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OBJECTIVE Endoscopic strip craniectomy with postoperative helmet orthosis therapy (ESCH) has emerged as a less invasive alternative to fronto-orbital remodeling for correction of trigonocephaly. However, there is no standardized objective method for monitoring morphological changes following ESCH. Such a method should be reproducible and avoid the use of ionizing radiation and general anesthesia for diagnostic imaging. The authors analyzed a number of metrics measured using 3D stereophotogrammetry (3DSPG) following ESCH, an imaging alternative that is free of ionizing radiation and can be performed on awake children. METHODS 3DSPG images obtained at two time points (perisurgical and 1-year follow-up [FU]) of children with metopic synostosis who had undergone ESCH were analyzed and compared to 3DSPG images of age-matched control children without craniofacial anomalies. In total, 9 parameters were measured, the frontal angle and anteroposterior volume in addition to 7 novel parameters: anteroposterior area ratio, anteroposterior width ratios 1 and 2, and right and left anteroposterior diagonal ratios 30 and 60. RESULTS Six eligible patients were identified in the operated group, and 15 children were in the control group. All 9 parameters differed significantly between perisurgical and age-matched controls, as well as from perisurgical to FU scans. Comparison of FU scans of metopic synostosis patients who underwent surgery to scans of age-matched controls without metopic synostosis revealed that all parameters were statistically identical, with the exception of the right anteroposterior diagonal ratio 30, which was not fully corrected in the treated patients. The left anterior part of the head showed the most change in surface area maps. CONCLUSIONS In this pilot study, ESCH showed satisfactory results at 1 year, with improvements in all measured parameters compared to perisurgical results and normalization of 8 of 9 parameters compared to an age-matched control group. The results indicate that these parameters may be useful for craniofacial units for monitoring changes in head shape after ESCH for trigonocephaly and that 3DSPG, which avoids the use of anesthesia and ionizing radiation, is a satisfactory monitoring method.

Atlantoaxial rotatory fixation (AARF) is a rare condition that occurs most commonly in children. The torticollis caused by AARF usually presents as abnormal posturing of the head and neck, with rotation of the chin to the opposite side. AARF in children could occur due to congenital bony malformation, minor trauma, upper respiratory tract infections (Grisel's syndrome), postoperatively after head and neck (ENT) surgery, and unknown reasons. AARF in the postoperative patient is a rare and poorly understood entity.The authors present a case report of a 2-year-old boy with Crouzon Syndrome undergoing posterior calvarial vault expansion (PVE) surgery that developed AARF as a complication.The authors believe that cranial vault surgery should be considered a potential risk procedure for AARF, especially if it is done in susceptible populations (syndromic craniosynostosis patients) with other underlying sequelae (tonsillar ectopia or syringomyelia). During surgery, careful attention should be paid to maintaining a neutral alignment of the patient's cervical spine as rotatory movements under anaesthesia and muscle relaxation may be contributory factors.AARF should be suspected and investigated in children with painful torticollis after craniofacial surgery.

Aims To assess the diagnostic accuracy of fundoscopy and visual evoked potentials (VEPs) in detecting intracranial hypertension (IH) in patients with craniosynostosis undergoing spring-assisted posterior vault expansion (sPVE). Methods Children with craniosynostosis undergoing sPVE and 48-hour intracranial pressure (ICP) monitoring were included in this single-centre, retrospective, diagnostic accuracy study. Data for ICP, fundoscopy and VEPs were analysed. Primary outcome measures were papilloedema on fundoscopy, VEP assessments and IH, defined as mean ICP > 20 mmHg. Diagnostic indices were calculated for fundoscopy and VEPs against IH. Secondary outcome measures included final visual outcomes. Results Fundoscopic examinations were available for 35 children and isolated VEPs for 30 children, 22 of whom had at least three serial VEPs. Sensitivity was 32.1% for fundoscopy (95% confidence intervals [CI]: 15.9–52.4) and 58.3% for isolated VEPs (95% CI 36.6–77.9). Specificity for IH was 100% for fundoscopy (95% CI: 59.0–100) and 83.3% for isolated VEPs (95% CI: 35.9–99.6). Where longitudinal deterioration was suspected from some prVEPs but not corroborated by all, sensitivity increased to 70.6% (95% CI: 44.0–89.7), while specificity decreased to 60% (95% CI: 14.7–94.7). Where longitudinal deterioration was clinically significant, sensitivity decreased to 47.1% (23.0–72.2) and specificity increased to 100% (47.8–100). Median final BCVA was 0.24 logMAR (n = 36). UK driving standard BCVA was achieved by 26 patients (72.2%), defined as ≥0.30 logMAR in the better eye. Conclusion Papilloedema present on fundoscopy reliably indicated IH, but its absence did not exclude IH. VEP testing boosted sensitivity at the expense of specificity, depending on method of analysis.

OBJECTIVESThe goal of this study was to characterize the complications and morbidity related to the surgical management of pediatric fourth ventricle tumors.METHODSAll patients referred to the authors’ institution with posterior fossa tumors from 2002 to 2018 inclusive were screened to include only true fourth ventricle tumors. Preoperative imaging and clinical notes were reviewed to extract data on presenting symptoms; surgical episodes, techniques, and adjuncts; tumor histology; and postoperative complications.RESULTSThree hundred fifty-four children with posterior fossa tumors were treated during the study period; of these, 185 tumors were in the fourth ventricle, and 167 fourth ventricle tumors with full data sets were included in this analysis. One hundred patients were male (mean age ± SD, 5.98 ± 4.12 years). The most common presenting symptom was vomiting (63.5%). The most common tumor types, in order, were medulloblastoma (94 cases) > pilocytic astrocytoma (30 cases) > ependymoma (30 cases) > choroid plexus neoplasms (5 cases) > atypical teratoid/rhabdoid tumor (4 cases), with 4 miscellaneous lesions. Of the 67.1% of patients who presented with hydrocephalus, 45.5% had an external ventricular drain inserted (66.7% of these prior to tumor surgery, 56.9% frontal); these patients were more likely to undergo ventriculoperitoneal shunt (VPS) placement at a later date (p = 0.00673). Twenty-two had an endoscopic third ventriculostomy, of whom 8 later underwent VPS placement. Overall, 19.7% of patients had a VPS sited during treatment.Across the whole series, the transvermian approach was more frequent than the telovelar approach (64.1% vs 33.0%); however, the telovelar approach was significantly more common in the latter half of the series (p < 0.001). Gross-total resection was achieved in 70.7%. The most common postoperative deficit was cerebellar mutism syndrome (CMS; 28.7%), followed by new weakness (24.0%), cranial neuropathy (18.0%), and new gait abnormality/ataxia (12.6%). Use of intraoperative ultrasonography significantly reduced the incidence of CMS (p = 0.0365). There was no significant difference in the rate of CMS between telovelar or transvermian approaches (p = 0.745), and multivariate logistic regression modeling did not reveal any statistically significant relationships between CMS and surgical approach.CONCLUSIONSSurgical management of pediatric fourth ventricle tumors continues to evolve, and resection is increasingly performed through the telovelar route. CMS is enduringly the major postoperative complication in this patient population.

OBJECTIVE Sagittal craniosynostosis (SC) is the most commonly encountered form of craniosynostosis. Despite its relative frequency, there remains significant heterogeneity in both operative management and follow-up between centers and a relative paucity of long-term outcome data in the literature. At the authors’ institution, families of children presenting with SC are offered the following options: 1) conservative management with ophthalmic surveillance, 2) minimally invasive surgery at < 6 months of age (spring-assisted cranioplasty [SAC]) or 3) calvarial vault remodeling at any age (CVR). The authors reviewed outcomes for all children presenting with SC during a 5-year period, regardless of the treatment received. METHODS Consecutive children born between January 1, 2008, and December 31, 2012, presenting with SC were identified, and detailed chart reviews were undertaken. Demographic, surgical, perioperative, head shape, scar, and neurodevelopmental (behavioral, education, speech, and language) data were analyzed. The cohort was divided by type of surgery (none, SAC, or CVR) and by age at surgery (early, defined as ≤ 6 months; or late, defined as > 6 months) for comparison purposes. RESULTS A total of 167 children were identified, 129 boys and 38 girls, with a median age at presentation of 5.0 (range 0.4–135) months. Three families opted for conservative management. Of the 164 children who underwent surgery, 83 underwent SAC, 76 underwent CVR, and 5 underwent a "hybrid" procedure (CVR with springs). At a median age of 7.0 (range 0.5–12.3) years, there was no significant difference in concerns regarding head shape, scar, or neurodevelopmental outcomes between the early and late intervention groups over all procedures performed, or between the early or late SAC and CVR cohorts. There were more head shape concerns in the SAC group than in the CVR group overall (25.7% vs 11.8%, respectively; p = 0.026), although most of these concerns were minor and did not require revision. CONCLUSIONS In this cohort, regardless of operative intervention and timing of intervention, infants achieved similar neurodevelopmental outcomes. Minimally invasive surgery (SAC) appears to result in less complete correction of head shape than CVR, but this may be balanced by advantages in reduced operative time, hospitalization, and blood loss. SAC was equal to CVR in neuropsychological outcomes.

Spring-assisted cranioplasty (SAC) is a minimally invasive technique for treating sagittal synostosis in young infants. Yet, follow-up data on cranial growth in patients who have undergone SAC are lacking. This project aimed to understand how the cranial shape develops during the postoperative period, from spring insertion to removal. 3D head scans of 30 consecutive infants undergoing SAC for sagittal synostosis were acquired using a handheld scanner pre-operatively, immediately postoperatively, at follow-up and at spring removal; 3D scans of 41 age-matched control subjects were also acquired. Measurements of head length, width, height, circumference, and volume were taken for all subjects; cephalic index (CI) was calculated. Statistical shape modeling was used to compute 3D average head models of sagittal patients at the different time points. SAC was performed at a mean age of 5.2 months (range 3.3-8.0) and springs were removed 4.3 months later. CI increased significantly (P < 0.001) from pre-op (69.5% ± 2.8%) to spring removal (74.4% ± 3.9%), mainly due to the widening of head width, which became as wide as for age-matched controls; however, the CI of controls was not reached (82.3% ± 6.8%). The springs did not constrain volume changes and allowed for natural growth. Population mean shapes showed that the bony prominences seen at the sites of spring engagement settle over time, and that springs affect the overall 3D head shape of the skull. In conclusion, results reaffirmed the effectiveness of SAC as a treatment method for nonsyndromic single suture sagittal synostosis.

Supplemental Digital Content is available in the text, Objective: Craniosynostosis can be associated with raised intracranial pressure (ICP), which can pose deleterious effects on the brain and vision if untreated. Estimating ICP in children is challenging, whilst gold standard direct intracranial measurement of ICP is invasive and carries risk. This systematic review aims to evaluate the role of optical coherence tomography (OCT), a noninvasive imaging technique, for detecting raised ICP in children with craniosynostosis. Methods: The authors conducted a systematic review of the literature published from inception until 19 August, 2019 in the Cochrane Central Register of Controlled Trials, PubMed, MEDLINE, and EMBASE. Eligible studies evaluated the role of OCT in detecting raised ICP in children aged 0 to 16 years with craniosynostosis. Main outcome measures were sensitivity and specificity of OCT parameters for raised ICP. Quality assessment was performed using the National Institutes of Health Quality Assessment Tool for Observational Cohort and Cross-sectional Studies. Results: Out of 318 records identified, data meeting the inclusion criteria were obtained from 3 studies. The quality of 2 studies was poor whilst 1 was fair. Optical coherence tomography demonstrated higher sensitivity and specificity for detecting raised ICP compared to fundus examination, clinical history, radiological testing, and visual field testing. Conclusions: This systematic review demonstrated a lack of quality evidence for OCT as a screening tool for children with craniosynostosis. Further research is required to clarify the strength of OCT in this role and to determine which OCT parameters are most appropriate.

OBJECTIVE Endoscopic strip craniectomy with postoperative molding helmet therapy (ESC-H) and spring-assisted cranioplasty (SAC) are commonly used minimally invasive techniques for correction of nonsyndromic sagittal craniosynostosis, but it is unclear which, if either, is superior. Therefore, the authors undertook a systematic review to compare ESC-H with SAC for the surgical management of nonsyndromic single-suture sagittal craniosynostosis. METHODS Studies were identified through a systematic and comprehensive search of four databases (Embase, MEDLINE, and two databases in the Cochrane Library). Databases were searched from inception until February 19, 2021. Pediatric patients undergoing either ESC-H or SAC for the management of nonsyndromic single-suture sagittal craniosynostosis were included. Systematic reviews and meta-analyses, single-patient case reports, mixed cohorts of nonsyndromic and syndromic patients, mixed cohorts of different craniosynostosis types, and studies in which no outcomes of interest were reported were excluded. Outcomes of interest included reoperations, blood transfusion, complications, postoperative intensive care unit (ICU) admission, operative time, estimated blood loss, length of hospital stay, and cephalic index. Pooled summary cohort characteristics were calculated for each outcome of interest. Methodological quality was assessed using the Newcastle-Ottawa Scale. The study was reported in accordance with the 2020 PRISMA statement. RESULTS Twenty-two studies were eligible for inclusion in the review, including 1094 patients, of whom 605 (55.3%) underwent ESC-H and 489 (44.7%) underwent SAC for nonsyndromic sagittal craniosynostosis. There was no difference between the pooled estimates of the ESC-H and SAC groups for operative time, length of stay, estimated blood loss, and cephalic index. There was no difference between the groups for reoperation rate and complication rate. However, ESC-H was associated with a higher blood transfusion rate and higher postoperative ICU admission. CONCLUSIONS The available literature does not demonstrate superiority of either ESC-H or SAC, and outcomes are broadly similar for the treatment of nonsyndromic sagittal craniosynostosis. However, the evidence is limited by single-center retrospective studies with low methodological quality. There is a need for international multicenter randomized controlled trials comparing both techniques to gain definitive and generalizable data.

IntroductionPaediatric intracranial hypertension (IH) is a rare but serious condition that can pose deleterious effects on the brain and vision. Estimating intracranial pressure (ICP) in children is difficult. Gold standard direct ICP measurement is invasive and carries risk. It is impractical to routinely perform direct ICP measurements over time for all children at risk of IH. This study proposes to assess the diagnostic accuracy of handheld optical coherence tomography (OCT), a non-invasive ocular imaging method, to detect IH in children.Methods and analysisThis is a prospective study evaluating the diagnostic accuracy of handheld OCT for IH in at risk children. Inclusion criteria include clinical and/or genetic diagnosis of craniosynostosis, idiopathic intracranial hypertension, space occupying lesion or other conditions association with IH and age 0–18 years old. Exclusion criteria include patients older than 18 years of age and/or absence of condition placing the child at risk of IH. The primary outcome measures are handheld OCT and 48-hour ICP assessments, which will be used for diagnostic accuracy testing (sensitivity, specificity, positive predictive value, negative predictive value and accuracy). Main secondary outcome measures include visual acuity, fundoscopic examination, contrast sensitivity, visual field testing and visual evoked potentials, wherever possible.Ethics and disseminationEthical approval was granted for this study by the East Midlands Nottingham 2 Research Ethics committee (UOL0348/IRAS 105137). Our findings will be disseminated through presentation at relevant meetings, peer-reviewed publication and via the popular media.Trial registration numberISRCTN52858719

Purpose To determine whether handheld optical coherence tomography (OCT) is feasible and repeatable in children with craniosynostosis. Methods This was a prospective cross-sectional study. Children with syndromic and non-syndromic craniosynostosis 0 to 18 years of age were recruited between February 13, 2020, and October 1, 2020. Main outcome measures included feasibility (patient recruitment and handheld OCT success rates) and repeatability, which were assessed using intraclass correlation coefficients (ICCs) where repeated images of the optic nerve head (ONH) within the same visit were available. ONH parameters used for repeatability analysis included cup depth, width, and area; disc width; rim height; retinal thickness; retinal nerve fiber layer thickness; and Bruch's membrane opening minimum rim width. Results Fifty children were approached, and all 50 (100%) were successfully recruited. Median age was 51.1 months (range, 1.9-156.9; interquartile range, 37.0-74.2), and 33 of the children (66%) were male. At least one ONH image was obtained in 43 children (86%), and bilateral ONH imaging was successful in 38 children (76%). Factors boosting the likelihood of success included good understanding and cooperation of the child and parent/guardian and availability of an assistant. Repeatability analysis was performed in 20 children, demonstrating good repeatability (ICC range, 0.77-0.99; the majority exceeded 0.90). OCT correctly identified two cases of intracranial hypertension, one of which was undetected by prior fundoscopy. Conclusions Handheld OCT is feasible and repeatable in children with syndromic and non-syndromic forms of craniosynostosis. Translational relevance Our handheld OCT approach could be used for the clinical surveillance of children with craniosynostosis.

Purpose Late deformity/indentation is well-recognised following fronto-orbital remodelling (FOR) for metopic synostosis. We hypothesise that if damage to temporalis muscle were a contributor, the thickness of soft tissue and bone in the affected area would be reduced. Materials and methods Soft tissues and bone were separately segmented and reconstructed three-dimensionally from computed tomograms of 8 patients 1.5–18 years post-FOR performed at 16 ± 2 months for metopic synostosis and from 8 age-matched controls. Soft tissue (taken as proxy for temporalis muscle) and bone thickness overall and in the indented areas were computed. Results Post-FOR, three-dimensional soft tissue thickness maps demonstrated temporalis extending upwards but falling short of the indented area. Overall skull thickness increased with age post-FOR (logarithmic fit R2 = 0.71) and for controls (R2 = 0.90). Although immediately post-FOR the future indented area had a thickness of 98% of control, it decreased linearly to 64% 16 years later (Pearson's r = 0.84). Conclusion These findings suggest that late post-FOR deformity/indentation is enhanced by limited upward extension (or retraction downwards) of temporalis muscle, while bone thickness in the affected area gradually decreases. This supports the hypothesis that aberrant re-attachment of the temporalis muscle makes a material contribution to late deformity following FOR for metopic synostosis.

BACKGROUND: Brain tumours are the most common solid tumours in childhood. Half of these tumours occur in the posterior fossa, where surgical removal is complicated by the risk of cerebellar mutism syndrome, of which postoperative speech impairment (POSI) is a cardinal symptom, in up to 25% of patients. The surgical approach to midline tumours, mostly undertaken by transvermian or telovelar routes, has been proposed to influence the risk of POSI. We aimed to investigate the risk of developing POSI, the time course of its resolution, and its association with surgical approach and other clinical factors. METHODS: In this observational prospective multicentre cohort study, we included children (aged

Monobloc and bipartition advancement by external distraction plays a major role in the treatment of syndromic craniosynostosis. They can reverse the associated facial deformity and play a role in the management of ocular exposure, intracranial hypertension, and upper airway obstruction. Facial bipartition distraction corrects the intrinsic facial deformities of Apert syndrome. Both procedures are associated with relatively high complication rates principally related to ascending infection and persistent cerebrospinal fluid leaks. Modern perioperative management has resulted in a significant decline in complications. External distractors allow fine tuning of distraction vectors and improve outcome but are less well tolerated than internal distractors.

To assess the diagnostic accuracy of fundoscopy and visual evoked potentials (VEPs) in detecting intracranial hypertension (IH) in patients with craniosynostosis undergoing spring-assisted posterior vault expansion (sPVE).Children with craniosynostosis undergoing sPVE and 48-hour intracranial pressure (ICP) monitoring were included in this single-centre, retrospective, diagnostic accuracy study. Data for ICP, fundoscopy and VEPs were analysed. Primary outcome measures were papilloedema on fundoscopy, VEP assessments and IH, defined as mean ICP 20 mmHg. Diagnostic indices were calculated for fundoscopy and VEPs against IH. Secondary outcome measures included final visual outcomes.Fundoscopic examinations were available for 35 children and isolated VEPs for 30 children, 22 of whom had at least three serial VEPs. Sensitivity was 32.1% for fundoscopy (95% confidence intervals [CI]: 15.9-52.4) and 58.3% for isolated VEPs (95% CI 36.6-77.9). Specificity for IH was 100% for fundoscopy (95% CI: 59.0-100) and 83.3% for isolated VEPs (95% CI: 35.9-99.6). Where longitudinal deterioration was suspected from some prVEPs but not corroborated by all, sensitivity increased to 70.6% (95% CI: 44.0-89.7), while specificity decreased to 60% (95% CI: 14.7-94.7). Where longitudinal deterioration was clinically significant, sensitivity decreased to 47.1% (23.0-72.2) and specificity increased to 100% (47.8-100). Median final BCVA was 0.24 logMAR (n = 36). UK driving standard BCVA was achieved by 26 patients (72.2%), defined as ≥0.30 logMAR in the better eye.Papilloedema present on fundoscopy reliably indicated IH, but its absence did not exclude IH. VEP testing boosted sensitivity at the expense of specificity, depending on method of analysis.

Craniopagus conjoined twins are extraordinarily rare and present unique challenges to the multidisciplinary team. There is a paucity of literature on optimizing neuro-ophthalmologic evaluation in craniopagus twins. Herein, we present our enhanced neuro-ophthalmologic evaluation and management in 17-month-old male craniopagus twins, uniquely using handheld optical coherence tomography (OCT) plus portable slit-lamp biomicroscopy, indirect ophthalmoscopy and modified forced-choice preferential looking assessment. Staged surgical separation was supported by enhanced neuro-ophthalmologic evaluation, detailed radiology, three-dimensional printing and virtual reality simulation. This represents the fourth separation of craniopagus twins by our unit.

Limited information is available on the effect of sagittal craniosynostosis (CS) on morphological and material properties of the parietal bone. Understanding these properties would not only provide an insight into bone response to surgical procedures but also improve the accuracy of computational models simulating these surgeries. The aim of the present study was to characterise the mechanical and microstructural properties of the cortical table and diploe in parietal bone of patients affected by sagittal CS. Twelve samples were collected from pediatric patients (11 males, and 1 female; age 5.2 ± 1.3 months) surgically treated for sagittal CS. Samples were imaged using micro-computed tomography (micro-CT); and mechanical properties were extracted by means of micro-CT based finite element modelling (micro-FE) of three-point bending test, calibrated using sample-specific experimental data. Reference point indentation (RPI) was used to validate the micro-FE output. Bone samples were classified based on their macrostructure as unilaminar or trilaminar (sandwich) structure. The elastic moduli obtained using RPI and micro-FE approaches for cortical tables (ERPI 3973.33 ± 268.45 MPa and Emicro-FE 3438.11 ± 387.38 MPa) in the sandwich structure and diploe (ERPI1958.17 ± 563.79 MPa and Emicro-FE 1960.66 ± 492.44 MPa) in unilaminar samples were in strong agreement (r = 0.86, p

The authors provide a comprehensive framework with which to approach paediatric calvarial injury sustained as a result of suspected abusive head trauma (AHT). This is achieved through the presentation of a case series set in the context of the unique morphology of the infant skull and the possible diagnostic pitfalls which may arise due to the presence of variant anatomy or other mimicking conditions. A retrospective analysis of sixty-three patients referred to our institution with suspected AHT was carried out. Seventeen patients with skull fractures were identified and their fractures were described in terms of anatomical location, type and course. Our data was then interpreted in the light of known anatomical fracture mimics and the available literature on the subject. Forty-two skull fractures were identified and described in our cohort, most of which were simple linear fractures of the parietal bones (33%). There were also a substantial number of complex stellate fractures, namely of the parietal (29%) and occipital (10%) bones. Eleven fracture mimics including accessory sutures and wormian bones were also identified in this cohort. Our study supports and builds on the existing literature, thereby offering a more complete view of the spectrum of calvarial damage sustained as a result of AHT in the context of its diagnostic pitfalls.

Introduction Craniosynostosis is characterised by the premature fusion of cranial sutures. This can be associated with raised intracranial pressure (ICP), which can lead to developmental delay, visual impairment and death. Treatment involves surgical expansion of the skull vault. There is no consensus over who to treat and when. Intracranial pressure is difficult to estimate in a child and existing methods possess sub-optimal diagnostic accuracy to be employed as screening tools. Here, we propose a systematic review protocol to examine the role of optical coherence tomography (OCT) in early detection of raised ICP in craniosynostosis. Methods Electronic searches in the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE and Embase will identify studies featuring OCT in detecting raised ICP in children with craniosynostosis. Two independent researchers will identify studies for inclusion using a screening questionnaire. Quality will be assessed using the National Institutes of Health (NIH) Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies. The primary outcome measure is the sensitivity and specificity of OCT in detecting raised ICP in children with craniosynostosis. Secondary outcomes measures include the sensitivity and specificity of other surrogate measures for raised ICP, OCT parameters used and normal ranges for ICP reported. A formal narrative synthesis with descriptive statistics will be presented. Discussion The proposed study will be the first to examine the role of optical coherence tomography in the early recognition of raised intracranial pressure in craniosynostosis, thereby addressing an important clinical problem in paediatric ophthalmology and craniofacial surgery. This systematic review protocol provides transparency to the proposed methods and reduces the possibility of duplication. The proposed methods reflect those prescribed by the Cochrane Collaboration. Systematic review registration International Prospective Register for Systematic Reviews (PROSPERO) number CRD42019147693

Spring-assisted surgery for the correction of scaphocephaly has gained popularity over the past 2 decades. Our unit utilizes standardized torsional springs with a central helix for spring-assisted surgery. This design allows a high degree of accuracy and reproducibility of the force vectors and force distance curves. In this manuscript, we expand on the biomechanical testing and properties of these springs. Standardization of design has enabled us to study the springs on bench and in vivo and a comprehensive repository of calvarial remodeling and spring dynamics has been acquired and analyzed.Finite element modeling is a technique utilized to predict the outcomes of spring-assisted surgery. We have found this to be a useful tool, in planning our surgical strategy and improving outcomes. This technique has also contributed significantly to the process of informed consent preoperatively. In this article, we expand on our spring design and dynamics as well as the finite element modeling used to predict and improve outcomes.In our unit, this practice has led to a significant improvement in patient outcomes and parental satisfaction and we hope to make our techniques available to a wider audience.

ObjectivesTo evaluate the diagnostic capability of optical coherence tomography (OCT) in children aged under 18 years old with intracranial hypertension (IH).DesignSystematic review.MethodsWe conducted a systematic review using the following platforms to search the keywords ‘optical coherence tomography’ and ‘intracranial hypertension’ from inception to 2 April 2020: Cochrane Central Register of Controlled Trials, EMBASE, MEDLINE, PubMed and Web of Science, without language restrictions. Our search returned 2729 records, screened by two independent screeners. Studies were graded according to the Oxford Centre for Evidence-Based Medicine and National Institutes of Health Quality Assessment Tool for observational studies.ResultsTwenty-one studies were included. Conditions included craniosynostosis (n=354 patients), idiopathic IH (IIH; n=102), space-occupying lesion (SOL; n=42) and other pathology (n=29). OCT measures included optic nerve parameters, rim parameters (notably retinal nerve fibre layer thickness) and retinal parameters. Levels of evidence included 2b (n=13 studies), 3b (n=4) and 4 (n=4). Quality of 10 studies was fair and 11 poor. There was inconsistency in OCT parameters and reference measures studied, although OCT did demonstrate good diagnostic capability for IH in craniosynostosis, IIH and SOL.ConclusionsThis systematic review identified various studies involving OCT to assist diagnosis and management of IH in children with craniosynostosis, IIH, SOL and other pathology, in conjunction with established clinical measures of intracranial pressure. However, no level 1 evidence was identified. Validating prospective studies are, therefore, required to determine optimal OCT parameters in this role and to develop formal clinical guidelines.PROSPERO registration numberCRD42019154254.

OBJECTIVEThe management of children with craniopharyngioma has evolved over time, with a trend toward less invasive neurosurgical approaches as surgeons have sought to balance oncological control and treatment-related morbidity. To this end, the aim of this study was to evaluate the safety and effectiveness of the current management of children with craniopharyngioma compared to the previous management methods used at the authors’ treatment center.METHODSA prospectively maintained database was searched over a 14-year period between January 1, 2005, and December 31, 2018, to identify all children 17 years of age or younger with a new diagnosis of craniopharyngioma. A retrospective case note review was performed for each child to extract data on the presentation, investigation, treatment, and outcome of their illness. Morbidity was assessed in the same fashion as in previous cohorts, according to the following categories: visual loss, pituitary dysfunction, hypothalamic dysfunction, neurological deficits, and cognitive impairment.RESULTSIn total, 59 children were identified with craniopharyngioma during the study period. A total of 92 operations were performed, including cyst drainage (35/92; 38.0%), craniotomy and resection (30/92; 32.6%), and transsphenoidal resection (16/92; 17.4%). Approximately two-thirds of all operations were performed using image guidance (66/92; 71.7%) and one-third were performed using endoscopy (27/92; 29.3%). The majority of children had adjuvant therapy comprising proton beam therapy (18/59; 30.5%) or conventional radiotherapy (16/59; 27.1%). The median follow-up duration was 44 months (range 1–142 months), and approximately one-half of the children had no evidence of residual disease on MRI studies (28/59; 47.5%). Of the remaining 31 children, there was a reduction in the volume of residual disease in 8 patients (8/59; 13.6%), stable residual disease in 18 (18/59; 30.5%), and tumor growth in 5 patients (5/59; 8.5%). There was significantly reduced morbidity (p < 0.05) in all categories in the current cohort compared with our last cohort (1996–2004).CONCLUSIONSThe authors’ institutional experience of pediatric craniopharyngioma confirms a trend toward less invasive neurosurgical procedures, most of which are now performed with the benefit of image guidance or endoscopy. Moreover, the authors have identified an expanding role for more targeted radiotherapy for children with residual disease. These advances have allowed for tumor control comparable to that achieved in previous cohorts, but with significantly reduced morbidity and mortality.

Three-dimensional surgical planning is used widely in orthognathic surgery. Although numerous computer programs exist, the accuracy of soft tissue prediction remains uncertain. The purpose of this study was to compare the prediction accuracy of Dolphin, ProPlan CMF, and a probabilistic finite element method (PFEM). Seven patients (mean age 18years; five female) who had undergone Le Fort I osteotomy with preoperative and 1-year postoperative cone beam computed tomography (CBCT) were included. The three programs were used for soft tissue prediction using planned and postoperative maxillary position, and these were compared to postoperative CBCT. Accurate predictions were obtained with each program, indicated by root mean square distances: RMSDolphin=1.8±0.8mm, RMSProPlan=1.2±0.4mm, and RMSPFEM=1.3±0.4mm. Dolphin utilizes a landmark-based algorithm allowing for patient-specific bone-to-soft tissue ratios, which works well for cephalometric radiographs but has limited three-dimensional accuracy, whilst ProPlan and PFEM provide better three-dimensional predictions with continuous displacements. Patient or population-specific material properties can be defined in PFEM, while no soft tissue parameters are adjustable in ProPlan. Important clinical considerations are the topological differences between predictions due to the three algorithms, the non-negligible influence of the mismatch between planned and postoperative maxillary position, and the learning curve associated with sophisticated programs like PFEM.

Copyright © 2014 by the American Society of Plastic Surgeons. Background: The incidence of midline frontonasal dermoid cysts is one in 20,000 to one in 40,000. These lesions may have intracranial extension. This is explained by the anatomy and embryology of nasofrontal development. Skin involvement may also be extensive. Incomplete excision frequently leads to recurrence. The authors report their experience and pathway for management of midline dermoids. Methods: Databases were searched to identify patients who had undergone surgery for removal of a dermoid cyst. Preoperative imaging and indications for surgery were reviewed. Cases were grouped according to surgical approach, and outcomes and complications were identified. Results: Fifty-five patients were treated. Magnetic resonance imaging or computed tomography was used to delineate the anatomy, and surgical excision was expedited if there was a history of infection, especially if imaging suggested intracranial extension. Twelve patients were treated endoscopically (one was converted to open). Eleven required transcranial approaches for intracranial extension (20 percent). Of these, one lesion breached the dura. The remaining 32 patients had dermoids excised with an open approach (direct, bicoronal, or rhinoplasty). There were no recurrences in the open group and there was one recurrence in the transcranial group. This was treated by reexcision. Conclusion: Midline dermoid cysts are relatively uncommon. However, knowledge of the pathogenesis of these lesions together with the authors' experience over 15 years has allowed them to develop a protocol-driven approach, with a low incidence of complications.

The frontofacial monobloc advancement is a safe, effective, and cosmetically acceptable treatment of frontal and midface retrusions present in craniofacial syndromes. Although a number of modifications have been put forward to supplement this procedure, the basic steps of cleaving the facial skeleton from the skull base remain the same. We present a case where intraoperative navigation was used to visualize the craniofacial anatomy while the bone cuts were made for this cleaving process. This technique visually demonstrates parts of this procedure that were hitherto performed solely using tactile and auditory inputs superimposed on a 3-dimensional model of the craniofacial anatomy in the surgeon's mind. This technique offers a significant advantage for teaching purposes, in performing cases with complex anatomy and for accurate positioning of the distractor devices.