Nonsyndromic Craniosynostosis Research Articles

High risk factors for craniosynostosis during pregnancy: A case-control study.

BackgroundCraniosynostosis is a birth defect involving premature cranial sutures’ fusion with an increasing prevalence and unknown underlying causes in nearly 80% of cases. The current study investigates a series of high-risk factors associated with a non-syndromic craniosynostosis.MethodsNinety-seven (97) children were included in the retrospective case-control study, 62 controls and 35 with craniosynostosis. A questionnaire with 143 questions was used in face-to-face interviews. After univariate analyses, stepwise multivariate logistic regression analysis was implemented.ResultsIn craniosynostosis group, 3 out of 4 were male subjects and 2 out of 3 born with caesarian section. History for central nervous system abnormalities in their younger siblings, low birth weight, extended use of mobile phone from the parents and medications’ use differed significantly between craniosynostosis and control group. After adjustment for all factors, only maternal medication use (aOR 6,1 [2.1 – 19], CI 95%) and oral progesterone intake (aOR 4 [1.2 – 14], CI 95%) were significantly associated with an increased risk in craniosynostosis group.ConclusionThe maternal medications’ use and particular oral progesterone intake is associated with an increased risk for non-syndromic craniosynostosis. However, due to the study’s limitations, further research is warranted.

Genetic Influence on Neurodevelopment in Nonsyndromic Craniosynostosis.

Nonsyndromic craniosynostosis is one of the most common anomalies treated by craniofacial surgeons. Despite optimal surgical management, nearly half of affected children have subtle neurocognitive deficits. Whereas timing and type of surgical intervention have been studied, the possibility of genetic influence on neurodevelopment in nonsyndromic craniosynostosis patients remains unexplored. The authors performed whole-exome sequencing for 404 case-parent trios with sporadic nonsyndromic craniosynostosis. Statistical analyses were performed to assess the burden of de novo mutations in cases compared to both expectation and 1789 healthy control trios. Individuals with and without each mutation class were analyzed, and the presence or absence of various types of neurodevelopmental delay were recorded alongside demographic information. The authors identified a highly significant burden of damaging de novo mutations in mutation-intolerant [probability of loss of function intolerance (pLI) >0.9] genes in nonsyndromic craniosynostosis probands (p = 5.9 × 10-6). Children with these mutations had a two-fold higher incidence of neurodevelopmental delay (p = 0.001) and a more than 20-fold greater incidence of intellectual disability (p = 7.2 × 10-7), and were 3.6-fold more likely to have delays that persisted past 5 years of age (p = 4.4 × 10-4) in comparison with children with nonsyndromic craniosynostosis without these mutations. Transmitted loss of function mutations in high-pLI genes also conferred a 1.9-fold greater risk of neurodevelopmental delay (p = 4.5 ×10-4). These findings implicate genetic lesions concurrently impacting neurodevelopment and cranial morphogenesis in the pathoetiology of nonsyndromic craniosynostosis and identify a strong genetic influence on neurodevelopmental outcomes in affected children. These findings may eventually prove useful in determining which children with nonsyndromic craniosynostosis are most likely to benefit from surgical intervention. Risk, III.

Polycystin-1 regulates cell proliferation and migration through AKT/mTORC2 pathway in a human craniosynostosis cell model.

Craniosynostosis is the premature fusion of skull sutures and has a severe pathological impact on childrens’ life. Mechanical forces are capable of triggering biological responses in bone cells and regulate osteoblastogenesis in cranial sutures, leading to premature closure. The mechanosensitive proteins polycystin‐1 (PC1) and polycystin‐2 (PC2) have been documented to play an important role in craniofacial proliferation and development. Herein, we investigated the contribution of PC1 to the pathogenesis of non‐syndromic craniosynostosis and the associated molecular mechanisms. Protein expression of PC1 and PC2 was detected in bone fragments derived from craniosynostosis patients via immunohistochemistry. To explore the modulatory role of PC1 in primary cranial suture cells, we further abrogated the function of PC1 extracellular mechanosensing domain using a specific anti‐PC1 IgPKD1 antibody. Effect of IgPKD1 treatment was evaluated with cell proliferation and migration assays. Activation of PI3K/AKT/mTOR pathway components was further detected via Western blot in primary cranial suture cells following IgPKD1 treatment. PC1 and PC2 are expressed in human tissues of craniosynostosis. PC1 functional inhibition resulted in elevated proliferation and migration of primary cranial suture cells. PC1 inhibition also induced activation of AKT, exhibiting elevated phospho (p)‐AKT (Ser473) levels, but not 4EBP1 or p70S6K activation. Our findings indicate that PC1 may act as a mechanosensing molecule in cranial sutures by modulating osteoblastic cell proliferation and migration through the PC1/AKT/mTORC2 cascade with a potential impact on the development of non‐syndromic craniosynostosis.

Application of Deep Learning Techniques for Automated Diagnosis of Non-Syndromic Craniosynostosis Using Skull.

Non-syndromic craniosynostosis (NSCS) is a disease, in which a single cranial bone suture is prematurely fused. The early intervention of the disease is associated with a favorable outcome at a later age, so appropriate screening of NSCS is essential for its clinical management. The present study aims to develop a classification and detection system of NSCS using skull X-ray images and a convolutional neural network (CNN) deep learning framework. A total of 56 NSCS cases (scaphocephaly [ n = 17], trigonocephaly [n = 28], anterior plagiocephaly [n = 8], and posterior plagiocephaly [n = 3]) and 25 healthy control infants were included in the study. All the cases underwent skull X-rays and computed tomography scan for diagnosis in our institution. The lateral views obtained from the patients were retrospectively examined using a CNN framework. Our CNN model classified the 4 NSCS types and control with high accuracy (100%). All the cases were correctly classified. The proposed CNN model may offer a safe and high-sensitivity screening of NSCS and facilitate early diagnosis of the disease and better neurocognitive outcome for patients.

"When the surgery was over, I felt like the worst part had passed": experiences of parents of children with craniosynostosis.

PurposeParents of children scheduled for surgery often experience emotional distress and anxiety. This study aimed to explore parents' experiences of hospital care after their child's craniosynostosis surgery and their perception of support during the year after discharge.Design and MethodsA purposive sample of 19 parents of 12 children with nonsyndromic craniosynostosis, who had undergone surgery, was recruited from one of two national centers in Sweden. An interview was conducted ~1 year after the child's surgery, from September 2017 to August 2018. The interviews followed a semistructured interview guide, were recorded, transcribed verbatim, and analyzed using inductive content analysis.ResultsThe analysis yielded six categories with subcategories as follows: (1) cared for and confident: the hospital staff was perceived as kind, professional, and reliable. (2) Alone and abandoned: sometimes, parents found it hard to initiate contact with professionals during hospitalization and after discharge. (3) The importance of information: thorough information was perceived as essential and the need for information varied during postsurgery period. (4) Feelings of worry: some parents remained worried about risks during recovery and were concerned about comorbidities and development. (5) Alright after all: parents felt that the worst part had been before surgery. (6) The need for support: parents were generally satisfied with the support offered and they often received support from family and friends, or other parents through social media/online forums.Practice ImplicationsHealthcare professionals must be responsive to what support parents need at different stages in the care process and be aware that parents sometimes hesitate to initiate contact and ask for help and support. Support from healthcare professionals to everyone in the follow‐up program, as a default, might be more accessible or acceptable for some parents. Providing online support from professionals should be considered and caregivers could also facilitate peer support among parents, either face‐to‐face or online.

Neurocognitive outcomes of children with non-syndromic single-suture craniosynostosis.

While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems that children with craniosynostosis face include cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems. Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis. Children with isolated single-suture subtypes of craniosynostosis such as sagittal, metopic, and unicoronal craniosynostosis can have distinct neurocognitive profiles. In this review, we discuss the unique neurodevelopmental profiles of children with single-suture subtypes of craniosynostosis.

Prevalence of Ocular Anomalies in Craniosynostosis: A Systematic Review and Meta-Analysis.

Background: The aim of this study was to describe the ophthalmic abnormalities and their prevalence in craniosynostosis prior to craniofacial surgery. Methods: A systematic search was conducted on Medline OVID, Embase, Cochrane, Google Scholar, Web of Science Core Collection. Inclusion criteria were English papers, children aged <18 years with non-syndromic and syndromic craniosynostosis, case reports, case series, and case-control studies. A system of domains was established consisting of an anatomic and functional ophthalmic domain. A meta-analysis of single proportions was carried out using random effects model and pooled mean proportions with 95% confidence intervals (CI) were calculated. Results: Thirty-two papers analyzing 2027 patients were included. Strabismus was the most common anomaly in non-syndromic craniosynostosis: Horizontal strabismus was highest prevalent in unicoronal craniosynostosis (UCS) 19% (95% CI 9–32), followed by vertical strabismus 17% (95% CI 5–33). In syndromic craniosynostosis, horizontal strabismus was most prevalent in Crouzon syndrome 52% (95 CI 26–76), followed by Apert syndrome 50% (95% CI 42–58). Vertical strabismus was most prevalent in Saethre-Chotzen 60% followed by Muenke’s syndrome 36%. Furthermore, astigmatism was the second most reported outcome in non-syndromic craniosynostosis and highest prevalent in UCS 35% (95% CI 21–51). In syndromic craniosynostosis, astigmatism was most frequently seen in Crouzon syndrome 43% (95% CI 22–65), followed by Apert syndrome 34% (95% CI 14–58). Moreover, in syndromic craniosynostosis, 5–40% had a decrease in visual acuity (VA) ≤ 0.3 LogMAR in the better eye and 11–65% had a VA ≤ 0.3 LogMAR in at least one eye. Discussion: This review demonstrates the high prevalence of ocular anomalies in non-syndromic and syndromic craniosynostosis. A multidisciplinary and systematic approach is needed for the screening and optimal treatment of these conditions in a timely manner.

An Investigation of the Relationship Between the Second-to-Fourth Digit Ratio and Sagittal Synostosis.

The most common presentation of nonsyndromic craniosynostosis is that of the sagittal suture. Amongst this subgroup there is a significant male preponderance. Although the etiology is largely unknown, androgen exposure in utero has been suggested as a contributing factor. The second-to-fourth digit ratio (2D:4D) is a sexually dimorphic trait, reflective of the levels of androgen and estrogen exposure in utero, with a lower 2D:4D ratio associated with higher androgen exposure.This study aimed to examine the difference in 2D:4D ratio between participants with sagittal synostosis (SS) and gender- matched controls, hypothesizing that alterations in androgen exposure would be reflected in participants' 2D:4D ratio. Participants with nonsyndromic SS and gender-matched controls were prospectively recruited from outpatients clinics. Photographs were taken of the right hand, and 3 independent researchers measured the length of the fingers and 2D:4D ratio, with the mean 2D:4D ratio then calculated. Fifty-six participants were recruited to both groups, with 35 males and 21 females in each. The mean age of the study and control groups were 5.6 and 6.3 years, respectively. There was no difference in the 2D:4D ratio between groups overall ( P = 0.126). However, males with SS had a significantly higher 2D:4D ratio in comparison to male controls (0.969 ± 0.379 versus 0.950 ± 0.354, P = 0.038). Our results suggest that 1 single hormonal pathway is not responsible for suture fusion. Subsequently we consider that an imbalance between testosterone and estrogen signaling may contribute to the development of sagittal craniosynostosis.

Parental Satisfaction from Telemedicine in the Follow-up of Children Operated for Craniosynostosis during COVID-19 Pandemic.

The current study aimed to evaluate the satisfaction level of parents from telemedicine use in the long-term follow-up of children operated for craniosynostosis during the COVID-19 pandemic. A cross-sectional 9-question survey analysis using Qualtrics survey software. An institutional study carried out at a national, tertiary level academic center in the Slovak Republic. All patients operated for non-syndromic and syndromic craniosynostosis in our department, who participated in the virtual postoperative follow-up consultations from March 23, 2020, through July 2, 2021, were included in our series. They were enrolled 498 patients, with the parents of 256 children being responding to the survey. The survey remained open until July 16, 2021, two weeks after the last virtual consultations. It was delivered to the parents by e-mail to generate closed 5-point Likert scale responses. Overall, 72.3% of parents were satisfied with the telemedicine program, and 67.2% found it convenient. Collectively, 89.1% reported that the received instructions were helpful to them. However, only 18.7% of them answered that they would prefer telemedicine consultations in the future. The results of this study showed a high satisfaction from the parents during the virtual follow-up of children operated for craniosynostosis. However, despite their positive experience with telemedicine, the vast majority of parents stated that they would prefer in-person visits in the future.

Incidence of Non-Syndromic and Syndromic Craniosynostosis in Sweden.

Premature craniosynostosis is a rare condition, with a wide range of incidence estimations in the literature. The aim of this study was to establish the current incidence among the Swedish population. Since the surgical care for these children is centralized to the 2 centers of Sahlgrenska University Hospital and Uppsala University Hospital, the 2 craniofacial hospital registries were examined for surgically treated children, all having a computed tomography verified diagnosis. Results show an incidence of 7.7 cases per 10,000 live births, including 0.60/10,000 syndromic craniosynostosis. Due to information programs among health care staff and a system for early diagnosis through rapid communication, these results seem to mirror the true incidence of craniosynostosis in the Swedish population. The updated incidence data will facilitate healthcare planning and make future studies of possible changes in craniosynostosis incidence more accurate.

Amount of reoperation following surgical repair of nonsyndromic craniosynostosis at a single center.

Craniosynostosis is a deformity of the skull that occurs as a result of early fusion of one or more cranial sutures and can be accompanied by neurological deficits. Craniosynostosis can be classified as syndromic or nonsyndromic according to the type of suture involved. Surgical treatment of craniosynostosis in infants basically involves loosening and opening the fused sutures to reduce intracranial pressure, allow the brain to grow, and also fix the skull shape. However, in such cases there is a risk of resynostosis after surgery. According to the literature, resynostosis rates vary between 0% and 70%. In this study, we aimed to evaluate the reoperation rate in craniosynostosis cases treated surgically in our clinic. A retrospective analysis of 70 nonsyndromic craniosynostosis cases treated surgically in the Neurosurgery Department of Bursa Uludağ University from 2005 to 2019 was performed. All patients had undergone total cranial vault remodeling surgically and had been followed up for at least a year. The study group included 70 patients, comprising 40 (57.1%) male and 30 (42.9%) female patients. The mean age of the group was 10.9 ± 7.8 months (range 3-34 months). Out of 70 patients, repeat surgery due to resynostosis had been performed once in 5 (7.1%) patients and twice in 1 (1.4%) patient. It should be kept in mind that resynostosis may occur in patients who have been operated for craniosynostosis. Patients should be examined cosmetically and if necessary, radiologically in the follow-up. Further studies based on larger sample size are recommended for more quantitative data and better results.

Psychological and Physical Health Outcomes in Adults With Craniosynostosis.

Within current research, little is known about the long-term outcomes of craniosynostosis. A priority-setting exercise by UK charity Headlines Craniofacial Support identified 2 key questions in this area: (1) What are the long-term physical and psychological effects for individuals with syndromic and non-syndromic craniosynostosis? and (2) Are individuals with craniosynostosis likely to suffer from mental health difficulties, or are they more resilient? The aim of the current study was to conduct an initial investigation of these priority questions. A comprehensive UK-wide survey consisting of 9 standardized psychological outcome measures and open-ended questions was distributed online. Thirty-six eligible adults (69.4% female) with a mean age of 30.8 years responded to the survey. Participants reported having single suture craniosynostosis (27.8%) or syndromic craniosynostosis (52.8%), with 19.4% being unsure of their diagnosis. Sample means were compared to published norms using independent samples t tests. Qualitative responses were analysed using inductive content analysis. Compared to the general population, participants reported significantly less favorable scores related to appearance concerns, attachment in adult relationships, anxiety, optimism, and resilience. Self-worth, depression, and social anxiety scores were similar to norms. Qualitative responses provided additional insight into participants' satisfaction with appearance, physical health, medical treatment, employment, relationships, and recurrence risks. Few participants had accessed psychological support. This preliminary study illustrates the potential long-term implications for individuals with craniosynostosis. Improved treatment protocols are needed to address physical health concerns in adulthood, while dedicated psychological resources are necessary to promote emotional well-being, social confidence, and resilience.

Anterior fontanelle closure and diagnosis of non-syndromic craniosynostosis: a comparative study using computed tomography

ObjectiveSuspicion of early anterior fontanel (AF) closure is a common reason for referral to a pediatric neurosurgeon because of the suspected increased risk of developing craniosynostosis (CS) in spite of the absence of evidence in the literature. The aim of this study was to analyze the association between AF closure and the diagnosis of non-syndromic CS in Brazilian children. MethodsAn observational and case-cohort study was conducted to compare the incidence of closed AF between healthy children (group 1) and children diagnosed with non-syndromic CS (group 2) at a pediatric neurosurgery referral center. The accuracies of completely closed AF and diagnosis of CS were assessed. ResultsHigh-resolution three-dimensional reconstruction computed tomography scans were obtained for 140 children aged < 13 months, of whom 62.9% were boys and 37.1% were girls (p < 0.001). The most common types of non-syndromic CS were trigonocephaly (34, 48%) and scaphocephaly (25, 35.7%). Closed fontanel (27, 38.6%) was observed in both groups, and a sensitivity of 36.1%, specificity of 72%, the positive predictive value of 59%, and negative predictive value of 51% were observed in the patients diagnosed with CS when AF closure occurred before the age of 6 months. ConclusionThe results of this comparative study of AF closure and CS diagnosis suggest that early AF closure does not imply a diagnosis of CS. Pediatricians should be aware of the risk of misdiagnosis of CS in cases with a widely open AF in spite of the presence of CS.

Craniosynostosis Surgery for Increased Intracranial Pressure.

Craniosynostosis is the premature fusion of 1 or more cranial sutures. The presentation may vary, and there are differing opinions regarding surgical indications and timing. Though increased intracranial pressure (ICP) is a well-established risk of craniosynostosis, its response to treatment is not uniform. This study aims to identify the signs and symptoms indicative of increased ICP that are most likely to improve after craniosynostosis surgery. Pre- and post-operative data were retrospectively collected from patients at our institution with syndromic and non-syndromic craniosynostosis from January 2009 to June 2020. Demographics, symptoms (headache, nausea, emesis, and lethargy), signs (visual disturbances and papilledema), and imaging characteristics (copper beaten changes), if available, were analyzed. One hundred fifty-three children with craniosynostosis were identified, and 56 with preoperative symptoms met inclusion criteria. Older age was significantly correlated with the number of symptoms improved postoperatively ( P = 0.015). Papilledema, headache, nausea, and irritability (if present preoperatively) were the features most likely to improve after craniosynostosis repair. Optic nerve or disc anomalies, feeding difficulties, seizures, and need for glasses were least likely to improve. Older children undergoing craniosynostosis surgery had a greater number of improved symptoms. Classical features of increased ICP were more likely to improve than developmental difficulties and multifactorial causes. These findings may be useful when considering craniosynostosis repair in a symptomatic child.

Progressive Myelinated Retinal Nerve Fibers in Children With Craniosynostosis.

The authors found the changes of myelinated retinal nerve fibers in one pair of identical twins with nonsyndromic craniosynostosis and two patients with Crouzon syndrome, who were at risk for impaired lamina cribrosa barrier function. This is the first report of progressive and presumed acquired myelinated retinal nerve fibers in craniosynostosis. [J Pediatr Ophthalmol Strabismus. 2021;58(6):e40-e43.].

Nonsyndromic craniosynostosis in Vietnam: initial surgical outcomes of subspecialty mentorship.

There is a global deficit of pediatric neurosurgical care, and the epidemiology and overall surgical care for craniosynostosis is not well characterized at the global level. This study serves to highlight the details and early surgical results of a neurosurgical educational partnership and subsequent local scale-up in craniosynostosis correction. A prospective case series was performed with inclusion of all patients undergoing correction of craniosynostosis by extensive cranial vault remodeling at Children's Hospital 2, Ho Chi Minh City, Vietnam, between January 1, 2015, and December 31, 2019. A total of 76 patients were included in the study. The group was predominantly male, with a male-to-female ratio of 3.3:1. Sagittal synostosis was the most common diagnosis (50%, 38/76), followed by unilateral coronal (11.8%, 9/76), bicoronal (11.8%, 9/76), and metopic (7.9%, 6/76). The most common corrective technique was anterior cranial vault remodeling (30/76, 39.4%) followed by frontoorbital advancement (34.2%, 26/76). The overall mean operative time was 205.8 ± 38.6 minutes, and the estimated blood loss was 176 ± 89.4 mL. Eleven procedures were complicated by intraoperative durotomy (14.5%, 11/76) without any damage of dural venous sinuses or brain tissue. Postoperatively, 4 procedures were complicated by wound infection (5.3%, 4/76), all of which required operative wound debridement. There were no neurological complications or postoperative deaths. One patient required repeat reconstruction due to delayed intracranial hypertension. There was no loss to follow-up. All patients were followed at outpatient clinic, and the mean follow-up period was 32.3 ± 18.8 months postoperatively. Surgical care for pediatric craniosynostosis can be taught and sustained in the setting of collegial educational partnerships with early capability for high surgical volume and safe outcomes. In the setting of the significant deficit in worldwide pediatric neurosurgical care, this study provides an example of the feasibility of such relationships in addressing this unmet need.

Long-term Outcomes of Non-syndromic and Syndromic Craniosynostosis: Analysis of Demographic, Morphologic, and Surgical Factors.

In this study, we analyzed the outcomes of patients (followed for 5–38 years, average 17.3 years) with craniosynostosis and evaluated their long-term prognosis. In all, 51 patients who underwent surgery for craniosynostosis between 1982 and 2015, including 12 syndromic and 39 non-syndromic cases, were included. The average age at the initial surgery was significantly lower in the syndromic group than that in the non-syndromic group (9.8 months old vs. 19.9 months, respectively). The surgical procedures did not significantly differ between the two groups, but repeat surgery was significantly more common in the syndromic group than in the non-syndromic group (4 children [30.8%] and 3 children [7.7%], respectively). The children requiring repeat surgery tended to be younger at the initial surgery than those who did not. Those patients who required repeat surgery did not have significantly different surgical procedures initially. The incidence of developmental retardation was 49.0% (43.5% in the non-syndromic group and 66.7% in the syndromic group), and only two children in the non-syndromic group displayed recovery. This study is the first to analyze the prognosis for patients who were followed for at least 5 years after cranioplasty. Repeat surgery was common, especially in syndromic patients. Severity of skull deformity and early initial surgery may be important factors determining the need for repeat surgery. Developmental retardation was also common, and improvement was rare even after surgery.

Genomic Findings in Bone Blood Paired DNA Comparison of Nonsyndromic Craniosynostosis

Genomic Findings in Bone Blood Paired DNA Comparison of Nonsyndromic Craniosynostosis

Craniometrics and Perioperative Variants in Non-Syndromic Single Suture Craniosynostosis with Cranial Vault Remodelling Surgery and Strip Craniotomy with and without Endoscopic Assistance: Systematic Review

Abstract Background Craniosynostosis is a major category in craniofacial anomalies defined as premature closure of one or more of cranial sutures. Diagnostic Indices and different measures and landmark points are being chosen according to age, the type of deformity and its severity, surgical procedure and targeted cosmetic outcome. Objectives Primary objective is to compare anthropometric measures Post-operative in short and long term follow up between different surgical modalities and impact on cosmetic outcome and need of second corrective surgical session. Secondary objective is to evaluate the Perioperative events between three surgical modalities in terms of Intra operative events; Estimated Blood Loss (EBL), rate of blood transfusion, Procedure duration and Post-operative events; Hospital Length of Stay, rate of post-operative complications and duration of needed Helmet therapy. Methods and Material The following electronic databases were searched from 2015 to 2019: PubMed, google scholar search engine. Cochrane database of systematic reviews, EMBASE for comparative studies between minimal invasive and open cranial vault remodelling techniques with different types of synostosis. Studies that were eligible if they contain the target keywords in title or abstract, addressing the age group up to 36 months with diagnosis of non- syndromic craniosynostosis by a plastic surgeon or neurosurgeon with or without confirmatory 3D skull reconstruction CT imaging. Follow-up outcomes were measured at 12 months or more. Exclusion criteria included studies lacking of quantitative comparison between open surgery and endoscopic assisted surgery, inclusion of patients with syndromic Craniosynostosis and editorials, abstracts and case reports. Results A total of 385 studies screened for eligibility, seven retrospective cohort studies were included in our systematic review for analysis of population demographics and management outcome with commenting of cosmetic outcome significance in comparison of different surgical modalities. Overall study population reaches 440 patients with different synostosis deformities with average age at surgery ranging from 2 to 6 months for endoscopic groups and 5 to 14 months for open surgery groups with average follow up duration reached 12 months. Analysis showed comparable postoperative cosmetic results between both techniques regardless type of synostosis with better perioperative outcomes such as less blood loss, shorter operations, shorter hospital stays and lower incidence of complications in minimal invasive and endoscopic assisted procedures groups. Conclusions We conclude that Minimal invasive approaches and especially endoscopic assisted craniotomies is a promising surgical option in craniosynostosis management. Regardless type of synostosis deformity, current literature comparing endoscopic and open CVR repair showed no statistical significant difference in craniometric analysis of cosmetic post-operative outcomes of both techniques. With improved perioperative outcomes, endoscopic assisted surgeries could be preferred for management team ideally for cases before 6 months age. Large population prospective studies and clinical trials are recommended for more high level evidence data for studying craniosynostosis management options for proper surgical decision making.

Utility of Preoperative Helmet Molding Therapy in Patients With Isolated Sagittal Craniosynostosis.

The purpose of this study was to review our institution's experience using helmet molding therapy in children with isolated non-syndromic sagittal craniosynostosis before placement of cranial springs and provide objective measurements of craniometric changes to help determine its role in treatment.Patients who underwent preoperative helmet molding therapy for sagittal craniosynostosis were retrospectively reviewed. Three-dimensional surface tomography scans were used to measure head circumference, cranial width, cranial length, cranial index (CI), and cranial vault asymmetry.Seventeen patients underwent orthotic helmeting therapy before spring mediated cranial vault expansion. Patients spent a median of 48 days (interquartile range [IQR] 32, 57) in preoperative orthotic helmeting therapy. There were increases in both cranial width and length post-helmeting (median: 107.5 mm [IQR 104.8, 110.4] versus 115.6 mm [IQR 114.5, 119.3]; P < 0.001) (median: 152.8 mm [IQR 149.2, 154.9] versus 156.8 mm [IQR 155.0, 161.5]; P < 0.001), respectively. There was a greater increase in cranial width (P = 0.015). Consequently, patients' CI improved after preoperative helmeting (median: 0.702 [IQR 0.693, 0.717] versus 0.739 [0.711, 0.752]; P < 0.001). There was no evidence of growth restriction from helmeting (pre-helmeting Head circumference [HC]: median 96.8 percentile [IQR 90.6, 99.9] versus post-helmeting HC: 98.7 percentile [IQR 94.7, 99.8]; P = 0.109).Preoperative helmeting in patients with non-syndromic isolated sagittal craniosynostosis can be used to improve CI before surgical correction. Significant benefits can be achieved in shorter preoperative helmeting durations than previously reported with no evidence of cranial growth restriction, which supports its feasibility and utility in children undergoing spring mediated cranial vault expansion.