Oncolytic HSV-1 G207 Immunovirotherapy for Pediatric High-Grade Gliomas.

BACKGROUND: Outcomes in children and adolescents with recurrent or progressive high-grade glioma are poor, with a historical median overall survival of 5.6 months. Pediatric high-grade gliomas are largely immunologically silent or "cold," with few tumor-infiltrating lymphocytes. Preclinically, pediatric brain tumors are highly sensitive to oncolytic virotherapy with genetically engineered herpes simplex virus type 1 (HSV-1) G207, which lacks genes essential for replication in normal brain tissue. METHODS: We conducted a phase 1 trial of G207, which used a 3+3 design with four dose cohorts of children and adolescents with biopsy-confirmed recurrent or progressive supratentorial brain tumors. Patients underwent stereotactic placement of up to four intratumoral catheters. The following day, they received G207 (107 or 108 plaque-forming units) by controlled-rate infusion over a period of 6 hours. Cohorts 3 and 4 received radiation (5 Gy) to the gross tumor volume within 24 hours after G207 administration. Viral shedding from saliva, conjunctiva, and blood was assessed by culture and polymerase-chain-reaction assay. Matched pre- and post-treatment tissue samples were examined for tumor-infiltrating lymphocytes by immunohistologic analysis. RESULTS: Twelve patients 7 to 18 years of age with high-grade glioma received G207. No dose-limiting toxic effects or serious adverse events were attributed to G207 by the investigators. Twenty grade 1 adverse events were possibly related to G207. No virus shedding was detected. Radiographic, neuropathological, or clinical responses were seen in 11 patients. The median overall survival was 12.2 months (95% confidence interval, 8.0 to 16.4); as of June 5, 2020, a total of 4 of 11 patients were still alive 18 months after G207 treatment. G207 markedly increased the number of tumor-infiltrating lymphocytes. CONCLUSIONS: Intratumoral G207 alone and with radiation had an acceptable adverse-event profile with evidence of responses in patients with recurrent or progressive pediatric high-grade glioma. G207 converted immunologically "cold" tumors to "hot." (Supported by the Food and Drug Administration and others; ClinicalTrials.gov number, NCT02457845.).

Characteristics and outcomes of pediatric dural arteriovenous fistulas: a systematic review.

BACKGROUND: Dural arteriovenous fistulas (dAVF) are arteriovenous shunts in communication with the dural vasculature in the brain or spine. Apart from single-center series, risk factors and treatment outcomes for pediatric dAVFs are largely undescribed. METHODS: We performed a systematic literature review of pediatric (< 18 years at diagnosis) intracranial and spinal dAVF according to PRISMA guidelines. We queried PubMed, CINAHL, SCOPUS, and Embase databases without time/date restriction. Search strings included a variety of MeSH keywords relating to dural AV fistulas in combination with MeSH keywords related to pediatric cases (see Appendix). Manuscripts describing patients diagnosed with dural sinus malformations or pial AVF were excluded. RESULTS: We identified 61 studies describing 69 individual patients. Overall, dAVF were more common in males (55.1%) with a mean age of diagnosis (5.17 ± 4.42 years). Approximately 20.2% of patients presented with cardiovascular disease (CVD), and 31.9% were discovered incidentally on neuroimaging studies. Transverse-sigmoid junction was the most common location (17.3%). Ninety-three percent (64 patients) were treated, most commonly using endovascular embolization (68.1%) followed by surgery (8.7%) and radiosurgery (2.9%). Almost half (43.8%) of dAVFs were completely obliterated. Of the 64 procedures, there were 19 neurological complications (29.7%) of varying severity where 12.5% were considered transient (i.e., pseudomeningocele) and 17.2% permanent (i.e., mortality secondary to acute sinus thrombosis, etc.). CONCLUSION: There is a paucity of information on pediatric dAVFs. This systematic review summarizes the published cases of dAVFs in the pediatric population. While the rate of missing data is high, there is publication bias, and precise details regarding complications are difficult to ascertain, this review serves as a descriptive summary of pediatric dAVFs.

Pediatric non-galenic pial arteriovenous fistula's characteristics and outcomes: a systematic review.

INTRODUCTION: Pediatric non-galenic pial arteriovenous fistulas (pAVFs) are rare vascular malformations that are characterized by a pial arterial-venous connection without an intervening capillary bed. Outcomes and treatment strategies for pAVFs are highly individualized, owing to the rarity of the disease and lack of large-scale data guiding optimal treatment approaches. METHODS: We performed a systematic review of pediatric patients (< 18 years at diagnosis) diagnosed with a pAVF by digital subtraction angiogram (DSA). The demographics, treatment modalities, and outcomes were documented for each patient and clinical outcome data was collected. Descriptive information stratified by outcome scores were classified as follows: 1 = excellent (no deficit and full premorbid activity), 2 = good (mild deficit and full premorbid activity), 3 = fair (moderate deficit and impaired activity), 4 = poor (severe deficit and dependent on others), 5 = death. RESULTS: A total of 87 studies involving 231 patients were identified. Median age at diagnosis was 3 years (neonates to 18 years). There was slight male preponderance (55.4%), and 150 subjects (81.1%*) experienced excellent outcomes after treatment. Of the 189 patients treated using endovascular approaches, 80.3% experienced excellent outcomes and of the 15 patients surgically treated subjects 75% had an excellent outcome. The highest rate of excellent outcomes was achieved in patients treated with Onyx (95.2%) and other forms of EvOH (100%). High output heart failure and comorbid vascular lesions tended to result in worse outcomes, with only 54.2% and 68% of subjects experiencing an excellent outcome, respectively. *Outcomes were reported in only 185 patients. CONCLUSION: pAVFs are rare lesions, necessitating aggregation of patient data to inform natural history and optimal treatment strategies. This review summarizes the current literature on pAVF in children, where children presenting with heart failure as a result of high flow through the lesion were less likely to experience an excellent outcome. Prospective, large-scale studies would further characterize pediatric pAVFs and enable quantitative analysis of outcomes to inform best treatment practices.

Optimizing Surgical Performance Using Preoperative Virtual Reality Planning: A Systematic Review.

BACKGROUND: Surgery is often a complex process that requires detailed 3-dimensional anatomical knowledge and rigorous interplay between team members to attain ideal operational efficiency or "flow." Virtual Reality (VR) represents a technology by which to rehearse complex plans and communicate precise steps to a surgical team prior to entering the operating room. The objective of this study was to evaluate the use of VR for preoperative surgical team planning and interdisciplinary communication across all surgical specialties. METHODS: A systematic review of the literature was performed examining existing research on VR use for preoperative surgical team planning and interdisciplinary communication across all surgical fields in order to optimize surgical efficiency. MEDLINE, SCOPUS, CINAHL databases were searched from inception to July 31, 2022 using standardized search clauses. A qualitative data synthesis was performed with particular attention to preoperative planning, surgical efficiency optimization, and interdisciplinary collaboration/communication techniques determined a priori. Preferred Reporting Items for Systematic Review and Meta-Analysis guidelines were followed. All included studies were appraised for their quality using the Medical Education Research Study Quality Instrument (MERSQI) tool. RESULTS: One thousand and ninety-three non-duplicated articles with abstract and full text availability were identified. Thirteen articles that examined preoperative VR-based planning techniques for optimization of surgical efficiency and/or interdisciplinary communication fulfilled inclusion and exclusion criteria. These studies had a low-to-medium methodological quality with a MERSQI mean score of 10.04 out of 18 (standard deviation 3.61). CONCLUSIONS: This review demonstrates that time spent rehearsing and visualizing patient-specific anatomical relationships in VR may improve operative efficiency and communication across multiple surgical specialties.

The utility and post-operative evolution of head circumference in nonsyndromic single-suture craniosynostosis: a prospective study in Vietnamese children.

PURPOSE: Head circumference (HC) is an important clinical tool for following head growth in children with craniosynostosis (CS). The purpose of this study is to quantify the usefulness of HC along continuum of CS care, from diagnosis to pre- and post-operative (pre-op, post-op) follow-up in Vietnamese children. METHODS: A prospective cohort of 54 nonsyndromic single-suture CS patients undergoing open surgery from January 2015 to January 2020 was collected at Children's Hospital 2, Vietnam. HC z-score on admission was compared with World Health Organization (WHO) standards to evaluate for utility in initial diagnosis. Pre-op and post-op HC were compared to demonstrate the evolution of head growth following reconstruction. RESULTS: Nonsyndromic single-suture CS was more predominant in males (79.6%) than in females (20.4%). The mean HC z-score was - 0.38 [Formula: see text] 1.29 similar to normal WHO standards regardless of which sutural involvement. The HC z-score increased above + 1 standard deviation (SD) significantly at 3 months of follow-up (p < 0.001); however, the trajectory gradually decreased after the first year of surgery. One patient (1.8%, 1/54) demonstrated restenosis and delayed intracranial hypertension (DIH) 4 years after reconstruction. CONCLUSIONS: The HC in nonsyndromic single-suture CS children presents similarly to the values of healthy children. Additionally, HC reliably increased after reconstruction and gradually normalized over subsequent years. This indicator is consistent in Southeast Asian populations and should be used to follow all patients to assess the normal progression of post-op head growth and as a useful indicator of suspected recurrent synostosis.

What has changed in pediatric neurosurgical care in spina bifida? A 30-year UAB/Children's of Alabama observational overview.

Spina bifida (SB) remains the most serious and most common congenital anomaly of the human nervous system that is compatible with life. The open myelomeningocele on the back is perhaps the most obvious initial problem, but the collective impact of dysraphism upon the entirety of the nervous system and innervated organs is an equal or greater longitudinal threat. As such, patients with myelomeningocele (MMC) are best managed in a multi-disciplinary clinic that brings together experienced medical, nursing, and therapy teams that provide high standards of care while studying outcomes and sharing insights and experiences. Since its inception 30 years ago, the spina bifida program at UAB/Children's of Alabama has remained dedicated to providing exemplary multi-disciplinary care for affected children and their families. During this time, there has been great change in the care landscape, but many of the neurosurgical principles and primary issues have remained the same. In utero myelomeningocele closure (IUMC) has revolutionized initial care and has favorable impact on several important co-morbidities of SB including hydrocephalus, the Chiari II malformation, and the functional level of the neurologic deficit. Hydrocephalus however is not solved by IUMC, and hydrocephalus management remains at the center of neurosurgical care in SB. Ventricular shunts were long the cornerstone of treatment for hydrocephalus, but we came to assess and incorporate endoscopic third ventriculostomy with choroid plexus coagulation (ETV-CPC). Educated and nurtured by an experienced senior mentor, we dedicated ourselves to fundamental concepts but persistently evaluated our care outcomes and evolved our protocols and paradigms for improvement. Active conversations amidst networks of treasured colleagues were central to this development and growth. While hydrocephalus support and treatment of tethered spinal cord remained our principal neurosurgical charges, we evolved to embrace a holistic perspective and approach that is reflected and captured in the Lifetime Care Plan. Our team engaged actively in important workshops and guideline initiatives and was central to the development and support of the National Spina Bifida Patient Registry. We started and developed an adult SB clinic to support our patients who aged out of pediatric care. Lessons there taught us the importance of a model of transition that emphasized personal responsibility and awareness of health and the crucial role of dedicated support over time. Support for sleep, bowel health, and personal intimate cares are important contributors to overall health and care. This paper details our growth, learning, and evolution of care provision over the past 30 years.

Immunotherapy for Pediatric Brain and Spine Tumors: Current State and Future Directions.

BACKGROUND: Brain tumors are the most common solid tumors and the leading cause of cancer-related deaths in children. Incidence in the USA has been on the rise for the last 2 decades. While therapeutic advances in diagnosis and treatment have improved survival and quality of life in many children, prognosis remains poor and current treatments have significant long-term sequelae. SUMMARY: There is a substantial need for the development of new therapeutic approaches, and since the introduction of immunotherapy by immune checkpoint inhibitors, there has been an exponential increase in clinical trials to adopt these and other immunotherapy approaches in children with brain tumors. In this review, we summarize the current immunotherapy landscape for various pediatric brain tumor types including choroid plexus tumors, embryonal tumors (medulloblastoma, AT/RT, PNETs), ependymoma, germ cell tumors, gliomas, glioneuronal and neuronal tumors, and mesenchymal tumors. We discuss the latest clinical trials and noteworthy preclinical studies to treat these pediatric brain tumors using checkpoint inhibitors, cellular therapies (CAR-T, NK, T cell), oncolytic virotherapy, radioimmunotherapy, tumor vaccines, immunomodulators, and other targeted therapies. KEY MESSAGES: The current landscape for immunotherapy in pediatric brain tumors is still emerging, but results in certain tumors have been promising. In the age of targeted therapy, genetic tumor profiling, and many ongoing clinical trials, immunotherapy will likely become an increasingly effective tool in the neuro-oncologist armamentarium.

A model for global surgical training and capacity development: the Children's of Alabama-Viet Nam pediatric neurosurgery partnership.

INTRODUCTION: Training capable and competent neurosurgeons to work in underserved regions of the world is an essential component of building global neurosurgical capacity. One strategy for achieving this goal is establishing longitudinal partnerships between institutions in low- and middle-income countries (LMICs) and their counterparts in high-income countries (HICs) utilizing a multi-component model. We describe the initial experience of the Children's of Alabama (COA) Global Surgery Program partnership with multiple Vietnamese neurosurgical centers. METHODS: The training model developed by the COA Global Surgery Program utilizes three complementary and interdependent methods to expand neurosurgical capacity: in-country training, out-of-country training, and ongoing support and mentorship. Multiple Vietnamese hospital systems have participated in the partnership, including three hospitals in Hanoi and one hospital in Ho Chi Minh City. RESULTS: During the 7 years of the partnership, the COA and Viet Nam teams have collaborated on expanding pediatric neurosurgical care in numerous areas of clinical need including five subspecialized areas of pediatric neurosurgery: cerebrovascular, epilepsy, neuroendoscopy for hydrocephalus management, craniofacial, and neuro-oncology. CONCLUSION: Long-term partnerships between academic departments in LMICs and HICs focused on education and training are playing an increasingly important role in scaling up global surgical capacity. We believe that our multi-faceted approach consisting of in-country targeted hands-on training, out-of-country fellowship training at the mentor institution, and ongoing mentorship using telecollaboration and Internet-based tools is a viable and generalizable model for enhancing surgical capacity globally.

Treatment of pediatric high-grade central nervous system tumors with high-dose methotrexate in combination with multiagent chemotherapy: A single-institution experience.

BACKGROUND: Effective treatment for pediatric embryonal brain tumors includes dose-intensive multiagent chemotherapy (DIMAC) followed by high-dose chemotherapy with stem cell rescue (HDCSCR). Use of repeated cycles of DIMAC including high-dose methotrexate (HDMTX) without HDCSCR has not been described. PROCEDURE: We retrospectively reviewed the responses/toxicities in 13 patients (aged 2-155 months, median 22 months) with central nervous system (CNS) tumors (atypical teratoid rhabdoid tumors, CNS embryonal tumors not otherwise specified, pineoblastoma, embryonal tumor with multilayered rosettes, and CNS sarcoma) treated over a 12-year period with repeated cycles of HDMTX followed by etoposide, cisplatin, cyclophosphamide, and vincristine. RESULTS: Six patients (46.2%) had disseminated disease at presentation and five (38.5%) had gross total resection. A total of 64 courses of therapy were administered with a median of five courses per patient.  Eight patients (61.5%) received radiation therapy (one at relapse). By completion of therapy, 11 patients (84.6%) achieved a response (six complete, five partial).  Six of the 13 patients (46.2%) remain alive with a median follow-up of 48 months (6-146).  Acute toxicities included fever/neutropenia (70.3%), bacteremia (15.6%), and grade 3 mucositis (18.8%).  Long-term complications included learning disability, seizure disorder, and brain necrosis, without treatment-related deaths. CONCLUSIONS: DIMAC with HDMTX without HDCSCR may be an effective treatment option for selected patients with embryonal or high-grade CNS tumors.

Styloidogenic jugular venous compression syndrome: a case report and review of the literature.

BACKGROUND: Styloidogenic jugular venous compression syndrome (SJVCS) has been shown to present with a similar symptomatology to idiopathic intracranial hypertension (IIH) and is caused by compression of the internal jugular vein (IJV) between the lateral tubercle of C1 and the styloid process. Treatments including venous stenting and styloidectomy have been reported with good outcomes; however, treatment of a pediatric patient with SJVCS with styloidectomy has not previously been reported in the literature. CASE REPORT: A 12-year-old male presented with refractory positional headaches, nausea, and vomiting, and after, workup including lumbar puncture (LP) and intracranial pressure (ICP) monitoring was found to have intracranial hypertension associated with contralateral neck turning. Computed tomography venogram (CTV) revealed severe bilateral compression of the IJV's between the styloid processes and C1 tubercle. The patient was successfully treated with unilateral right-sided styloidectomy with symptomatic relief. CONCLUSIONS: This is the first reported pediatric case of SJVCS treated successfully with styloidectomy to our knowledge and adds to the limited literature that styloidectomy is a durable treatment option for SJVCS.

Incidence of delayed intracranial hypertension in children with isolated sagittal synostosis following open calvarial vault reconstruction.

PURPOSE: Delayed intracranial hypertension (DIH) occurs most frequently in children with syndromic or multi-suture synostosis after surgical correction. The rarity of DIH in children with isolated non-syndromic sagittal synostosis (ISS) warrants follow-up evaluation by large craniofacial centers until skeletal maturity. This study reports the incidence of DIH in children following open repair for ISS by our center's craniofacial team. METHODS: A single-center retrospective study of patients who underwent open calvarial vault remodeling (CVR) for ISS at our institution between November 2000 and November 2012 was performed. Syndromic and multi-suture synostosis patients were excluded. Demographic and follow-up data were extracted from the medical record for analysis until July 2017. RESULTS: One hundred five patients with ISS were identified who had undergone CVR in the aforementioned timeframe. Average age at initial surgery was 11.7 ± 15.32 months. Mean follow-up in our craniofacial clinic was 4.94 ± 3.53 years, with 69 patients (65.7%) having follow-up in craniofacial clinic ≥ 3 years and 74 (70.5%) having follow-up ≥ 3 years in any clinic at our institution. Four patients (3.8%) had intracranial pressure (ICP) monitors placed for symptoms concerning for DIH, one of which (0.95%) had confirmed DIH and underwent a second surgical procedure at 7.4 years of age. The patient presented late initially, having his first operation at 1.56 years of age. CONCLUSION: One patient out of 105 (0.95%) developed DIH, confirmed by ICP monitoring, and required reoperation. The occurrence of DIH, albeit rare, remains an important topic to include in parental discussions and mandates long-term follow-up in this population.

The initial experience of InterSurgeon: an online platform to facilitate global neurosurgical partnerships.

OBJECTIVE: Despite general enthusiasm for international collaboration within the organized neurosurgical community, establishing international partnerships remains challenging. The current study analyzes the initial experience of the InterSurgeon website in partnering surgeons from across the world to increase surgical collaboration. METHODS: One year after the launch of the InterSurgeon website, data were collected to quantify the number of website visits, average session duration, total numbers of matches, and number of offers and requests added to the website each month. Additionally, a 15-question survey was designed and distributed to all registered members of the website. RESULTS: There are currently 321 surgeon and institutional members of InterSurgeon representing 69 different countries and all global regions. At the time of the survey there were 277 members, of whom 76 responded to the survey, yielding a response rate of 27.4% (76/277). Twenty-five participants (32.9%) confirmed having either received a match email (12/76, 15.8%) or initiated contact with another user via the website (13/76, 17.1%). As expected, the majority of the collaborations were either between a high-income country (HIC) and a low-income country (LIC) (5/18, 27.8%) or between an HIC and a middle-income country (MIC) (9/18, 50%). Interestingly, there were 2 MIC-to-MIC collaborations (2/18, 11.1%) as well as 1 MIC-to-LIC (1/18, 5.6%) and 1 LIC-to-LIC partnership. At the time of response, 6 (33.3%) of the matches had at least resulted in initial contact via email or telephone. One of the partnerships had involved face-to-face interaction via video conference. A total of 4 respondents had traveled internationally to visit their partner's institution. CONCLUSIONS: Within its first year of launch, the InterSurgeon membership has grown significantly. The partnerships that have already been formed involve not only international visits between HICs and low- to middle-income countries (LMICs), but also telecollaboration and inter-LMIC connections that allow for greater exchange of knowledge and expertise. As membership and site features grow to include other surgical and anesthesia specialties, membership growth and utilization is expected to increase rapidly over time according to social network dynamics.

Pediatric neurosurgical workforce, access to care, equipment and training needs worldwide.

OBJECTIVE: The presence and capability of existing pediatric neurosurgical care worldwide is unknown. The objective of this study was to solicit the expertise of specialists to quantify the geographic representation of pediatric neurosurgeons, access to specialist care, and equipment and training needs globally. METHODS: A mixed-question survey was sent to surgeon members of several international neurosurgical and general pediatric surgical societies via a web-based platform. Respondents answered questions on 5 categories: surgeon demographics and training, hospital and practice details, surgical workforce and access to neurosurgical care, training and equipment needs, and desire for international collaboration. Responses were anonymized and analyzed using Stata software. RESULTS: A total of 459 surgeons from 76 countries responded. Pediatric neurosurgeons in high-income and upper-middle-income countries underwent formal pediatric training at a greater rate than surgeons in low- and lower-middle-income countries (89.5% vs 54.4%). There are an estimated 2297 pediatric neurosurgeons in practice globally, with 85.6% operating in high-income and upper-middle-income countries. In low- and lower-middle-income countries, roughly 330 pediatric neurosurgeons care for a total child population of 1.2 billion. In low-income countries in Africa, the density of pediatric neurosurgeons is roughly 1 per 30 million children. A higher proportion of patients in low- and lower-middle-income countries must travel > 2 hours to seek emergency neurosurgical care, relative to high-income countries (75.6% vs 33.6%, p < 0.001). Vast basic and essential training and equipment needs exist, particularly low- and lower-middle-income countries within Africa, South America, the Eastern Mediterranean, and South-East Asia. Eighty-nine percent of respondents demonstrated an interest in international collaboration for the purposes of pediatric neurosurgical capacity building. CONCLUSIONS: Wide disparity in the access to pediatric neurosurgical care exists globally. In low- and lower-middle-income countries, wherein there exists the greatest burden of pediatric neurosurgical disease, there is a grossly insufficient presence of capable providers and equipped facilities. Neurosurgeons across income groups and geographic regions share a desire for collaboration and partnership.

Surgical treatment of epilepsy in Vietnam: program development and international collaboration.

OBJECTIVE: The purpose of this report was to describe an international collaboration model to facilitate the surgical treatment of children with epilepsy in Vietnam. METHODS: This model uses three complementary methods to achieve a meaningful expansion in epilepsy surgery capacity: US-based providers visiting Hanoi, Vietnam; Vietnamese providers visiting the US; and ongoing telecollaboration, including case review and real-time mentorship using internet-based communication platforms. RESULTS: Introductions took place during a US neurosurgeon's visit to Vietnam in 2014. Given the Vietnamese surgeon's expertise in intraventricular tumor surgery, the focus of the initial visit was corpus callosotomy. After two operations performed jointly, the Vietnamese surgeon went on to perform 10 more callosotomy procedures in the ensuing 6 months with excellent results. The collaborative work grew and matured in 2016-2017, with 40 pediatric epilepsy surgeries performed from 2015 through 2017. Because pediatric epilepsy care requires far more than neurosurgery, teams traveling to Vietnam included a pediatric neurologist and an electroencephalography (EEG) technologist. Also, in 2016-2017, a neurosurgeon, two neurologists, and an EEG nurse from Vietnam completed 2- to 3-month fellowships at Children's of Alabama (COA) in the US. These experiences improved EEG capabilities and facilitated the development of intraoperative electrocorticography (ECoG), making nonlesional epilepsy treatment more feasible. The final component has been ongoing, i.e., regular communication. The Vietnamese team regularly sends case summaries for discussion to the COA epilepsy conference. Three patients in Vietnam have undergone resection guided by ECoG without the US team present, although there was communication via internet-based telecollaboration tools between Vietnamese and US EEG technologists. To date, two of these three patients remain seizure free. The Vietnamese team has presented the results of their epilepsy experience at two international functional and epilepsy surgery scientific meetings. CONCLUSIONS: Ongoing international collaboration has improved the surgical care of epilepsy in Vietnam. Experience suggests that the combination of in-country and US-based training, augmented by long-distance telecollaboration, is an effective paradigm for increasing the capacity for highly subspecialized, multidisciplinary neurosurgical care.

Extremely large sinus pericranii with involvement of the torcular and associated with Crouzon's syndrome.

INTRODUCTION: Sinus pericranii is a rare vascular malformation that connects the intracranial dural sinuses to the extracranial venous drainage system and is caused by either trauma or congenital defects. Although the majority of these vascular structures are due to trauma, some are congenital. CASE REPORT: Herein, we report a 5-month-old patient with a very large and fluctuating subcutaneous mass over the occiput and the diagnosis of Crouzon's syndrome. The child presented with a large midline mass that on imaging, connected to the underlying torcular and was diagnosed as a sinus pericranii. At long-term follow-up and without operative intervention, the sinus pericranii resolved. This uncommon relationship is reviewed. CONCLUSION: Premature closure of posterior fossa sutures as part of Crouzon's syndrome can present with large sinus pericranii. Such subcutaneous swellings might resolve spontaneously.

A never before reported variant of the sacrum.

INTRODUCTION: Several important steps occur in order for the sacrum to develop properly. Embryological derailment can result in several different anatomical variants. CASE REPORT: Herein, we report, to our knowledge, the first case of a duplicated sacrum in the sagittal plane. CONCLUSIONS: Clinicians and radiologists should be aware of such a finding if found during imaging. We would hypothesize that such a variant occurred near the 29th day of development and probably arose from an error in the HOX gene. To our knowledge, this represents the first reported case.

A History of the Professional Credentialing of Applied Behavior Analysts.

The need for a credible professional credential became apparent early in the history of applied behavior analysis. The first efforts to develop a system that identified behavior-analytic practitioners having a specified level of expertise in the profession began in the early 1970s. Over the years, a number of credentialing initiatives were developed in an effort to meet the profession's growing needs for a means of establishing a meaningful professional identity. This article reviews the evolution of these initiatives, culminating with the Behavior Analyst Certification Board and the more recent movement toward state licensure.

Rate of shunt revision as a function of age in patients with shunted hydrocephalus due to myelomeningocele.

OBJECTIVE It is generally accepted that cerebrospinal fluid shunts fail most frequently in the first years of life. The purpose of this study was to describe the risk of shunt failure for a given patient age in a well-defined cohort with shunted hydrocephalus due to myelomeningocele (MMC). METHODS The authors analyzed data from their institutional spina bifida research database including all patients with MMC and shunted hydrocephalus. For the entire population, the number of shunt revisions in each year of life was determined. Then the number of patients at risk for shunt revision during each year of life was calculated, thus enabling them to calculate the rate of shunt revision per patient in each year of life. In this way, the timing of all shunt revision operations for the entire clinic population and the likelihood of having a shunt revision during each year of life were calculated. RESULTS A total of 655 patients were enrolled in the spina bifida research database, 519 of whom had a diagnosis of MMC and whose mean age was 17.48 ± 11.7 years (median 16 years, range 0-63 years). Four hundred seventeen patients had had a CSF shunt for the treatment of hydrocephalus and thus are included in this analysis. There were 94 shunt revisions in the 1st year of life, which represents a rate of 0.23 revisions per patient in that year. The rate of shunt revision per patient-year initially decreased as age increased, except for an increase in revision frequency in the early teen years. Shunt revisions continued to occur as late as 43 years of age. CONCLUSIONS These data substantiate the idea that shunt revision surgeries in patients with MMC are most common in the 1st year of life and decrease thereafter, except for an increase in the early teen years. A persistent risk of shunt failure was observed well into adult life. These findings underscore the importance of routine follow-up of all MMC patients with shunted hydrocephalus and will aid in counseling patients and families.

Necessity and Opportunity: the 1-Year Master's ABA Program at Auburn University.

The Auburn University Master of Science program in Applied Behavior Analysis was designed to accommodate not only the requirements of the Behavior Analyst Certification Board for approved course sequences and practicum training, but unavoidable limitations in faculty and other resources. These limitations were incompatible with the more traditional 2-year curriculum model, so a 1-year program was designed that met the necessary conditions. This article describes the resulting academic and practicum curriculum, the key funding mechanisms that allowed the program to develop, and the opportunities and benefits that this model afforded.

Lambdoidal synostosis in dizygotic twins with a family history of an undiagnosed connective tissue disorder.

INTRODUCTION: Unilateral lambdoidal craniosynostosis is a rare disorder that occurs in approximately 3 % of all craniosynostosis phenotypes and only 0.03 % of one million live births. It is even more unusual for this type of synostosis to occur in siblings with only two other cases reported in the literature. CASE REPORT: We report a set of full-term dizygotic twins born with lambdoidal synostosis and a family history of connective tissue and cardiovascular anomalies. One of the twins also had concomitant bicoronal craniosynostosis. CONCLUSION: True familial lambdoidal synostosis is exceedingly rare. The present cases in dizygotic twins occurred in a family with a significant history of connective tissue disease suggesting a possible association.