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.

A Constellation of Atypical Findings in a Nine-Year-Old Child With Dysembryoplastic Neuroepithelial Tumors: A Case Report and Review of the Literature.

Dysembryoplastic neuroepithelial tumors (DNETs) are rare, generally benign, mixed neuronal-glial neoplasms occurring most often between 10 and 14 years of age. These lesions are classically cortically based and solitary, found preferentially in the temporal lobe, and most commonly present with seizures. On magnetic resonance imaging (MRI), these lesions are generally cystic and have variable contrast enhancement, which, when present, often involves the periphery. Rarely, lesions followed radiographically may demonstrate delayed contrast enhancement. Here, we present a case of multifocal DNETs involving the cerebellum that demonstrated delayed contrast enhancement. In addition, these occurred in a patient with Noonan syndrome (NS), a "RASopathy" disorder associated with low-grade glial and glioneuronal tumors. We present a summary of all previously reported cases of cerebellar DNETs. Our patient was successfully treated surgically and is doing well clinically, now one year status post his last procedure, and is being closely monitored with serial MRIs for progression. Gross total resection is often curative without adjuvant therapy for most DNETs. Our case emphasizes the importance of radiographic surveillance, as multifocality and recurrence may necessitate more than one procedure. Lastly, clinicians should be suspicious for DNETs and other low-grade glial tumors when treating patients with NS, acknowledging their predisposition for multifocal involvement and atypical presentations.

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.

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.

Malignant spindle cell tumors of the posterior fossa in children: case series and review of management.

OBJECTIVE: The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population. METHODS: The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with "posterior fossa" or "spindle cell tumors" or "Ewing sarcoma" or "high-grade" or "spindle cell sarcoma" or "leptomeningeal melanocytoma" as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome. RESULTS: The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease. CONCLUSIONS: A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.

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.

Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups. CONCLUSION: Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD.

Suprasellar central nervous system ganglioneuroblastoma: a case in a 9-year-old child and review of the literature.

PURPOSE: Intracranial ganglioneuroblastomas are incredibly rare neuroectodermal tumors with only 8 described cases total, 5 of those having imaging findings METHODS: Here we present a 9-year-old female patient with 4 months progressive headaches, personality changes, and vomiting. We also present a review of the current literature of intracranial ganglioneuroblastomas. RESULTS: Imaging demonstrated a partially calcified suprasellar mass measuring 4.6 × 6.3 × 5 cm composed of both solid and cystic components, diagnosed to be a ganglioneuroblastoma, with mass effect on the lateral and 3rd ventricles, with a midline shift of right to left of 6-7 mm. She was treated with subtotal surgical resection, an intensive chemotherapeutic regimen, and radiation and has no residual disease on imaging 1 year and 4 months status post-surgery. CONCLUSION: To our knowledge, this is the first case of a ganglioneuroblastoma to mimic a craniopharyngioma based upon imaging findings and suprasellar location. As these cases are extremely rare, an optimal therapeutic regimen has not been defined. However, a combination of surgical resection, chemotherapy, and radiation therapy can be effective, as shown here with successful treatment and no evidence of residual disease.

Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established. METHODS: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length. RESULTS: The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16-0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67-5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate. CONCLUSIONS: These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.

Radiological and clinical predictors of scoliosis in patients with Chiari malformation type I and spinal cord syrinx from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors' goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis. METHODS: A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°). RESULTS: Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB-C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude. CONCLUSIONS: Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.

Intracranial malignant peripheral nerve sheath tumor variant: an unusual neurovascular phenotype sarcoma case invading through the petrous bone.

INTRODUCTION: Intracranial malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. Previously reported cases of intracranial MPNST have been associated with development within a prominent cranial nerve. METHODS: This is the first report of an MPNST with both nerve sheath and vascular phenotype that follows the neurovascular bundle, without arising in a major cranial nerve or in the setting of neurofibromatosis type 1 (NF1). RESULTS: The patient is a 14-year-old boy with a history of worsening headaches for the past several months, left-sided hearing loss, nausea, vomiting, and vertigo. MRI was performed that demonstrated a large extra-axial tumor compressing the left infratemporal posterior temporal region. The tumor was associated with significant destruction of the superior portion of the petrous bone and extension through the petrous into the upper posterior fossa, immediately below the tentorium. The patient underwent surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide. Pathology demonstrated a variant malignant peripheral nerve sheath tumor with both nerve sheath and vascular phenotype by immunostains. The patient's symptoms improved following treatment. CONCLUSION: We present the first reported case of an intracranial MPNST variant that developed along the neurovascular bundle as a sarcoma with both nerve sheath and vascular phenotype through the petrous bone and not associated with a major cranial nerve or with stigmata of neurofibromatosis type 1 (NF1). Although this is an extremely unusual presentation due to location and lack of prominent cranial nerves in that location, it is not unusual for benign nerve sheath tumors to follow the neurovascular bundle through foramen of cortical long bone or pelvis. This case suggests that physicians should incorporate intracranial MPNST variant into their differential diagnosis in the cranium, even when tumor is not located near a prominent cranial nerve. Surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide has led to improvement in patient symptoms.

Improving Responsiveness to Patient Phone Calls: A Pilot Study.

Optimal patient-physician communication in the outpatient clinical setting is critical for safe and effective patient care. Keeping track of multiple patient telephone messages can be difficult and hazardous if a structured system is not in place. A multidisciplinary group at Hershey Medical Center developed a standardized approach for addressing patient telephone calls at their outpatient surgical clinics. This program was designed to improve the patient experience by providing a realistic time frame for phone calls to be returned and requests fulfilled. Additionally, this system permitted phone calls to be tracked and documented appropriately and allowed for prioritization of urgent and emergent messages. Our intent for this program was to close potential gaps within the communication chain at our outpatient surgical clinics, improve overall communication between clinicians and their patients, and improve both patient and employee satisfaction.

A Pivotal Randomized Clinical Trial Evaluating the Safety and Effectiveness of a Novel Hydrogel Dural Sealant as an Adjunct to Dural Repair.

BACKGROUND: A watertight dural repair is critical to minimizing the risk of postoperative complications secondary to cerebrospinal fluid (CSF) leaks. OBJECTIVE: To evaluate the safety and efficacy of a novel hydrogel, Adherus Dural Sealant, when compared with control, DuraSeal Dural Sealant System, as an adjunct to standard methods of dural repair. METHODS: In this 17-center, prospective, randomized clinical trial designed as a noninferiority, single-blinded study, 124 patients received Adherus Dural Sealant (test sealant) and 126 received DuraSeal (control). The primary composite endpoint was the proportion of patients who were free of any intraoperative CSF leakage during Valsalva maneuver after dural repair, CSF leak/pseudomeningocele, and unplanned retreatment of the surgical site. Each component was then analyzed individually as a secondary endpoint. Patients were followed for 4 mo after surgery. RESULTS: The primary composite endpoint at the 120-d follow-up was achieved in 91.2% of the test sealant group compared with 90.6% of the control, thus showing that the test sealant was statistically significantly noninferior to DuraSeal ( P = .0049). Post hoc analysis of the primary composite endpoint at 14 d demonstrated superiority of the test sealant over the control ( P = .030). Primary endpoint failures in the control group tended to occur early in follow-up period, while a majority of test dural sealant failures were identified through protocol-required radiographic imaging at the 120-d follow-up visit. CONCLUSION: The test sealant, Adherus Dural Sealant, is a practical, safe, and effective adjunct to achieving a watertight dural closure after primary dural closure in cranial procedures.

Dermal Sinus Tract of Lumbosacral Spine in Children: Patterns on Magnetic Resonance Imaging and Scoring System.

Purpose Dermal sinus tract (DST) is a challenging clinical diagnosis in children. The purpose of our study was to analyze the added value of magnetic resonance imaging (MRI) in the diagnosis of DST involving the lumbosacral spine. We also sought to establish an MRI-based scoring system to simplify the diagnosis of DST. Methods MRI images of 20 patients with clinically suspected DST were retrospectively assessed by two neuroradiologists blinded to the surgical results. The MRI studies were performed from July 2003 to July 2013. Institutional Review Board (IRB) approval was obtained. All MRI studies were assessed with respect to five imaging signs: A) visualization on both sagittal and axial images, B) dural penetration, C) associated tethered cord, D) presence of tumor or inflammation, and E) attachment to conus medullaris. The frequency of each imaging sign in the study population was calculated. For the 20 patients who underwent surgery, sensitivity and specificity of each neuroradiologist's interpretation of the MRI data were calculated using operative findings as the gold standard. Results Twelve of the 20 had confirmed DSTs. The incidences of the five imaging signs were as follows: A) visualization on both sagittal and axial images (12/12, 100%), B) dural penetration (10/12, 83.3%), C) associated tethered cord (7/12, 58.3%), D) presence of tumor or inflammation (4/12, 33.3%), and E) attachment to the conus medullaris (4/12, 33.3%). The best combination of findings predictive of DST was simply the appearance of DST on both axial and sagittal imaging, which resulted in a sensitivity of 100% and a specificity of 75-100%. Conclusion Visualization of DST on both axial and sagittal imaging is the best marker for pathology proven DST. Using a higher threshold score on the five-point scoring system that we proposed did not increase sensitivity or specificity in the diagnosis of DST; however, it may still prove clinically helpful in standardizing reporting leading to a more accurate and detailed assessment.

Pneumorrhachis Resulting in Transient Paresis after PICC Line Insertion into the Ascending Lumbar Vein.

Obtaining intravascular access in the neonatal intensive care unit (NICU) is not only critical but also technically challenging. Malposition of the catheter tip is a known and well-documented complication. Specifically, peripherally inserted central venous catheter (PICC) line insertion into the ascending lumbar vein can lead to neurological dysfunction and, in some cases, even death. We present the first reported case of pneumorrhachis (PR) following PICC line insertion into the ascending lumbar vein. Our patient presented with lower extremity weakness and imaging confirmed the presence of air within the spinal canal. After conservative treatment, the strength deficit resolved and subsequent imaging revealed resolution of the air within the spinal canal. Insertion of central venous catheters into the ascending lumbar vein is a well-documented complication that can lead to neurologic injury and even death. This should be considered in the evaluation of any neonate presenting with an abnormal neurological examination or unexplained change in exam after line insertion.

Intracranial venous drainage through spinal veins.

There is extensive collateral networking at the craniocervical junction with a substantial anatomical and functional continuity between the veins, venous sinuses, and venous plexuses of the brain and spine. The predominant pathway for intracranial blood outflow may depend on the level and degree of obstruction. We are presenting an unusual case of predominant egress of intracranial blood through enlarged spinal canal venous collaterals due to thrombosis of the intracranial venous sinuses. Awareness of this unique pattern of venous drainage of the cranium is important and should be considered in the differential diagnosis of spinal arteriovenous malformation (AVM) or arteriovenous fistula (AVF). Magnetic Resonance Venography (MRV) of the brain should be considered to exclude intracranial thrombosis in these cases.

Impact of improved documentation on an academic neurosurgical practice.

OBJECT: Accuracy in documenting clinical care is becoming increasingly important; it can greatly affect the success of a neurosurgery department. As patient outcomes are being more rigorously monitored, inaccurate documentation of patient variables may present a distorted picture of the severity of illness (SOI) of the patients and adversely affect observed versus expected mortality ratios and hospital reimbursement. Just as accuracy of coding is important for generating professional revenue, accuracy of documentation is important for generating technical revenue. The aim of this study was to evaluate the impact of an educational intervention on the documentation of patient comorbidities as well as its impact on quality metrics and hospital margin per case. METHODS: All patients who were discharged from the Department of Neurosurgery of the Penn State Milton S. Hershey Medical Center between November 2009 and June 2012 were evaluated. An educational intervention to improve documentation was implemented and evaluated, and the next 16 months, starting in March 2011, were used for comparison with the previous 16 months in regard to All Patient Refined Diagnosis-Related Group (APR-DRG) weight, SOI, risk of mortality (ROM), case mix index (CMI), and margin per discharge. RESULTS: The APR-DRG weight was corrected from 2.123 ± 0.140 to 2.514 ± 0.224; the SOI was corrected from 1.8638 ± 0.0855 to 2.154 ± 0.130; the ROM was corrected from 1.5106 ± 0.0884 to 1.801 ± 0.117; and the CMI was corrected from 2.429 ± 0.153 to 2.825 ± 0.232, and as a result the average margin per discharge improved by 42.2%. The mean values are expressed ± SD throughout. CONCLUSIONS: A simple educational intervention can have a significant impact on documentation accuracy, quality metrics, and revenue generation in an academic neurosurgery department.

Multifocal lymphangioendotheliomatosis with devastating intracranial hemorrhage.

An in utero female was found to have a small hemorrhage at the foramen of Monro, hydrocephalus, and what was originally interpreted as a Dandy-Walker variant. At birth she had macrocephaly and numerous cutaneous, multifocal, red-pink blanchable macules. Postnatal MRI demonstrated a hemorrhagic soft-tissue mass involving the upper brainstem, thalamus, and basal ganglia most consistent with in utero complex multifocal intracranial hemorrhage. The skin lesions were thought to be consistent with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). The size and location of the hemorrhage precluded operative intervention, although the hydrocephalus was treated with a ventricular shunt. The child continues to have severe developmental delays. Multifocal lymphangioendotheliomatosis with thrombocytopenia is a multifocal vascular disorder most commonly involving the skin and gastrointestinal tract. Intracranial hemorrhages are rare in this context. This case is the third reported instance of MLT with associated intracranial hemorrhage and the only case described in the neurosurgical literature. The authors review the presenting features and pathophysiology of this condition.

Perceived benefits and barriers to a career in pediatric neurosurgery: a survey of neurosurgical residents.

OBJECT: Research suggests that there may be a growing disparity between the supply of and demand for both pediatric specialists and neurosurgeons. Whether pediatric neurosurgeons are facing such a disparity is disputable, but interest in pediatric neurosurgery (PNS) has waxed and waned as evidenced by the number of applicants for PNS fellowships. The authors undertook a survey to analyze current neurosurgical residents' perceptions of both benefits and deterrents to a pediatric neurosurgical career. METHODS: All residents and PNS fellows in the United States and Canada during the academic year 2008-2009 were invited to complete a Web-based survey that assessed 1) demographic and educational information about residents and their residency training, particularly as it related to training in PNS; 2) residents' exposure to mentoring opportunities from pediatric neurosurgical faculty and their plans for the future; and 3) residents' perceptions about how likely 40 various factors were to influence their decision about whether to pursue a PNS career. RESULTS: Four hundred ninety-six responses were obtained: 89% of the respondents were male, 63% were married, 75% were in at least their 3rd year of postgraduate training, 61% trained in a children's hospital and 29% in a children's "hospital within a hospital," and 72% were in programs having one or more dedicated PNS faculty members. The residencies of 56% of respondents offered 6-11 months of PNS training and nearly three-quarters of respondents had completed 2 months of PNS training. During medical school, 92% had been exposed to neurosurgery and 45% to PNS during a clinical rotation, but only 7% identified a PNS mentor. Nearly half (43%) are considering a PNS career, and of these, 61% are definitely or probably considering post-residency fellowship. On the other hand, 68% would prefer an enfolded fellowship during residency. Perceived strengths of PNS included working with children, developing lasting relationships, wider variety of operations, fast healing and lack of comorbidities, and altruism. Perceived significant deterrents included shunts, lower reimbursement, cross-coverage issues, higher malpractice premiums and greater legal exposure, and working with parents and pediatric health professionals. The intrinsic nature of PNS was listed as the most significant deterrent (46%) followed by financial concerns (25%), additional training (12%), longer work hours (12%), and medicolegal issues (4%). The majority felt that fellowship training and PNS certification should be recommended for surgeons treating of all but traumatic brain injuries and Chiari I malformations and performing simple shunt-related procedures, although they felt that these credentials should be required only for treating complex craniosynostosis. CONCLUSIONS: The nature of PNS is the most significant barrier to attracting residents, although reimbursement, cross-coverage, and legal issues are also important to residents. The authors provide several recommendations that might enhance resident perceptions of PNS and attract trainees to the specialty.