Management Of Craniosynostosis Research Articles

The Top 100 Most-Cited Publications in Clinical Craniofacial Research.

Craniosynostosis is a birth defect defined as premature closure of sutures leading to possible neurological deficits and cosmetic deformities. Most of the current literature to date focuses on craniosynostosis etiology by analyzing genetics. This paper is a bibliometric analysis of the most influential works related to the clinical management of craniosynostosis to help guide clinicians in their decision-making. Clarivate Web of Science database was used to identify the top 100 most-cited articles addressing the clinical management of craniosynostosis. A bibliometric review was performed to analyze publication metrics and track research trends. The 100 most-cited publications pertaining to craniosynostosis management were cited a cumulative 12,779 times. The highest cited article was Shillito and colleagues' "Craniosynostosis: A Review Of 519 Surgical Patients" with 352 citations. The oldest clinical craniosynostosis article dates back to 1948, and the most recent was published in 2016. The year with the most clinical-focused publications was 2011. The most prolific author was Renier, D. The United States produced 56 of the 100 articles. Most articles (n=52) were level 3 evidence. This bibliometric evaluation of craniosynostosis provides insight into the most impactful literature on this topic. The highest cited articles retrospectively analyze large sample sizes, outline proper evaluation, discuss intervention timelines, and highlight specific treatment plans for this birth defect. By filtering through existing literature, this analysis can guide clinicians on the management of craniosynostosis to maximize patient outcomes.

Machine learning applications in craniosynostosis diagnosis and treatment prediction: a systematic review.

Craniosynostosis refers to the premature fusion of one or more of the fibrous cranial sutures connecting the bones of the skull. Machine learning (ML) is an emerging technology and its application to craniosynostosis detection and management is underexplored. This systematic review aims to evaluate the application of ML techniques in the diagnosis, severity assessment, and predictive modeling of craniosynostosis. A comprehensive search was conducted on the PubMed and Google Scholar databases using predefined keywords related to craniosynostosis and ML. Inclusion criteria encompassed peer-reviewed studies in English that investigated ML algorithms in craniosynostosis diagnosis, severity assessment, or treatment outcome prediction. Three independent reviewers screened the search results, performed full-text assessments, and extracted data from selected studies using a standardized form. Thirteen studies met the inclusion criteria and were included in the review. Of the thirteen papers examined on the application of ML to the identification and treatment of craniosynostosis, two papers were dedicated to sagittal craniosynostosis, five papers utilized several different types of craniosynostosis in the training and testing of their ML models, and six papers were dedicated to metopic craniosynostosis. ML models demonstrated high accuracy in identifying different types of craniosynostosis and objectively quantifying severity using innovative metrics such as metopic severity score and cranial morphology deviation. The findings highlight the significant strides made in utilizing ML techniques for craniosynostosis diagnosis, severity assessment, and predictive modeling. Predictive modeling of treatment outcomes following surgical interventions showed promising results, aiding in personalized treatment strategies. Despite methodological diversities among studies, the collective evidence underscores ML's transformative potential in revolutionizing craniosynostosis management.

Scoping Review of Healthcare Disparities in Treatment of Craniosynostosis: Assessing Impacts of Race, Insurance, and Socioeconomic Status

Background: Racial and socioeconomic factors persist as barriers to timely diagnosis and treatment for children with craniosynostosis, contributing to inequitable care. This scoping review aims to better understand how disparities influence differences in services, approach, and outcomes for patients undergoing surgical corrections for craniosynostosis. Methods: We conducted a PRISMA systematic review of all articles published between 2003 and 2023 within PubMed/Medline, Embase, and a Scopus cited review. Variables of interest included sociodemographic factors such as race, socioeconomic status (SES), and insurance status, and measurements of treatment access or outcomes within craniosynostosis surgical repair. Results: The final analysis included 28 studies, of which 21 studies examined race, 13 examined insurance status, and 5 examined household income or other socioeconomic factors. Overall, non-White, minority patients, patients with Medicaid or government-funded insurance, and patients from lower SES were more likely to experience delays in diagnosis and presentation for surgery compared to White and privately-insured patients. Black and Hispanic patients and patients with public insurance were more likely to undergo open cranial vault reconstruction (CVR) at an older age and experience longer total surgery time, increasing perioperative complication risk, compared to patients who underwent endoscopic procedures. Sociodemographic factors including non-White race, lower SES, and being underinsured or uninsured predisposed patients to the more expensive CVR procedure, increased hospital length of stay (LOS), and a higher rate of readmission, accumulating to greater total cost of care. Conclusion: Significant disparities continue to exist in craniosynostosis management. It is still unknown whether these discrepancies in the type of operation lead to significant clinical differences in neurocognitive outcomes, revision operations, or patient/parent satisfaction with appearance. This study highlights gaps in the current body of knowledge to better inform future aeas of investigation to ensure the best outcomes for all children born with craniosynostosis regardless of patient demographic.

Insights into craniosynostosis management in low- and middle-income countries: A narrative review of outcomes, shortcomings and paediatric neurosurgery capacity.

Craniosynostosis, marked by premature cranial suture fusion, necessitates prompt intervention to avert developmental, neurological, and aesthetic issues. While high-income countries have advanced in managing this condition, low- and middle-income countries grapple with substantial healthcare access disparities. This narrative review explores current craniosynostosis management in low- and middle-income countries. The review focused on studies published between 2008 and 2023. The focus was neurosurgical outcomes, and the search utilised databases like PubMed, EMBASE, Google Scholar, the Cochrane Library and Scopus, incorporating specific keywords and phrases. An in-depth analysis of 21 included studies reveals noteworthy positive outcomes, including low mortality, successful corrections and sustained efficacy. These advancements stem from enhanced pre-operative strategies, surgical techniques and postoperative care. Nonetheless, challenges persist, encompassing complications, mortality, reoperations, and treatment disparities, particularly in low- and middle-income countries constrained by financial and expertise limitations. The enhancement of clinical practice and the formulation of effective policies in the future entail several key strategies. These include the reinforcement of specialised healthcare infrastructure and diagnostic capabilities, the ongoing training and retention of neurosurgeons, the improvement of funding mechanisms, and the promotion of equitable access. Additionally, a crucial focus is placed on fortifying paediatric neurosurgical care in low- and middle-income countries. The recommendations underscore the importance of collaborative initiatives, the development of specialised healthcare infrastructure, and the implementation of strategic policies to not only advance pediatric neurosurgical care but also to address existing gaps in management.

The Enhanced Recovery After Surgery protocol for the surgical management of craniosynostosis: Lausanne experience.

Over the past decade, the Enhanced Recovery After Surgery (ERAS) program has demonstrated its effectiveness and efficiency in improving postoperative care and enhancing recovery across various surgical fields. Preliminary results of ERAS protocol implementation in craniosynostosis surgery are presented. An ERAS protocol was developed and implemented for cranial pediatric neurosurgery, focusing on craniosynostosis repair. The study incorporated a pre-ERAS group consisting of a consecutive series of patients who underwent craniosynostosis repair surgery prior to the implementation of the ERAS protocol; the results were compared with a consecutive group of patients who had been prospectively collected since the introduction of the ERAS for craniosynostosis protocol. The safety, feasibility, and efficiency of the ERAS protocol in pediatric neurosurgery was evaluated, through the collection of clinical data from the pre-, intra-, and postoperative phase. Surgery-related complications were evaluated according to the Clavien-Dindo classification. Costs of the stays were obtained using a microcosting approach. A total of 35 pre-ERAS patients and 10 ERAS patients were included. Scaphocephaly was the most common pathology in both groups. The overall compliance with the pre-, intra-, and postoperative criteria significantly increased-from 35.5%, 64.4%, and 54.7%, respectively, in each phase to 94%, 90%, and 84% (p < 0.001). The authors noticed a reduction in the average opioid dose used per patient in the ERAS group (p = 0.004), and they observed a trend toward a decreased mean length of stay from 5.2 days in the pre-ERAS group to 4.6 days in the ERAS group, without an increase of the rate of readmission within 30 days of surgery. The rate of complications decreased but this difference was not statistically significant. The hospital costs lowered significantly: from 21,958 Confederatio Helvetica Francs (CHF) in the pre-ERAS group to 18,936 CHF in the ERAS group (p = 0.02). The ERAS protocol represents a safe and cost-effective tool for the perioperative management of craniosynostosis. It showed its positive impact on the analgesia provided and on the reduction of in-hospital costs for these patients. ERAS protocols may thus be interesting options in the pediatric neurosurgical field.

Artificial Intelligence Applications in Diagnosing and Managing Non-syndromic Craniosynostosis: A Comprehensive Review.

Craniosynostosis is characterised by the premature fusion of one or more cranial sutures, resulting in an abnormal head shape. The management of craniosynostosis requires early diagnosis, surgical intervention, and long-term monitoring. With the advancements in artificial intelligence (AI) technologies, there is great potential for AI to assist in various aspects of managing craniosynostosis. The main aim of this article is to review available literature describing the current uses of AI in craniosynostosis. The main applications highlighted include diagnosis, surgical planning, and outcome prediction. Many studies have demonstrated the accuracy of AI in differentiating subtypes of craniosynostosis using machine learning (ML) algorithms to classify craniosynostosis based on simple photographs. This demonstrates its potential to be used as a screening tool and may allow patients to monitor disease progression reducing the need for CT scanning. ML algorithms can also analyse CT scans to aid in the accurate and efficient diagnosis of craniosynostosis, particularly when training junior surgeons. However, the lack of sufficient data currently limits this clinical application. Virtual surgical planning for cranial vault remodelling using prefabricated cutting guides has been shown to allow more precise reconstruction by minimising the subjectivity of the clinicians' assessment. This was particularly beneficial in reducing operating length and preventing the need for blood transfusions. Despite the potential benefits, there are numerous challenges associated with implementing AI in craniosynostosis. The integration of AI in craniosynostosis holds significant promise for improving the management of craniosynostosis. Further collaboration between clinicians, researchers, and AI experts is necessary to harness its full potential.

Management of Craniosynostosis in Lethal Perinatal Hypophosphatasia.

Although perinatal lethal hypophosphatasia (HPP) was once a disease with a universally poor prognosis, it has now become a rare but treatable condition with the advent of enzyme replacement therapy with asfotase alfa. As a result, a greater population of patients with perinatal HPP are presenting with abnormal head shape and craniosynostosis. The authors present here 3 cases of perinatal lethal HPP, 1 treated with traditional open cranial vault remodeling and 2 treated utilizing distraction osteogenesis techniques. All patients demonstrated outcomes comparable to those previously reported with traditional observation or open cranial vault repair. Thorough consideration and discussion between the surgical team and patient's family is needed to determine a treatment plan that best addresses the goals of patient and family in light of recent advances in medical treatment in this rare patient population in which surgical interventions were previously nearly impossible. This article further supports the safety and efficacy of surgical intervention and explores the utility of distraction osteogenesis to address craniosynostosis in this patient population.

Technical evolution of pediatric neurosurgery: craniosynostosis from 1972 to 2023 and beyond.

Very few clinical entities have undergone so many different treatment approaches over such a short period of time as craniosynostosis. Surgical treatments for this condition have ranged from simple linear craniectomies, accounting for the specific role of cranial sutures in assuring the normal growth of the skull, to more complex cranial vault reconstructions, based on the perceived role of the skull base in affecting the growth of the skull. While a great deal of evolution has occurred, there remains controversy regarding the ideal treatment including the best surgical technique, the optimal age for surgery, and the long-term morphological and neurodevelopmental outcomes. The evolution of the surgical management of craniosynostosis in the last 50years has been affected by several factors. This includes the awareness of needing to operate on affected children during infancy to achieve the best results, the use of multistage operations, the availability of more sophisticated surgical tools, and improved perioperative care. In some forms of craniosynostosis, the operations can be carried out at a very young age with low morbidity, and with the postoperative use of a molding helmet, springs, or distractors, these operations prove to be as effective as traditional larger cranial reconstructions performed in older children. As a consequence, complex surgical operations have become progressively less utilized. A second relevant advance was the more recent advent of a molecular diagnosis, which allowed us to understand the pathogenesis of some associated malformations and neurodevelopmental issues that were observed in some children despite appropriate surgical treatment. Future research should focus on improving the analysis of longer-term outcomes and understanding the natural history of craniofacial conditions, including what issues persist despite optimal surgical correction. Progress in molecular investigations concerning the normal and pathological development of cranial sutures could be a further significant step in the management of craniosynostosis, possibly favoring a "medical" treatment in the near future. Artificial intelligence will likely have a role in establishing the diagnosis with less reliance on radiographic studies and in assisting with surgical planning. Overall, much progress has been made, but there remains much to do.

Surgical Management of Craniosynostosis—Between the Past and the Future: A Comprehensive Review of the Literature

AbstractCranial sutures are fibrous joints between the bones of the cranial vault, acting as centers of osteogenesis of the embryonic mesenchyme. Skull growth is a strictly regulated process, controlled by several genetic pathways. A minor perturbation of these pathways may lead to premature fusion of the cranial sutures. Craniosynostosis occurs as a result of the premature fusion of the cranial sutures. The incidence of craniosynostosis is approximately 1 in 2,500 live births. Syndromic craniosynostoses such as Apert, Crouzon, and Pfeiffer comprise 15% of patients, while nonsyndromic craniosynostosis represents 85% of all patients. By the late 1800s, Lannelongue (Paris, 1890) and Lane (San Francisco, 1892) attempted the first surgical intervention (strip craniectomy) for the management of craniosynostosis. The inadequacy of simple suturectomies and strip craniectomies in the management of craniosynostosis led to the innovation of more complex procedures such as fronto-orbital advancement and posterior cranial vault distraction. However, these extensive surgical interventions are lengthy procedures and associated with more blood volume loss which requires blood transfusion. These limitations led to the modern era of minimally invasive endoscopic techniques. In this paper, we reviewed the body of the literature on the evolution of surgical management of craniosynostosis over the last century and the possible future directions.

Early bone reformation after cranial vault remodelling for sagittal craniosynostosis: A retrospective 3D analysis

This study aims to measure postoperative bone reformation percentage, rates and patterns after cranial vault remodelling (CVR) in isolated non-syndromic sagittal craniosynostosis. Volumetric bone measurements were performed starting from the DICOM files of previously available postoperative CT scans. The 3D images were then resampled into the master box, and ‘Skull 3D models’ were derived. The percentage of bone reformation was investigated using automated 3D analysis software. The intra-rater reliability analysis revealed high reliability (Intraclass correlation coefficient = 0.99, p < 0.001). The median bone reformation volume and rate were 11.2 ml and 1.98 ml/week, respectively. The median percentage of bone reformation was 56.7% when the median postoperative CT timing was 6.1 weeks. As a statistic model, the linear plateau showed the highest Pseudo R2 in both volume and percentage of bone reformation predicting patterns. By using the calculated model at 9 weeks postoperatively, the re-osteogenesis reaches 80% of the total cranial defect. After CVR, the early bone reformation pattern was demonstrated as a linear plateau model rather than logarithmic. This study gives a better understanding of the pattern and quantity of re-osteogenesis at cranial defects after CVR. The statistic model can facilitate healthcare practitioners to predict bone reformation and improve postoperative care protocol in sagittal craniosynostosis management.

Craniosynostosis in Patients With X-Linked Hypophosphatemia: A Review.

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

Craniosynostosis: Existing and Restructuring of Rehabilitation

Craniosynostosis is a developmental craniofacial anomaly, resulting in impairment of brain development and abnormally shaped skull. Restricted normal growth of the skull can lead to increased intracranial pressure and changes in brain morphology, which in turn may contribute to neurocognitive deficiency. The management of craniosynostosis, especially in the setting of craniofacial syndromes, is ideally done in a multidisciplinary clinic with a team focused toward comprehensive care. However, evidence has suggested the role of physical therapy in managing craniosynostosis, especially deformational plagiocephaly. The present review aims to find relevant evidence-based studies regarding the role of physical therapy in managing neonates with deformational plagiocephaly.

Knowledge, attitude, and practice related to craniosynostosis among physicians in Saudi Arabia: a national survey

Objectives: To evaluate the knowledge, attitude, and practice of physicians in Saudi Arabia about various aspects of the craniosynostosis. Methods: A cross-sectional study was conducted through a self-administered online survey that was distributed to a targeted sample of clinical specialists in the field of pediatric, family medicine, radiology, plastic surgery, obstetrics and gynecology, and neurosurgery in Saudi Arabia from November 2021 to February 2022. Results: Physicians&amp;apos; overall knowledge was poor, which seemed to influence their attitudes. In addition, plastic surgeons and neurosurgeons showed significantly higher knowledge scores than other specialists. Consultants and specialists showed better attitudes toward craniosynostosis management than residents. Finally, scores of clinical practices related to craniosynostosis were notably higher among physicians in public (ministry of health), academic, and military hospitals than in private hospitals. Conclusion: Poor knowledge of craniosynostosis might negatively affect physicians&amp;apos; behavior and clinical practice. Efforts should be made to increase awareness of this condition among physicians.

Cranioplasty for Bone Defects after Craniosynostosis Surgery in Pediatric Patient: A Case Report

Background: Craniosynostosis, the premature closure of skull sutures, can cause a misshapen head and neurologic issues. Surgical correction may result in bone defects requiring additional surgery. Case Description: We present a 5-year-old female who underwent cranioplasty for a skull defect and bulging brain after craniosynostosis surgery. Cranioplasty corrected the defect&amp;apos;s functional and cosmetic aspects. CT with 3D reconstruction confirmed findings and aided planning. The patient underwent cranioplasty using mesh over the defect and had an unremarkable recovery. Result: A 5-year-old had residual craniosynostosis bone defect and bulging brain. Cranioplasty used mesh over the defect, had unremarkable recovery. Early intervention and a multidisciplinary approach are key. More research is needed on this condition&amp;apos;s prevalence, causes and management. Conclusion: This case emphasizes early intervention and a multidisciplinary approach for craniosynostosis and related defects. A team approach addressing the condition&amp;apos;s physical and psychological impacts on the child and family is crucial for optimal outcomes. Cranioplasty is critical, and success depends on expertise. This case highlights the importance of timely, appropriate craniosynostosis management and related defect care. We hope this report contributes to literature and improves outcomes for patients undergoing cranioplasty after craniosynostosis surgery.

Guideline on Treatment and Management of Craniosynostosis: Patient and Family Version.

The care and treatment of patients with craniosynostosis and the new developments were described for health care professionals involved in this in the guideline "Treatment and Management of Craniosynostosis", which was revised in 2020. A patient version was written based on the professional guideline to make the information accessible to patients and parents too. In this patient version, each chapter consists of several sections. Firstly, an introduction and background information is provided in each chapter. Various questions are then answered based on scientific literature. Finally, the recommendations indicate the importance of the literature for care in practice and how this care should be provided in practice. This patient version is an abbreviated and simplified representation of the professional guideline. The introduction, conclusions, and recommendations sections of each chapter were revised and, where necessary, rewritten. With some surgical techniques, links to animation videos (recognizable by underlined references) have been added in the text for clarification. An attempt was made to stay as close as possible to the original guideline in terms of content, questions, numbering, and classification. The patient version can therefore easily be read side by side with the professional guideline if more information is required about a specific subject. As this patient version is a summary and does not deal with all aspects in detail, no rights can be derived from its content and the professional guideline takes precedence at all times. Originally, this patient version has been written in response to the established Dutch guideline on craniosynostosis for health care professionals.2 This professional guideline has been specifically tailored to the Dutch health care setting and policy. There are however differences between health care systems and national health policies of other countries and the Netherlands. It is important to keep in mind that this may, at some points, result in the management of care in your country and/or hospital different from outlined here.

Three-Dimensional Printed Polycaprolactone Mesh in Pediatric Cranial Vault Remodeling Surgery.

The surgical management of craniosynostosis has greatly evolved with improvements in both technology and understanding of the disease process. Some drawbacks remain regarding bone regeneration within the surgical bony gaps. Generally, bony gaps improve in the 12 to 24 months after surgery, but some gaps may remain for longer and cause deformity and/or require additional bony reconstruction. These considerations make tissue-engineered bone very attractive. Novel 3-dimensional printed bioresorbable mesh implants made of Polycaprolactone (PCL) can be used to fill the surgical bony defects. The authors seek to investigate how the use of a 3-dimensional printed biodegradable PCL mesh applied to bony defects in cranial vault surgery affects bone healing. Case series analysis of 8 pediatric patients who have undergone surgical intervention using PCL mesh implants for reconstruction of bony defects during craniosynostosis correction surgery. Radiological evaluation of 3 patients at random time points between 9 and 12 months postoperative revealed persistent bony gaps in areas where PCL mesh was laid. One patient who underwent a subsequent cranial vault surgery at 9 months was found to have less bone regeneration in the defect area where PCL mesh was used when compared with an adjacent area where a particulate bone graft was used. Based on our experience, the use of PCL mesh on its own did not augment bone regeneration. It is possible that a greater amount of time or increased vascularization of the scaffold is required, which supports the concept of regenerative matching axial vascularization or the further addition of osteogenic factors to increase the rate of bone formation.

Historical Perspectives on the Management of Craniosynostosis.

The history of craniofacial surgery is one of many fundamental advances by monumental figures. Although craniosynostosis has been known to exist for multiple centuries, modern management has evolved over roughly the last century. An overview of early history, early scientific exploration, the advancement of surgical treatment of craniofacial deformities and the current state of craniosynostosis management is discussed. To fully appreciate the evolution of craniosynostosis surgery, one must understand the gradual advancements that have brought the specialty to this modern era.

Update in Management of Craniosynostosis.

After studying this article, the participant should be able to: 1. Understand the craniofacial dysmorphology of craniosynostosis, and the variation of each type. 2. Identify the functional concerns and learn the rationale behind timing of operative intervention. 3. Approach each dysmorphology critically and identify the operative intervention needed to improve form and function 4. Understand and address the specific issues related to syndromic craniosynostosis and be able to delineate management plan. Craniosynostosis is a condition in which premature fusion of one or more cranial sutures lead to abnormal head shape and growth restriction of the brain. Nonsyndromic craniosynostosis occurs in isolation, and usually involves a single suture, whereas syndromic craniosynostosis may involve multiple sutures and is associated with extracraniofacial findings. Although surgical management can be similar, the treatment plan must take into consideration issues specific to the syndromes. This article aims to provide a concise overview of the authors' current understanding regarding the presentation, treatment principle, surgical option, and debates in craniosynostosis.

Establishing Standards of Care in Craniosynostosis: Results from a Survey of ERN CRANIO Member Institutions

European Reference Networks (ERNs) are networks involving hospitals with particular expertise in rare conditions. ERN-CRANIO focuses on rare disorders of the skull and face including craniosynostosis. We undertook a pilot study in the form of an electronic survey to understand current practice in craniosynostosis management across ERN-CRANIO, which at the time consisted of 29 member institutions across 11 countries. Most (19 of 29) units replied; however, some answered the survey only partially. The majority (87.5%) of units have specific management protocols. For single-suture cases, 8 of 15 units see >50 new cases per year, 4 of 15 see 21-50 cases, and 3 of 15 see fewer than 20 cases. Duration of follow-up ranges from age 10 years or less (3 of 6) to indefinite (2 of 6). A variety of surgical techniques are used. For sagittal synostosis, the endoscopic and helmet technique is the most common (5 of 14), and for metopic and unicoronal synostosis, preferred by 9 of 14 centers was the frontoorbital remodeling (bandeau) technique. For multisutural syndromic craniosynostosis, 2 of 16 centers see >20 new cases per year, 4 of 16 see 11-20 cases, 5 of 16 see 6-10 cases, and 4 of 16 see fewer than 5 cases. Most centers (12 of 15) either never discharge syndromic patients or follow until adulthood. Eleven of 14 units perform prophylactic vault expansion, while 3 of 14 wait for clinical indication. Nine of 13 units operate at 6-12 months. Again, a wide variety of techniques are used, most commonly frontal advancement (4 of 13) initially. This study provides a useful snapshot of current standards of care in craniosynostosis across the high-volume centers of the ERN. Going forward, these results can be used to direct more detailed analysis of current practice, which will then be useful for constructing a management guideline for patients presenting with both single-suture and multisutural craniosynostosis.

Assessment of paediatric head shape and management of craniosynostosis.

The presentation of a child with an abnormal head shape can be challenging and should be met with an appropriate clinical approach. Craniosynostosis is a common cause of paediatric skull deformity and is best managed by a multispecialty tertiary referral unit with regular follow-up. As craniosynostosis frequently requires time-sensitive surgery, it is important to differentiate between craniosynostosis and common self-limiting conditions such as deformational plagiocephaly. The aim of this article is to outline the clinical approach to paediatric skull deformity in the general practice setting, and to highlight the importance of early referral if there is clinical suspicion of craniosynostosis. Parental concern regarding infant head shape is common. General practitioners (GPs) have an important role in assessment, diagnosis and referral for paediatric skull deformities. GPs are well placed to clinically differentiate between deformational plagiocephaly and craniosynostosis and provide timely referrals to optimise patient outcomes.