The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Approaches to ventriculoperitoneal shunt scalp erosion: countersinking into the calvarium. Illustrative case.

BACKGROUND: Ventriculoperitoneal shunting (VPS) is a standard procedure for the treatment of hydrocephalus, and the management of its complications is common in the practice of pediatric neurosurgery. Shunt exposure, though a rare complication, can occur because of thin, fragile skin, a young patient age, protuberant hardware, poor scalp perfusion, and a multitude of other patient factors. OBSERVATIONS: The authors report a complex case of VPS erosion through the scalp in a young female with Pfeiffer syndrome treated with external ventricular drainage, empirical antibiotics, and reinternalization with countersinking of replaced shunt hardware into the calvarium to prevent internal skin pressure points, reduce wound tension, and allow wound healing. LESSONS: Recessing the shunt hardware, or countersinking the implant, into the calvarium is a simple technique often used in functional neurosurgical implantation surgeries, providing a safe surgical strategy to optimize wound healing in select cases in which the skin flap is unfavorable.

Cervical corpectomy in a pediatric patient with chondrodysplasia punctata and C5 dysplasia with spinal cord compression: illustrative case.

BACKGROUND: Chondrodysplasia punctata (CDP) describes skeletal dysplasia secondary to a variety of genetic underpinnings characterized by cartilaginous stippling from abnormal calcium deposition during endochondral bone formation. Approximately 20%-38% of patients with CDP have cervical spine abnormalities, resulting in stenosis and cord compression. However, approaches to management differ among patients. OBSERVATIONS: The authors present an 18-year-old male with a known history of CDP and cervical kyphosis with worsening paresthesias and increased spasticity. Imaging confirmed dysplastic C4 and C5 vertebra with focal kyphosis, bony retropulsion, spinal cord compression, and myelomalacia. To treat the stenosis and deformity, the patient underwent C4 and C5 vertebrectomies with C3 to C6 anterior fusion with resolution of symptoms. LESSONS: Despite many CDP patients having cervical deformities with spinal cord compression and associated neurological symptoms, there is a paucity of data on surgical management and outcomes. There are only scattered reports, and most authors recommend initial conservative management because of the high risk of operative morbidity and mortality secondary to comorbidities. When surgery is performed, long-term follow-up is recommended because of the high rates of progression of deformity, requiring subsequent operations. The authors hope that their experience adds to the literature describing the surgical management of cervical deformities in these patients.

The Clinical Impact of Vagal Nerve Stimulator Implantation on Laryngopharyngeal Function in Children: A Single-Center Experience.

OBJECTIVE: A vagal nerve stimulator (VNS) has been established as the treatment of choice for children with refractory epilepsy. The outcomes of the procedure have been well documented in adults but are less clear in children. The goal of our study was to review laryngopharyngeal (LP) function following VNS implantation in children. STUDY DESIGN: Case series with chart review. SETTING: Tertiary-care children's hospital. METHODS: Voice, swallowing, and sleep apnea symptoms were extracted from the charts of children who underwent VNS implantation between 2013 and 2021. A questionnaire was sent to parents of implanted children to ascertain the degree of the social and functional impact of the implant. RESULTS: There were 69 patients, aged 2.3 to 21.4 years old, who met the inclusion criteria. LP symptoms were most common during the first year following implantation; 26 patients (37.6%) demonstrated at least 1 symptom (voice alteration, chronic cough, sleep-disordered breathing, or dysphagia), and 15 patients required adjustments to their implant settings. The incidence of symptoms and the need to adjust VNS settings significantly dropped during years 2 to 5 and 6 to 8 (22% vs 7% and 5%, respectively, p = .0002). The mean score of the Pediatric Voice Handicap Index differed greatly from a normal control group on each subscale and the total score. CONCLUSION: LP dysfunction in children following VNS implantation is comparable to adults, with the most burden noticed during the first year after implantation. The presence of voice alterations did not correlate with the presence of dysphagia and sleep-disordered breathing. Thorough evaluation, preferably by a multidisciplinary team, is required to assess LP dysfunction postoperatively.

An adolescent case of sellar osteochondromyxoma in the setting of spondyloepiphyseal dysplasia.

PURPOSE: Osteochondromyxomas (OMX) are rare congenital bone tumors that have only been described in the context of Carney complex syndrome (CNC). Data on OMX as a separate entity and in association with other disorders remain limited, making both diagnosis and treatment difficult. METHODS: A case report of a 17-year-old female diagnosed with sellar OMX is presented in the setting of spondyloepiphyseal dysplasia (SED). We discuss the radiographic and histopathological interpretations in addition to reviewing the current literature on OMX. RESULTS: A successful gross total resection of the tumor was achieved via an endonasal endoscopic transsphenoidal approach. A diagnosis was established radiographically and pathologically. CONCLUSION: The diagnosis and treatment of OMX are best achieved via tissue biopsy. Following confirmed osteochondromyxoma cases long term for recurrence and outcomes will be essential in understanding its natural tumor history and in establishing standard treatments.

Pediatric Endoscopic Endonasal Skull Base Surgery: A Retrospective Review Over 11 Years.

OBJECTIVE: To show the safety and efficacy of the endoscopic endonasal approach (EEA) for skull base surgery in pediatric patients through descriptive analysis of cases over an 11-year period. METHODS: The study comprised 94 patients undergoing EEA for skull base surgery, between January 2007 and June 2018, at 2 tertiary pediatric hospitals. Descriptive statistics are presented regarding the presentation, intraoperative details, and complications. RESULTS: Over the study period, 130 surgeries were performed in 94 patients: 94 primary surgeries and 36 reoperations. The mean patient age was 13.8 years and 48.9% of patients were female. Presenting signs/symptoms included endocrinopathies (56.4%), vision abnormalities (37.2%), and cranial nerve deficits (20.2%). EEA alone was used in 95.7% of primary surgeries and 91.7% of reoperations. Diseases treated included craniopharyngioma (18.1%), pituitary adenoma (17.0%), Rathke cleft cyst (13.8%), chordoma (9.6%), osteosarcoma (5.3%), juvenile nasopharyngeal angiofibroma (4.3%), skull base fracture (4.3%), and encephalocele (3.2%). A lumbar drain was used in 20.2% of primary surgeries and 25% of reoperations. A nasoseptal flap was used in 36.2% of primary surgeries and 25% of reoperations. Postoperative complications included cerebrospinal fluid leak (12.8%), sinusitis (7.4%), bacterial meningitis (3.2%), and carotid artery injury in 1 reoperation. CONCLUSIONS: EEA for anterior cranial base disease is safe and efficacious in pediatric patients and can be used to treat many of the rare and heterogenous diseases that arise in this anatomic location. Management strategies and rates of sinonasal and intracranial complications including cerebrospinal fluid leak rate are similar to those reported in adult cohorts.

Pediatric pineal region tumors: institutional experience of surgical managements with posterior interhemispheric transtentorial approach.

PURPOSE: Resecting pineal region tumors in children is often challenging. Several approaches have been proposed and practiced. A personal series of pediatric pineal region tumors resected through craniotomy with posterior interhemispheric occipital transtentorial (OT) approach are reviewed. We present the surgical techniques, pitfalls, and their results. MATERIAL AND METHODS: Eighty patients ranging in age from 3 months to 21 years old, and treated over 3 decades were reviewed. Hydrocephalus caused the main presenting symptoms and was noted in 74 patients. It was treated prior to the craniotomy for tumor resection with endoscopic third ventriculostomy (ETV) in 33, external ventricular drainage in 26, and precraniotomy shunt in 15. Nine patients had ETV together with endoscopic biopsy. All patients had a parieto-occipital craniotomy in a prone position. Through a tentorial section, a gross total resection of the tumor was attempted except for germinomas. RESULTS: The tumor pathology showed 32 germ cell tumors (GCT), 22 benign astrocytomas, 13 pineal parenchymal tumors, 5 ATRTs, 3 papillary tumors, and 5 others. Of GCTs, 18 were teratomas. The extent of resection consisted of 55 gross total resections, 13 subtotal resections, 10 partial, and 2 biopsies with one postoperative death. Hemiparesis in 2, cerebellar ataxia in another 2, and hemiballismus in 1 were transient and improved over time. One had permanent hemisensory loss and another patient had bilateral oculomotor palsy. Postoperative homonymous hemianopia occurred in 2 patients but subsided over a short period of time. Parinaud's sign was noted in 24 patients, of which 16 were transient. CONCLUSION: The posterior interhemispheric OT approach provides a safe route and comfortable access to the pineal region in children. A great majority of postoperative neurological complications are the results of direct manipulations of the midbrain at tumor resection. Identification and preservation of the tumor-brain interface are of paramount importance. GCTs other than teratomas are treated with neoadjuvant chemotherapy and may eliminate the need for craniotomy. Exophytic midbrain JPAs are amenable to resection.

Endoscopic endonasal optic nerve decompression: treatment of fibrous dysplasia in a pediatric population.

OBJECTIVE: Patients with fibrous dysplasia (FD) of the anterior skull base can experience progressive visual loss and impairment. The authors reviewed their experience with endonasal decompression of the optic nerve (ON) in this patient population. Endoscopic ON decompression (EOND) is a feasible surgical approach for children with FD and visual deficit due to structural ON compression. METHODS: Electronic medical records of children between 1 and 17 years of age with unilateral FD of the anterior skull base and concomitant ON compression, who required EOND between 2017 and 2022 (n = 4), were reviewed for demographic data, both pre- and postoperative imaging, and evaluations by an otolaryngologist, neurosurgeon, and ophthalmologist in a multidisciplinary fashion. RESULTS: EOND was found to be a safe and effective surgery for children with FD. Visual acuity was stable in 80% of the eyes postoperatively. Visual fields improved in 40% of the eyes and remained stable in the rest. CONCLUSIONS: EOND is beneficial for progressive optic neuropathy that is unresponsive to steroid therapy and can prevent permanent disability if performed prior to irreversible damage to the nerve. EOND can decompress the edematous ON with proper exposure of the optic canal and orbital apex, without any major complications.

Optimizing the patient handoff and progress note documentation efficiency in the EPIC EMR system within a neurosurgery residency: A quality improvement initiative.

BACKGROUND: Handoffs and documentation are a potentially modifiable source of medical error. However, little attention has been given toenhancementof these within the neurosurgical field. We aim to increase efficiency and accuracy of neurosurgical handoffs, including the neurological exam, thus decreasing medical documentation time within current duty-hour restrictions. METHODS: The existing Epic electronic medical record system was modified to include the neurological exam in the handoff: a tool used to generate lists including relevant patient clinical details and plans. The handoff tool was also converted into a subjective, objective, assessment, and plan (SOAP) format, which was leveraged to efficiently generate daily progress notes. A four-question survey was developed to assess the effectiveness of this new format. Mean note times were compared before and after the EPIC update using an independent samples t-test. RESULTS: All of the surveyed neurosurgery residents at our institution reported a decrease in documentation time per progress note, felt the notes were more accurate, and found it easier to recall the neurological exams of patients. 8/9 residents felt that the new handoff made in-house call less stressful. There was a significant difference in mean note time, with the mean note time of 37.9 s after the EPIC upgrade compared to 120 s prior the upgrade. We project that over 241 h of documentation will be saved annually at our institution. CONCLUSIONS: This QI project demonstrates how a low-effort initiative improved resident recall of patients' neurological exams while saving time spent documenting daily progress notes.

Pediatric benign triton tumor of trigeminal nerve: a case report and literature review.

PURPOSE: Benign triton tumors (BTTs) in the pediatric population are extremely rare occurrences. Paucity of data on BTTs poses both diagnostic and therapeutic challenges, particularly when found intracranially. METHODS: A case report of a 10-year-old male diagnosed with incidental maxillary trigeminal (V2) BTT is presented. We discuss radiographic and histopathological interpretations. Furthermore, we provide a brief review of current literature and historical background on pediatric trigeminal BTT diagnosis, histopathology, and management. RESULTS: Successful gross total resection of the tumor was achieved via Dolenc approach to the cavernous sinus. Management options with consideration of outcomes from the few prior cases reported in the literature are presented. CONCLUSION: Treatment of trigeminal nerve tumors requires a broad differential diagnosis and understanding rare tumors is essential in the diagnosis and treatment algorithm.

Presentation, management and outcome of surgically managed pediatric thoracic outlet syndrome.

PURPOSE: Thoracic outlet syndrome (TOS) is a rare disorder involving compression of the brachial plexus, subclavian artery, and subclavian vein. There is a paucity of data for this pathology's surgical treatment within pediatrics. The objective of this study is to explore the presentation, management, and outcome of pediatric TOS. METHODS: A retrospective chart review was conducted for 44 patients at a single institution undergoing surgery for TOS. Data was collected on demographics, pre- and postoperative factors, and outcomes. RESULTS: Forty-four patients underwent 50 surgeries (8 bilaterally). The average age was 15.5 years with 72% female. The most common symptoms were numbness (72%) and pain (66%), with a normal exam in 58%. The average symptom duration prior to surgery was 35.2 months. A supraclavicular approach was performed in all patients, with anterior scalene section (90%), rib resection (72%), neurolysis (92%), and intraoperative EMG (84%) commonly used. Two patients had a lymphatic leak. All patients reported subjective improvement of preoperative symptoms of numbness (26%), pain (22%), and weakness (6%). Differences between vTOS (n = 9) and nTOS (n = 35) included higher preop swelling (p < 0.012), decreased symptom duration (p < 0.022), higher venogram usage (p < 0.0030), and higher preoperative thrombolytics/angioplasty (p < 0.001) in vTOS compared to nTOS. A comparison of soft tissue and soft tissue with bone decompression did not reveal any outcome differences. CONCLUSION: Pediatric TOS benefits from a multidisciplinary approach, showing good outcomes in postoperative symptom resolution. In our cohort, a supraclavicular approach provided an effective window for decompression with a low complication rate.

Middle meningeal artery embolization in pediatric patient.

PURPOSE: There is paucity of data in management of recurrent and expanding subdural hematomas (SDH) within the pediatric population, who are otherwise not surgical candidates. Middle meningeal artery (MMA) embolization has been utilized minimally in this population and here, we explore the utility of this procedure in a 15-month-old-child, along with review of the literature. METHODS: A case report of a 15-month-old child who underwent MMA embolization for recurrent and expanding SDH in the setting of anticoagulation for cardiac condition. A literature review of MMA embolization in pediatric patients was conducted. RESULTS: Initially stabilization of SDH was noted on serial imaging; however, recurrent hemorrhages were noted with subsequent boluses of antiplatelet and anticoagulating agents. There are only 5 total reported cases, included ours, of MMA embolization in pediatrics with an overall success rate of 80%. CONCLUSION: Treatment of chronic or recurrent subdural hematoma by MMA embolization in the pediatric population is understudied. Our case notes limitation of this procedure and impact on long-term success, specifically in patients with systemic illness and ongoing anticoagulation.

The role of neurosurgery in the management of tuberous sclerosis complex-associated epilepsy: a systematic review.

OBJECTIVE: Tuberous sclerosis complex (TSC) is an autosomal dominant, multisystem neurocutaneous disorder associated with cortical tubers, brain lesions seen in nearly all patients with TSC, which are frequently epileptogenic. Seizures are often the earliest clinical manifestation of TSC, leading to epilepsy in over 70% of patients. Medical management with antiepileptic drugs constitutes early therapy, but over 50% develop medically refractory epilepsy, necessitating surgical evaluation and treatment. The objective of this study was to summarize the literature and report seizure outcomes following surgical treatment for TSC-associated epilepsy. METHODS: A systematic literature review was performed in accordance with the PRISMA guidelines. The PubMed and Embase databases were searched for journal articles reporting seizure outcomes following epilepsy surgery in TSC patients. Included studies were placed into one of two groups based on the surgical technique used. Excellent and worthwhile seizure reductions were defined for each group as outcomes and extracted from each study. RESULTS: A total of 46 studies were included. Forty of these studies reported seizure outcomes following any combination of resection, disconnection, and ablation on a collective 1157 patients. Excellent and worthwhile seizure reductions were achieved in 59% (683/1157) and 85% (450/528) of patients, respectively. Six of these studies reported seizure outcomes following treatment with neuromodulation. Excellent and worthwhile seizure reductions were achieved in 34% (24/70) and 76% (53/70) of patients, respectively. CONCLUSIONS: Surgery effectively controls seizures in select patients with TSC-associated epilepsy, but outcomes vary. Further understanding of TSC-associated epilepsy, improving localization strategies, and emerging surgical techniques represent promising avenues for improving surgical outcomes.

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Patient-Specific Characteristics Associated with Favorable Response to Vagus Nerve Stimulation.

OBJECTIVE: The expansion in treatments for medically refractory epilepsy heightens the importance of identifying patients who are likely to benefit from vagus nerve stimulation (VNS). Here, we identify predictors with a positive VNS response. METHODS: We present a retrospective analysis of 158 patients with medically refractory epilepsy. Patients were categorized as VNS responders or nonresponders. Baseline characteristics and time to VNS response were recorded. Univariate and multivariate Cox regression were used to identify predictors of response. Recursive partitioning analysis was used to identify likely VNS responders. RESULTS: Eighty-nine (56.3%) patients achieved ≥50% seizure frequency reduction. Left-hand dominance (hazard ratio [HR] 1.703, P = 0.038), age at epilepsy onset ≥15 years (HR 2.029, P = 0.005), duration of epilepsy ≥8 years (HR 1.968, P = 0.007) and age at implantation ≥35 years (HR 1.809, P = 0.020), and baseline seizure frequency <5/month (HR 1.569, P = 0.044) were significant univariate predictors of VNS response. Following multivariate Cox regression, left-hand dominance, age at epilepsy onset ≥15 years, and duration of epilepsy ≥8 years remained significant. With recursive partitioning analysis, patients with either age at epilepsy onset ≥15 years, left-hand dominance, or baseline seizure frequency <5/month were stratified into Group A and had a 73.9% responder rate; the remaining patients stratified into Group B had a 43.8% responder rate. CONCLUSIONS: Patients with age at epilepsy onset ≥15 years, left-hand dominance, or baseline seizure frequency <5/month are ideal candidates for VNS.

Chiari malformation type 1 presenting as isolated unilateral foot drop with rapid recovery following posterior fossa decompression.

BACKGROUND: Chiari malformation (CM) type 1 is characterized by descent of the cerebellar tonsils resulting from crowding of the posterior fossa. In 30% of cases, it is associated with syringomyelia. When symptomatic, it may result in a classic constellation of symptoms. CASE PRESENTATION: Here we describe a case of a 16-year-old male who presented with isolated, unilateral foot drop due to CM type 1 and holosyrinx. This unique presentation is extremely rare, and we additionally present a review of all other reported cases in the literature. After undergoing posterior fossa decompression with C1 laminectomy and duraplasty, our patient made a complete neurological recovery within 2 weeks postoperatively and his MRI entire spine at 3 months postoperatively demonstrated a nearly complete resolution of the holosyrinx with significant decompression of the foramen magnum. CONCLUSION: This rare presentation highlights the importance of maintaining a broad differential, particularly in pediatric patients, and expediting the workup in order to offer a surgical decompression within 1-2 months of foot weakness to maximize the probability of a full neurological recovery.

Pediatric spinal intramedullary anaplastic myxopapillary ependymoma: a case report.

A 6-year-old girl presented with a 1-week history of progressive upper and lower extremity weakness and bilateral upper extremity dysesthesia. Imaging demonstrated a 4.7 × 1.2-cm enhancing intramedullary lesion in the cervical spine from level C2 to C5 with associated cystic components and syringomyelia. The patient underwent a C2-C5 laminoplasty, with gross total resection of the intramedullary lesion. Histological analysis showed small to medium-sized epithelioid cells, with predominantly a solid architecture focally infiltrating into the adjacent spinal cord tissue. Focal papillary differentiation was present along with peri-vascular pseudorosettes, mucin microcysts, and globules of dense collagen. Focal anaplasia was noted with mitosis (5/10 HPF), focal necrosis, and elevated Ki67 10-15%. These findings were consistent with a myxopapillary ependymoma with anaplastic features. CSF cytology was negative for tumor cells. MYCN amplification was not present. She was treated with targeted proton-beam radiation therapy. This is the fourth case of an intramedullary anaplastic myxopapillary ependymoma to date, and the first case in the cervical spine reported in the literature.

Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia.

OBJECTIVE: The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS: The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS: A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS: Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Extradural decompression versus duraplasty in Chiari malformation type I with syrinx: outcomes on scoliosis from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Scoliosis is common in patients with Chiari malformation type I (CM-I)-associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS: A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS: In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS: In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

Dural augmentation approaches and complication rates after posterior fossa decompression for Chiari I malformation and syringomyelia: a Park-Reeves Syringomyelia Research Consortium study.

OBJECTIVE: Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM. METHODS: The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft. RESULTS: A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain. CONCLUSIONS: In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.