Cranial Vault Surgery Research Articles

Effective Pediatric Blood Management in Craniosynostosis Surgery: A Long-Term Update.

Blood transfusion is common in cranial vault surgery, and protocolized efforts to minimize transfusion have been reported in recent years. This study assesses the long term, prospective outcomes of a pediatric blood management protocol for the cranial vault reconstruction (CVR) population. Data from a retrospective control cohort and a prospective cohort employing a protocol for preoperative hematologic optimization of patients undergoing CVR from January 2015 to October 2023 was reviewed. Preoperative hemoglobin (Hgb) determined the preoperative protocol. Intraoperative tranexamic acid (TXA) and/or aminocaproic acid, cell-saver technology, and postoperative iron or erythropoietin alfa supplementation were also used in the protocol. For statistical analysis, P<0.05 was deemed significant. The cohort consisted of 194 successive patients (20 control and 174 treatment). Age, sex, and weight were not significantly different between groups. Mean postoperative Hgb was significantly higher in the control group (P<0.01). No difference was observed in Hgb at discharge between control and treatment groups. Mean estimated blood loss, volume of intraoperative packed red blood cells, rate of packed red blood cell transfusion, and mean total transfusion volume during hospitalization were significantly higher in the control group compared with the treatment group (P<0.01). Mean length of stay did not differ between groups. Efforts to optimize blood management in the CVR population are critical. This prospective study represents a robust and reproducible protocol for pediatric blood management with significant reductions in transfusion requirements.

Changes in Ventricular Volume After Posterior Vault Distraction Osteogenesis in Patients With Syndromic and Nonsyndromic Craniosynostosis.

Little is known about the response of the ventricular system to cranial vault surgery in patients with craniosynostosis. This study aims to evaluate the changes in the cerebral ventricular system in response to posterior vault distraction osteogenesis (PVDO) in patients with syndromic and nonsyndromic craniosynostosis. A single-institution retrospective review of all patients with craniosynostosis undergoing PVDO from 2000 to 2022 was completed. Patients were included for analysis if they had pre and postoperative cranial computed tomography scans. Ventricular volume (VV) and intracranial volume (ICV) were calculated using segmentation software. Both patients with syndromic synostosis and nonsyndromic synostosis (NSS) experienced a significant increase in ICV after PVDO, but only patients with NSS experienced a significant VV change ( P = 0.004). After normalization by ICV, total, lateral, and third VV changes retained significance with percentage increases of 114%, 117%, and 89%, respectively ( P < 0.05 for all). The differing results between cohorts reinforce the concept that the intracranial milieu is different between patients with syndromic synostosis and NSS. The results of the NSS cohort suggest that these patients may exist in a compensated state in which a reduction in cerebral blood flow and VV allows for the maintenance of parenchymal health to prevent the development of intracranial hypertension. Further studies may explore VV as a surrogate marker of ICP elevation, and the utility of cranial vault remodeling on nonsynostotic pathologies with cephalocranial disproportion.

Subtemporal Decompression in Resistant Slit Ventricle Syndrome in Children: An Observational Study and Survival Analysis

Introduction: Slit ventricle syndrome (SVS) remains a challenging problem in the early-shunted paediatric population. Various surgical and non-surgical treatments have been devised for this condition. However, there is currently no gold standard for its optimal management. Among various treatment modalities, subtemporal decompression (STD) is often performed as a last resort. We present our experience of STD in paediatric patients with SVS in whom initial treatment with programmable valves and anti-syphon device were not successful. Methods: This is a single-centre retrospective observational study and survival analysis. Patients who underwent STD for SVS were included. Pre- and post-operative imaging data and clinical outcomes were collected. Results: There were 20 patients (12 M, 8 F) with a mean age of 9 years (SD: 4) at first STD. 90% (n = 18) of patients had multiple shunt revisions pre-STD. At first STD, 70% (n = 14) and 30% (n = 6) of patients had unilateral or bilateral STD, respectively. STD led to a reduction in the frequency of shunt revisions in 60% (n = 12) of patients. The median time required before further STD, shunt surgery, or cranial vault surgery was 14 months. The median time before a further STD was required (either revision or contralateral side) was 89 months. At a median follow-up of 66.5 months (range: 1–159), 65% (n = 13) of patients had improvement in symptoms. Conclusions: A large proportion of patients with persistent SVS symptoms, refractory to multiple shunt revisions, benefitted from STD in combination with shunt optimization. It was also safe and well-tolerated. Therefore, in patients who have multiple failed shunts, STD may reduce the morbidity associated with further shunt revisions and can significantly improve symptomatology.

Re-imagining early cloverleaf skull deformity management from front to back approach-30years on.

The cloverleaf skull deformity remains among the most complicated craniofacial conditions to successfully manage. Many cases achieve largely unsatisfactory outcomes due to the requirement for frequent reoperation on the cranial vault and failure to deal with all the elements of the craniofaciostenosis in a timely fashion. Early cranial vault surgery without addressing the cranial base deformity and its attendant cerebrospinal fluid flow changes is invariably challenging and disappointing. A recent focus on the expansion of the posterior cranial vault as a primary procedure with the greater volume change allows a delay in fronto-orbital advancement and reduced need for repeat surgery. We herein describe three cases of complex multisuture craniosynostosis with cloverleaf skull deformity who underwent neonatal posterior cranial vault decompression along with foramen magnum decompression. Our report examines the safety and rationale for this pre-emptive surgical approach to simultaneously deal with the cranial vault and craniocervical junction abnormalities and thus change the early trajectory of these complex cases.

Investigation of the Effffect of Cranial Vault Surgery On Parenchymal Perfusion Before and After Surgery in Craniosynostosis Patients

Objective: In our study, it is aimed to examine the effect of surgery on brain perfusion with ASL sequence perfusion MRI taken before surgery and 12 weeks after surgery in cases of non-syndromic craniosynostosis treated in our clinic. Materials and Methods: The study was conducted between 28.12.2021-16.09.2022 with 10 patients who evaluated preoperatively and treated surgically at the Neurosurgery Clinic of Ankara City Hospital. Perfusion MRI was performed preoperatively and 12 weeks postoperatively. The preoperative and postoperative images obtained were evaluated comparatively at the workstation. Results: After the exclusion of patients who did not appropriate the study criteria, the results of 9 patients were examined. MRI scans taken preoperatively and 87 days as average postoperatively. After surgery, there is perfusion enhancement in 7 patients and a perfusion impairment in 2 patients. About the average perfusion rates, the results were found to be statistically significant in four of the seven patients in whom an increase was detected (p≤0.05), while the results were found to be statistically significant in one of the patients in whom a decrease was observed. (p≤0.05) Conclusion: Surgical treatment is effective on brain perfusion in patients with non-syndromic craniosynostosis. Perfusion alteration may be different according to surgical technique, patient age at the time of surgery and type of craniosynostosis. In addition to cosmetic improvement, surgical treatment can also be effective in changing neurological functions. ASL sequence perfusion MRI, which doesn’t require contrast and is non-invasive, is a successful method of demonstrating the quantitative effectiveness of surgery in patients with craniosynostosis.

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.

Epidemiology, Genetics, and Pathophysiology of Craniosynostosis.

Craniosynostosis, the premature fusion of the infant cranial skulls, can be recognized by characteristic head shape differences that worsen with head growth. Craniosynostosis can be syndromic or nonsyndromic and can involve one suture or multiple sutures. Timely cranial vault surgery is recommended to expand and reshape the skull, with a goal of preventing increased intracranial pressure and providing sufficient space for brain growth. Several gene variants and environmental exposures are known to increase the risk of single suture craniosynostosis (SSC), including in utero constraint, exposure to specific toxins and medications, and medical conditions such as thyroid dysregulation and metabolic bone disorders.

Preoperative imaging patterns and intracranial findings in single-suture craniosynostosis: a study from the Synostosis Research Group.

The diagnosis of single-suture craniosynostosis can be made by physical examination, but the use of confirmatory imaging is common practice. The authors sought to investigate preoperative imaging use and to describe intracranial findings in children with single-suture synostosis from a large, prospective multicenter cohort. In this study from the Synostosis Research Group, the study population included children with clinically diagnosed single-suture synostosis between March 1, 2017, and October 31, 2020, at 5 institutions. The primary analysis correlated the clinical diagnosis and imaging diagnosis; secondary outcomes included intracranial findings by pathological suture type. A total of 403 children (67% male) were identified with single-suture synostosis. Sagittal (n = 267), metopic (n = 77), coronal (n = 52), and lambdoid (n = 7) synostoses were reported; the most common presentation was abnormal head shape (97%), followed by a palpable or visible ridge (37%). Preoperative cranial imaging was performed in 90% of children; findings on 97% of these imaging studies matched the initial clinical diagnosis. Thirty-one additional fused sutures were identified in 18 children (5%) that differed from the clinical diagnosis. The most commonly used imaging modality by far was CT (n = 360), followed by radiography (n = 9) and MRI (n = 7). Most preoperative imaging was ordered as part of a protocolized pathway (67%); some images were obtained as a result of a nondiagnostic clinical examination (5.2%). Of the 360 patients who had CT imaging, 150 underwent total cranial vault surgery and 210 underwent strip craniectomy. The imaging findings influenced the surgical treatment 0.95% of the time. Among the 24% of children with additional (nonsynostosis) abnormal findings on CT, only 3.5% required further monitoring. The authors found that a clinical diagnosis of single-suture craniosynostosis and the findings on CT were the same with rare exceptions. CT imaging very rarely altered the surgical treatment of children with single-suture synostosis.

Evaluating the Effects of Enhanced Recovery Pathways in Craniosynostosis: National Trends in Hospitalization Charges and Length of Stay in Craniosynostosis Surgery.

Enhanced Recovery After Surgery (ERAS) pathways are multimodal approaches aimed at minimizing postoperative surgical stress, reducing hospitalization time, and lowering hospitalization charges. Enhanced Recovery After Surgery is broadly and increasingly implemented in hospitals across the country. Early reports have shown ERAS to reduce length of stay (LOS) after commonly performed pediatric surgeries. However, LOS and hospital charges after craniosynostosis have not been studied. We hypothesized that extended hospital LOS is correlated with increased hospitalization charges associated with open cranial vault surgery (CVS) and that over a multiyear timeframe, LOS and cost would decrease because of the increased adoption of ERAS in pediatric surgery. The Healthcare Cost and Utilization Project's National Inpatient Sample database was analyzed from January 2007 to December 2014. All patients who were diagnosed with craniosynostosis who underwent CVS were included. Variables of interest included demographic data, hospital characteristics, hospitalization data, and total hospital charges. Univariate and generalized linear regression models were used to examine associations between selected variables and the hospitalization charges. There were 54,583 patients diagnosed with craniosynostosis between 2007 and 2014. Of these patients, 22,916 (41.9%) received CVS. The median total hospital charge was $66,605.77 (interquartile range, $44,095.60-$101,071.17). The median LOS was 3 days (interquartile range, 2-4 days), and there was no significant change in LOS by year (P = 0.979). However, despite a stable LOS, mean hospitalization charge increased significantly by year (P < 0.01). Regression analysis demonstrated the proportion of eligible patients who underwent CVS substantially increased over the selected timeframe (P < 0.01). Most procedures were performed in urban teaching hospitals and high-volume hospitals. There was no significant association between hospital volume and hospitalization charge (P = 0.331). Increasing hospital charges despite constant LOS for craniosynostosis CVS procedures was observed between 2007 and 2014. Although ERAS has reduced LOS for common pediatric surgical procedures, no decrease in LOS for CVS has been observed. The charges significantly increased over the same period including high-volume centers. Further study to safely lower LOS and hospitalization charges for this procedure may reduce the overall health care burden.

The Pediatric Craniofacial Collaborative Group (PCCG) Consensus Conference Methodology.

This article describes the methodology used for the Pediatric Craniofacial Collaborative Group (PCCG) Consensus Conference. This is a novel Consensus Conference of national experts in Pediatric Craniofacial Surgery and Anesthesia, who will follow standards set by the Institute of Medicine and using the Research and Development/University of California, Los Angeles appropriateness method, modeled after the Pediatric Critical Care Transfusion and Anemia Expertise Initiative. Topics related to pediatric craniofacial anesthesia for open cranial vault surgery were divided into twelve subgroups with a systematic review of the literature. A group of 20 content experts met virtually between 2019 and 2020 and will collaborate in their selected topics related to perioperative management for pediatric open cranial vault surgery for craniosynostosis. These groups will also identify where future research is needed. Experts in pediatric craniofacial surgery and anesthesiology are developing recommendations on behalf of the Pediatric Craniofacial Collaborative Group for perioperative management of patients undergoing open cranial vault surgery for craniosynostosis and identifying future research priorities.

Cranial Vault Remodeling in Children With Ventricular Shunts.

Cranial vault surgery in children with ventricular shunts is more complex due to the possible interference of shunt location with surgical planning and increased risk of shunt related complications. The study evaluated the management of ventricular shunts during cranial vault remodeling (CVR) and subsequent outcomes and complications following CVR. An IRB-approved retrospective chart review was performed including patients who underwent CVR in presence of a ventricular shunt. Measured outcomes were number of shunt revisions following CVR, 30-day complication rate, 3-month complication rate, and post-CVR Whitaker classification. Finally, a review of the literature was performed, and our results were compared to previous reports. Eleven patients met the inclusion criteria. Ventricular shunt was not exposed in 18%; exposed and not revised in 54%; exposed and left on a bone peninsula in 18% and simultaneously exposed and revised in 18%. Two patients experienced complications within 30-days. Three additional patients experienced complications within 3-months. An average of 2.2 shunt revisions per patient were performed following CVR. Whitaker classification was I in 7 patients, II in 1 patient and III in 2 patients. Shunt revision rate for patients undergoing CVR for hydrocephalic macrocephaly was on average of 1.2 and 1.9 for shunt-induced craniosynostosis in literature. Cranial vault abnormalities in the presence of a ventricular shunt can be effectively treated with CVR. The presence of a shunt does not seem to interfere with final head shape. While leaving the shunt unexposed may appear less morbid, shunt related complications can still occur with this approach.

Routine Postoperative Admission to the Intensive Care Unit Following Repair of Nonsyndromic Craniosynostosis: Is it Necessary?

Cranial vault surgery for craniosynostosis is generally managed postoperatively in the intensive care unit (ICU). The purpose of the present study was to examine our center's experience with the postoperative management of otherwise healthy patients with nonsyndromic craniosynostosis (NSC) without routine ICU admission. A retrospective cohort study of patients with NSC operated using a variety of vault reshaping techniques in our pediatric center between 2009 and 2017 was carried out. Patients with documented preexisting comorbidities that would have required admission to the ICU regardless of the surgical intervention were excluded. A total of 102 patients were included in the study. Postoperatively, 100 patients (98%) were admitted as planned to a general surgical ward following observation in the recovery room. Two patients (2%) required ICU admission due to adverse intraoperative events. There were no patients who required transfer to the ICU from the recovery area or surgical ward. Within the surgical ward cohort, 6 patients (6%) had minor postoperative complications that were readily managed on the surgical floor. Postoperative anemia requiring transfusion was the most common complication. The results from this study suggest that otherwise healthy patients with NSC undergoing cranial vault surgery can potentially be safely managed without routine admission to the ICU postoperatively. Key elements are proper preoperative screening, access to ICU should an adverse intraoperative event occur and necessary postoperative surgical care. The authors hope that this experience will encourage other craniofacial surgeons to reconsider the dogma of routine ICU admission for this patient population.

S2-14 SESSION 2: POSTERIOR VAULT DISTRACTION QUANTITATIVE OUTCOMES IN POSTERIOR AND TRANSVERSE CRANIAL VAULT DISTRACTION FOR CRANIOSYNOSTOSIS

Introduction: CVD osteogenesis has been shown to establish greater cranial volume overall than conventional remodeling. Quantitative measurement with systematic evaluation of outcomes is vital for the comparison of surgical outcomes across international institutions. Computed tomography (CT) or magnetic resonance imaging (MRI) in addition to image analysis software has been increasingly used in recent times to predict ICV changes after cranial vault surgery. The primary study aim was to quantify ICV increase in CVD and secondarily to gauge acceptability of using a simple modality to measure ICV. Methods: A retrospective review of a prospectively maintained database of posterior and transverse cranial vault distraction procedures at the National Paediatric Craniofacial Centre in Temple Street Children’s University Hospital from May 2010 to September 2016 was performed. ICV was calculated using Osirix® Version 3.3 from post-operative CT-scans. Cephalometric analysis was also performed using lateral and anteroposterior radiographic views of the cranium. Data was analyzed with GraphPad Prism®. Results: 21 patients (9 PCVD and 13 TransCVD), underwent successful cranial vault distraction. Patients in the PCVD group achieved a mean ICV increase of 2947mm, 32.5% (range: 12.8% to 80.2%, p<0.0001).95% CI 2135 to 3758mm3. In the TransCVD group, an average ICV increase of 1912.96,15.8% (range: 4.4% to 24.2%, p<0.0001) was achieved. 95% CI 1507 to 2320 mm3. Cephalic index values moved towards normal range, average change of 10.1% (range: 2.04% to 19.83%, p<0.0552) in the PVD group and 6.8% in the TransCVD (range: 2.7% to 14.8%, p<0.0001) respectively. Conclusion: This institutional review of outcomes post cranial vault distraction for syndromic and non-syndromic synostosis contributes to the existing literature and confirms the satisfactory reliable results afforded by distraction surgery along with a favorable morbidity profile. Quantitative analysis of cranial vault expansion by distraction has shown a reproducible increase in total ICV and is an effective method for rapid expansion of ICV with normalization of head morphology. We found Osirix® used with CT scans an acceptable modality that does not compromise accuracy and confer excellent interactive user-software interface.

S1-07 SESSION 1: NEUROSURGERY COHORT VALIDATION OF OPTICAL COHERENCE TOMOGRAPHY TO DETECT ELEVATED INTRACRANIAL PRESSURE IN CHILDREN WITH CRANIOSYNOSTOSIS

Introduction: Better diagnosis of elevated intracranial pressure (ICP) in patients with craniosynostosis may facilitate more timely intervention to improve neurocognitive outcomes. Spectral-domain optical coherence tomography (OCT) of the retinal laminae can non-invasively diagnose elevated ICP. We previously reported thresholds of maximal retinal nerve fiber layer (RNFL) thickness, retinal thickness, and anterior retinal projection measured with OCT that appeared to correlate with subacutely elevated ICP. This study sought to prospectively validate this OCT methodology in a cohort of patients with craniosynostosis. Methods: Infants and children with craniosynostosis were prospectively enrolled, and demographic, clinical, and radiographic characteristics collected. Retinal parameters were prospectively assessed in both eyes using spectral-domain OCT under general anesthesia. Subsequently, ICP was measured directly using a subdural catheter during cranial vault surgery. OCT retinal analysis was performed in a masked fashion, then compared to directly-measured ICP data. Results: Seventeen Nineteen subjects (aged 0.4-6.75.6 years) with craniosynostosis were enrolled; among these 3 (16%) were syndromic with bicoronal fusion, 8 (42%) had sagittal, 4 (21%) unicoronal, and 4 (21%) metopic suture fusion. The sample (mean age 1.9 years) included many patients with late diagnosis; 2 (67%) syndromic and 8 (50%) nonsyndromic patients had elevated ICP (>= 15mmHg.) OCT parameters (either maximal RNFL thickness or anterior retinal projection exceeding 2 standard deviations, in either eye) predicted elevated ICP in 10 patients, 7 of which were diagnosed with elevated ICP directly. This yielded OCT parameter sensitivity of 70%, and specificity of 80% when compared to the gold standard of direct ICP measurement. Sensitivity analysis of a modified protocol assessing bilateral anterior retinal projection was associated with increased specificity of 100%, but reduced sensitivity of 60%. Conclusions: Prospective assessment of a optical coherence tomography protocol to non-invasively detect elevated ICP suggests superior sensitivity and similar specificity to conventional methods in a small cohort of patients with craniosynostosis.

S4A-25 SESSION 4A: GENERALITIES IN CRANIOSYNOSTOSIS – PART I INTERNATIONAL ESTABLISHMENT OF CRANIOFACIAL PROGRAM: 15 YEARS IN THE REPUBLIC OF MOLDOVA

Introduction: The prevalence of craniosynostosis is estimated to be 1 in 2100 births in the US. The global prevalence is unknown due to a lack of recognition, limited technology for diagnosis, and limited resources for treatment. Moldova, a country of 2.8 million people. The infrastructure for surgical treatment of craniosynostosis in Moldova was non-existent in 2003. Given the clear need and lack of alternatives, we will describe a replicable pathway to develop local surgical care for craniofacial patients through education and training. Methods: A bilateral state partnership for collaboration began between North Carolina and Moldova in 1999. This facilitated the beginning of our relationship in 2003 between the surgical departments of our Universities. Stake holders were identified and jointly committed to the establishment of a program. Yearly surgical educational exchanges were conducted. A local team was established including pediatric oral maxillofacial surgeons, neurologists, pediatricians, dentists, neurosurgeons, and anesthesiologists. Data was collected from 2011 to 2018 on more than 33 patients who underwent surgical intervention from the joint team. The patients were assessed including gender, age, blood loss, blood transfusion, duration of surgery, complications, cephalic index (CI), length of stay, and cost. Additional patients treated by the Moldova team were also monitored for the same outcome parameters. Results: Surgical intervention began with minimally invasive surgery utilizing springs for treatment of sagittal synostosis. This allowed the local team to provide time sensitive surgery with limited resources. As the team built confidence and resources were added, complete cranial vault surgery was successfully performed. The joint cases included treatment of metopic (6), sagittal (13), unicoronal (9), bicoronal (4) and multi-suture (1) synostosis. There were no significant complications during the follow-up period. For the past several years the Moldova team has been independently treating a wide range of craniofacial deformities with minimal morbidity and excellent aesthetic outcomes. Conclusion: Organizations desiring to develop craniofacial programs should begin with establishing relationships at the local level. A commitment is needed to build trust among the physicians, administrators and patients. Financial resources are helpful to provide the needed educational exchange programs and equipment needed for the perioperative care of the patients. Significantly more patients can receive needed and life-saving surgical care in their own country when there is a dedicated local team trained to provide the surgery.

Onset and Resolution of Chiari Malformations and Hydrocephalus in Syndromic Craniosynostosis following Posterior Vault Distraction.

Patients with syndromic craniosynostosis have an increased incidence of progressive hydrocephalus and Chiari malformations, with few data on the relative benefit of various surgical interventions. The authors compare the incidence and resolution of Chiari malformations and hydrocephalus between patients undergoing posterior vault distraction osteogenesis (PVDO) and patients undergoing conventional cranial vault remodeling. Patients with syndromic craniosynostosis who underwent cranial vault surgery from 2004 to 2016 at a single academic hospital, with adequate radiographic assessments, were reviewed. Demographics, interventions, the presence of a Chiari malformation on radiographic studies and hydrocephalus requiring shunt placement were recorded. Mann-Whitney U and Fisher's exact tests were used as appropriate. Forty-nine patients underwent PVDO, and 23 patients underwent cranial vault remodeling during the study period. Median age at surgery (p = 0.880), sex (p = 0.123), and types of syndrome (p = 0.583) were well matched. Patients who underwent PVDO had a decreased incidence of developing Chiari malformations postoperatively compared with the cranial vault remodeling cohort (2.0 percent versus 17.4 percent; p = 0.033). Not surprisingly, no significant difference was found between the groups with regard to the incidence of postoperative hydrocephalus requiring shunt placement (PVDO, 4.1 percent; cranial vault remodeling, 4.3 percent; p = 0.999). As expected, PVDO did not significantly affect intracranial hydrodynamics to the extent that hydrocephalus shunting rates were different for patients with syndromic craniosynostosis. However, PVDO was associated with a reduced risk of developing a Chiari malformation; however, prospective evaluation is needed to determine causality. Therapeutic, III.

ERF-related craniosynostosis: The phenotypic and developmental profile of a new craniosynostosis syndrome.

Mutations in the ERF gene, coding for ETS2 repressor factor, a member of the ETS family of transcription factors cause a recently recognized syndromic form of craniosynostosis (CRS4) with facial dysmorphism, Chiari‐1 malformation, speech and language delay, and learning difficulties and/or behavioral problems. The overall prevalence of ERF mutations in patients with syndromic craniosynostosis is around 2%, and 0.7% in clinically nonsyndromic craniosynostosis. Here, we present findings from 16 unrelated probands with ERF‐related craniosynostosis, with additional data from 20 family members sharing the mutations. Most of the probands exhibited multisutural (including pan‐) synostosis but a pattern involving the sagittal and lambdoid sutures (Mercedes‐Benz pattern) predominated. Importantly the craniosynostosis was often postnatal in onset, insidious and progressive with subtle effects on head morphology resulting in a median age at presentation of 42 months among the probands and, in some instances, permanent visual impairment due to unsuspected raised intracranial pressure (ICP). Facial dysmorphism (exhibited by all of the probands and many of the affected relatives) took the form of orbital hypertelorism, mild exorbitism and malar hypoplasia resembling Crouzon syndrome but, importantly, a Class I occlusal relationship. Speech delay, poor gross and/or fine motor control, hyperactivity and poor concentration were common. Cranial vault surgery for raised ICP and/or Chiari‐1 malformation was expected when multisutural synostosis was observed. Variable expressivity and nonpenetrance among genetically affected relatives was encountered. These observations form the most complete phenotypic and developmental profile of this recently identified craniosynostosis syndrome yet described and have important implications for surgical intervention and follow‐up.

Variation in the management of isolated craniosynostosis: a survey of the Synostosis Research Group.

OBJECTIVEThe authors created a collaborative network, the Synostosis Research Group (SynRG), to facilitate multicenter clinical research on craniosynostosis. To identify common and differing practice patterns within the network, they assessed the SynRG surgeons' management preferences for sagittal synostosis. These results will be incorporated into planning cooperative studies.METHODSThe SynRG consists of 12 surgeons at 5 clinical sites. An email survey was distributed to SynRG surgeons in late 2016, and responses were collected through early 2017. Responses were collated and analyzed descriptively.RESULTSAll of the surgeons-7 plastic/craniofacial surgeons and 5 neurosurgeons-completed the survey. They varied in both experience (1-24 years) and sagittal synostosis case volume in the preceding year (5-45 cases). Three sites routinely perform preoperative CT scans. The preferred surgical technique for children younger than 3 months is strip craniectomy (10/12 surgeons), whereas children older than 6 months are all treated with open cranial vault surgery. Pre-incision cefazolin, preoperative complete blood count panels, and an arterial line were used by most surgeons, but tranexamic acid was used routinely at 3 sites and never at the other 2 sites. Among surgeons performing endoscopic strip craniectomy surgery (SCS), most create a 5-cm-wide craniectomy, whereas 2 surgeons create a 2-cm strip. Four surgeons routinely send endoscopic SCS patients to the intensive care unit after surgery. Two of the 5 sites routinely obtain a CT scan within the 1st year after surgery.CONCLUSIONSThe SynRG surgeons vary substantially in the use of imaging, the choice of surgical procedure and technique, and follow-up. A collaborative network will provide the opportunity to study different practice patterns, reduce variation, and contribute multicenter data on the management of children with craniosynostosis.

Early postoperative follow-up after craniosynostosis surgery

Background/aim: Declined morbidity rates after craniosynostosis surgery indicate bypassing the pediatric intensive care unit (PICU) course to minimize treatment costs and bed usage. The aim of this study is to examine the incident rates of PICU admission and assess its necessity. Materials and methods: A retrospective analysis of 41 patients (operated on by open surgical techniques) between July 2011 and December 2015 was carried out. Intraoperative/postoperative vital signs, hemodynamic and metabolic parameters, estimated blood loss (EBVloss), blood transfusions, length of PICU, and hospitalizations were recorded. Results: Major and minor events reached 51.2% and 82.9%, respectively. EBVloss within 24 h was calculated as 39.58 ± 8.19 (median: 38.44, 25.68-66.34) with 75.6% blood transfusion rate. Hypotension and hypothermia were associated with prolonged surgery (P = 0.001 and P = 0.007, respectively), but were not related to age (P = 0.054, P = 0.162) or procedure types (P = 0.558, P = 0.663). Prolonged surgery and younger age had an impact on the complications. One patient died of persistent hemorrhage at 96 h. Conclusion: Monitoring cardiovascular and metabolic dynamics at PICU during the first 24 h after surgery is crucial. Additional studies are needed to define the threshold values of several metabolic and hemodynamic markers in risk assessment after cranial vault surgery.

Longitudinal Assessment of Developmental Outcomes in Infants Undergoing Late Craniosynostosis Repair.

Evaluation of infants with craniosynostosis for surgical intervention, as opposed to conservative management, remains a challenge within the field of craniofacial surgery. Studies have consistently demonstrated that surgical repair of craniosynostosis is ideally performed between 3 and 12 months of age. As such, there is limited data regarding neurocognitive development in infants who initially present with uncorrected craniosynostosis after 12 months of age. Moreover, the impact of cranial vault surgery on neurocognitive development at all ages remains under investigation. A prospective, nonrandomized study was performed. All children with nonsyndromic craniosynostosis who presented for initial evaluation after 12 months of age were enrolled. The Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) was utilized to assess pre- and postoperative cognitive development and comparisons were made to normative values. Developmental delay is defined as scoring < 85. Five infants, average age 26 months (13-43 months) at initial presentation, underwent cranial vault remodeling and developmental testing. Fused cranial sutures involved: metopic (n = 4), and right coronal (n = 1). Cognitive testing demonstrated that 4 of 5 infants (80%) were developmentally delayed at presentation (scores: 60, 70, 72, and 80), and 1 infant was within normal limits (score: 100). Postoperative testing was performed between 2 and 12 months postoperatively. Universal improvement was observed in infants who were delayed prior to surgery (80, 80, 75, and 90, respectively). The infant who was not delayed prior to surgery remained within normal limits after surgery. This study demonstrates an association between cranial vault surgery and cognitive improvement in infants presenting late with developmental delay.