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OBJECTIVE Quantitative measurements of trigonocephaly can be used to characterize and track this phenotype, which is associated with metopic craniosynostosis. Traditionally, trigonocephaly metrics were extracted from CT scans; however, this method exposes patients to ionizing radiation. Three-dimensional optical scans are another option but are not routinely available in most outpatient settings. Recently, the authors developed semiautomated artificial intelligence algorithms that extract craniometric data from orthogonal 2D photographs. Although 2D photographs are safe, inexpensive, and straightforward to obtain, the accuracy of photograph-based craniometrics in comparison to CT and 3D optical scan correlates has not been established. In this study the authors compared the classification power of 2D photograph–based metrics of trigonocephaly with four CT-based metrics and one 3D optical scan–based metric in a heterogeneous series of patients who presented to an outpatient craniofacial clinic. METHODS In this study the authors performed retrospective craniometric analyses of patient 2D photographs, 3D optical scans, and CT scans. Imaging-derived craniometrics include the 2D photograph–based anterior arc angle (AAA2D-photo), anterior-posterior ratio (APR2D-photo), and anterior-middle ratio (AMR2D-photo); the CT-based anterior arc angle (AAACT), metopic index (MICT), endocranial-bifrontal angle (eBFACT), and interfrontal angle (IFACT); and the 3D optical scan–based anterior arc angle (AAA3D-optical). Receiver operating characteristics (ROCs) were used to identify craniometrics strongly descriptive of trigonocephaly. Interrater comparisons were made between paired trigonocephaly measurements obtained from photographs and either CT scans or 3D optical scans. RESULTS There were 13 photograph-based and CT-based pairs and 22 paired measurements from 2D photographs and 3D optical scans. AAA displayed the strongest classification capacity across all three imaging modalities. Significant agreement was observed between AAACT and AAA2D-photo (intraclass correlation coefficient [ICC] = 0.68 [95% CI 0.24–0.89], p = 0.0035), and AAA3D-optical and AAA2D-photo (ICC = 0.70 [95% CI 0.41–0.87], p < 0.0001). There was no significant correlation between APR2D-photo or AMR2D-photo and conventional CT-based metrics describing longitudinal width ratios (MICT). CONCLUSIONS Photograph-based craniometrics are powerful tools that can be used to quantify the severity of trigonocephaly and exhibit high concordance with standard measurements derived from CT scans and 3D optical scans. The authors developed and freely share a research-use application to calculate trigonocephaly metrics from 2D photographs. Given the availability of digital photography, lack of ionizing radiation, and low cost of photograph-based craniometric derivation, this technique may be useful to supplement routine ambulatory care and objectively track outcomes following treatment.

1. Kaitlin N. Leopold, MD* 2. Edward S. Ahn, MD† 3. Molly J. Youssef, MD*,‡ 4. Seth W. Gregory, MD* 1. *Department of Pediatric and Adolescent Medicine 2. †Department of Neurologic Surgery 3. ‡Department of Dermatology, Mayo Clinic, Rochester, MN A 1-day-old girl is seen for routine care in the newborn nursery. The patient was born at 40 weeks and 1 day of gestation to a 21-year-old gravida 2 now para 2 mother by vaginal delivery. Pregnancy was complicated by maternal obesity, mild intermittent asthma, hyperthyroidism, allergic rhinitis, anemia, and sickle cell trait. The patient’s mother had adequate prenatal care and a normal prenatal anatomic ultrasonographic image. The mother’s medications during pregnancy included prenatal vitamins, ferrous sulfate, fluticasone nasal spray, and albuterol inhaler as needed. The patient’s newborn course has been uneventful. She has been breastfeeding without difficulty and is voiding and stooling normally. There are no parental concerns. On examination the infant is well appearing and shows no dysmorphic facial features. Her temperature is 98.1°F (36.7°C), heart rate is 128 beats/min, and respiratory rate is 48 breaths/min. Her head is normocephalic, with a flat, open, and soft anterior fontanelle. Neurologic examination reveals normal Moro, grasp, and suck reflexes and normal tone with no focal deficits. External genitalia appear normal, and a patent anus is visualized. Upper and lower extremities are without deformity. The spine examination reveals a straight spine with no sacral dimples or hair tufts. Skin examination is significant for sacral dermal melanosis and an erythematous macular patch over the lumbosacral region. The upper aspect of the gluteal cleft is characterized by a mildly asymmetrical Y-shaped crease (Fig 1). The remainder of her newborn examination findings are within normal limits. Ultrasonography and magnetic resonance imaging (MRI) reveal the diagnosis. Figure 1. A lumbosacral nevus simplex, asymmetrical Y-shaped upper gluteal cleft, and sacral dermal melanosis of a 1-day-old girl. The differential diagnosis of the patient’s lumbosacral birthmark includes a nevus simplex, hemangioma, port-wine stain, or arteriovenous malformation. The location, size, and appearance are most consistent with a nevus simplex. The midline …

Objective To cope with the changing health care services in the era of SARS-CoV-2 pandemic. We share the institutional framework for the management of anomalous fetuses requiring fetal intervention at Mayo Clinic, Rochester, Minnesota. To assess the success of our program during this time, we compare intraoperative outcomes of fetal interventions performed during the pandemic with the previous year. Patients We implemented our testing protocol on patients undergoing fetal intervention at our institution between March 1, and May 15, 2020, and we compared it with same period a year before. A total of 17 pregnant patients with anomalous fetuses who met criteria for fetal intervention were included: 8 from 2019 and 9 from 2020. Methods Our testing protocol was designed based on our institutional perinatal guidelines, surgical requirements from the infection prevention and control (IPAC) committee, and input from our fetal surgery team, with focus on urgency of procedure and maternal SARS-CoV-2 screening status. We compared the indications, types of procedures, maternal age, gestational age at procedure, type of anesthesia used, and duration of procedure for cases performed at our institution between March 1, 2020, and May 15, 2020, and for the same period in 2019. Results There were no statistically significant differences among the number of cases, indications, types of procedures, maternal age, gestational age, types of anesthesia, and duration of procedures (P values were all >.05) between the pre–SARS-CoV-2 pandemic in 2019 and the SARS-CoV-2 pandemic in 2020. Conclusions Adoption of new institutional protocols during SARS-CoV-2 pandemic, with appropriate screening and case selection, allows provision of necessary fetal intervention with maximal benefit to mother, fetus, and health care provider.

OBJECTIVEPhenotypic differences between moyamoya disease (MMD) and moyamoya syndrome (MMS) remain unclear. The purpose of this study was to evaluate whether such differences exist when presentation, procedure-related, and outcome variables are compared quantitatively.METHODSThe study cohort included 185 patients with moyamoya presenting to the Johns Hopkins Medical Institutions between 1994 and 2015. Baseline demographic, angiographic, and clinical characteristics were compared between patients with MMS and MMD, in addition to procedure-related complications and length of stay (LOS) after surgery. Stroke-free survival was compared between both disease variants after diagnosis. Kaplan-Meier analysis and Cox proportional hazards regression were used to compare stroke-free survival between surgically treated and conservatively managed hemispheres in both types of disease, while evaluating interaction between disease variant and management.RESULTSThe cohort consisted of 137 patients with MMD (74%) with a bimodal age distribution and 48 patients with MMS (26%) who were mostly under 18 years of age (75%). Underlying diseases included sickle cell disease (48%), trisomy 21 (12%), neurofibromatosis (23%), and other disorders (17%). Patients with MMS were younger (p < 0.001) and less likely to be female (p = 0.034). Otherwise, baseline characteristics were statistically comparable. The rate of surgical complications was 33% in patients with MMD and 16% in patients with MMS (p = 0.097). Both groups of patients had a similar LOS after surgery (p = 0.823). Survival analysis (n = 330 hemispheres) showed similar stroke-free survival after diagnosis (p = 0.856) and lower stroke hazard in surgically managed patients in both MMD (hazard ratio [HR] 0.29, p = 0.028) and MMS (HR 0.62, p = 0.586). The disease variant (MMD vs MMS) did not affect the relationship between management approach (surgery vs conservative) and stroke hazard (p = 0.787).CONCLUSIONSMMD and MMS have largely comparable clinical and angiographic phenotypes with analogously favorable responses to surgical revascularization.

No abstract available Keywords: CNS Tumors; Childhood Neuropsychiatric Disorders; Mood; Pediatrics; Psychosis.

OBJECTIVE Complex tethered spinal cord (cTSC) release in children is often complicated by surgical site infection (SSI). Children undergoing this surgery share many similarities with patients undergoing correction for neuromuscular scoliosis, where high rates of gram-negative and polymicrobial infections have been reported. Similar organisms isolated from SSIs after cTSC release were recently demonstrated in a single-center pilot study. The purpose of this investigation was to determine if these findings are reproducible across a larger, multicenter study. METHODS A multicenter, retrospective chart review including 7 centers was conducted to identify all cases of SSI following cTSC release during a 10-year study period from 2007 to 2017. Demographic information along with specific microbial culture data and antibiotic sensitivities for each cultured organism were collected. RESULTS A total of 44 SSIs were identified from a total of 655 cases, with 78 individual organisms isolated. There was an overall SSI rate of 6.7%, with 43% polymicrobial and 66% containing at least one gram-negative organism. Half of SSIs included an organism that was resistant to cefazolin, whereas only 32% of SSIs were completely susceptible to cefazolin. CONCLUSIONS In this study, gram-negative and polymicrobial infections were responsible for the majority of SSIs following cTSC surgery, with approximately half resistant to cefazolin. Broader gram-negative antibiotic prophylaxis should be considered for this patient population.

OBJECTIVE Arteriovenous malformations (AVMs) are a major cause of intracerebral hemorrhage in children, resulting in significant morbidity and mortality. Moreover, the rate of AVM recurrence in children is significantly higher than in adults. The aim of this study was to define the risk of delayed pediatric AVM (pAVM) recurrence following confirmed radiological obliteration. Further understanding of this risk could inform the role of long-term radiological surveillance. METHODS The authors conducted a retrospective review of ruptured and unruptured pAVM cases treated at a single tertiary care referral center between 1994 and 2019. Demographics, clinical characteristics, treatment modalities, and AVM recurrence were analyzed. RESULTS A total of 102 pediatric patients with intracranial AVMs, including 52 (51%) ruptured cases, were identified. The mean patient age at presentation was 11.2 ± 4.4 years, and 51 (50%) patients were female. The mean nidus size was 2.66 ± 1.44 cm. The most common Spetzler-Martin grades were III (32%) and II (31%). Stereotactic radiosurgery was performed in 69.6% of patients. AVM obliteration was radiologically confirmed in 68 (72.3%) of 94 patients with follow-up imaging, on angiography in 50 (73.5%) patients and on magnetic resonance imaging in 18 (26.5%). AVM recurrence was identified in 1 (2.3%) of 43 patients with long-term surveillance imaging over a mean follow-up of 54.7 ± 38.9 months (range 2–153 months). This recurrence was identified in a boy who had presented with a ruptured AVM and had been surgically treated at 5 years of age. The AVM recurred 54 months after confirmed obliteration on surveillance digital subtraction angiography. Two other cases of presumed AVM recurrence following resection in young children were excluded from recurrence analysis because of incomplete sets of imaging available for review. CONCLUSIONS AVM recurrence following confirmed obliteration on imaging is a rare phenomenon, though it occurs more frequently in the pediatric population. Regular long-term follow-up with dedicated surveillance angiography is recommended even after obliteration following resection.

Spinal cord intramedullary arteriovenous malformations (AVMs) have classically been considered congenital lesions that are present from birth. The reason for this dogmatic principal is the fact that a vast majority of these lesions present in pediatric and young adult patients. Interestingly, while many authors have demonstrated the development of de novo nidus-type brain AVMs, there have been no reported cases of a de novo intramedullary or perimedullary AVM of the spine. In this paper the authors describe what they believe to be the first reported case of a de novo AVM of the spinal cord in a young patient who underwent serial imaging from birth for evaluation of a syrinx. Potential pathophysiological mechanisms for the development of de novo vascular malformations of the spinal cord are discussed.

OBJECTIVEThere remains uncertainty regarding the appropriate level of care and need for repeating neuroimaging among children with mild traumatic brain injury (mTBI) complicated by intracranial injury (ICI). This study’s objective was to investigate physician practice patterns and decision-making processes for these patients in order to identify knowledge gaps and highlight avenues for future investigation.METHODSThe authors surveyed residents, fellows, and attending physicians from the following pediatric specialties: emergency medicine; general surgery; neurosurgery; and critical care. Participants came from 10 institutions in the United States and an email list maintained by the Canadian Neurosurgical Society. The survey asked respondents to indicate management preferences for and experiences with children with mTBI complicated by ICI, focusing on an exemplar clinical vignette of a 7-year-old girl with a Glasgow Coma Scale score of 15 and a 5-mm subdural hematoma without midline shift after a fall down stairs.RESULTSThe response rate was 52% (n = 536). Overall, 326 (61%) respondents indicated they would recommend ICU admission for the child in the vignette. However, only 62 (12%) agreed/strongly agreed that this child was at high risk of neurological decline. Half of respondents (45%; n = 243) indicated they would order a planned follow-up CT (29%; n = 155) or MRI scan (19%; n = 102), though only 64 (12%) agreed/strongly agreed that repeat neuroimaging would influence their management. Common factors that increased the likelihood of ICU admission included presence of a focal neurological deficit (95%; n = 508 endorsed), midline shift (90%; n = 480) or an epidural hematoma (88%; n = 471). However, 42% (n = 225) indicated they would admit all children with mTBI and ICI to the ICU. Notably, 27% (n = 143) of respondents indicated they had seen one or more children with mTBI and intracranial hemorrhage demonstrate a rapid neurological decline when admitted to a general ward in the last year, and 13% (n = 71) had witnessed this outcome at least twice in the past year.CONCLUSIONSMany physicians endorse ICU admission and repeat neuroimaging for pediatric mTBI with ICI, despite uncertainty regarding the clinical utility of those decisions. These results, combined with evidence that existing practice may provide insufficient monitoring to some high-risk children, emphasize the need for validated decision tools to aid the management of these patients.

OBJECTIVE CSF shunt insertion is the most commonly performed neurosurgical procedure for pediatric patients with hydrocephalus, and complications including infections and catheter obstruction are common. The rate of readmission in the first 30 days after surgery has been used across surgical disciplines to determine healthcare quality. In the current study, the authors sought to assess factors associated with early shunt revision within 30 days using real-world data. METHODS Targeted shunt data set participant user files of the National Surgical Quality Improvement Program (NSQIP) from 2016 to 2019 were queried for patients undergoing a shunt procedure. A multivariable logistic regression model was performed to assess the impact of demographics, etiologies, comorbidities, congenital malformations, and shunt adjuncts on shunt revision within 30 days, as well as shunt revision due to infection within 30 days. RESULTS A total of 3919 primary pediatric shunt insertions were identified in the NSQIP database, with a mean (± SD) patient age of 26.3 ± 51.6 months. There were a total of 285 (7.3%) unplanned shunt revisions within 30 days, with a mean duration of 14.9 ± 8.5 days to first intervention. The most common reason for intervention was mechanical shunt failure (32.6% of revision, 2.4% overall, n = 93), followed by infection (31.2% of all interventions, 2.3% overall, n = 89) and wound disruption or CSF leak (22.1% of all interventions, 1.6% overall, n = 63). Patients younger than 6 months of age had the highest overall unplanned 30-day revision rate (8.5%, 203/2402) as well as the highest 30-day shunt infection rate (3%, 72/2402). Patients who required a revision were also more likely to have a cardiac risk factor (34.7%, n = 99, vs 29.2%, n = 1061; p = 0.048). Multivariable logistic regression revealed that compared to patients 9–18 years old, those aged 2–9 years had significantly lower odds of repeat shunt intervention (p = 0.047), while certain etiologies including congenital hydrocephalus (p = 0.0127), intraventricular hemorrhage (IVH) of prematurity (p = 0.0173), neoplasm (p = 0.0005), infection (p = 0.0004), and syndromic etiology (p = 0.0136), as well as presence of ostomy (p = 0.0095), were associated with higher odds of repeat intervention. For shunt infection, IVH of prematurity was found to be associated with significantly higher odds (p = 0.0427) of shunt infection within 30 days, while use of intraventricular antibiotics was associated with significantly lower odds (p = 0.0085). CONCLUSIONS In this study of outcomes after pediatric shunt placement using a nationally derived cohort, early shunt failure and infection within 30 days were found to remain as considerable risks. The analysis of this national surgical quality registry confirms that, in accordance with other multicenter studies, hydrocephalus etiology, age, and presence of ostomy are important predictors of the need for early shunt revision. IVH of prematurity is associated with early infections while intraventricular antibiotics may be protective. These findings could be used for benchmarking in hospital efforts to improve quality of care for pediatric patients with hydrocephalus.

The authors describe an endoscopic strip craniectomy through a single incision for the treatment of sagittal craniosynostosis in a young infant. The endoscopic strip craniectomy was first introduced with the use of two incisions on either end of the fused suture. This single-incision technique offers several advantages. There is a cosmetic advantage and a reduced risk of wound complications. This technique also allows for early control of emissary veins and an inside-out identification of the lambdoid sutures. Endoscopic visualization is optimized to reduce the risk of blood loss, especially because circulating blood volume is very limited in these young infants. The video can be found here: https://vimeo.com/514366415

OBJECTIVE The authors sought to evaluate the accuracy of a novel telehealth-compatible diagnostic software system for identifying craniosynostosis within a newborn (< 1 year old) population. Agreement with gold standard craniometric diagnostics was also assessed. METHODS Cranial shape classification software accuracy was compared to that of blinded craniofacial specialists using a data set of open-source (n = 40) and retrospectively collected newborn orthogonal top-down cranial images, with or without additional facial views (n = 339), culled between April 1, 2008, and February 29, 2020. Based on image quality, midface visibility, and visibility of the cranial equator, 351 image sets were deemed acceptable. Accuracy, sensitivity, and specificity were calculated for the software versus specialist classification. Software agreement with optical craniometrics was assessed with intraclass correlation coefficients. RESULTS The cranial shape classification software had an accuracy of 93.3% (95% CI 86.8–98.8; p < 0.001), with a sensitivity of 92.0% and specificity of 94.3%. Intraclass correlation coefficients for measurements of the cephalic index and cranial vault asymmetry index compared to optical measurements were 0.95 (95% CI 0.84–0.98; p < 0.001) and 0.67 (95% CI 0.24–0.88; p = 0.003), respectively. CONCLUSIONS These results support the use of image processing–based neonatal cranial deformity classification software for remote screening of nonsyndromic craniosynostosis in a newborn population and as a substitute for optical scanner– or CT-based craniometrics. This work has implications that suggest the potential for the development of software for a mobile platform that would allow for screening by telemedicine or in a primary care setting.

OBJECTIVEPediatric Chiari I malformation decompression is a common neurosurgical procedure. Liposomal bupivacaine (LB) is a novel formulation that can have an impact on postoperative recovery for particular procedures, but its potential role in pediatric neurosurgery is largely unexplored. The authors sought to describe and assess their initial experience with LB in pediatric Chiari I malformation decompression to better define its potential role as an analgesic agent in a procedure for which the postoperative course is often remarkably painful.METHODSA retrospective review of all pediatric Chiari procedures performed at the authors’ institution between 2018 and 2020 was conducted. Patients were divided into those who were treated with a single intraoperative dose of LB (LB group) and those who were not (control group). Comparisons of total opioid use and pain control were made using chi-square and Wilcoxon rank-sum tests.RESULTSA total of 18 patients were identified, 9 (50%) in the LB group and 9 (50%) in the control group. Overall, there were 13 (72%) female and 5 (28%) male patients with a mean age of 15.9 years. No surgical complications were observed over a mean length of stay of 2.7 days. Within the first 24 hours after surgery, the LB group had significantly lower total opioid use than the control group (17.5 vs 47.9 morphine milligram equivalents, respectively; p = 0.03) as well as lower mean pain scores reported by patients using a 10-point visual analog scale (3.6 vs 5.5 for the LB vs control groups, p = 0.04). However, from the first 24 postoperative hours to discharge, total opioid use (p = 0.51) and mean pain scores (p = 0.09) were statistically comparable between the two groups. There were 2/9 (22%) LB patients versus 0/9 (0%) control patients who did not require opioid analgesia at any point during hospitalization.CONCLUSIONSThe use of a single intraoperative dose of LB in pediatric Chiari I malformation surgery appears to be safe and has the potential to reduce pain scores and opioid use when administered during the first 24 postoperative hours. From that time period to discharge, however, there may be no significant difference in total opioid use or pain scores.

The modern era of surgery has been characterized by the growing permeation of robotic surgery. Smaller incisions, enhanced dexterity in confined spaces, and magnification make robotic surgery ideally suited for the growing field of prenatal intervention, wherein fetal conditions are surgically addressed within the confines of the womb. In this chapter, we review the history of fetal surgery and the features that make it both challenging and unique, particularly the multidisciplinary nature of maternal-fetal intervention and the risk of precipitating premature labor. We discuss the growing bodies of evidence for fetal myelomeningocele closure, interventions for lower urinary tract obstruction, and various other conditions well-suited for robotic application. As robotic technology rapidly improves with newer platforms, smaller instruments, enhanced simulation, and telesurgery capabilities, the frontier of robotic fetal surgery will be propelled into the next era.

OBJECTIVEPediatric cerebral venous sinus thrombosis has been previously described in the setting of blunt head trauma; however, the population demographics, risk factors for thrombosis, and the risks and benefits of detection and treatment in this patient population are poorly defined. Furthermore, few reports differentiate between different forms of sinus pathology. A series of pediatric patients with skull fractures who underwent venous imaging and were diagnosed with intrinsic cerebral venous sinus thrombosis or extrinsic sinus compression is presented.METHODSThe medical records of patients at 2 pediatric trauma centers were retrospectively reviewed. Patients who were evaluated for blunt head trauma from January 2003 to December 2013, diagnosed with a skull fracture, and underwent venous imaging were included.RESULTSOf 2224 pediatric patients with skull fractures following blunt trauma, 41 patients (2%) underwent venous imaging. Of these, 8 patients (20%) had intrinsic sinus thrombosis and 14 patients (34%) displayed extrinsic compression of a venous sinus. Three patients with intrinsic sinus thrombosis developed venous infarcts, and 2 of these patients were treated with anticoagulation. One patient with extrinsic sinus compression by a depressed skull fracture underwent surgical elevation of the fracture. All patients with sinus pathology were discharged to home or inpatient rehabilitation. Among patients who underwent follow-up imaging, the sinus pathology had resolved by 6 months postinjury in 80% of patients with intrinsic thrombosis as well as 80% of patients with extrinsic compression. All patients with intrinsic thrombosis or extrinsic compression had a Glasgow Outcome Scale score of 4 or 5 at their last follow-up.CONCLUSIONSIn this series of pediatric trauma patients who underwent venous imaging for suspected thrombosis, the yield of detecting intrinsic thrombosis and/or extrinsic compression of a venous sinus was high. However, few patients developed venous hypertension or infarction and were subsequently treated with anticoagulation or surgical decompression of the sinus. Most had spontaneous resolution and good neurological outcomes without treatment. Therefore, in the setting of pediatric skull fractures after blunt injury, venous imaging is recommended when venous hypertension or infarction is suspected and anticoagulation is being considered. However, there is little indication for pervasive venous imaging after pediatric skull fractures, especially in light of the potential risks of CT venography or MR venography in the pediatric population and the unclear benefits of anticoagulation.

Few reports have compared surgical intervention with conservative treatment for moyamoya disease (MMD) in non-Asian cohorts. This study describes the effectiveness of follow-up stroke prevention by indirect revascularization relative to conservative management in a Northeast United States study population.We retrospectively reviewed records of patients with MMD at our institution from 1990 to 2014. Baseline characteristics and follow-up results including subsequent ipsilateral strokes were collected, and compared between an indirect revascularization group and a conservatively treated group on a per-hemisphere basis.A total of 94 patients with 184 hemispheres were included. The average age was 23.9 ± 18.1 years, with 76.6% (n = 141) being female. Racial distribution comprised white (n = 75, 40.8%), African-American (n = 57, 31.0%), Asian (n = 30, 16.3%), and other (n = 22, 12.0%). Eighty-three hemispheres (45.1%) presented with ipsilateral stroke and 54 (29.3%) with ipsilateral TIA. Management strategies included either conservative management (51.1%) or indirect bypass (48.9%). Patients who were male (P 0.001), on baseline antiplatelets (P = 0.043), or with speech disturbance (P = 0.002) were more likely to receive indirect revascularization. Patients with headache history were more likely to be treated conservatively (P = 0.046). History of ipsilateral stroke was borderline associated with indirect bypass (P = 0.058). During a follow-up period of 6.37 ± 5.81 years, the annual risk of stroke for indirect revascularization group was 0.93% and 2.70% for the conservative group. Multivariate analysis found that increasing age (P = 0.029), posterior cerebral artery involvement (P = 0.040), and conservative treatment (P = 0.048) were associated with follow-up stroke.Our results suggests that indirect revascularization provides symptom relief and lower risk of stroke during follow-up compared with conservative management. Indirect revascularization should be considered for symptomatic MMD patients with a low surgical risk profile in a similar patient population.

Background Children with moyamoya vasculopathy are at high risk of perioperative cerebral ischemia or hyperperfusion. Maintaining blood pressure within the range of functional cerebrovascular blood pressure autoregulation might reduce the risk of perioperative neurologic injury. Aims We tested whether blood pressure autoregulation is associated with postoperative transient ischemic attack in a study of patients with pediatric moyamoya vasculopathy. Methods We conducted an observational study of 15 pediatric patients undergoing surgical revascularization with pial synangiosis. Nine patients had bilateral moyamoya and 6 had unilateral moyamoya. We measured autoregulatory vasoreactivity intraoperatively and during the first postoperative night with the hemoglobin volume index, a value derived from near-infrared spectroscopy. We also identified the optimal mean arterial blood pressure at which autoregulation was most robust in each patient. Results Of the 15 children monitored, 3 with bilateral moyamoya and one with unilateral moyamoya experienced a transient ischemic attack. Poorer autoregulation during surgery was associated with postoperative transient ischemic attack among those with bilateral vasculopathy (P = .048, difference in hemoglobin volume index medians: 0.023, 95% confidence interval: 0.003-0.071). This relationship was not observed with postoperative autoregulation. The optimal mean arterial blood pressure was identifiable during surgery in all monitored patients, varied among patients, and often differed between the intraoperative and postoperative periods. Conclusion Dysfunctional intraoperative autoregulation may increase the risk of TIA in patients with pediatric moyamoya vasculopathy. The blood pressure range that supports autoregulation appears to vary among patients. Using autoregulation monitoring to guide individualized blood pressure goals should be studied as a potential method to reduce perioperative neurologic morbidity in pediatric patients with moyamoya.

Cranial vault remodeling surgery for craniosynostosis carries the potential risk of dural venous sinus injury given the extensive bony exposure. Identification of the dural venous sinuses can be challenging in patients with craniosynostosis given the lack of accurate surface-localizing landmarks. Computer-aided design and manufacturing (CAD/CAM) has allowed surgeons to pre-operatively plan these complex procedures in an effort to increase reconstructive efficiency. An added benefit of this technology is the ability to intraoperatively map the dural venous sinuses based on pre-operative imaging. We utilized CAD/CAM technology to intraoperatively map the dural venous sinuses for patients undergoing reconstructive surgery for craniosynostosis in an effort to prevent sinus injury, increase operative efficiency, and enhance patient safety. Here, we describe our experience utilizing this intraoperative technology in pediatric patients with craniosynostosis. We retrospectively reviewed the charts of children undergoing reconstructive surgery for craniosynostosis using CAD/CAM surgical planning guides at our institution between 2012 and 2016. Data collected included the following: age, gender, type of craniosynostosis, estimated blood loss, sagittal sinus deviation from the sagittal suture, peri-operative outcomes, and hospital length of stay. Thirty-two patients underwent reconstructive cranial surgery for craniosynostosis, with a median age of 11 months (range, 7–160). Types of synostosis included metopic (6), unicoronal (6), sagittal (15), lambdoid (1), and multiple suture (4). Sagittal sinus deviation from the sagittal suture was maximal in unicoronal synostosis patients (10.2 ± 0.9 mm). All patients tolerated surgery well, and there were no occurrences of sagittal sinus, transverse sinus, or torcular injury. The use of CAD/CAM technology allows for accurate intraoperative dural venous sinus localization during reconstructive surgery for craniosynostosis and enhances operative efficiency and surgeon confidence while minimizing the risk of patient morbidity.

OBJECTIVEEndoscopic strip craniectomy (ESC) with postoperative helmet orthosis is a well-established treatment option for sagittal craniosynostosis. There are many technical variations to the surgery ranging from simple strip craniectomy to methods that employ multiple cranial osteotomies. The purpose of this study was to determine whether the addition of lateral barrel-stave osteotomies during ESC improved morphological outcomes.METHODSAn IRB-approved retrospective review was conducted on a consecutive series of cases involving ESC for sagittal craniosynostosis at 2 different institutions from March 2008 to August 2014. The patients in Group A underwent ESC and those in Group B had ESC with lateral barrel-stave osteotomies. Demographic and perioperative data were recorded; postoperative morphological outcomes were analyzed using 3D laser scan data acquired from a single orthotic manufacturer who managed patients from both institutions.RESULTSA total of 73 patients were included (34 in Group A and 39 in Group B). Compared with Group B patients, Group A patients had a shorter mean anesthetic time (161.7 vs 195 minutes; p < 0.01) and operative time (71.6 vs 111 minutes; p < 0.01). The mean hospital stay was similar for the 2 groups (1.2 days for Group A vs 1.4 days for Group B; p = 0.1). Adequate postoperative data on morphological outcomes were reported by the orthotic manufacturer for 65 patients (29 in Group A and 36 in Group B). The 2 groups had similar improvement in the cephalic index (CI): Group A, mean change 10.5% (mean preoperative CI 72.6, final 80.4) at a mean follow-up of 13.2 months; Group B, mean change 12.2% (mean preoperative CI 71.0, final 79.6) at a mean follow-up of 19.4 months. The difference was not statistically significant (p = 0.15).CONCLUSIONSBoth ESC alone and ESC with barrel staving produced excellent outcomes. However, the addition of barrel staves did not improve the results and, therefore, may not be warranted in the endoscopic treatment of sagittal craniosynostosis.

OBJECTIVERecent reports have described early endoscopic suturectomy as a treatment option for patients with syndromic craniosynostosis, but such patients often require subsequent calvarial remodeling. The authors describe their experience with this patient population and seek to identify predictors of sufficiency of endoscopic surgery alone.METHODSThe medical records of patients with syndromic craniosynostosis who underwent endoscopic repair were retrospectively reviewed. Demographic data, operative details, and follow-up data were collected.RESULTSA total of 6 patients with syndromic craniosynostosis underwent endoscopic surgery followed by helmet therapy during the study period. Of these, 3 patients were male. The involved syndromes included Crouzon, Pfeiffer, Jackson-Weiss, Muenke, Saethre-Chotzen, and craniosynostosis-3 (n = 1 each). The patients underwent endoscopic surgery at a median age of 2.1 months (range 0.9–4.1 months). The median estimated blood loss was 30 ml (range 20–100 ml), with 2 patients requiring a transfusion. The median length of stay in the hospital was 1.5 days (range 1–4 days), and the median follow-up was 29.0 months (range 16.8–81.7 months), with 1 patient (16.7%) requiring an open revision. Three patients (50%) were classified as Whitaker Category I at the last follow-up. The patients for whom additional open surgery was performed or recommended (Whitaker Category IV) were the oldest patients in the cohort, ranging from 2.6 to 4.1 months at the time of surgery.CONCLUSIONSThis series demonstrates that endoscopic surgery can be sufficient to treat syndromic craniosynostosis without subsequent open calvarial remodeling over a median follow-up period of at least 2 years. The findings suggest that younger age at the time of endoscopic surgery may be an important factor in determining the sufficiency of this procedure. Even among patients who require subsequent open calvarial remodeling, early endoscopic surgery may allow for growth and development of the brain and skull while delaying the need for open remodeling until the patient is older and can better tolerate the procedure.

Background Seizure risk has not been fully characterized in pediatric patients with arteriovenous malformations (AVMs). Objective To describe the progression and risk factors of post-treatment seizure in children with AVMs. Methods We retrospectively reviewed pediatric patients diagnosed with intracranial AVMs at our institution between 1990 and 2013. Clinical and angiographic variables were included in univariate and multivariate Cox proportional hazard models to explore risk factors associated with time-related seizure outcomes. The outcome event is defined as first seizure occurrence after initial treatment. Kaplan-Meier survival curve is depicted for each significant variable, and survival differences were confirmed by Log-rank test. Results We included 90 pediatric patients with complete data in our study cohort. Average age is 13.3 ± 3.8 years with 43.3% male patients. Thirty-seven patients had a hemorrhagic presentation, and 39 patients presented with seizure. Post-treatment seizure occurred in 33 patients (36.7%) over an average follow-up period of 8.1 ± 10.6 years; average time to onset is 5.3 ± 8.2 years, with partial seizures as the most common presentation. Multivariate Cox proportional hazard regression revealed seizure presentation ( P = .005), male gender ( P = .026), and nonconservative treatment modality to be significantly associated with earlier onset of post-treatment seizure after adjusting for AVM location and deep venous drainage. Overall annualized seizure risk is 7.4% for patients with pretreatment seizure, and 1.4% for those without. Conclusion Risk of seizure persists after treatment in pediatric AVM patients. Seizure presentation, male gender, and nonconservative management are associated with increased risk of post-treatment seizure. Early identification of these risk factors provides important information for determining seizure management strategies.

OBJECTIVE Subjective evaluations typically guide craniosynostosis repair. This study provides normative values of anthropometric cranial indices that are clinically useful for the evaluation of multiple types of craniosynostosis and introduces 2 new indices that are useful in the evaluation and management of metopic and bicoronal synostosis. The authors hypothesize that normative values of the new indices as well as for established measures like the cephalic index can be drawn from the evaluation of CT scans of normal individuals. METHODS High-resolution 3D CT scans obtained in normal infants (age 0–24 months) were retrospectively reviewed. Calvarial measurements obtained from advanced imaging visualization software were used to compute cranial indices. Additionally, metopic sutures were evaluated for patency or closure. RESULTS A total of 312 participants were included in the study. Each monthly age group (total 24) included 12–18 patients, yielding 324 head CT scans studied. The mean cephalic index decreased from 0.85 at age 0–3 months to 0.81 at 19–24 months, the mean frontoparietal index decreased from 0.68 to 0.65, the metopic index from 0.59 to 0.55, and the towering index remained comparatively uniform at 0.64 and 0.65. Trends were statistically significant for all measured indices. There were no significant differences found in mean cranial indices between sexes in any age group. Metopic suture closure frequency for ages 3, 6, and 9 months were 38.5%, 69.2%, and 100.0%, respectively. CONCLUSIONS Radiographically acquired normative values for anthropometric cranial indices during infancy can be used as standards for guiding preoperative decision making, surgical correction, and postoperative helmeting in various forms of craniosynostosis. Metopic and towering indices represent new cranial indices that are potentially useful for the clinical evaluation of metopic and bicoronal synostoses, respectively. The present study additionally shows that metopic suture closure appears ubiquitous after 9 months of age.

Fetoscopic myelomeningocele (MMC) repair is performed using intrauterine carbon dioxide (CO2 ) insufflation. Sheep experiments have shown that CO2 insufflation is associated with significant fetal acidemia; however, corresponding data for human pregnancy are not available. We performed umbilical venous cord blood sampling in three patients during fetoscopic MMC repair at 25 + 1, 25 + 3 and 24 + 0 weeks' gestation, respectively. Fetal venous pH at the beginning of CO2 insufflation was 7.36, 7.46 and 7.37, respectively in the three fetuses, and repeat values were 7.28, 7.35 and 7.36 after 181, 159 and 149 min, respectively. The partial pressure of oxygen and CO2 was maintained in the normal range during these times, and pH decrease was less in Patient 3 who received humidified CO2 insufflation. Our observations suggest that, in contrast to sheep experiments, CO2 insufflation during fetoscopic myelomeningocele repair does not cause acidemia in human fetuses. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

OBJECTIVES: We sought to characterize patterns of in utero dilation in isolated severe fetal ventriculomegaly (ISVM) and investigate their value in predicting obstetrical and postnatal outcomes. METHODS: This is a retrospective cohort study. ISVM was defined as a sonographic cerebral ventricle atrial width ≥15 mm in the absence of additional cerebral or other anatomic anomalies. The aim of this study was to characterize two ISVM groups using a receiver operator curve to evaluate the rate of ventricular progression versus need for ventriculoperitoneal (VP) shunt postnatally. Outcomes were compared between the groups using Pearson’s chi-squared test, Student t-test, and descriptive statistics. RESULTS: Based on the ROC analysis, ventricular growth of ≥3 mm/week versus

The optimal clinical management and outcomes of rare pediatric spinal aneurysmal bone cysts (spABC) is largely anecdotal. Current practice is based on bigger adult series, although given the disparities in spine growth of adults versus children, what impact this difference may have on long-term outcomes has yet to be substantiated. Correspondingly, the aim of this study was to describe the clinical course of all pediatric spABC cases managed at our institution to better understand this. A retrospective cohort study of all pediatric spABC cases presenting to our institution between 1993 and 2017 was performed using a predetermined set of selection criteria. Primary outcomes of interest were treatment modalities and their outcomes, recurrence status, and functional status. A total of 24 pediatric spABC cases satisfied all criteria. Median age of diagnosis was 13.5 years, with 15 females and 9 males. Radicular pain was the presenting symptom in 21 (88%) cases. Diagnostic biopsy was pursued in 9 (38%) cases, pre-operative embolization in 8 (33%) cases, surgical intervention in 23 (96%) cases, and sclerotherapy in 2 (8%) cases. In terms of surgery, there were no intraoperative complications, and gross total resection (GTR) was achieved in 14 of the 23 (61%) cases. Overall, there were 5 (21%) cases which experienced recurrence by a median time of 8 months after initial surgery, all of which had initial subtotal resection. Median follow-up was 5 years, by which all patients demonstrated excellent functional status. There are a number of feasible therapeutic modalities and combinations that can be utilized to maximize control of pediatric spABCs and optimize long-term function similar to that of adults, irrespective of developing versus developed spines. The incidence of recurrence is not negligible, and therefore, rigorous long-term surveillance is highly encouraged, particularly within the first post-operative year following mono-modal non-GTR treatment.

OBJECTIVEOpen prenatal myelomeningocele (MMC) repair is typically associated with reversal of in utero hindbrain herniation (HBH) and has been posited to be associated with a reduction in both postoperative prenatal and immediate postnatal hydrocephalus (HCP) risks. However, the long-term postnatal risk of HCP following HBH reversal in these cases has not been well defined. The authors describe the results of a long-term HCP surveillance in a cohort of patients who underwent prenatal MMC repair at their institution.METHODSA retrospective review of all prenatal MMC repair operations performed at the Mayo Clinic between 2012 and 2017 was conducted. Pertinent data regarding the clinical courses of these patients before and after MMC repair were summarized. Outcomes of interest were occurrences of HBH and HCP and the need for intervention.RESULTSA total of 9 prenatal MMC repair cases were identified. There were 7 cases in which MRI clearly demonstrated prenatal HBH, and of these 86% (6/7) had evidence of HBH reversal after repair and prior to delivery. After a mean postnatal follow-up of 20 months, there were 3 cases of postnatal HCP requiring intervention. One case that failed to show complete HBH reversal after MMC repair required early ventriculoperitoneal shunting. The other 2 cases were of progressive, gradual-onset HCP despite complete prenatal HBH reversal, requiring endoscopic third ventriculostomy with choroid plexus cauterization at ages 5 and 7 months.CONCLUSIONSAlthough prenatal MMC repair can achieve HBH reversal in a majority of well-selected cases, the prevention of postnatal HCP requiring intervention appears not to be predicated on this outcome alone. In fact, it appears that in a subset of cases in which HBH reversal is achieved, patients can experience a progressive, gradual-onset HCP within the 1st year of life. These findings support continued rigorous postnatal surveillance of all prenatal MMC repair patients, irrespective of postoperative HBH outcome.

Objective To assess our initial experience with prenatal restoration of hindbrain herniation following in utero repair of myelomeningocele (MMC). Patients and Methods Three consecutive patients with prenatally diagnosed MMC (between January 1, 2018 and September 30, 2018) were managed with open in utero surgery. As per institutional review board approval and following a protocol designed at the Mayo Clinic Maternal & Fetal Center, fetal intervention was offered between 19 0/7 and 25 6/7 weeks of gestation. Prenatal improvement of hindbrain herniation was the declared restorative end point. Obstetrical and perinatal outcomes were also assessed. Results Diagnosis of MMC was confirmed upon referral between 20 and 21 weeks' gestation by using fetal ultrasound and magnetic resonance imaging. In all cases reported here, the spinal defect was lumbosacral with evidence of hindbrain herniation. Open in utero MMC repair was performed between 24 and 25 weeks' gestation with no notable perioperative complications. Postprocedure fetal magnetic resonance imaging performed 6 weeks after in utero repair documented improvement of hindbrain herniation. Deliveries were at 37 weeks by cesarean section without complications. Most recent postnatal follow-ups were unremarkable at both 11 months (baby 1) and 3 months of age (baby 2), with mild ventriculomegaly. Antenatal and postnatal follow-up of baby 3 at 1 month of age was also unremarkable. Conclusion Our study highlights the prenatal restoration of hindbrain herniation following in utero MMC repair in all cases presented here as an example of a prenatal regenerative therapy program in our institution.

OBJECTIVE Because the metopic suture normally fuses during infancy, there are varying degrees of severity in head shape abnormalities associated with premature fusion. A method for the objective and reproducible assessment of metopic synostosis is needed to guide management, as current methods are limited by their reliance on aesthetic markers. The object of this study was to describe the metopic index (MI), a simple anthropometric cranial measurement. The measurements can be obtained from CT scans and, more importantly, from palpable cranial landmarks, and the index provides a rapid tool for evaluating patients in both pre- and postoperative settings. METHODS High-resolution head CT scans obtained in 69 patients (age range 0–24 months) diagnosed with metopic craniosynostosis were retrospectively reviewed. Preoperative 3D reconstructions were available in 15 cases, and these were compared with 3D reconstructions of 324 CT scans obtained in a control group of 316 infants (age range 0–24 months) who did not have any condition that might affect head size or shape and also in a subset of this group, comprising 112 patients precisely matched to the craniosynostosis patients with respect to age and sex. Postoperative scans were available and reviewed in 9 of the craniosynostosis patients at a mean time of 7.1 months after surgical repair. 3D reconstructions of these scans were matched with controls based upon age and sex. RESULTS The mean preoperative MI for patients with trigonocephaly was 0.48 (SD 0.05), significantly lower than the mean values of 0.57 (SD 0.04) calculated on the basis of all 324 scans obtained in controls (p < 0.001) and 0.58 (SD 0.04) for the subset of 112 age- and sex-matched controls (p < 0.001). For 7 patients with both pre- and postoperative CT scans available for evaluation, the mean postoperative MI was 0.55 (SD 0.03), significantly greater than their preoperative MIs (mean 0.48 [SD 0.04], p = 0.001) and comparable to the mean MI of the controls (p = 0.30). In 4 patients, clinically obtained postoperative MIs by caliper measurement were comparable to measurements derived from CT (p = 0.141). CONCLUSIONS The MI is a useful measurement of the severity of trigonocephaly in patients with metopic synostosis. This simple quantitative assessment can potentially be used in the clinical setting to guide preoperative evaluation, surgical repair, and postoperative degree of correction.

OBJECTIVE Compared with the general population, the specific natural history of arteriovenous malformations (AVMs) in pediatric patients is less well understood. Furthermore, few pediatric studies have compared posttreatment hemorrhagic risk and functional outcome across different treatment modalities. The objective of this study was to elucidate these points. METHODS The authors retrospectively reviewed all pediatric patients with AVMs evaluated at their institution between 1990 and 2013. The AVM natural history was represented by hemorrhagic risk during the observation period. For treated patients, the observation period was defined as the interval between diagnosis and treatment. Posttreatment hemorrhagic risk and functional outcomes were also assessed. RESULTS A total of 124 pediatric patients with AVMs were evaluated, and 90 patients (72.6%) were retained through follow-up. The average patient age was 13.3 ± 3.8 years, with a mean follow-up period of 9.95 years. The overall AVM obliteration rate was 59.7%. Radiosurgery had an obliteration rate of 49.0%. Thirteen patients were managed conservatively. Four patients under observation hemorrhaged during a total interval of 429.4 patient-years, translating to an annual risk of 0.9%. Posttreatment hemorrhagic risk by treatment modalities were categorized as follows: surgery ± embolization (0.0%), radiosurgery ± embolization (0.8%), embolization alone (2.8%), surgery + radiosurgery ± embolization (3.5%), and observation (0.8%). A significantly higher risk of posttreatment hemorrhage was observed for patients with hemorrhagic presentation (p = 0.043) in multivariate analysis. Seizure presentation, frontal lobe location, nonheadache presentation, and treatment modality were significantly associated with increased risk of poor functional outcomes. CONCLUSIONS In this study of pediatric patients with AVMs, the natural history of hemorrhage was relatively low at 0.9%. Resection remained the optimal management for hemorrhage control and functional outcome perseverance in these pediatric patients with AVMs. AVM obliteration is a valid treatment goal, especially for patients with ruptured presentation, to prevent further hemorrhages later in life.

Although optic pathway gliomas are the most common brain tumors associated with neurofibromatosis type 1 (NF1), extra-optic gliomas occur and may behave more aggressively with outcomes that differ by age. A retrospective case-control study was designed to describe the clinical course of adult NF1 patients with progressive extra-optic pilocytic astrocytomas (PAs) and compare to a pediatric cohort. Data for patients treated at the Johns Hopkins Comprehensive Neurofibromatosis Center from 2003 to 2013 were reviewed to identify cases (adults, age >18) and controls (pediatric, age

OBJECT Chiari Type I malformation involves caudal displacement of the cerebellar tonsils below the foramen magnum, which obstructs normal cerebrospinal fluid flow and increases intracranial pressure. Certain aspects of its surgical treatment remain controversial. A retrospective study was conducted to assess the efficacy of tonsillar cautery on syrinx resolution among pediatric Chiari patients undergoing cervicomedullary decompression. METHODS A retrospective cohort study was performed for patients 0–18 years of age who underwent surgical correction for Chiari Type I malformation with syrinx between 1995 and 2013. Basic demographic information was collected as well as data for preoperative symptoms, prior surgical history, perioperative characteristics, and postsurgical outcomes. Descriptive statistics were performed in addition to bivariate analyses. Candidate predictor variables were identified based on an association with tonsillar cautery with p < 0.10. Forward stepwise likelihood ratio was used to select candidate predictors in a binary logistic regression model (Pin = 0.05, Pout = 0.10) most strongly associated with the outcome. RESULTS A total of 171 patients with Chiari Type I malformation with syrinx were identified, and 43 underwent tonsillar cautery. Patients who underwent tonsillar cautery had 6.11 times greater odds of improvement in their syrinx (95% CI 2.57–14.49, p < 0.001). There was no effect of tonsillar cautery on increased perioperative complications as well as the need for repeat decompressions. CONCLUSIONS Tonsillar cautery is safe and effective in the treatment of Chiari Type I malformation with syrinx and may decrease time to syrinx resolution after cervicomedullary decompression. Tonsillar cautery does not increase postoperative complications in pediatric Chiari Type I malformation patients.

To report a case of pediatric recurrent meningitis that is associated with an underlying petrous apex encephalocele in a patient with bilateral cochlear implants and reviews the literature of similar petrous apex lesions. A 5-year-old girl presented during her third episode of meningitis. Her first episode of meningitis left her with profound bilateral sensorineural hearing loss for which she received cochlear implants. During her assessment, she was found to have a petrous apex encephalocele, which was successfully repaired via a middle cranial fossa approach. Literature review demonstrates that petrous apex cephaloceles (PAC) are associated with meningitis, particularly in children. No previous reports exist of PAC in patients with cochlear implants. This case illustrates the importance of identifying an underlying anatomic abnormality in recurrent meningitis. PAC are rare abnormalities that can cause cerebrospinal fluid leaks leading to intracranial hypotension, cranial neuropathies, and infect...

Intervention (surgery, embolization, and radiosurgery) is critical in maximizing outcomes of pediatric arteriovenous malformations (pAVMs). Although short-term functional outcomes following intervention have been stablished to be favorable, long-term outcomes have yet to be thoroughly consolidated. Searches of 7 electronic databases from inception to April 2019 were conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. Favorable functional were modified Rankin Scale (mRS) scores ranging from 0 to 2, and the incidences were extracted and pooled by random-effects meta-analysis of proportions. Fourteen pertinent studies were identified describing outcomes of 699 pAVM patients, with median 75 % presenting with hemorrhage. Surgery, embolization and radiosurgery use were reported by 12 (86 %), 14 (100 %) and 10 (71 %) studies respectively. By median study follow-up time of 4.1 years, a favorable functional outcome was estimated to occur in 87 % (95 % CI, 82-91 %) of subjects respectively. Hemorrhagic versus non-hemorrhagic presentations did not statistically differ in incidence of this long-term outcome, 78 % (95 % CI, 67-87 %) and 91 % (95 % CI, 80-98 %) respectively. This study demonstrates that favorable long-term functional outlook of pAVM subjects after intervention can persist for many years after initial intervention. The certainty of achieving this outcome is moderate, irrespective of hemorrhagic presentation or intervention modality. Long-term functional deficit risk should not be the sole factor in deciding if intervention should be pursued.

To analyze the impact of an intervention of using telemedicine during interhospital transport on time to surgery in children with operative intracranial hemorrhage.We performed a retrospective chart review of children with intracranial hemorrhage transferred for emergent neurosurgical intervention between January 1, 2011 and December 31, 2016. We identified those patients whose neuroimaging was transmitted via telemedicine to the neurosurgical team prior to arrival at our center and then compared the telemedicine and nontelemedicine groups. Mann-Whitney U and Fisher exact tests were used to compare interval variables and categorical data.Single-center study performed at Johns Hopkins Hospital.Patients less than or equal to 18 years old transferred for operative intracranial hemorrhage.Pediatric transport implemented routine telemedicine use via departmental smart phones to facilitate transfer of information and imaging and reduce time to definitive care by having surgical services available when needed.Fifteen children (eight in telemedicine group; seven in nontelemedicine group) met inclusion criteria. Most had extraaxial hemorrhage (87.5% telemedicine group; 85.7% nontelemedicine group; p = 1.0), were intubated pre transport (62.5% telemedicine group; 71.4% nontelemedicine group; p = 1.0), and arrived at our center's trauma bay during night shift or weekend (87.5% telemedicine group; 57.1% nontelemedicine group; p = 0.28). Median trauma bay Glasgow Coma Scale scores did not differ (eight in telemedicine group; seven in nontelemedicine group; p = 0.24). Although nonsignificant, when compared with the nontelemedicine group, the telemedicine group had decreased rates of repeat preoperative neuroimaging (37.5% vs 57%; p = 0.62), shorter median times from trauma bay arrival to surgery (33 min vs 47 min; p = 0.22) and from diagnosis to surgery (146.5 min vs 157 min; p = 0.45), shorter intensive care stay (2.5 vs 5 d) and hospitalization (4 vs 5 d), and higher home discharge rates (87.5% vs 57.1%; p = 0.28).Telemedicine use during interhospital transport appears to expedite definitive care for children with intracranial hemorrhage requiring emergent neurosurgical intervention, which could contribute to improved patient outcomes.

OBJECTIVEComplications after complex tethered spinal cord (cTSC) surgery include infections and cerebrospinal fluid (CSF) leaks. With little empirical evidence to guide management, there is variability in the interventions undertaken to limit complications. Expert-based best practices may improve the care of patients undergoing cTSC surgery. Here, authors conducted a study to identify consensus-driven best practices.METHODSThe Delphi method was employed to identify consensual best practices. A literature review regarding cTSC surgery together with a survey of current practices was distributed to 17 board-certified pediatric neurosurgeons. Thirty statements were then formulated and distributed to the group. Results of the second survey were discussed during an in-person meeting leading to further consensus, which was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree).RESULTSSeventeen consensus-driven best practices were identified, with all participants willing to incorporate them into their practice. There were four preoperative interventions: (1, 2) asymptomatic AND symptomatic patients should be referred to urology preoperatively, (3, 4) routine preoperative urine cultures are not necessary for asymptomatic AND symptomatic patients. There were nine intraoperative interventions: (5) patients should receive perioperative cefazolin or an equivalent alternative in the event of allergy, (6) chlorhexidine-based skin preparation is the preferred regimen, (7) saline irrigation should be used intermittently throughout the case, (8) antibiotic-containing irrigation should be used following dural closure, (9) a nonlocking running suture technique should be used for dural closure, (10) dural graft overlay should be used when unable to obtain primary dural closure, (11) an expansile dural graft should be incorporated in cases of lipomyelomeningocele in which primary dural closure does not permit free flow of CSF, (12) paraxial muscles should be closed as a layer separate from the fascia, (13) routine placement of postoperative drains is not necessary. There were three postoperative interventions: (14) postoperative antibiotics are an option and, if given, should be discontinued within 24 hours; (15) patients should remain flat for at least 24 hours postoperatively; (16) routine use of abdominal binders or other compressive devices postoperatively is not necessary. One intervention was prioritized for additional study: (17) further study of additional gram-negative perioperative coverage is needed.CONCLUSIONSA modified Delphi technique was used to develop consensus-driven best practices for decreasing wound complications after cTSC surgery. Further study is required to determine if implementation of these practices will lead to reduced complications. Discussion through the course of this study resulted in the initiation of a multicenter study of gram-negative surgical site infections in cTSC surgery.

OBJECTIVEMany infants with sagittal craniosynostosis undergo effective surgical correction with endoscopic strip craniectomy (ESC) and postoperative helmet therapy (PHT). While PHT is essential to achieving optimal cosmesis following ESC, there has been little comprehensive analysis of the ideal PHT duration needed to attain this goal.METHODSThe authors retrospectively reviewed the charts of infants undergoing ESC and PHT for sagittal synostosis at our institution between 2008 and 2015. Data collected included age at surgery, follow-up duration, and PHT duration. Cephalic index (CI) was evaluated preoperatively (CIpre), at its peak level (CImax), at termination of helmet therapy (CIoff), and at last follow-up (CIfinal). A multivariate regression analysis was performed to determine factors influencing CIfinal.RESULTSThirty-one patients (27 male, 4 female) were treated in the studied time period. The median age at surgery was 2.7 months (range 1.6 to 3.2) and the median duration of PHT was 10.4 months (range 8.4 to 14.4). The mean CImax was 0.83 (SD 0.01), which was attained an average of 8.4 months (SD 1.2) following PHT initiation. At last follow-up, there was an average retraction of CIfinal among all patients to 0.78 (SD 0.01). Longer helmet duration after achieving CImax did not correlate with higher CIfinal values. While CImax was a significant predictor of CIfinal, neither age at surgery nor CIpre were found to be predictive of final outcome.CONCLUSIONSPatients undergoing ESC and PHT for sagittal synostosis reach a peak CI around 7 to 9 months after surgery. PHT beyond CImax does not improve final anthropometric outcomes. CIfinal is significantly dependent on CImax, but not on age, nor CIpre. These results imply that helmet removal at CImax may be appropriate for ESC patients, while helmeting beyond the peak does not change final outcome.

Highlights • Factor VII deficiency is a rare autosomal disorder with genotypic and phenotypic variability. • Preoperative lab evaluation should at a minimum consist of a hematocrit, platelet count, type and screen, and coagulation studies for high-risk surgeries. • Treatment of acute hemorrhage in factor VII deficient patients primarily consists of factor VII (FVII) replacement therapy., Introduction Congenital factor VII deficiency is a rare bleeding disorder with high phenotypic variability. It is critical that children with congenital Factor VII deficiency be identified early when high-risk surgery is planned. Cranial vault surgery is common for children with craniosynostosis, and these surgeries are associated with significant morbidity mostly secondary to the risk of massive blood loss. Presentation of case A two-month old infant who presented for elective craniosynostosis repair was noted to have an elevated prothrombin time (PT) with a normal activated partial thromboplastin time (aPTT) on preoperative labs. The infant had no clinical history or reported family history of bleeding disorders, therefore a multidisciplinary decision was made to repeat the labs under general anesthesia and await the results prior to incision. The results confirmed the abnormal PT and the case was canceled. Hematologic workup during admission revealed factor VII deficiency. The patient underwent an uneventful endoscopic strip craniectomy with perioperative administration of recombinant Factor VIIa. Discussion Important considerations for perioperative laboratory evaluation and management in children with factor VII deficiency are discussed. Anesthetic and surgical management of the child with factor VII deficiency necessitates meticulous planning to prevent life threatening bleeding during the perioperative period. Conclusion A thorough history and physical examination with a high clinical suspicion are vital in preventing hemorrhage during surgeries in children with coagulopathies. Abnormal preoperative lab values should always be confirmed and addressed before proceeding with high-risk surgery. A multidisciplinary discussion is essential to optimize the risk-benefit ratio during the perioperative period.

Introduction Children born premature who require ventricular shunt placement for post hemorrhagic hydrocephalus are at increased risk for neurodevelopmental delay. We hypothesized that preterm infants with bronchopulmonary dysplasia (BPD) who require ventricular shunt (VS) placement are at additive risk for long-term respiratory morbidities due to their higher likelihood of neurodevelopmental delay. We also hypothesized that children with BPD and ventricular shunts would require frequent shunt revisions during early childhood following initial shunt placement. Methods All subjects were recruited from the Johns Hopkins Bronchopulmonary Dysplasia Clinic between January 2008 and November 2016. A review of demographic and clinical information was undertaken and a respiratory outcomes questionnaire was completed by the primary caregiver. Results Fifty-five (8.9%) of 623 subjects in the study population had ventricular shunts, with a mean 4.6 ± 2.3 years of follow-up data (range: 0.5-10.1). Subjects with VS were more likely to be born at earlier gestational ages and be discharged on supplemental oxygen compared to BPD subjects alone. Outpatient respiratory symptoms and acute care usage were similar between the two groups. BPD subjects with VS were more likely to have had gastric tubes placed. A total of 270 neurosurgical procedures were performed in subjects with VS with a mean of 4.9 ± 5.4 procedures/subject. Conclusion Children with ventricular shunts and BPD had similar outpatient respiratory outcomes compared to children with BPD alone; however respiratory morbidities may have been mitigated by the greater use of gastric tubes in the VS + BPD group. Multiple neurosurgical procedures were common in children with ventricular shunts.

Introduction. This study investigates radiographically acquired normative ranges of anterior fontanelle closure (AFC) and surface area (SA) in healthy full-term infants. Methods. High-resolution head computed tomography (CT) scans were retrospectively reviewed for AFC and AF dimensions to allow approximation of AF SA. Results. Between 15 and 23 head CT scans per monthly age-group (0-24 months) were reviewed, totaling 464 scans. AFC frequency increased steadily from age 10 (16%) to 20 months (88%), reaching higher than 50% at age 16 months (53%). The AF was closed in 3% to 5% of infants at 5 to 6 months. AF median SA increased from 769.3 mm2(age 0 months) to 1022.2 mm2(2 months), then declined steadily. Conclusions. This study provides reference charts detailing AFC frequency and AF SA as a function of age. Wide variability of AFC timing and AF size among healthy infants suggest that early or delayed AFC may represent normal variants.

Object Civilian gunshot wounds to the head (GSWH) are often deadly, but some patients with open cranial wounds need medical and surgical management and are potentially good candidates for acceptable functional recovery. The authors analyzed predictors of favorable clinical outcome (Glasgow Outcome Scale scores of 4 and 5) after GSWH over a 24-month period. Methods The authors posited 2 questions: First, what percentage of civilians with GSWH died in the state of Maryland in a given period of time? Second, what were the predictors of favorable outcome after GSWH? The authors examined demographic, clinical, imaging, and acute care data for 786 civilians who sustained GSWH. Univariate and logistic regression analyses were used to analyze the data. Results Of the 786 patients in this series, 712 (91%) died and 74 (9%) completed acute care in 9 trauma centers. Of the 69 patients admitted to one Maryland center, 46 (67%) eventually died. In 48 patients who were resuscitated, the Injury Severity Score was 26.2, Glasgow Coma Scale (GCS) score was 7.8, and an abnormal pupillary response (APR) to light was present in 41% of patients. Computed tomography indicated midline shift in 17%, obliteration of basal cisterns in 41.3%, intracranial hematomas in 34.8%, and intraventricular hemorrhage in 49% of cases. When analyzed for trajectory, 57.5% of bullet slugs crossed midcoronal, midsagittal, or both planes. Two subsets of admissions were studied: 27 patients (65%) who had poor outcome (25 patients who died and 2 who had severe disability) and 15 patients (35%) who had a favorable outcome when followed for a mean period of 40.6 months. Six patients were lost to follow-up. Univariate analysis indicated that admission GCS score (p < 0.001), missile trajectory (p < 0.001), surgery (p < 0.001), APR to light (p = 0.002), patency of basal cisterns (p = 0.01), age (p = 0.01), and intraventricular bleed (p = 0.03) had a significant relationship to outcome. Multivariable logistic regression analysis indicated that GCS score and patency of the basal cistern were significant determinants of outcome. Exclusion of GCS score from the regression models indicated missile trajectory and APR to light were significant in determining outcome. Conclusions Admission GCS score, trajectory of the missile track, APR to light, and patency of basal cisterns were significant determinants of outcome in civilian GSWH.

Macrocephaly is a rare presentation of untreated hydrocephalus. In cases where medical management has failed, macrocephaly can be managed with reduction cranioplasty. Traditionally, reduction cranioplasty is highly dependent on intraoperative tailoring of bone segments to obtain the best possible result and involves high risks of associated severe blood loss and mortality. In this clinical report, we describe a patient with hydrocephalus macrocephaly treated with reduction cranioplasty with the innovative use of computer-aided design and manufacturing to reduce intraoperative risks and improve efficiency. We used computer-aided design to plan osteotomy sites and the final positioning of bone segments. We also utilized computer-aided manufacturing to produce cutting guides, positioning guides, and models to increase precision and improve the final positioning of the cranium. Computer-aided design and manufacturing technology has enabled a shift of the planning burden of complicated craniofacial reconstructions from the intraoperative to the preoperative phase. With a completed plan and premade guides and models, it is possible to minimize the risks, improve efficiency, and obtain a precise, aesthetic result.

OBJECTIVE Due to the changing properties of the infant skull, there is still no clear consensus on the ideal time to surgically intervene in cases of nonsyndromic craniosynostosis (NSC). This study aims to shed light on how patient age at the time of surgery may affect surgical outcomes and the subsequent need for reoperation. METHODS A retrospective cohort review was conducted for patients with NSC who underwent primary cranial vault remodeling between 1990 and 2013. Patients' demographic and clinical characteristics and surgical interventions were recorded. Postoperative outcomes were assessed by assigning each procedure to a Whitaker category. Multivariate logistic regression analysis was performed to determine the relationship between age at surgery and need for minor (Whitaker I or II) versus major (Whitaker III or IV) reoperation. Odds ratios (ORs) for Whitaker category by age at surgery were assigned. RESULTS A total of 413 unique patients underwent cranial vault remodeling procedures for NSC during the study period. Multivariate logistic regression demonstrated increased odds of requiring major surgical revisions (Whitaker III or IV) in patients younger than 6 months of age (OR 2.49, 95% CI 1.05–5.93), and increased odds of requiring minimal surgical revisions (Whitaker I or II) in patients older than 6 months of age (OR 2.72, 95% CI 1.16–6.41). CONCLUSIONS Timing, as a proxy for the changing properties of the infant skull, is an important factor to consider when planning vault reconstruction in NSC. The data presented in this study demonstrate that patients operated on before 6 months of age had increased odds of requiring major surgical revisions.

Recently a two-port open fetoscopic myelomeningocele (MMC) repair technique, performed together by a pediatric neurosurgeon and maternal-fetal medicine specialist was described.1 Acquiring skills for this type of fetoscopic surgery is complex since MMC lesions are anatomically variable and collaboration of two surgical specialists is required. We utilized ultrasound-based 3D multi-material printing to generate a patient–matched medical model to prepare the surgical team for fetoscopic MMC repair.