Spontaneous thrombosis of high flow pediatric arteriovenous fistulae: Case series of two patients and a comprehensive literature review.

Pediatric pial arteriovenous shunts in the brain and spine are challenging to understand because of low incidence, variable presentation, and associations with genetic syndromes. What is known about their natural history comes from reviews of small series. To better understand the natural history and role for intervention, two cases are presented followed by a review of the literature. In the first case, an infant with a prior history of intracranial hemorrhage from a ruptured pial fistula returns for elective embolization for a second pial fistula which was found to be spontaneously thrombosed 2 weeks later. In the second case, a 5-year-old with a vertebro-vertebral fistula, identified on work up for a heart murmur and documented with diagnostic angiography, is brought for elective embolization 6 weeks later where spontaneous thrombosis is identified. In reviewing the literature on pediatric single-hole fistulae of the brain and spine, the authors offer some morphologic considerations for identifying which high-flow fistulae may undergo spontaneous thrombosis to decrease the potentially unnecessary risk associated with interventions in small children.

Clinical outcomes of stereotactic biopsy on children with pontine diffuse midline glioma.

INTRODUCTION: Diffuse midline glioma (DMG) of the pons occurs in pediatric patients and carries a dismal prognosis. Biopsy is not necessary for diagnosis but provides information, particularly H3K27M status, with prognostic implications. Additionally, biopsy information may open therapeutic options such as clinical trials that require mutation status. Therefore, we sought to assess the safety of surgical biopsy in DMG patients as well as its potential impact on clinical course. METHODS: Retrospective analysis of patients who were radiographically and clinically diagnosed with pontine DMG in the last 5 years was performed. We assessed demographic, clinical, radiographic, surgical, and follow-up data. RESULTS: 25 patients were included; 18 (72%) underwent biopsy while 7 (28%) declined. 12 biopsies (67%) were performed with robotic arm and 5 (27%) with frameless stereotaxy. Three biopsied patients (17%) experienced new post-operative neurologic deficits (1 facial palsy, 1 VI nerve palsy and 1 ataxia) that all resolved at 2-week follow-up. All biopsies yielded diagnostic tissue. Fourteen patients (78%) had H3K27M mutation. Median OS for H3K27M patients was 10 months compared to 11 months in the wild-type patients (p = 0.30, log-rank test). Median OS for patients enrolled in clinical trials was 12 months compared to 8 months for non-trial patients (p = 0.076). CONCLUSION: In our series, stereotactic pontine DMG biopsies did not carry any permanent deficit or complication and yielded diagnostic tissue in all patients. Similar post-operative course was observed in both robot-assisted and frameless stereotactic approaches. There was no significant difference in survival based on mutation status or clinical trial enrollment.

Seizure outcomes after resection of primary brain tumors in pediatric patients: a systematic review and meta-analysis.

PURPOSE: Primary brain neoplasms are the most common solid tumors in pediatric patients and seizures are a common presenting symptom. Surgical intervention improves oncologic outcomes and seizure burden. A better understanding of factors that influence seizure outcomes in the surgical management of primary brain tumors of childhood can guide treatment approach thereby improving patient quality of life. METHODS: We performed a systematic analysis using articles queried from PubMed, EMBASE, and Cochrane published from January 1990 to August 2022 to determine predictors of seizure outcomes in pediatric patients undergoing resection of primary brain tumors. RESULTS: We identified 24 retrospective cohort studies, one prospective cohort study, and one mixed retrospective and prospective study for the systematic analysis. A total of 831 pediatric patients were available for analysis. 668 (80.4%) patients achieved seizure freedom after surgery. Complete tumor resection increased the likelihood of a seizure-free (Engel I) outcome compared to subtotal resection (OR 7.1, 95% CI 2.3-21.9). Rates of Engel I seizure outcomes did not significantly differ based on factors such as age at seizure onset, duration of epilepsy, gender, tumor laterality, or age at surgery, but trended towards significance for improved outcomes in temporal lobe tumors. CONCLUSION: Primary brain tumors in the pediatric population are commonly associated with seizures. Resection of these lesions reduces seizure burden and is associated with high rates of seizure freedom. Complete resection, compared to subtotal resection, significantly increases the likelihood of seizure-free outcomes.

An Initial Experience of Completion Hemispherotomy via Magnetic Resonance-Guided Laser Interstitial Therapy.

INTRODUCTION: In carefully selected patients with medically refractory epilepsy, disconnective hemispherotomy can result in significant seizure freedom; however, incomplete disconnection can result in ongoing seizures and poses a significant challenge. Completion hemispherotomy provides an opportunity to finish the disconnection. We describe the use of magnetic resonance-guided laser interstitial thermal ablation (MRgLITT) for completion hemispherotomy. METHODS: Patients treated with completion hemispherotomy using MRgLITT at our institution were identified. Procedural and seizure outcomes were evaluated retrospectively. RESULTS: Five patients (3 males) underwent six MRgLITT procedures (one child treated twice) for completion hemispherotomy at a median age of 6 years (range 1.8-12.9). Two children had hemimegalencephaly, two had Rasmussen encephalitis, and one had polymicrogyria. All five children had persistent seizures likely secondary to incomplete disconnection after their functional hemispherotomy. The mean time from open hemispherotomy to MRgLITT was 569.5 ± 272.4 days (median 424, range 342-1,095). One patient underwent stereoelectroencephalography before MRgLITT. The mean number of ablation targets was 2.3 ± 0.47 (median 2, range 2-3). The mean length of the procedure was 373 min ± 68.9 (median 374, range 246-475). Four of the five patients were afforded improvement in their neurocognitive functioning and speech performance after ablation, with mean daily seizure frequency at 1 year of 1.03 ± 1.98 (median 0, range 0-5). Two patients achieved Engel Class I outcomes at 1 year after ablation, one was Engel Class III, and two were Engel Class IV. The mean follow-up time was 646.8 ± 179.5 days (median 634, range 384-918). No MRgLITT-related complications occurred. Delayed retreatment (>1 year) occurred in three patients: one child underwent redo ablation and two underwent anatomic hemispherectomy. CONCLUSION: We have demonstrated the feasibility of a minimally invasive approach for completion hemispherotomy using MRgLITT. Delayed retreatment was needed in three patients; thus, further study of this technique with comparison to other surgical techniques is warranted.

Primary spinal intramedullary anaplastic ganglioglioma in a pediatric patient.

Background: Gangliogliomas (GGs) are rare tumors of the central nervous system composed of neoplastic neural and glial cells and are typically low-grade. Intramedullary spinal anaplastic GGs (AGG) are rare, poorly understood, and often aggressive tumors that can result in widespread progression along the craniospinal axis. Due to the rarity of these tumors, data are lacking to guide clinical and pathologic diagnosis and standard of care treatment. Here, we present a case of pediatric spinal AGG to provide information on our institutional approach to work-up and to highlight unique molecular pathology. Case Description: A 13-year-old female presented with signs of spinal cord compression including right sided hyperreflexia, weakness, and enuresis. Magnetic resonance imaging (MRI) revealed a C3-C5 cystic and solid mass which was treated surgically with osteoplastic laminoplasty and tumor resection. Histopathologic diagnosis was consistent with AGG, and molecular testing identified mutations in H3F3A (K27M), TP53, and NF1. She received adjuvant radiation therapy and her neurological symptoms improved. However, at 6-month follow-up, she developed new symptoms. MRI revealed metastatic recurrence of tumor with leptomeningeal and intracranial spread. Conclusion: Primary spinal AGGs are rare tumors, but a growing body of literature shows some trends that may improve diagnosis and management. These tumors generally present in adolescence and early adulthood with motor/sensory impairment and other spinal cord symptoms. They are most commonly treated by surgical resection but frequently recur due to their aggressive nature. Further reports of these primary spinal AGGs along with characterization of their molecular profile will be important in developing more effective treatments.

Behavioral Improvements following Lesion Resection for Pediatric Epilepsy: Pediatric Psychosurgery?

INTRODUCTION: Resection of brain lesions associated with refractory epilepsy to achieve seizure control is well accepted. However, concurrent behavioral effects of these lesions such as changes in mood, personality, and cognition and the effects of surgery on behavior have not been well characterized. We describe 5 such children with epileptogenic lesions and significant behavioral abnormalities which improved after surgery. CASE DESCRIPTIONS: Five children (ages 3-14 years) with major behavioral abnormalities and lesional epilepsy were identified and treated at our center. Behavioral problems included academic impairment, impulsivity, self-injurious behavior, and decreased social interaction with diagnoses of ADHD, oppositional defiant disorder, and autism. Pre-operative neuropsychiatric testing was performed in 4/5 patients and revealed low-average cognitive and intellectual abilities for their age, attentional difficulties, and poor memory. Lesions were located in the temporal (2 gangliogliomas, 1 JPA, 1 cavernoma) and parietal (1 DNET) lobes. Gross total resection was achieved in all cases. At mean 1-year follow-up, seizure freedom (Engel 1a in 3 patients, Engel 1c in 2 patients) and significant behavioral improvements (academic performance, attention, socialization, and aggression) were achieved in all. Two patients manifested violence pre-operatively; one had extreme behavior with violence toward teachers and peers despite low seizure burden. Since surgery, his behavior has normalized. CONCLUSION: We identified 5 patients with severe behavioral disorders in the setting of lesional epilepsy, all of whom demonstrated improvement after surgery. The degree of behavioral abnormality was disproportionate to epilepsy severity, suggesting a more complicated mechanism by which lesional epilepsy impacts behavior. We propose a novel paradigm in which lesionectomy may offer behavioral benefit even when seizures are not refractory. Thus, behavioral improvement may be an important novel goal for neurosurgical resection in children with epileptic brain lesions.

Flow diversion of a dissecting PICA aneurysm.

A 47-year-old female with a history of a ruptured left posterior inferior cerebellar artery (PICA) aneurysm, status post coil embolization and retreatment for recurrence, presented with evidence of a recurrent dissecting PICA aneurysm. Given that these aneurysms are considered high risk and have a greater propensity for rupture than anterior circulation aneurysms, retreatment was recommended. With the patient's strong preference for endovascular therapy, flow diversion with a Silk Vista Baby was performed. Given the low-profile design of the device, a radial artery approach and coaxial technique were used to deploy the flow diverter. The device was successfully placed, with complete obliteration of the aneurysm after 1 year. The video can be found here: https://stream.cadmore.media/r10.3171/2022.7.FOCVID2247.

Natural history of posterior fetal cephaloceles and incidence of progressive cephalocele herniation.

OBJECTIVE: In utero repair of fetal posterior cephaloceles (meningocele and encephalocele) is being performed based on the premise that fetal surgery prevents progressive herniation of neural tissue and brain damage during pregnancy. However, the extent to which progressive herniation occurs during pregnancy, specifically from prenatal diagnosis to after delivery, is not well known. The objective of this study was to describe the natural history of patients with fetal cephaloceles focusing on the incidence of progressive herniation. METHODS: The authors conducted a retrospective cohort study of all patients referred to their center for posterior fetal cephalocele between 2006 and 2021. All patients underwent prenatal and postnatal MRI. Progressive herniation (primary outcome) was defined as an increase in the absolute volume of neural tissue within the cephalocele of > 5% or new herniation of a critical structure into the cephalocele. Total brain and cephalocele volumes were calculated to determine herniation progression from prenatal to postnatal MRI. Information on the presence of hydrocephalus, epilepsy, and developmental delay (secondary outcomes) was collected at 1 year of age. RESULTS: Twenty patients met all study criteria. Ten patients (50%; 95% CI 0.27-0.73) demonstrated progressive herniation from prenatal to postnatal MRI. Three patients with progressive herniation were diagnosed with a meningocele prenatally and had an encephalocele postnatally. Two patients without progression had meningocele identified prenatally that regressed and became atretic by birth. Both prenatal hindbrain herniation (p = 0.03) and prenatal microcephaly (p = 0.05) were predictive of progressive herniation. The rates of hydrocephalus (44%), epilepsy (44%), and developmental delay (63%) were not associated with the occurrence of progressive herniation in this study. CONCLUSIONS: In this study, progressive herniation was not a rare event (50%). Fetal hindbrain herniation and fetal microcephaly were associated with the occurrence of progressive herniation. These results support further investigations into why progressive herniation occurs in utero and if progressive cerebral herniation in utero plays a significant role in determining clinical outcome.

Comparison of Surgical Outcomes in Individuals With Hypothalamic Hamartoma Alone or With Other Potentially Epileptogenic Focal Lesions.

BACKGROUND: Hypothalamic hamartoma is a rare condition associated with refractory seizures. It can occur in isolation or with additional epileptogenic lesions. The aim of this study was to investigate the effects of additional potentially epileptogenic lesions on surgical outcomes in individuals with hypothalamic hamartoma. METHODS: We conducted a chart review of 112 patients with hypothalamic hamartoma who underwent magnetic resonance imaging (MRI)-guided laser interstitial thermal therapy targeted to the hypothalamic hamartoma. We compared surgical outcomes after at least six months of postoperative follow-up (N = 65) between patients with hypothalamic hamartoma alone and those with hypothalamic hamartoma plus additional potentially epileptogenic lesions. RESULTS: Sixteen out of 112 (14%) patients had additional epileptogenic lesions, including focal cortical dysplasia, gray matter heterotopia, and polymicrogyria. Ten out of 16 patients with additional lesions and 55 out of 96 patients with hypothalamic hamartoma alone had more than six months of follow-up and are included in the outcome analysis. Mean follow-up in these patients is 21.5 ± 17.3 months (standard deviation, range: 7.3-76.8 months) for patients with hypothalamic hamartoma alone and 16.1 ± 15.0 months (standard deviation, range: 6.6-58.2 months) for those with hypothalamic hamartoma plus additional epileptogenic lesions. Fewer patients with hypothalamic hamartoma plus other lesions had Engel class I/II outcomes than patients with hypothalamic hamartoma alone (5/10 [50%] vs 46/55 [83.6%]; P = 0.031). CONCLUSIONS: MRI-guided laser interstitial thermal therapy remains an effective treatment option for patients with hypothalamic hamartoma. However, the outcome of surgical procedures targeted to the hypothalamic hamartoma may be less favorable in patients who have hypothalamic hamartoma coexisting with other potentially epileptogenic focal lesions. Thus, an additional surgical workup is warranted for these patients who have failed surgical treatment of hypothalamic hamartoma.

Endovascular Intervention for Refractory Pediatric Cerebral Venous Sinus Thrombosis.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare but potentially morbid disease in the pediatric population, and the optimal treatment is not fully understood. Endovascular intervention for this condition has been rarely reported. METHODS: The Stroke Thrombectomy and Aneurysm Registry was queried for patients aged less than or equal to 18 years undergoing endovascular treatment for CVST in the past 10 years. Clinical charts and radiographic data were retrospectively reviewed. Modified Rankin Score (mRS) at 90 days postprocedure was determined as the primary outcome. RESULTS: A total of seven patients across five pediatric centers ranging from 7 to 16 years of age were identified with a mean follow-up of 28 months. All had underlying conditions predisposing to CVST. Endovascular intervention was undertaken due to neurological deterioration despite systemic anticoagulation; venous infarct was evident preoperatively in six of seven patients. Mechanical venous thrombectomy was attempted in all individuals, and intrasinus thrombolytic therapy was also performed in three cases. Six patients had favorable outcome with mRS 0 or 1 at 90 days postprocedure; one remained neurologically devastated. CONCLUSIONS: Endovascular treatment by an experienced interventionalist may be safe and effective in severe cases of CVST in children failing frontline therapy. Children with radiographic or clinical progression despite anticoagulation may be considered for endovascular intervention in a timely manner.

Pediatric neurosurgery at Texas Children's Hospital: the legacy of Dr. William R. Cheek.

Texas Children's Hospital opened its doors in 1954, and since that time the institution has remained dedicated to a three-part mission: patient care, education, and research. Dr. William R. Cheek developed an early interest in pediatric neurosurgery, which led to his efforts in building and developing a service at Texas Children's Hospital at a time when the field was just emerging. His work with other early pioneers in the field led to the establishment of organized societies, educational texts, and governing bodies that have led to significant advances in the field over the past 50 years.

Neurenteric Cyst: Case Report and Operative Video.

Neurenteric cysts are rare, congenital lesions of the spinal axis composed of endodermal tissue arising from poor segmentation of the notochord. A 36-year-old patient presented with arm paresthesias and incontinence with imaging revealing a lesion in the C6-C7 region most consistent with neurenteric cyst. After partial resection of the lesion, the patient regained all neurological function. Here, we provide a brief overview of this rare neuropathologic entity and demonstrate surgical resection of neurenteric cyst through operative video.

Sociodemographic Factors in Pediatric Epilepsy Surgery.

BACKGROUND: Despite documented efficacy of surgical treatment in carefully selected patients, surgery is delayed and/or underutilized in both adult and children with focal onset epilepsy. The reasons for surgical delay are often assumed or theorized, and studies have predominantly targeted the adult population. To focus on a more targeted pediatric population and to determine identifiable reasons for intervention, this study aimed to investigate time to epilepsy surgery among pediatric patients with medically intractable epilepsy associated with focal cortical dysplasia and to identify sociodemographic and clinical associations in time to epilepsy surgery. METHODS: We reviewed 96 consecutive pediatric patients who underwent surgery for medically intractable epilepsy with a diagnosis of focal cortical dysplasia. Descriptive statistics, univariate and multivariate analyses were conducted to study the association of sociodemographic variables of patients with focal cortical dysplasia and time to epilepsy surgery and postoperative seizure control. RESULTS: We identified that non-white patients on average had a longer duration of epilepsy before surgery and traveled shorter distances for care. Non-white patients were more likely to have government-funded insurance. Patients who traveled the shortest distance to the surgical center underwent epilepsy surgery at an older age. CONCLUSIONS: Sociodemographic factors of travel distance, insurance, and race influenced time to epilepsy surgery for children with focal cortical dysplasia. Further research is warranted to target barriers in access to subspecialty care and develop ways to identify earlier the patients who may benefit from evaluation and deployment of surgical intervention.

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.

Pediatric intracranial arteriovenous malformations: Examining rehabilitation outcomes.

PURPOSE: Arteriovenous malformation (AVM) rupture in children can cause debilitating neurological injury. Rehabilitation is key to recovery, though literature details little regarding rehabilitation outcomes. We examined a single-center experience with pediatric AVMs as related to acute inpatient rehabilitation outcomes. METHODS: At our institution, a retrospective chart review was completed examining all cases of intracranial AVMs in patients age 18 and younger who completed our acute inpatient rehabilitation program between 2012-2018. Patient characteristics, clinical data, treatment modality, and functional outcomes were reviewed. RESULTS: 14 patients with AVMs underwent acute inpatient rehabilitation; nine (64.3%) treated surgically at our institution, two (14.3%) non-surgically at our institution, and three (21.4%) surgically at an outside facility prior to transitioning care at our institution. Eight (57.1%) were male, seven (50.0%) Caucasian, and seven (50.0%) Hispanic. Seven (50.0%) presented with AVM rupture; six (42.9%) were found incidentally on imaging. Clinical courses, treatment outcomes, and post-treatment complications varied. Several patients underwent repeat treatment or additional procedures. Neurological deficits identified included hemiparesis, dystonia, spasticity, epilepsy, hydrocephalus, and ataxia. Inpatient rehabilitation unit length of stay was on average 21 days (SD 9.02, range 9-41). Functional Independence Measure for Children (WeeFIM®) scores, including self-care, mobility, and cognition, demonstrated improvement upon discharge. The mean total change was 36.7 points in those treated surgically, 16.5 in those treated non-surgically, and 25.7 in those treated surgically at another facility. CONCLUSION: We found that all pediatric patients with intracranial AVMs, across all treatment modalities, demonstrated improved outcomes across all functional domains after an acute inpatient rehabilitation program.

Staged Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Hypothalamic Hamartoma: Analysis of Ablation Volumes and Morphological Considerations.

BACKGROUND: Hypothalamic hamartomas (HH) are a challenging pathology that cause gelastic seizures. Magnetic Resonance Imaging-guided Laser Interstitial Thermal Therapy (MRgLITT) offers a safe and effective treatment for HHs via a minimally invasive technique. OBJECTIVE: To determine how clinical outcome correlates to residual tumor volume and surgical strategy by analyzing radiographic data and reconstructing volumetric imaging. METHODS: Clinical and radiographic information of 58 pediatric patients who underwent MRgLITT for HH with at least 6 mo of follow-up were retrospectively reviewed. MR imaging was volumetrically reconstructed to analyze the impact of hamartoma and ablation volumes on outcome. Primary outcome measure was freedom from gelastic seizures. RESULTS: Eighty-one percent of patients were completely free of gelastic seizures at last follow-up; of 22 patients with secondary nongelastic epilepsy, 15 were free of additional seizures. Postoperative complication rate was low. There was no significant difference in gelastic seizure outcome related to pre- or postoperative hamartoma size. Residual hamartoma percentage in those free of gelastic seizures was 43% compared to 71% in those with continued seizures (P = .021). Larger hamartomas required multiple ablations to achieve seizure freedom. CONCLUSION: This large series of patients confirms the safety and efficacy of MRgLITT for pediatric HH and describes morphological considerations that predict success. Our data suggest that complete ablation of the lesion is not necessary, and that the focus should be on appropriate disconnection of the epileptogenic network. We have found that a staged approach to hamartoma ablation allows adequate disconnection of the hamartoma while mitigating risk to surrounding structures.

Preoperative computed tomography perfusion in pediatric moyamoya disease: a single-institution experience.

OBJECTIVE: Moyamoya disease is a progressive occlusive arteriopathy for which surgical revascularization is indicated. In this retrospective study, the authors investigated the use of preoperative CT perfusion with the aim of establishing pathological data references. METHODS: The authors reviewed the medical records of children with moyamoya disease treated surgically at one institution between 2016 and 2019. Preoperative CT perfusion studies were used to quantify mean transit time (MTT), cerebral blood volume (CBV), cerebral blood flow (CBF), and time to peak (TTP) for the anterior, middle, and posterior cerebral artery vascular territories for each patient. CT perfusion parameter ratios (diseased/healthy hemispheres) and absolute differences were compared between diseased and normal vascular territories (defined by catheter angiography studies). Sensitivity, specificity, and positive (PPV) and negative (NPV) predictive values for CT perfusion parameters for severe angiographic moyamoya were calculated. RESULTS: Nine children (89% female) had preoperative CT perfusion data; 5 of them had evidence of unilateral hemispheric disease and 4 had bilateral disease. The mean age at revascularization was 77 months (range 40-144 months). The etiology of disease was neurofibromatosis type 1 (3 patients), Down syndrome (2), primary moyamoya disease (2), cerebral proliferative angiopathy (1), and sickle cell disease (1). Five patients had undergone unilateral revascularization. Among these patients, pathological vascular territories demonstrated increased MTT in 66% of samples, increased TTP in 66%, decreased CBF in 47%, and increased CBV in 87%. Severe moyamoya (Suzuki stage ≥ 4) had diseased/healthy ratios ≥ 1 for MTT in 78% of cases, for TTP in 89%, for CBF in 67%, and for CBV in 89%. The MTT and TTP region of interest ratio ≥ 1 demonstrated 89% sensitivity, 67% specificity, 80% PPV, and 80% NPV for the prediction of severe angiographic moyamoya disease. CONCLUSIONS: Pathological hemispheres in these children with moyamoya disease demonstrated increased MTT, TTP, and CBV and decreased CBF. The authors' results suggest that preoperative CT perfusion may, with high sensitivity, be useful in deciphering perfusion mismatch in brain tissue in children with moyamoya disease. More severe angiographic disease displays a more distinct correlation, allowing surgeons to recognize when to intervene 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.

Quality of Life in Pediatric Neurosurgery: Comparing Parent and Patient Perceptions.

BACKGROUND: In the pediatric neurosurgical population, understanding how to mitigate the effects of disease on children's physical ability, social and psychological well-being, and education can have lasting effects on their development and family. Understanding parents' perceptions of their children's health-related quality of life (HRQoL) is crucial in proper counseling, management of expectations, establishment of a healthy patient/parent-physician relationship, and understanding the role and impact of health care. In this study we sought to examine the differences between patient and parent perceptions of HRQoL and the factors that influence these differences. METHODS: A standardized survey (PedsQL Inventory) to assess physical, psychological, and social function and school performance was administered to both parents and patients in a general pediatric neurosurgery outpatient clinic from 2015-2018. RESULTS: 197 encounters with 179 patients occurred in the study period. There were 105 male and 92 female patients across various ethnic backgrounds. The most common diagnoses were tumor (23.4%) and vascular malformation (16.2%). Parent overall scores were lower than their children's (72.5 vs. 76.4, P < 0.01). Parent scores of physical function (74.3 vs. 78.7, P < 0.01) and overall psychosocial function (71.5 vs. 75.3, P < 0.01) were also lower. Parent scores in all domains of the PedsQL were correlated with overall score difference (P < 0.01), but only child scores of overall psychosocial (P < 0.03) and school function (P < 0.04) were correlated with overall score difference. CONCLUSIONS: Parents have more negative assessments of their children's HRQoL compared with their children in all domains assessed by the PedsQL. Further research is needed to identify factors that contribute to these discrepancies.

Postoperative 30-day outcomes after craniotomy for supratentorial AVM resection in children.

PURPOSE: To describe 30-day outcomes following craniotomy for arteriovenous malformation in children and identify risk factors for readmission, reoperation, and perioperative complication using the National Surgical Quality Improvement Program (NSQIP) Pediatric database. METHODS: Patients aged 0-18 years who underwent surgery for arteriovenous malformations (years 2015-2016) were identified from the NSQIP Pediatric database. Descriptive statistics, uni-variate, and multi-variate regression analysis were performed using preoperative and perioperative data. The outcome of interest was postoperative adverse event including reoperation within 30 days, readmission within 30 days, discharge to rehab, and the complications wound infection/dehiscence, pneumonia, unplanned reintubation, pulmonary embolism (PE), renal insufficiency, urinary tract infection (UTI), stroke, venous thromboembolism (VT), and sepsis. RESULTS: 167 patients were identified who met study criteria. 58% were male, and the majority had an ASA classification of 3 or greater (68%). 96 (57%) patients were found to have a preoperative comorbidity, with the most common comorbidity being seizure disorder (54 patients, 32%). 76 patients (46%) had documented perioperative events or complications. The incidence of wound infection/dehiscence was 4%; and of pneumonia, PE, unplanned reintubation, renal insufficiency, UTI, stroke, VT, sepsis were <1%. There were no deaths. The incidence of unplanned reoperation was 10% and unplanned readmission was 12%. Most (90%) were discharged to home. Operative time (p = .0001, OR = 9.53), emergent surgery status (p = .0001, OR = 8.19) and preoperative comorbidities (p = .007) were found to be significant predictors of poor outcome. CONCLUSION: In the NSQIP-P dataset, the incidence of perioperative complications and suboptimal outcomes among patients undergoing AVM resection were low.