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.

The occipital interhemispheric transtentorial approach in infants and toddlers: efficacy and complications.

INTRODUCTION: Outcomes for pineal region and superior cerebellar tumors in young children often hinge on extent of microsurgical resection, and thus choosing an approach that provides adequate visualization of pathology is essential. The occipital interhemispheric transtentorial (OITT) approach provides excellent exposure while minimizing cerebellar retraction. However, this approach has not been widely accepted as a viable option for very young children due to concerns for potential blood loss when incising the tentorium. The aim of this paper is to characterize our recent institutional experience with the occipital interhemispheric transtentorial approach (OITT) for tumor resection in infants and toddlers. METHODS: A retrospective study was performed between 2016 and 2023 of pediatric patients less than 36 months of age who underwent OITT for tumor resection at a high-volume referral center. Patients with at least 3 months of postoperative follow-up and postoperative MRI were included. Primary outcomes included extent of resection, intraoperative and postoperative complications, and neurologic outcome. Secondary outcomes included length of stay and estimated blood loss. RESULTS: Eight patients, five male, were included. The median age at the time of surgery was 10 months (range 5-36 months). Presenting symptoms included macrocephaly, nausea/vomiting, strabismus, gait instability, or milestone regression. Hydrocephalus was present preoperatively in all patients. Average tumor volume was 38.6 cm3, ranging from 1.3 to 71.9 cm3. All patients underwent an OITT approach for tumor resection with stereotactic guidance. No intraoperative complications occurred, and no permanent neurologic deficits developed postoperatively. Gross total resection was achieved in all cases per postoperative MRI report, and no instances of new cerebellar, brainstem, or occipital lobe ischemia were noted. CONCLUSIONS: OITT approach for tumor resection in very young children (≤ 36 months) is an effective strategy with an acceptable safety profile. In our series, no significant intraoperative or postoperative complications occurred. To our knowledge, this is the first report describing this technique specifically in patients less than 36 months of age.

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.

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.

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.

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.

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.

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.

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.

Evaluating the National Surgical Quality Improvement Program-Pediatric Surgical Risk Calculator for Pediatric Craniosynostosis Surgery.

BACKGROUND: The American College of Surgeons' National Surgical Quality Improvement Program-Pediatric (NSQIP-P) risk calculator was developed based on national data. There have been no studies assessing the risk calculator's performance in pediatric neurosurgery. The authors aimed to evaluate the predictions from the risk calculator compared to our single institution experience in craniosynostosis surgery. METHODS: Outcomes from craniosynostosis surgeries performed between 2012 and 2016 at our academic pediatric hospital were evaluated using the NSQIP-P risk calculator. Descriptive statistics were performed comparing predicted 30-day postoperative events and clinically observed outcomes. The performance of the calculator was evaluated using the Brier score and receiver operating characteristic curve (ROC). RESULTS: A total of 202 craniosynostosis surgeries were included. Median age was 0.74 years (range 0.15-6.32); 66% were males. Blood transfusion occurred in 162/202 patients (80%). The following clinical characteristics were statistically correlated with surgical complications: American Society of Anesthesiologists physical status classification >1 (P < 0.001), central nervous system abnormality (P < 0.001), syndromic craniosynostosis (P = 0.001), and redo operations (P = 0.002). Postoperative events occurred in <3%, including hardware breakage, tracheal-cartilaginous sleeve associated with critical airway, and surgical site infection. The calculator performed well in predicting any complication (Brier = 0.067, ROC = 73.9%), and for pneumonia (Brier = 0.0049, ROC 99%). The calculator predicted a low rate of cardiac complications, venous thromboembolism, renal failure, reintubation, and death; the observed rate of these complications was 0. CONCLUSIONS: The risk calculator demonstrated reasonable ability to predict the low number of perioperative complications in patients undergoing craniosynostosis surgery with a composite complications outcome. Efforts to improve the calculator may include further stratification based on procedure-specific risk factors.

Focal selective dorsal rhizotomy and concurrent deformity correction: a combined approach.

OBJECTIVE: Selective dorsal rhizotomy (SDR) is a neurosurgical procedure to reduce spasticity in children with cerebral palsy and spastic diplegia. The authors developed a procedure called focal SDR for children with spasticity predominantly in the L5 or S1 motor distribution, which can be combined with orthopedic correction of fixed soft-tissue or bony deformity. The authors describe in detail the technique of minimally invasive focal SDR and propose selection criteria. METHODS: The authors conducted a retrospective study of patients who underwent focal SDR at their institution and underwent baseline and 1-year postoperative 3D gait analysis. Modified Ashworth scale (MAS) and Gait Deviation Index (GDI) scores were the primary outcome measures. RESULTS: Ten patients met the study criteria, all with an underlying diagnosis of cerebral palsy. All underwent focal SDR at the unilateral or bilateral S1 level, and 4 additionally underwent focal SDR at the L5 level unilaterally or bilaterally. All but 1 patient underwent concurrent orthopedic surgery. The improvement in spasticity of the plantar flexors, as measured by the MAS score, was 2.2 (p < 0.001). In the patients who underwent L5 focal SDR, there was an improvement in the hamstring MAS score of 1.4 (p = 0.004). The mean improvement in the GDI score following focal SDR was 11 (range -6 to 29, p < 0.001). CONCLUSIONS: Focally impairing spasticity in the gastrocsoleus complex and/or hamstrings muscle group in the setting of less functionally impactful proximal tone is extremely common in cerebral palsy. The novel technique of focal SDR, combined with orthopedic intervention, improves spasticity scores and overall gait mechanics. Further investigation is warranted to define the ideal candidacy and outcomes.

Treatment of contact dermatitis associated with spinal cord stimulator pulse generator--technical note.

BACKGROUND: Expanded polytetrafluoroethylene sheets have been successfully used as a biologic barrier in the treatment of pacemaker contact dermatitis. TECHNIQUE: In this report, we describe two patients with contact dermatitis related to the implantable pulse generator of a spinal cord stimulator who were treated with expanded polytetrafluoroethylene as a barrier. RESULTS: Resolution of the dermatitis was achieved in both cases. CONCLUSIONS: This technique is an effective way of treating contact dermatitis related to implantable pulse generators, without the need for explantation of the neuromodulation system.

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.

DREZotomy in the treatment of cancer pain: a review.

BACKGROUND: Cancer-related pain is a common problem that may be intractable by medical and neuromodulatory treatment. The dorsal root entry zone (DREZ) is a hyperactive focus in neuropathic pain syndromes, and DREZotomy has been used in selective cases of neuropathic cancer pain. OBJECTIVE: The aim of this study was to describe the technique of spinal DREZotomy in the treatment of cancer pain and review the relevant published literature. METHODS: A PubMed database search for 'DREZ', 'dorsal root entry zone' and 'cancer', and a search of the references of these manuscripts, was undertaken. RESULTS: 14 papers were identified and reviewed that described a total of 123 patients with cancer pain or radiation-induced pain who have been treated with DREZotomy. Though heterogeneous, these studies reported an overall favorable outcome in carefully selected patients with topographically limited pain syndromes. CONCLUSION: For patients with well-localized neuropathic cancer pain intractable to medical and first-line surgical management, DREZotomy is a viable treatment option. Further prospective studies are needed to evaluate the outcomes of this procedure.

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.

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.

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.

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.