no evidence of haemorrhage, ischaemia or bAVM progression on follow-up imaging. SR using indirect techniques may have a role in the management of inoperable deep-seated bAVMs with symptoms due to vascular steal phenomena. The successful revascularization of these cases may also provide evidence to support the hypothesis of vascular steal phenomenon in rapid shunting bAVM causing a local perinidal hypoxemia and neural damage.

This study aimed to identify factors influencing brain volumes at term-equivalent age (TEA) in preterm infants with post-haemorrhagic ventricular dilatation (PHVD) and explore how these volumes, and other factors, correlate with neurodevelopmental outcome (NDO). A retrospective cohort study of 84 infants with PHVD managed on a standardised pathway at a single centre between 2012 and 2020. Brain volumes were measured from TEA MRI scans in 31 infants. NDO was assessed at one and two years. Primary analyses included the 31 infants with measured brain volumes. Sensitivity analysis included all 84 infants using multiple imputation for missing brain volumes. Sepsis (-12.75mL, 95 Understanding associations of independent variables with NDO is essential to evaluate the impact of neurosurgical interventions. - Among infants with post-haemorrhagic ventricular dilatation: - Sepsis was associated with smaller cerebellar volumes at term-equivalent age. - Intubation and inotrope use were associated with smaller deep grey matter volumes at term-equivalent age. - Larger deep grey matter volume at term-equivalent age was associated with improved neurodevelopmental outcome at two years.

BACKGROUND:Endoscopic 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. METHOD:Six 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. RESULTS:Comparison 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). CONCLUSIONS:In 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.

Brain arteriovenous malformations (bAVMs) are aberrant arteriovenous shunts through a vascular nidus with no intervening capillary beds. They are one of the commonest causes of spontaneous intracranial haemorrhage in children and may be associated with significant morbidity and mortality in cases of rupture. Treatment strategies include microsurgical resection, endovascular embolisation, stereotactic radiosurgery, multimodality treatment with a combination thereof, and particularly in high-grade bAVMs, conservative management. Clinicians involved in treating bAVMs need to have familiarity with the natural history pertaining to bAVMs in terms of risk of rupture, risk factors elevating rupture risk as well as understanding the clinical manifestations of bAVMs. This invited review serves to provide a synthesis on natural history and clinical presentation of bAVMs with particular focus in children to inform decision-making pertaining to management.

OBJECTIVE: Outcomes of surgical repair of trigonocephaly are well reported in the literature, but there is a paucity of information on the natural history of unoperated children. The authors evaluated a group of unoperated children with metopic synostosis to describe the natural change in head shape over time. METHODS: A database was screened for scans of children with unoperated trigonocephaly (2010-2021). Multisuture cases and those with a metopic ridge were excluded. Three-dimensional surface scans (3D stereophotogrammetry/CT) were used for morphological analysis. Nine previously published parameters were used: frontal angle (FA(30 degrees)), anteroposterior (AP) volume ratio (APVR), AP area ratio (APAR), AP width ratios 1 and 2 (APWR1 and APWR2), and 4 AP diagonal ratios (30 degrees right APDR( )[rAPDR(30)], 30 degrees left APDR [lAPDR(30)], 60 degrees right APDR [rAPDR(60)], and 60 degrees left APDR [lAPDR(60)]). RESULTS: Ninety-seven scans were identified from a cohort of 316 patients with a single metopic suture, in which the male-to-female ratio was 2.7:1. Ages at the time of the scan ranged from 9 days to 11 years and were stratified into 4 groups: group 1, < 6 months; group 2, 6-12 months; group 3, 1-3 years; and group 4, > 3 years. Significant improvements were detected in 5 parameters (APVR, APAR, APWR1, rAPDR(30), and lAPDR(30)) over time, whereas no significant differences were found in FA(30), APWR2, rAPDR(60), and lAPDR(60) between age groups. CONCLUSIONS: Forehead shape (surface area and volume), as well as narrowing and anterolateral contour at the frontal points, differed significantly over time without surgery. However, forehead angulation, narrowing, and anterolateral contour at temporal points did not show significant differences. This knowledge will aid in surgical and parental decision-making.

Brain arteriovenous malformations (bAVMs) present complex challenges in neurosurgery, requiring precise pre-surgical planning. In this context, 3D printing technology has emerged as a promising tool to aid in understanding bAVM morphology and enhance surgical outcomes, particularly in pediatric patients. This study aims to assess the feasibility and effectiveness of using 3D AVM models in pediatric bAVM surgery. The study was conducted at Great Ormond Street Hospital, and cases were selected sequentially between October 2021 and February 2023. Eight pediatric bAVM cases with 3D models were compared to eight cases treated before the introduction of 3D printing models. The 3D modelling fidelity and clinical outcomes were assessed and compared between the two cohorts. The study demonstrated excellent fidelity between 3D models and actual operative anatomy, with a median difference of only 0.31 mm. There was no statistically significant difference in angiographic cure rates or complications between the 3D model group and the non-3D model group. Surgical time showed a non-significant increase in cases involving 3D models. Furthermore, the 3D model cohort included higher-grade bAVMs, indicating increased surgical confidence. This study demonstrates the feasibility and efficacy of utilizing 3D AVM models in pediatric bAVM surgery. The high fidelity between the models and actual operative anatomy suggests that 3D modelling can enhance pre-surgical planning and intraoperative guidance without significantly increasing surgical times or complications. Further research with larger cohorts is warranted to confirm and refine the application of 3D modelling in clinical practice.

Objective Endoscopic suturectomy for craniosynostosis with helmet therapy (ESCH) has emerged as a successful treatment for craniosynostosis initially in North America. We present early outcomes from the first cohort of ESCH patients treated in the United Kingdom. Methods Retrospective cohort study with review of electronic records. Results 18 consecutive patients from the first procedure in the UK (May 2017) until January 2020 were identified. Our cohort consisted of 12 male and 6 female infants with craniosynostosis, with mean age of 4.6 months (range: 2.5–7.8 months) and weight of 6.8 kg (range 4.8–9.8 kg). The most commonly operated diagnosis was metopic synostosis (n=8) followed by unicoronal (n=7), sagittal (n=2) and multi-sutural (n=1) synostoses. Median length of skin incision was 3 cm (range 2–10 cm), with 15 patients having a single skin incision. 16/18 received no blood products with 2 requiring transfusion (1 donor exposure). Mean operative time (including anaesthesia) was 96 mins (range 40–127 mins). The median length of hospital stay was 1 night with no patients staying more than 2 nights (n=3). Only 1 surgical complication was noted (a suture abscess requiring oral antibiotics). All patients are currently undergoing helmet orthosis with 100% compliance so far. No patients have required revisional surgery. Conclusion Early peri-operative experience from the first UK cohort of ESCH patients suggests this is a safe and well tolerated technique with low morbidity, need for transfusion and short hospital stay. We hope the success of these results leads to developing discussions to facilitate funding of the helmet orthotic therapy as part of NHS highly specialised commissioning services at Great Ormond Street.

OBJECTIVE:Despite growing published evidence of the merits of endoscopic third ventriculostomy (ETV) instead of shunt revision at the time of shunt malfunction (secondary ETV), concerns about its efficacy and complications remain and ETV is still not used widely in this context. This study aimed to carry out a comprehensive meta-analysis and reports on the success and safety of secondary ETV in the pediatric age group. METHODS:In accordance with the PRISMA guidelines, systematic searches of Medline, Embase, and Cochrane Central were undertaken from database inception to September 7, 2022. ETV success was defined as the lack of need for a shunt and was the primary outcome measure. Secondary outcome measures were the rates of complications and mortality. A random-effects model was used. Summary-level meta-regression was performed to identify predictors for success in accordance with the ETV Success Score (ETVSS). RESULTS:Sixteen studies reporting on 584 patients who underwent secondary ETV for shunt malfunction were included in the meta-analysis. The overall pooled mean (95% CI) age was 6.1 (3-9) years, and 57.0% of patients were male. The pooled prevalence rates of the hydrocephalus etiologies were as follows: aqueduct stenosis (39.3%); myelomeningocele (27.6%); postinfectious (17.1%); posthemorrhagic (13.0%); neoplasm (13.0%); and malformation (11.3%). The overall pooled success rates of ETV for shunt malfunction at 3 months, 6 months, and 12 months were 65.69% (95% CI 52%-77%, prediction interval 47%-81%, I2 = 0, p = 0.775); 63.25% (95% CI 54%-72%, prediction interval 38%-83%, I2 = 65, p < 0.001); and 53.37% (95% CI 24%-81%, prediction interval 1%-99%, I2 = 47, p = 0.154). The overall pooled prevalence of intraoperative bleeding was 4.96% (95% CI 0%-64%, prediction interval 0%-99%, I2 = 85, p < 0.001). The overall rates of complications were low, with new neurological deficit (transient or permanent) having the highest rate at 1.61% (95% CI 0.68%-3.72%, prediction interval 0.67%-3.78%, I2 = 0, p > 0.999). On meta-regression, age (p = 0.138), proportion of patients with postinfectious hydrocephalus (p = 0.8736), and number of shunt revisions (p = 0.1775) were not statistically significant predictors of secondary ETV success at 6 months. CONCLUSIONS:This meta-analysis demonstrates that secondary ETV after shunt malfunction in pediatric patients is a feasible option with acceptable success rates and low complication rates. Clinical trial registration no.: CRD42022359573 (PROSPERO).

Intracranial vascular malformations can be classified depending on the presence or absence of arteriovenous shunting, the vessel type involved, and the location of the lesion. Tumours primarily affecting the vascular tree are rare in children; however, rapidly growing tumours may present with haemorrhage and may mimic ruptured arteriovenous malformations (AVMs). Most intracranial abnormalities with arteriovenous shunting in the paediatric population are high-flow abnormalities. These can be further classified depending on their angioarchitecture into AVMs and arteriovenous fistulae. Intracranial vascular anomalies with low-flow shunts are rare. Cerebral proliferative angiopathy (CPA) is a poorly understood condition, in which there are nidal vessels, but in comparison with AVMs, they are generally more diffuse and interspersed with normal brain tissue. Intracranial vascular abnormalities without arteriovenous shunting include developmental venous anomalies (DVAs). DVAs are the most common intracranial vascular anomalies, estimated to occur in 3% of the general population.

Introduction There is no clear consensus regarding the technique of surgical revascularization for moyamoya disease and syndrome (MMD/MMS) in the pediatric population. Previous meta-analyses have attempted to address this gap in literature but with methodological limitations that affect the reliability of their pooled estimates. This meta-analysis aimed to report an accurate and transparent comparison between studies of indirect (IB), direct (DB), and combined bypasses (CB) in pediatric patients with MMD/MMS. Methods In accordance with PRISMA guidelines, systematic searches of Medline, Embase, and Cochrane Central were undertaken from database inception to 7 October 2022. Perioperative adverse events were the primary outcome measure. Secondary outcomes were rates of long-term revascularization, stroke recurrence, morbidity, and mortality. Results Thirty-seven studies reporting 2460 patients and 4432 hemispheres were included in the meta-analysis. The overall pooled mean age was 8.6 years (95% CI: 7.7; 9.5), and 45.0% were male. Pooled proportions of perioperative adverse events were similar between the DB/CB and IB groups except for wound complication which was higher in the former group (RR = 2.54 (95% CI: 1.82; 3.55)). Proportions of post-surgical Matsushima Grade A/B revascularization favored DB/CB over IB ( RR = 1.12 (95% CI 1.02; 1.24)). There was no significant difference in stroke recurrence, morbidity, and mortality. After meta-regression analysis, year of publication and age were significant predictors of outcomes. Conclusions IB, DB/CB are relatively effective and safe revascularization options for pediatric MMD/MMS. Low-quality GRADE evidence suggests that DB/CB was associated with better long-term angiographic revascularization outcomes when compared with IB, although this did not translate to long-term stroke and mortality benefits.

OBJECTIVE:Proton beam therapy (PBT) is an increasingly used treatment modality for pediatric patients with brain tumors. Moyamoya syndrome (MMS) is well recognized as a complication of traditional photon radiotherapy, however its association with PBT is less well described. The authors discuss their initial experience with the neurosurgical management of MMS secondary to PBT in a large-volume pediatric neurovascular service. METHODS:The authors performed a retrospective case review of consecutive children referred for neurosurgical management of MMS after PBT between 2009 and 2022. Patient demographic characteristics, oncological history and treatment, interval between PBT and MMS diagnosis, and MMS management were recorded. Clinical outcome at last review was classified as good if the modified Rankin Scale (mRS) score was ≤ 2 and/or the patient attended mainstream education without additional assistance. Poor outcome was defined as mRS score ≥ 3 and/or the patient received additional educational support. The recorded radiological outcomes included angiographic analysis of stenosis, evidence of brain ischemia/infarction on MRI, and postsurgical angiographic revascularization. RESULTS:Ten patients were identified. Oncological diagnosis included craniopharyngioma (n = 6), optic pathway glioma (1), ependymoma (1), Ewing sarcoma (1), and rhabdosarcoma (1). The median (interquartile range [IQR]) age at PBT was 5.1 (2.7-7.9) years. The median (IQR) age at MMS diagnosis was 7.8 (5.7-9.3) years. The median time between PBT and diagnosis of MMS was 20 (15-41) months. Six patients had poor functional status after initial oncological treatment and prior to diagnosis of MMS. All 10 patients had endocrine dysfunction, 8 had visual impairment, and 4 had behavioral issues prior to MMS diagnosis. Four patients had a perioperative ischemic event: 2 after tumor surgery, 1 after MMS surgical revascularization, and 1 after receiving a general anesthetic for an MRI scan during oncological surveillance. Seven children were treated with surgical revascularization, whereas 3 were managed medically. The incidence of ischemic events per cerebral hemisphere was reduced after surgical revascularization: only 1 patient of 7 had an ischemic event during the follow-up period after surgery. No children moved from good to poor functional status after MMS diagnosis. CONCLUSIONS:MMS can occur after PBT. Magnetic resonance angiography sequences should be included in surveillance MRI scans to screen for MMS, and families should be counseled about this complication. Management at a high-volume pediatric neurovascular center, including selective use of revascularization surgery, appears to maintain functional status in these children.