Visual outcomes after treatment of craniopharyngioma in children: A systematic review.

OBJECTIVE: Craniopharyngiomas (CP) are rare brain tumors that often result in visual impairment due to their proximity to the optic pathway. The optimal management approach to preserve visual function in these patients remains controversial. We sought to investigate visual outcomes of children with craniopharyngiomas based on treatment modality. METHODS: A systematic review was performed according to PRISMA guidelines. PubMed, Embase, and Scopus databases were searched in December 2022 for relevant articles. Articles were screened by title/abstract for relevance, then by full-text. Relevant demographic, intervention, and outcome data were extracted from included studies. RESULTS: A total of 59 studies were included, representing 2655 patients. The overall visual status (OVS) of patients receiving surgery alone was improved in 27.6% of reported outcomes, unchanged in 50.3%, and deteriorated in 22.1%. The OVS for patients receiving radiation alone was improved in 21.1%, unchanged in 42.1%, and deteriorated in 36.8%. Patients receiving surgery plus adjuvant radiotherapy had OVS improvement in 27.4%, unchanged in 63.2%, and deteriorated in 9.4%. Of those receiving intracystic bleomycin, 23.1% had improvement in OVS, 46.2% remained unchanged, and 30.8% deteriorated. Of patients receiving interferon-α, 34.8% improved, 54.5% remained unchanged, and 10.6% deteriorated. CONCLUSION: OVS most frequently remained unchanged regardless of intervention. The greatest improvement in OVS was seen in those receiving interferon-α or surgery alone. The greatest OVS deterioration was noted with radiation alone. Future standardized, randomized, large-scale studies with focused assessment of ophthalmologic findings are key to further understanding the impact different interventions have on visual outcomes in these children.

Experience in endoscope choice for neuroendoscopic lavage for intraventricular hemorrhage of prematurity: a systematic review.

OBJECTIVE: Intraventricular hemorrhage (IVH) of prematurity occurs in 20-38% of infants born < 28 weeks gestational age and 15% of infants born in 28-32 weeks gestational age. Treatment has evolved from conservative management and CSF diversion of temporizing and shunting procedures to include strategies aimed at primarily clearing intraventricular blood products. Neuroendoscopic lavage (NEL) aims to decrease the intraventricular blood burden under the same anesthetic as temporizing CSF diversion measures in cases of hydrocephalus from IVH of prematurity. Given the variety of neuroendoscopes, we sought to review the literature and practical considerations to help guide neuroendoscope selection when planning NEL. METHODS: We conducted a systematic review of the literature on neuroendoscopic lavage in IVH of prematurity to examine data on the choice of neuroendoscope and outcomes regarding shunt rate. We then collected manufacturer data on neuroendoscopic devices, including inflow and outflow mechanisms, working channel specifications, and tools compatible with the working channel. We paired this information with the advantages and disadvantages reported in the literature and observations from the experiences of pediatric neurosurgeons from several institutions to provide a pragmatic evaluation of international clinical experience with each neuroendoscope in NEL. RESULTS: Eight studies were identified; four neuroendoscopes have been used for NEL as reported in the literature. These include the Karl Storz Flexible Neuroendoscope, LOTTA® system, GAAB system, and Aesculap MINOP® system. The LOTTA® and MINOP® systems were similar in setup and instrument options. Positive neuroendoscope features for NEL include increased degrees of visualization, better visualization with the evolution of light and camera sources, the ability to sterilize with autoclave processes, balanced inflow and outflow mechanisms via separate channels, and a working channel. Neuroendoscope disadvantages for NEL may include special sterilization requirements, large outer diameter, and limitations in working channels. CONCLUSIONS: A neuroendoscope integrating continuous irrigation, characterized by measured inflow and outflow via separate channels and multiple associated instruments, appears to be the most commonly used technology in the literature. As neuroendoscopes evolve, maximizing clear visualization, adequate inflow, measured outflow, and large enough working channels for paired instrumentation while minimizing the footprint of the outer diameter will be most advantageous when applied for NEL in premature infants.

Patient family engagement and partnership: Pilot survey results in assessing behavior, communication, and quality of life in children with Lennox-Gastaut syndrome and other drug-resistant epilepsy.

OBJECTIVES: Lennox-Gastaut Syndrome (LGS) and other drug-resistant epilepsy (DRE) can impact behavior, communication, and quality of life (QoL). In collaboration with community engagement efforts with the Lennox-Gastaut Syndrome Foundation (LGSF), we aimed to gain an initial snapshot of patient and family perspectives and experiences with evaluation of behavior, communication, and QoL. METHODS: A cross-sectional survey was conducted to collect self-reported information from caregivers of children with LGS and other DRE regarding their perspectives and experiences with healthcare providers' evaluation of behavior, communication, and QoL. The survey tool was developed by the study investigators in partnership with the LGS Foundation and had diffused to caregivers online by epilepsy advocacy groups including the Pediatric Epilepsy Surgery Alliance (PESA). Responses were analyzed. Descriptive statistics were calculated. The survey asked for caregiver perspectives and assessed which instruments the caregivers had previously been given for measuring these domains. RESULTS: Responses from 245 caregivers were included, with 132 (54%) caregivers of an individual with LGS and 113 (46%) caregivers of an individual with non-LGS related DRE. Respondents reported that 66% of their loved ones had undergone epilepsy-related surgery. Over 90% agreed that measuring behavior, communication, and QoL was important, but fewer than half felt that their healthcare providers evaluated these domains well. LGS caregivers largely shared non-LGS caregivers' perspectives; however, they reported more frequently that communication was not evaluated enough. Barriers to measuring these domains included a lack of good surveys (developmentally appropriate and specific to the type of epilepsy) or not receiving any survey instruments for these domains during clinic appointments. Caregivers play a crucial role for individuals with DRE, and their input is essential in identifying challenges and needs. Caregivers believe that measuring behavior, communication, and quality of life is important, and most of them feel that their loved ones are not adequately evaluated during their healthcare encounters. There is a need for appropriately scaled survey instruments to measure areas of importance for patients and caregivers, as well as incorporation of these outcomes in the healthcare discussion.

Disparities in pediatric drug-resistant epilepsy care.

INTRODUCTION: Epilepsy affects millions of children worldwide, with 20-40% experiencing drug-resistant epilepsy (DRE) who are recommended for epilepsy surgery evaluation and may benefit from surgical management. However, many patients live with DRE for multiple years prior to surgical epilepsy referral or treatment or are never referred at all. OBJECTIVE: We aimed to describe factors associated with referral for epilepsy surgery in the USA, in order to identify disparities in DRE, characterize why they may exist, and recognize areas for improvement. METHODS: Pediatric patients diagnosed with DRE between January 1, 2004 and December 31, 2020 were identified from the Pediatric Health Information System (PHIS) Database. Patients treated with antiseizure medications (ASMs) only, ASMs plus vagus nerve stimulation (VNS), and ASMs plus cranial epilepsy surgery were studied regarding access to epilepsy surgery and disparities in care. This study used chi-square tests to determine associations between treatment time and preoperative factors. Preoperative factors studied included epilepsy treatment type, age, sex, race/ethnicity, insurance type, geographic region, patient type, epilepsy type, and presence of pediatric complex chronic conditions (PCCCs). RESULTS: A total of 18,292 patients were identified; 10,240 treated with ASMs, 5019 treated with ASMs + VNS, and 3033 treated with ASMs + cranial epilepsy surgery. Sex was not found to significantly vary among groups. There was significant variation in age, census region, race/ethnicity, patient type, presence of PCCCs, diagnosis, and insurance (p < 0.001). Those treated surgically, either with VNS or cranial epilepsy surgery, were 2 years older than those medically treated. Additionally, those medically treated were less likely to be living in the Midwest (25.46%), identified as non-Hispanic white (51.78%), have a focal/partial epilepsy diagnosis (8.74%), and be privately insured (35.82%). CONCLUSIONS: We studied a large administrative US database examining variables associated with surgical epilepsy evaluation and management. We found significant variation in treatment associated with age, US census region, race/ethnicity, patient type, presence of PCCCs, diagnosis, and health insurance type. We believe that these disparities in care are related to access and social determinants of health, and we encourage focused outreach strategies to mitigate these disparities to broaden access and improve outcomes in children in the USA with DRE.

Vagus nerve stimulator revision in pediatric epilepsy patients: a technical note and case series.

INTRODUCTION: Vagus nerve stimulation (VNS) is an adjunctive treatment in children with intractable epilepsy. When lead replacement becomes necessary, the old leads are often truncated and retained and new leads are implanted at a newly exposed segment of the nerve. Direct lead removal and replacement are infrequently described, with outcomes poorly characterized. We aimed to describe our experience with feasibility of VNS lead removal and replacement in pediatric patients. METHODS: Retrospective review examined 14 patients, at a single, tertiary-care, children's hospital, who underwent surgery to replace VNS leads, with complete removal of the existing lead from the vagus nerve and placement of a new lead on the same segment of the vagus nerve, via blunt and sharp dissection without use of electrocautery. Preoperative characteristics, stimulation parameters, and outcomes were collected. RESULTS: Mean age at initial VNS placement was 7.6 years (SD 3.5, range 4.5-13.4). Most common etiologies of epilepsy were genetic (5, 36%) and cryptogenic (4, 29%). Lead replacement was performed at a mean of 6.0 years (SD 3.8, range 2.1-11.7) following initial VNS placement. Reasons for revision included VNS lead breakage or malfunction. There were no perioperative complications, including surgical site infection, voice changes, dysphagia, or new deficits postoperatively. Stimulation parameters after replacement surgery at last follow-up were similar compared to preoperatively, with final stimulation parameters ranging from 0.25 mA higher to 1.5 mA lower to maintain baseline seizure control. The mean length of follow-up was 7.9 years (SD 3.5, range 3.1-13.7). CONCLUSION: Removal and replacement of VNS leads are feasible and can be safely performed in children. Further characterization of surgical technique, associated risk, impact on stimulation parameters, and long-term outcomes are needed to inform best practices in VNS revision.

The role of blood product removal in intraventricular hemorrhage of prematurity: a meta-analysis of the clinical evidence.

INTRODUCTION: Premature neonates have a high risk of intraventricular hemorrhage (IVH) at birth, the blood products of which activate inflammatory cascades that can cause hydrocephalus and long-term neurological morbidities and sequelae. However, there is no consensus for one treatment strategy. While the mainstay of treatment involves CSF diversion to reduce intracranial pressure, a number of interventions focus on blood product removal at various stages including extraventricular drains (EVD), intra-ventricular thrombolytics, drainage-irrigation-fibrinolytic therapy (DRIFT), and neuroendoscopic lavage (NEL). METHODS: We performed a systematic review and meta-analysis to compare the risks and benefits commonly associated with active blood product removal treatment strategies. We searched MEDLINE, Embase, Scopus, Cochrane Library, and CINAHL databases through Dec 2020 for articles reporting on outcomes of EVDs, thrombolytics, DRIFT, and NEL. Outcomes of interest were rate of conversion to ventriculoperitoneal shunt (VPS), infection, mortality, secondary hemorrhage, and cognitive disability. RESULTS: Of the 10,398 articles identified in the search, 23 full-text articles representing 22 cohorts and 530 patients were included for meta-analysis. These articles included retrospective, prospective, and randomized controlled studies on the use of EVDs (n = 7), thrombolytics (n = 8), DRIFT therapy (n = 3), and NEL (n = 5). Pooled rates of reported outcomes for EVD, thrombolytics, DRIFT, and NEL for ventriculoperitoneal shunt (VPS) placement were 51.1%, 43.3%, 34.3%, and 54.8%; for infection, 15.4%, 12.5%, 4.7%, and 11.0%; for mortality, 20.0%, 11.6%, 6.0%, and 4.9%; for secondary hemorrhage, 5.8%, 7.8%, 20.0%, and 6.9%; for cognitive impairment, 52.6%, 50.0%, 53.7%, and 50.9%. Meta-regression using type of treatment as a categorical covariate showed no effect of treatment modality on rate of VPS conversion or cognitive disability. CONCLUSION: There was a significant effect of treatment modality on secondary hemorrhage and mortality; however, mortality was no longer significant after adjusting for year of publication. Re-hemorrhage rate was significantly higher for DRIFT (p < 0.001) but did not differ among the other modalities. NEL also had lower mortality relative to EVD (p < 0.001) and thrombolytics (p = 0.013), which was no longer significant after adjusting for year of publication. Thus, NEL appears to be safer than DRIFT in terms of risk of hemorrhage, and not different than other blood-product removal strategies in terms of mortality. Outcomes-in terms of shunting and cognitive impairment-did not differ. Later year of publication was predictive of lower rates of mortality, but not the other outcome variables. Further prospective and randomized studies will be necessary to directly compare NEL with other temporizing procedures.

Arteriovenous Malformations-Associated Epilepsy in Pediatrics.

PURPOSE: Seizures are the second most common presentation of cerebral arteriovenous malformations (AVMs); pediatric patients are more likely to develop AVM-associated epilepsy. We examined the role of multimodality AVM treatment in pediatric AVM-associated epilepsy to characterize long-term epilepsy outcomes. METHODS: A retrospective chart review identified pediatric patients with AVM-associated epilepsy seen at our institution from 2005 to 2018. Variables measured included demographic and descriptive data. Primary outcomes included seizure freedom, seizure control, and functional outcomes. RESULTS: Of 105 pediatric patients with AVMs, 18 had AVM-related epilepsy. Thirteen underwent surgical resection, of which 6 underwent preoperative embolization. Twelve (92.31%) had complete resection; one (7.69%) with residual underwent redo craniotomy with subsequent complete resection. All had radiographic cure at most recent follow-up, with no recurrence seen during length of follow-up (mean 2.17 years, SD 1.40, range 0.25-4.41). Eight (61.54%) experienced seizure freedom postoperatively; 12 (92.31%) were modified Engel Class I at last follow-up. Five patients underwent treatment without open surgical resection, with conservative management (3, 60%) or endovascular embolization (2, 40%). None in our cohort underwent radiosurgery. Of those embolized, one had complete AVM obliteration and two had partial obliteration. Four of the 5 patients (80%) treated without open surgery achieved seizure freedom. CONCLUSION: Long-term outcomes of AVM-related epilepsy are poorly characterized in children. We found that in addition to improved AVM outcomes regarding obliteration, treatment of residual, and recurrence, pediatric patients undergoing surgical AVM treatment had improved AVM-associated epilepsy outcomes, with 61.54% achieving seizure freedom and 92.31% classified as modified Engel Class I seizure control.

Predicting postoperative tracheostomy requirement in children undergoing surgery for posterior fossa tumors.

PURPOSE: Posterior fossa tumor (PFT) resection can be associated with postoperative respiratory failure. We aimed to identify risk factors predicting tracheostomy dependence in children after PFT resection. METHODS: Retrospective chart review of all children undergoing PFT resection from April 2007 to May 2017 at our institution was performed. RESULTS: A total of 197 patients were included; 12 (6.1%) required tracheostomy placement at a mean 69.1 days postoperatively (SD 112.7, range 7-388). Patients requiring tracheostomy were younger (3.4 vs. 6.8 years, p < 0.01), more likely to have postoperative dysphagia (91.7% vs. 17.3%, p < 0.01), and more likely to have an ependymoma (41.7% vs. 15.1%, p < 0.01) or astrocytoma (25.0% vs. 8.1%, p < 0.01). Patients with eventual tracheostomy were less likely extubated immediately postoperatively (45.5% vs. 79.6%, p < 0.01), had longer intubation duration postoperatively (5.7 vs. 0.5 days, p < 0.01), and had higher rates of reintubation within 48 h (63.6% vs. 1.3%, p < 0.01). Patients requiring tracheostomy had longer hospital length of stay (45.8 vs. 15.3 days, p < 0.01) and ICU stay postoperatively (13.5 vs. 2.1 days, p < 0.01). Of those requiring tracheostomy, three (25.0%) were decannulated by 1 year postoperatively. Decannulation rates did not vary by age (p < 0.47), extubation failure (p < 0.24), duration of intubation (p < 0.10), tumor histology (p < 0.23), or tumor grade (p < 0.13). CONCLUSION: Lower cranial neuropathy following PFT resection is common. Identifying risk factors correlated with need for tracheostomy can help identify patients who may benefit from early tracheostomy, reducing prolonged intubation trauma, time on mechanical ventilation, and length of stay.

Natural history of high-grade pediatric arteriovenous malformations: implications for management options.

PURPOSE: Cerebral arteriovenous malformations are a common cause of pediatric intracranial hemorrhage. Often, small, superficial, lesions are treated surgically; however, more complex, deeper, eloquently located lesions portend higher-risk features and suffer from limitations in treatment. We sought to examine our institution's experience with the natural history of these high-grade arteriovenous malformations to explore outcomes with conservative treatment. METHODS: A retrospective chart review was performed to identify all pediatric cases of intracranial arteriovenous malformations seen at our institution from 2005 to 2018. Subjects with Spetzler-Martin grade IV or V lesions, treated conservatively, were examined for primary outcomes including rupture rate, progression, and functional outcomes. RESULTS: A total of 14 patients were included in the study, of which, 78.57% were classified as Spetzler-Martin grade IV and 21.43% Spetzler-Martin grade V. All patients in this study were treated conservatively, with surveillance, followed for a mean of 32.17 months (range 9.43-79.10). 7.14% experienced delayed hemorrhage or re-rupture, 7.14% had hydrocephalus, and 14.29% had seizures. Neurological sequelae included weakness, visual impairment, speech impairment, sensory changes, and dystonia; functionally independent outcomes, defined as modified Rankin Score of 0-2, were seen in 85.71% of patients. CONCLUSION: Our experience suggests that patients with large, deep lesions have significant morbidity with high rates of rupture and subsequent neurologic deficits. However, intervention of these lesions may carry high risk, and the literature suggests such lesions may have less favorable outcomes when treated. We propose conservative treatment for high-grade arteriovenous malformations as a viable option with good functional outcomes in a cohort often without good options for conventional treatment.

Surgical strategies for management of pediatric arteriovenous malformation rupture: the role of initial decompressive craniectomy.

INTRODUCTION: Arteriovenous malformations (AVMs) are a common cause of intracranial hemorrhage in children, which can result in elevated intracranial pressure (ICP) and cerebral edema. We sought to explore the role of initial decompressive craniectomy at time of rupture, followed by interval surgical AVM resection, compared to treatment with initial resection, in clinical outcomes and recovery in children. METHODS: A retrospective chart review was conducted examining patients age 0-18 with AVM rupture between 2005 and 2018 who underwent resection for ruptured AVM either initially at presentation or underwent initial decompressive craniectomy followed by interval AVM resection. Clinical, radiographic, surgical, and outcome data were examined. Primary outcomes measured included functional status, AVM obliteration rate, AVM recurrence/residual, and re-hemorrhage. RESULTS: Thirty-six cases were included; 28 (77.8%) underwent initial AVM resection, and 7 (19.4%) underwent initial decompressive craniectomy with interval resection. The mean time between craniectomy and resection was 66.9 days (SD 59.3). Patients undergoing initial decompressive craniectomy with interval resection were younger (mean age 6.1 vs. 9.8 years, p = 0.05) and had a higher mean hematoma volume (52.9 vs. 22.2 mL, p = 0.01), mean midline shift (5.1 vs. 2.1 mm, p = 0.01), and presence of cisternal effacement (p = 0.01). There were no statistically significant associations between surgical strategy and postoperative outcomes, including complications, radiographic outcomes, complete resection, residual, recurrence, and functional outcomes. Those treated by initial craniectomy followed by interval resection were associated with undergoing additional procedures. CONCLUSIONS: Children presenting with AVM rupture who require emergent decompression may safely undergo emergent craniectomy with interval AVM resection and cranioplasty without additional risk of morbidity or mortality. This is reasonable in those with elevated intracranial pressure. This strategy may provide time for initial recovery and allow for natural degradation of the hematoma enhancing the plane for interval AVM resection, perhaps improving outcomes.

The role of spinal angiography in the evaluation and treatment of pediatric spinal vascular pathology: a case series and systematic review.

OBJECT: While it has been extensively studied in adults, the role of spinal angiography in children with suspected spinal vascular malformations is not fully characterized. With special implications regarding technique, radiation dose, and pathology, we sought to review our single-center experience with pediatric spinal vascular pathology and use a systematic review of the literature to further identify its role in pediatrics. METHODS: A retrospective chart review was conducted under IRB approval for all patients age 0-18 years old who underwent spinal angiography at our institution between 2007 and 2018 for concern for spinal vascular pathology. A simultaneous systematic review was conducted via dedicated search terms in two distinct databases and reviewed to identify all studies referring to spinal angiography or angiograms in pediatric patients. RESULTS: Six patients were included. Three patients (50%) had vascular malformations confirmed on diagnostic angiography and underwent subsequent endovascular treatment. Two patients (33.3%), one with hematomyelia and one with spinal cord infarction, had negative diagnostic angiograms. One patient (16.7%) had a spinal tumor and underwent angiography for further evaluation preoperatively. Spinal angiography was used to aid in diagnosis, preoperative planning, and treatment in these cases. It was demonstrated to be safe in this patient population, with no untoward events, minimal radiation dose, and possible therapeutic applications in select cases. The systematic review identified 11 studies regarding pediatric spinal angiography. These ranged from single case reports to case series of up to 38 patients and highlighted the role of spinal angiography in diagnosis, endovascular treatment, preoperative planning, and postoperative follow-up. CONCLUSIONS: Spinal angiography may be used in a variety of scenarios to better understand the architecture of vascular spinal lesions and facilitate endovascular intervention. While its application in both adult and pediatric pathology is limited to select cases, spinal angiography remains a key diagnostic procedure when approaching vascular lesions or tumors of the spine, assessing for etiology of spinal cord infarcts, and in the evaluation of unexplained hemorrhage in the spinal canal.

Treatment of pediatric cerebral venous sinus thromboses: the role of anticoagulation.

BACKGROUND: Cerebral venous sinus thromboses (CVST) occur in children with a variety of etiologies. However, no standard treatment paradigm is established. We sought to identify what treatments have been applied, their outcomes, and the role of anticoagulation in pediatric patients with CVST. METHODS: A systematic review was conducted exploring all treatments of pediatric CVSTs using PubMed, Embase, Scopus, and Cochrane Library and Cochrane Central Register of Controlled Trials. Studies meeting inclusion criteria were reviewed in full and analyzed for study design, aim, population, interventions, and outcomes. RESULTS: Of 2946 resultant articles, 51 full-text articles were included. Management of infectious CVST included broad-spectrum antibiotics, surgery, and anticoagulation. Neoplastic and traumatic CVST treatment included anticoagulation. Treatment of CVSTs associated with metabolic abnormalities centered on correction of metabolic derangements, or supplementation where appropriate, and anticoagulation. Autoimmune, congenital, and thrombotic pathway CVSTs were treated with anticoagulation and treatment of the underlying disorder. Unfractionated heparin and low molecular weight heparin were most commonly used and seen to be effective and safe. Uncommonly, endovascular interventions including venous thrombectomy and intravenous injection of thrombolytic therapy were used with varying success. CONCLUSIONS: While conservative, medical, thombolytic, endovascular, and surgical treatment all have a role in in the treatment of pediatric CVSTs, anticoagulation is commonly applied and found to be safe and effective in pediatrics. Risks and benefits of anticoagulation must be considered on an individual basis as no randomized trials have established a standard of care. Based on our findings, we propose an approach to CVST treatment and look to future study aimed at more clearly delineating treatment dose, duration, and timing of re-evaluation in these patients.

Predicting dysphagia in children undergoing surgery for posterior fossa tumors.

PURPOSE: Posterior fossa tumors (PFTs) are the most common type of brain tumor in children. Dysphagia is a known complication of PFT resection in children, but data regarding risk factors and clinical course are sparse. METHODS: The records of all children who underwent resection of posterior fossa tumor between April 2007 and May 2017 at our institution were analyzed. Clinical, radiographic, histologic data were gathered. Swallowing function was assessed immediately postoperatively and at 1-year follow-up. RESULTS: A total of 197 patients were included. Forty-three (21.8%) patients developed dysphagia after surgery. Patients who developed dysphagia were younger (4.5 vs. 7.2 years, p < 0.01), were more likely to have brainstem compression (74.4% vs. 57.8%, p < 0.03) or invasion (14.0 vs. 9.7%, p < 0.03), and were more likely to have ependymoma (27.9% vs. 13.6%, p < 0.01) or ATRT (atypical teratoid/rhabdoid tumor) (9.3% vs. 3.9%, p < 0.01). Patients with postoperative dysphagia also had a longer length of stay (33.7 vs. 12.7 days, p < 0.01) and were more likely to be discharged to inpatient rehabilitation (25.6% vs. 9.1%, p < 0.01). Ten patients (5.1%) were PEG-dependent by 1-year follow-up. These patients were younger (2.7 vs. 5.6 years, p < 0.01), had a longer length of stay (55.5 vs. 27.4 days, p < 0.01), and were more likely to have ATRT (30.0% vs. 0.0%, p < 0.01). Recovery was not associated with tumor grade or extent of resection. CONCLUSIONS: Dysphagia after PFT resection is associated with younger age, aggressive tumor histology, and increased healthcare utilization. While most patients recover, a small percentage are still dependent on enteral feeding at 1-year follow-up. Further research is needed to identify factors associated with persistent deficits.

Systematic review of clinician awareness of mycotoxin impact in neural tube defects and best practices for pediatric neurosurgeons: implications for public health and policy.

PURPOSE: In lower-income populations, high rates of neural tube defects (NTDs) are a concern. Nutritional folate deficiencies and mycotoxins in contaminated food supplies increase risk of NTDs. As physicians in public health and involved in the care of children with NTDs, pediatric neurosurgeons have an interest in the treatment and prevention of NTDs. We aimed to evaluate the literature to assess the awareness and the existence of best practices/educational materials on this issue to better guide management. METHODS: A systematic review using the National Library of Medicine PubMed database was conducted to find articles related to mycotoxins in foods causing neural tube defects. Additional citation searches of key publications and personal collections were used. Two reviewers evaluated the resulting studies for subject area analysis. Best practice recommendations were drawn from articles selected for full-text review. RESULTS: Seventy-three articles were identified. Most articles were found in "nutritional sciences" (18), "teratology" (14), and "toxicology" (13). No articles were found in neurosurgery. Thirty-two additional articles were identified through other sources to screen best practice recommendations. Of the 105 articles, 34 journal articles were included in best practice recommendation guidelines. Key recommendations included education of proper food storage, hygienic agricultural practices, decontamination techniques, diet diversification, folate supplementation, risk assessment, and food safety policy and public health initiatives. CONCLUSION: There is an absence of neurosurgical literature-related mycotoxins and NTDs. We suggest a set of best practices/educational materials on this topic and advocate pediatric neurosurgery engagement in public health initiatives targeted towards populations most affected by mycotoxins.

Outcomes and resource utilization in surgery for Chiari I malformation in a national network of children's hospitals.

INTRODUCTION: Chiari malformation type 1 (CM-1) is a common congenital or acquired malformation of the posterior fossa. We aimed to characterize preoperative risk factors, perioperative complications, and postoperative outcomes related to CM-1 surgery in pediatric populations across a nationwide network of pediatric hospitals in the United States (US). METHODS: The Children's Hospital Association's Pediatric Health Information System (PHIS) database was used to examine patients < 21 years old in the US-based nationwide database who underwent inpatient surgery for CM-1 from 2007 to 2015. Data analyzed included patient characteristics, preoperative comorbidities, perioperative outcomes, short-term postoperative surgical and medical complications, and healthcare resource utilization. RESULTS: Among the 5976 patients identified, those age 0-4 years had higher medical and surgical complication rates compared to older patients. Those with pre-existing comorbidity of hydrocephalus had higher odds of 30- and 90-day medical and surgical complications. Those with any complications at 90 days had an increased length of stay and higher healthcare costs compared to those without complications. 6.88% of complications were surgical and 1.67% medical. Patients with medical complications had the longer mean stay and associated costs compared to those with surgical complications (13 vs. 6.9 at 95% CI, and $71,300-94,500 vs. $110,400-195,000 at 95% CI). CONCLUSIONS: Use of a US-based national children's hospital database presents outcomes and resource utilization from a multi-institutional, real-world experience in pediatric hospitals. There was a higher risk of perioperative complications in younger patients and those with pre-existing comorbidities, namely hydrocephalus. Understanding preoperative risk factors, perioperative complications, and postoperative outcomes, as well as healthcare utilization and costs, can help target areas for improvement and guide preoperative counseling and risk stratification.

Quality of the Patient Experience during Radiosurgery: Measurement toward Improvement.

BACKGROUND: All physicians seek to improve the patient experience. In an awake surgical procedure, the patient has a unique opportunity to comment on all aspects of care. The provision of a positive experience is part of quality health care. Our purpose was to define this experience to determine areas for improvement. METHODS: We evaluated 125 patients who underwent stereotactic radiosurgery using a frame-based, gamma knife technique. Patients were surveyed by a nurse practitioner across all elements of their procedural experience prior to same-day discharge. RESULTS: The radiosurgery was completed in all patients with same-day discharge. In an initial 100-patient cohort, 89 patients said they had received adequate oral and/or intravenous sedation before the procedure. All 100 patients said that they felt comfortable before stereotactic frame application, and all patients later remembered frame application. These patients described frame application as very or adequately comfortable (n = 73), minimally uncomfortable (n = 18), or very uncomfortable (n = 9). Neuroimaging was described as very or adequately comfortable (n = 93), minimally uncomfortable (n = 3), or very uncomfortable (n = 4). Radiosurgery in the gamma knife unit was found to be very or adequately comfortable (n = 99) or very uncomfortable (n = 1). We evaluated how 8 separate factors may have contributed to survey responses related to procedural comfort. These factors included intravenous line placement, delivery of sedation medications, application of the head frame, having the MRI, having radiosurgery on the gamma knife bed, removal of the stereotactic frame, communication with caregivers, and knowing what to expect beforehand. We asked the patients to rate their nursing care during the radiosurgery experience, and 'excellent' was chosen by all initial 100 patients. Other elements of the procedure were also studied as well as suggestions for improvement. As a secondary objective, we then modified our protocol to include sodium bicarbonate added to the local anesthetic for frame application and evaluated an additional cohort of 25 patients. CONCLUSIONS: Utilizing a system of physician and nursing education, together with pharmacological sedation and efficient procedural steps, patients said that intravenous line placement (91%), stereotactic frame application (74%), MRI (93%), receiving radiosurgery in the unit (99%), frame removal (84%), communication with caregivers (100%), and knowing what to expect beforehand (97%) were either very or adequately comfortable. Specific evaluations of care processes can lead to care improvement.

Trends in epidemiology and hospitalization utilization for myelomeningocele repair from 2000 to 2009.

PURPOSE: Although the incidence of myelomeningocele (MMC) has declined over the past decades with folic acid supplementation and prenatal screening, neural tube defects remain the most common birth defect in the USA. A majority of affected neonates require surgical repair. To characterize US trends in the epidemiology and hospital utilization of MMC repair over the past decade, we analyzed a nationally representative database. METHODS: We queried the Healthcare Cost and Utilization Project (HCUP) Kid's Inpatient Database (KID) for all discharges with procedure code for MMC repair for the years 2000, 2003, 2006, and 2009. The cohorts from these time points were compared for their demographic and in-hospital variables. Results are reported as estimated frequencies and means with 95 % confidence intervals (CI). RESULTS: Sex, race, insurance status, family income level, and mortality of affected infants have not changed significantly over the decade. A majority of neonatal MMC repairs occur in larger hospital bed size and more specialized children's hospital centers. Of patients, 52.3 to 60 % receive VPS placement during the same admission as the primary MMC repair. Total hospital costs for the MMC hospitalizations have remained relatively stable from 42,843 dollars in 2003 to 46,749 dollars in 2009 (adjusted to 2009 dollars). CONCLUSION: Demographics of children having MMC repair have not changed significantly over the past decade, while these surgeries have become more concentrated in pediatric-specialized centers. There appears to be a plateau in public health and access advances with relatively stable cost of MMC hospital care.

The impact of social determinants of health on utilization of pediatric epilepsy surgery: a systematic review.

OBJECTIVE: A minority of pediatric patients who may benefit from epilepsy surgery receive it. The reasons for this utilization gap are complex and not completely understood. Patient and caregiver social determinants of health (SDOH) may impact which patients undergo surgery and when. The authors conducted a systematic review examining SDOH and surgical intervention in children with drug-resistant epilepsy (DRE). They aimed to understand which factors influenced time to surgical program referral or receipt of epilepsy surgery among children with DRE, as well as identify areas to characterize the SDOH impacting epilepsy surgery in children and guide efforts aimed to promote health equity in epilepsy. METHODS: A systematic review was conducted using the PubMed, Embase, and Scopus databases in January 2022. Studies were analyzed by title and abstract, then full text, to identify all studies examining the impact of SDOH on utilization of epilepsy surgery. Studies meeting inclusion criteria were analyzed for SDOH examined, outcomes, and key findings. Quality was assessed using the Grading of Recommendations Assessment, Development and Evaluation system. RESULTS: Of 4545 resultant articles, 18 were included. Studies examined social, cultural, and environmental factors that contributed to SDOH impacting epilepsy surgery. Patients who underwent surgical evaluation were found to be most commonly White and privately insured and have college-educated caregivers. Five studies found differences in time to referral/surgery or rates of surgery by racial group, with most finding an increased time to referral/surgery or lower rates of surgery for those who were Hispanic and/or non-White. Four studies found that private insurance was associated with higher surgical utilization. Three studies found higher household income was related to surgical utilization. No studies examined biological, psychological, or behavioral factors that contributed to SDOH impacting epilepsy surgery. CONCLUSIONS: The authors conducted a systematic review exploring the impact of SDOH in DRE surgery utilization. They found that race, ethnicity, insurance type, caregiver educational attainment, and household income demonstrate relationships with pediatric epilepsy surgery. Further study is necessary to understand how these factors, and others not identified in this study, contribute to the low rates of utilization of epilepsy surgery and potential target areas for interventions aiming to increase equity in access to epilepsy surgery in children.

Cervicothoracic ventral-dorsal rhizotomy for bilateral upper-extremity hypertonia in cerebral palsy: illustrative case.

BACKGROUND: Management of medically refractory limb-specific hypertonia is challenging. Neurosurgical options include deep brain stimulation, intrathecal baclofen, thalamotomy, pallidotomy, or rhizotomy. Cervical dorsal rhizotomy has been successful in the treatment of upper-extremity spasticity. Cervical ventral and cervical ventral-dorsal rhizotomy (VDR) has been used in the treatment or torticollis and traumatic hypertonia; however, the use of cervicothoracic VDR for the treatment of upper-extremity mixed hypertonia is not well described. OBSERVATIONS: A 9-year-old girl with severe quadriplegic mixed hypertonia secondary to cerebral palsy (CP) underwent cervicothoracic VDR. Modified Ashworth Scale scores, provision of caregiving, and examination improved. Treatment was well tolerated. LESSONS: Cervicothoracic VDR can afford symptomatic and quality of life improvement in patients with medically refractory limb hypertonia. Intraoperative positioning and nuances in surgical techniques are particularly important based on spinal cord position as modified by scoliosis. Here, the first successful use of cervicothoracic VDR for the treatment of medically refractory upper-limb hypertonia in a pediatric patient with CP is described.