Building consensus for the medical management of children with moderate and severe acute spinal cord injury: a modified Delphi study.

OBJECTIVE: The focus of this modified Delphi study was to investigate and build consensus regarding the medical management of children with moderate and severe acute spinal cord injury (SCI) during their initial inpatient hospitalization. This impetus for the study was based on the AANS/CNS guidelines for pediatric SCI published in 2013, which indicated that there was no consensus provided in the literature describing the medical management of pediatric patients with SCIs. METHODS: An international, multidisciplinary group of 19 physicians, including pediatric neurosurgeons, orthopedic surgeons, and intensivists, were asked to participate. The authors chose to include both complete and incomplete injuries with traumatic as well as iatrogenic etiologies (e.g., spinal deformity surgery, spinal traction, intradural spinal surgery, etc.) due to the overall low incidence of pediatric SCI, potentially similar pathophysiology, and scarce literature exploring whether different etiologies of SCI should be managed differently. An initial survey of current practices was administered, and based on the responses, a follow-up survey of potential consensus statements was distributed. Consensus was defined as ≥ 80% of participants reaching agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A final meeting was held virtually to generate final consensus statements. RESULTS: Following the final Delphi round, 35 statements reached consensus after modification and consolidation of previous statements. Statements were categorized into the following eight sections: inpatient care unit, spinal immobilization, pharmacological management, cardiopulmonary management, venous thromboembolism prophylaxis, genitourinary management, gastrointestinal/nutritional management, and pressure ulcer prophylaxis. All participants stated that they would be willing or somewhat willing to change their practices based on consensus guidelines. CONCLUSIONS: General management strategies were similar for both iatrogenic (e.g., spinal deformity, traction, etc.) and traumatic SCIs. Steroids were recommended only for injury after intradural surgery, not after acute traumatic or iatrogenic extradural surgery. Consensus was reached that mean arterial pressure ranges are preferred for blood pressure targets following SCI, with goals between 80 and 90 mm Hg for children at least 6 years of age. Further multicenter study of steroid use following acute neuromonitoring changes was recommended.

Cumulative strain-based metrics for predicting subconcussive head impact exposure-related imaging changes in a cohort of American youth football players.

OBJECTIVE: Youth football athletes are exposed to repetitive subconcussive head impacts during normal participation in the sport, and there is increasing concern about the long-term effects of these impacts. The objective of the current study was to determine if strain-based cumulative exposure measures are superior to kinematic-based exposure measures for predicting imaging changes in the brain. METHODS: This prospective, longitudinal cohort study was conducted from 2012 to 2017 and assessed youth, male football athletes. Kinematic data were collected at all practices and games from enrolled athletes participating in local youth football organizations in Winston-Salem, North Carolina, and were used to calculate multiple risk-weighted cumulative exposure (RWE) kinematic metrics and 36 strain-based exposure metrics. Pre- and postseason imaging was performed at Wake Forest School of Medicine, and diffusion tensor imaging (DTI) measures, including fractional anisotropy (FA), and its components (CL, CP, and CS), and mean diffusivity (MD), were investigated. Included participants were youth football players ranging in age from 9 to 13 years. Exclusion criteria included any history of previous neurological illness, psychiatric illness, brain tumor, concussion within the past 6 months, and/or contraindication to MRI. RESULTS: A total of 95 male athletes (mean age 11.9 years [SD 1.0 years]) participated between 2012 and 2017, with some participating for multiple seasons, resulting in 116 unique athlete-seasons. Regression analysis revealed statistically significant linear relationships between the FA, linear coefficient (CL), and spherical coefficient (CS) and all strain exposure measures, and well as the planar coefficient (CP) and 8 strain measures. For the kinematic exposure measures, there were statistically significant relationships between FA and RWE linear (RWEL) and RWE combined probability (RWECP) as well as CS and RWEL. According to area under the receiver operating characteristic (ROC) curve (AUC) analysis, the best-performing metrics were all strain measures, and included metrics based on tensile, compressive, and shear strain. CONCLUSIONS: Using ROC curves and AUC analysis, all exposure metrics were ranked in order of performance, and the results demonstrated that all the strain-based metrics performed better than any of the kinematic metrics, indicating that strain-based metrics are better discriminators of imaging changes than kinematic-based measures. Studies relating the biomechanics of head impacts with brain imaging and cognitive function may allow equipment designers, care providers, and organizations to prevent, identify, and treat injuries in order to make football a safer activity.

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.

Incidental durotomy in the pediatric spine population.

OBJECTIVESpine surgery is less common in children than adults. These surgeries, like all others, are subject to complications such as bleeding, infection, and CSF leak. The rate of incidental durotomy in the pediatric population, and its associated complications, has scarcely been reported in the literature.METHODSThis is a retrospective chart review of all pediatric patients operated on at Wake Forest Baptist Health from 2012 to 2017 who underwent spine surgeries. The authors excluded any procedures with intended durotomy, such as tethered cord release or spinal cord tumor resection.RESULTSFrom 2012 to 2017, 318 pediatric patients underwent surgery for a variety of indications, including adolescent idiopathic scoliosis (51.9%), neuromuscular scoliosis (27.4%), thoracolumbar fracture (2.83%), and other non-fusion-related indications (3.77%). Of these patients, the average age was 14.1 years, and 71.0% were female. There were 6 total incidental durotomies, resulting in an overall incidence of 1.9%. The incidence was 18.5% in revision operations, compared to 0.34% for index surgeries. Comparison of the revision cohort to the durotomy cohort revealed a trend toward increased length of stay, operative time, and blood loss; however, the trends were not statistically significant. The pedicle probe was implicated in 3 cases and the exact cause was not ascertained in the remaining 3 cases. The 3 durotomies caused by pedicle probe were treated with bone wax; 1 was treated with dry Gelfoam application and 2 were treated with primary repair. Only 1 patient had a persistent leak postoperatively that eventually required wound revision.CONCLUSIONSIncidental durotomy is an uncommon occurrence in the pediatric spinal surgery population. The majority occurred during placement of pedicle screws, and they were easily treated with bone wax at the time of surgery. Awareness of the incidence, predisposing factors, and treatment options is important in preventing complications and disability.

The use of dual growing rods to correct spinal deformity secondary to a low-grade spinal cord astrocytoma.

Pediatric intramedullary spinal cord astrocytomas are rare, and the majority are low grade, typically carrying a low risk of mortality, but a high risk of morbidity. Quality of life is, therefore, an important consideration in treating concomitant progressive kyphoscoliosis. Compared with fusion-based spinal stabilization, fusionless techniques may limit some complications related to early instrumentation of the developing spine. Another consideration is the timing of radiation therapy relative to both spinal maturity and spinal instrumentation. To date, there have been no reports of the use of a fusionless technique to treat spinal deformity secondary to an intramedullary spinal cord tumor. Herein, we report the use of fusionless spinal stabilization with dual growing rods in a boy with low-grade spinal cord astrocytoma after radiation therapy.

Intracranial pseudolymphoma presenting with grand mal seizures.

Primary central nervous system lymphoproliferative disorders comprise a heterogenous group of intracranial disease, predominantly of the high-grade non-Hodgkin's lymphoma type. We report a 56-year-old woman who developed new-onset grand mal seizures and was found to have two small uniformly enhancing dural-based lesions, which were radiologically concerning for meningiomas. Biopsy demonstrated findings consistent with benign, reactive lymphoid tissue. The patient's seizures resolved post-operatively. To our knowledge, this is the first reported patient with intracranial pseudolymphoma presenting as grand mal seizures. This case highlights this rare differential consideration in a patient with symptomatic dural-based lesion.

Vacuum-assisted closure for complex cranial wounds involving the loss of dura mater.

The aim in this study was to describe the safety and efficacy of vacuum-assisted closure (VAC) in patients with complex cranial wounds with extensive scalp, bone, and dural defects who were not candidates for immediate free tissue transfer. Five patients (4 men and 1 woman) ages 24-73 years with complex cranial wounds were treated with VAC at Wake Forest Baptist Medical Center. Etiologies included trauma, squamous cell carcinoma, and malignant meningioma. Cutaneous wound defects measured as large as 15 cm in diameter. Four of the 5 patients had open skull defects with concomitant dural defects, and 1 patient had dural dehiscence. After surgical debridement, all 5 patients were treated with the direct application of a VAC device to a reapproximated dura mater (1 patient), to a pericranial flap (1 patient), or to a regenerative tissue matrix overlying CNS tissue (3 patients). In all cases involving open cranial wounds, the VAC device promoted granulation tissue formation over the dural substitute, prevented CSF leakage, and kept the wounds free from local infection. The duration of VAC therapy ranged from 16 to 91 days. Although VAC therapy was intended as a temporary measure until these patients could be stabilized for larger tissue transfer procedures or they succumbed to their primary pathology, 1 patient had a successful skin graft following VAC therapy. Hydrocephalus requiring shunt placement developed in 2 patients during VAC therapy. The VAC dressings applied to a tissue matrix or other barrier over brain tissue in extensive cranial wounds are safe and well tolerated, providing a functional barrier and preventing infection.

Sacroiliac joint pain in the pediatric population.

OBJECT: The sacroiliac (SI) joint can be a pain generator in 13%-27% of cases of back pain in adults. These numbers are largely unknown for the pediatric population. In children and especially girls, development of the pelvic girdle makes the SI joint prone to misalignment. Young athletes sustain repeated stress on their SI joints, and sometimes even minor trauma can result in lasting pain that mimics radiculopathy. The authors present a series of 48 pediatric patients who were evaluated for low-back pain and were found to have SI joint misalignment as the cause of their symptoms. They were treated with a simple maneuver described in this paper that realigned their SI joint and provided significant improvement of symptoms. METHODS: A retrospective review of the electronic records identified 48 patients who were referred with primary complaints of low-back pain and were determined to have SI joint misalignment during bedside examination maneuvers described here. Three patients did not have a record of their response to treatment and were excluded. Patients were evaluated by a physical therapist and had the realignment procedure performed on the day of initial consultation. The authors collected data regarding the immediate effect of the procedure, as well as the duration of pain relief at follow-up visits. RESULTS: Eighty percent of patients experienced dramatic improvement in symptoms that had a lasting effect after the initial treatment. The majority of them were given a home exercise program, and only 2 of the 36 patients who experienced significant relief had to be treated again. Fifty-three percent of all patients had immediate and complete resolution of symptoms. Three of the 48 patients had missing data from the medical records and were excluded from computations. CONCLUSIONS: Back pain is multifactorial, and the authors' data demonstrate the potential importance of SI joint pathology. Although the technique described here for treatment of misaligned SI joints in the pediatric patients is not effective in all, the authors have observed significant improvement in 80% of cases. Often it is difficult to determine the exact cause of back pain, but when the SI joint is suspected as the primary pathology, the authors have described a simple and effective bedside treatment that should be attempted prior to the initiation of further testing and surgery.

Patients with Chiari malformation Type I presenting with acute neurological deficits: case series.

OBJECT: A subset of patients with Chiari malformation Type I (CM-I) presented with acute onset of a neurological deficit. In this study the authors summarize their experience with these patients' clinical presentation, imaging results, timing of surgery, and outcome following decompression. METHODS: The authors reviewed clinical records, imaging studies, and operative notes from all patients undergoing posterior fossa decompression for CM-I at St. Louis Children's Hospital from 1990 to 2008. Of the 189 patients who underwent surgery, 6 were identified with the acute onset of a neurological deficit at presentation. RESULTS: All 6 children (age range 3-14 years, 3 boys and 3 girls) had either syringomyelia (5 patients) or T2 signal changes in the spinal cord (1 patient) and CM-I on initial MR imaging. Three patients presented after minor trauma (1 with paraparesis, 2 with sensory deficits). Three patients presented without a clear history of trauma (1 with abrupt onset of spontaneous dysphagia and ataxia, 2 with sensory deficits). Decompression was performed at a mean 7.7 ± 4.9 days after symptom onset (7.0 ± 1.6 days after neurosurgical evaluation). In 1 patient, symptoms had resolved by the time of surgery; in the remainder of the patients, clear improvements were noted within 2 weeks of surgery, with complete resolution of symptoms by 12 months postoperatively. Follow-up MR images were obtained in 4 patients, demonstrating improvement in the extent of the syrinx in each patient. CONCLUSIONS: Children with CM-I and syringomyelia can develop acute spinal cord or bulbar deficits with relatively minor head or neck injuries. The prognosis for symptomatic improvement in the observed deficit is good, with each patient in our series showing resolution of deficits over time. However, based on this relatively limited experience, the authors suggest that patients who present with an acute neurological deficit and are found to have CM-I be managed with early posterior fossa decompression. Patients with CM-I and syringomyelia may be at higher risk of acute neurological deficit than those without a syrinx.

A review of surgical intervention in the setting of traumatic central cord syndrome.

BACKGROUND CONTEXT: Surgical treatment in the setting of central cord syndrome (CCS) has become safer since Schneider's original description. It is generally accepted that a decompressive surgical intervention is a valid treatment option in a patient with CCS and radiographic evidence of spinal cord compression. The optimal timing of surgical intervention for CCS remains controversial. PURPOSE: To review a single institution's experience managing CCS, with particular emphasis on surgical versus medical management, timing of surgery, neurologic outcomes, hospital length of stay, and complications. STUDY DESIGN: Retrospective review. PATIENT SAMPLE: One hundred twenty-six patients diagnosed with CCS were treated at Wake Forest University Baptist Medical Center between June 1985 and September 2006. OUTCOME MEASURES: Neurological outcomes were measured using the Frankel grading scale. Other outcome measures included hospital and intensive care unit (ICU) length of stay and complication profiles. METHODS: A retrospective chart review was performed on patients admitted to Wake Forest University Baptist Medical Center with the diagnosis of traumatic central cord injury from June 1985 to September 2006 with institutional review board approval. Neurologic status was recorded on presentation and at maximum follow-up using the Frankel classification. The surgical cohort was stratified into three subgroups with regard to the timing of surgical intervention after injury: surgery less than 24 hours after injury, surgery greater than 24 hours after injury but during the initial admission, and delayed operative intervention on a second hospital admission. Other variables collected included ICU and hospital length of stay and complication profiles. Data analyses were performed using SPSS (SPSS, Chicago, IL, USA) and Excel 2002 (Microsoft, Seattle, WA, USA). RESULTS: A total of 126 patients treated for CCS were reviewed. Sixty-seven patients received surgery compared with 59 patients managed nonoperatively. Of those managed operatively, 16 patients received surgery within 24 hours of the time of injury. There were 34 patients who received surgery greater than 24 hours after the time of injury but during their initial admission with a mean time to surgery of 6.4 days (5-52 days). There were 17 patients who received their operation on a second hospital admission with a mean time interval of 137 days between injury and surgery (3-209). Mean follow-up was 32 months (1-210 months). An improvement in Frankel grade was seen in the overall operative cohort compared with those patients who received medical management alone. No statistically significant difference in neurologic outcome using Frankel grades was identified between the surgical subgroups with regard to timing of surgery. A trend toward decreased length of stay was seen in the surgical subgroup that received surgery during their initial admission. No statistically significant difference was identified between complication rates for the operative and nonoperative groups; however, a trend toward fewer complications and deaths was seen in those who received surgery in the first 24 hours or during the initial hospitalization. CONCLUSIONS: Surgical treatment in the setting of CCS has become safer since Schneider's original description. Acknowledging its numerous limitations, this retrospective study supports surgical intervention in the setting of CCS as a safe effective management option. Improved Frankel grades were identified in those patients managed surgically compared with those receiving medical management alone. The data further shed light on the safety and potential benefits of early operative intervention for acute CCS compared with delayed surgical treatment. A prospective randomized controlled trial is needed to definitively compare surgical versus medical management and/or early versus delayed surgical treatment in the setting of traumatic CCS.

Hemispherotomy: efficacy and analysis of seizure recurrence.

OBJECT: Hemispherotomy generally is performed in hemiparetic patients with severe, intractable epilepsy arising from one cerebral hemisphere. In this study, the authors evaluate the efficacy of hemispherotomy and present an analysis of the factors influencing seizure recurrence following the operation. METHODS: The authors performed a retrospective review of 49 patients (ages 0.2-20.5 years) who underwent functional hemispherotomy at their institution. The first 14 cases were traditional functional hemispherotomies, and included temporal lobectomy, while the latter 35 were performed using a modified periinsular technique that the authors adopted in 2003. RESULTS: Thirty-eight of the 49 patients (77.6%) were seizure free at the termination of the study (mean follow-up 28.6 months). Of the 11 patients who were not seizure free, all had significant improvement in seizure frequency, with 6 patients (12.2%) achieving Engel Class II outcome and 5 patients (10.2%) achieving Engel Class III. There were no cases of Engel Class IV outcome. The effect of hemispherotomy was durable over time with no significant change in Engel class over the postoperative follow-up period. There was no statistical difference in outcome between surgery types. Analysis of factors contributing to seizure recurrence after hemispherotomy revealed no statistically significant predictors of treatment failure, although bilateral electrographic abnormalities on the preoperative electroencephalogram demonstrated a trend toward a worse outcome. CONCLUSIONS: In the present study, hemispherotomy resulted in freedom from seizures in nearly 78% of patients; worthwhile improvement was demonstrated in all patients. The seizure reduction observed after hemispherotomy was durable over time, with only rare late failure. Bilateral electrographic abnormalities may be predictive of posthemispherotomy recurrent seizures.

Occult dural arteriovenous fistula causing rapidly progressive conus medullaris syndrome and paraplegia after lumbar microdiscectomy.

BACKGROUND CONTEXT: A spinal dural arteriovenous fistula (DAVF) is a known cause of venous congestive myelopathy. These lesions are thought to be acquired. There exist two prior reports that describe DAVF presenting with slowly progressive myelopathy years after lumbar disc surgery. PURPOSE: We report the first case of a preexistent, asymptomatic DAVF that became acutely symptomatic after lumbar microdiscectomy causing rapidly progressive conus medullaris syndrome and paraplegia. STUDY DESIGN: Case report. METHODS: A 53-year-old Caucasian male presented with urinary retention, fecal incontinence, and progressive loss of function in bilateral lower extremities less than 3 months after a lumbar discectomy at an outside facility. The patient underwent microdiscectomy at L4/L5 and L5/S1 for left-sided radicular symptoms and evidence of herniated discs on magnetic resonance imaging (MRI). The patient's preoperative pain improved after the discectomy, but his bowel, bladder, and lower extremity function deteriorated steadily after discharge. Postoperative MRI showed salient serpentine vessels in the region of L1 and L2 with an enlarged T2 bright conus medullaris. Retrospective review of preoperative MRI revealed the presence of similar but less pronounced findings. A spinal arteriogram confirmed concerns of an arteriovenous malformation. The patient was transferred to our facility for definitive management of his DAVF via catheter embolization. RESULTS: Complete fistula obliteration was confirmed at the time of embolization and at 8 weeks follow-up with selective spinal angiography. The appearance of the conus medullaris normalized on follow-up MRI. The patient made a slow but significant recovery in rehabilitation. He regained the ability to independently ambulate but remains significantly disabled secondary to residual lower extremity weakness and spasticity. He continues self-catheterization for persistent neurogenic bladder dysfunction. CONCLUSIONS: This represents the first case of an occult spinal DAVF becoming acutely symptomatic after lumbar disc surgery. Although the etiology of the lesion and its symptomatic progression remains unknown, an alteration of blood flow through the DAVF as a result of surgery may have resulted in progressive venous congestive myelopathy. The present case highlights the importance of considering a spinal DAVF in the differential diagnosis of any patient with signs of myelopathy or conus medullaris syndrome after lumbar discectomy. In addition, this case underscores the gravity of recognizing subtle features that suggest the presence of an occult vascular malformation on preoperative imaging, as symptomatic progression of these lesions carries significant potential morbidity.