A systematic review of the power of standardization in pediatric neurosurgery.

In the current neurosurgical field, there is a constant emphasis on providing the best care with the most value. Such work requires the constant optimization of not only surgical but also perioperative services. Recent work has demonstrated the power of standardized techniques in limiting complication while promoting optimal outcomes. In this review article, protocols addressing operative and perioperative care for common pediatric neurosurgical procedures are discussed. These articles address how various institutions have optimized procedures through standardization. Our objective is to improve patient outcomes through the optimization of protocols.

Multidisciplinary neurocutaneous syndrome clinics: a systematic review and institutional experience.

OBJECTIVE: Neurocutaneous syndromes have variable multisystem involvement. The multiorgan involvement, potential pathologies, and various treatment options necessitate collaboration and open discussion to ensure optimal treatment in any given patient. These disorders provide quintessential examples of chronic medical conditions that require a lifelong, multidisciplinary approach. The objectives of this study were to 1) perform a systematic review, thoroughly assessing different multidisciplinary clinic layouts utilized in centers worldwide; and 2) characterize an institutional experience with the management of these conditions, focusing on the patient demographics, clinical presentation, complications, and therapeutic strategies seen in a patient population. METHODS: A systematic review of studies involving multidisciplinary clinics and their reported structure was performed according to PRISMA guidelines using the PubMed database. Then a retrospective chart review of patients enrolled in the Oklahoma Children's Hospital Neurocutaneous Syndromes Clinic was conducted. RESULTS: A search of the PubMed database yielded 251 unique results. Of these, 15 papers were included in the analysis, which identified 16 clinics that treated more than 2000 patients worldwide. The majority of these clinics treated patients with neurofibromatosis (13/16). The remaining clinics treated patients with von Hippel-Lindau syndrome (n = 1), tuberous sclerosis complex (n = 1), and multiple neurocutaneous syndromes (n = 1). The most commonly represented subspecialties in these clinics were genetics (15/16) and neurology (13/16). Five clinics (31%) solely saw pediatric patients, 10 clinics saw a combination of children and adults, and the final clinic had separate pediatric and adult clinics. The retrospective chart review of the Neurocutaneous Syndromes Clinic demonstrated that 164 patients were enrolled and seen in the clinic from April 2013 to December 2021. Diagnoses were made based on clinical findings or results of genetic testing; 115 (70%) had neurofibromatosis type 1, 9 (5.5%) had neurofibromatosis type 2, 35 (21%) had tuberous sclerosis complex, 2 (1%) had von Hippel-Lindau syndrome, 2 (1%) had Gorlin syndrome, and the remaining patient (0.6%) had Aarskog-Scott syndrome. Patient demographics, clinical presentation, complications, and therapeutic strategies are also discussed. CONCLUSIONS: To the best of the authors' knowledge, this is the first detailed description of a comprehensive pediatric neurocutaneous clinic in the US that serves patients with multiple syndromes. There is currently heterogeneity between described multidisciplinary clinic structures and practices. More detailed accounts of clinic compositions and practices along with patient data and outcomes are needed in order to establish the most comprehensive and efficient multidisciplinary approach for neurocutaneous syndromes.

Spinal cord stimulation for treatment of chronic neuropathic pain in adolescent patients: a single-institution series, systematic review, and individual participant data meta-analysis.

OBJECTIVE: Neuropathic pain is undertreated in children. Neurosurgical treatments of pediatric chronic pain are limited by the absence of both US Food and Drug Administration approval and pediatric-specific hardware, as well as weak referral patterns due to a lack of physician education. This study presents a single-institution retrospective case series of spinal cord stimulation (SCS) in children ≤ 19 years of age and a systematic review of SCS in children. The authors' findings may further validate the role of SCS as an effective treatment modality for varied neuropathic pain syndromes found in pediatric patients. METHODS: The study was a single-center, single-surgeon, retrospective case series of individuals treated between July 2017 and May 2022. The outcomes for pediatric patients with chronic neuropathic pain syndromes indicated by the multidisciplinary pain clinic for evaluation for SCS were cataloged. A systematic review and individual participant data (IPD) meta-analysis was performed for cases treated until May 2022, using PubMed, EMBASE, and Scopus to characterize outcomes of children with neuropathic pain treated with SCS. RESULTS: Twelve patients were evaluated and 9 were indicated for percutaneous or buried lead trials. Seven female and 2 male patients between the ages of 13 and 19 years were implanted with trial leads. Eight of 9 (89%) patients went on to receive permanent systems. The average trial length was 6 days, and the length of stay for both trial and implant was less than 1 day. Complication rates due to CSF leaks were 22% and 0% for trial and implant, respectively. Visual analog scale pain scores decreased from 9.2 to 2.9 (p = 0.0002) and the number of medications decreased from 4.9 to 2.1 (p = 0.0005). Functional status also improved for each patient. A systematic review identified 13 studies describing pediatric patients with SCS, including 12 providing IPD on 30 patients. In the IPD meta-analysis, pain was reduced in 16/16 (100%) of patients following surgery and in 25/26 (96.2%) at last follow-up. Medication use was decreased in 16/21 (76.2%), and functional outcomes were improved in 29/29 (100%). The complication rate was 5/30 (16.7%). CONCLUSIONS: SCS effectively decreases pain and medication use for pediatric neuropathic pain syndromes. Patients also report improved functional status, including improved matriculation, gainful employment, and physical activity. There is minimal high-quality literature describing neuromodulation for pain in children. Neuromodulation should be considered earlier as a viable alternative to escalating use of multiple drugs and as a potential mechanism to address tolerance, dependence, and addiction in pediatric patients.

Characterization of standard work tools for intrathecal baclofen therapy.

PURPOSE: Intrathecal baclofen (ITB) has been an effective therapy since the 1980s, with widely reported revision, infection, and complication rates. Publications targeting surgical workflow have resulted in decreased infection and revision rates, but a standard workflow for the entire pathway has not been described. To present, define, and test standard work tools for patients receiving ITB to promote uniformity and standard of care in the field. METHODS: A multidisciplinary approach from the movement disorder program of a tertiary care center defined all steps comprising the ITB pathway, and then developed standard work tools to decrease variability with respect to preoperative workup, day of surgery protocol, post-operative care, and also evaluation and treatment with respect to pump infection or malfunction. RESULTS: Defined steps used at specific points of ITB pathway are presented with a single institution's outcome using the protocol from July 2017 to November 2020. A total of 60 procedures were performed. The overall complication rate was 14.5% at 6 months. Complications included an infection rate of 3.6% at 6 months, wound revision rate of 1.8% at 6 months, CSF leak rate of 1.7% at 6 months, and a 30-day readmission rate related to initial surgery of 6.7%. CONCLUSIONS: Workflow efficiency and optimization for ITB patients can be used to obtain lower complication rates compared to historical cohorts in literature. A single-center, retrospective review highlights this.

Network-targeted approach and postoperative resting-state functional magnetic resonance imaging are associated with seizure outcome.

OBJECTIVE: Postoperative resting-state functional magnetic resonance imaging (MRI) in children with intractable epilepsy has not been quantified in relation to seizure outcome. Therefore, its value as a biomarker for epileptogenic pathology is not well understood. METHODS: In a sample of children with intractable epilepsy who underwent prospective resting-state seizure onset zone (SOZ)-targeted epilepsy surgery, postoperative resting-state functional MRI (rs-fMRI) was performed 6 to 12 months later. Graded normalization of the postoperative resting-state SOZ was compared to seizure outcomes, patient, surgery, and anatomical MRI characteristics. RESULTS: A total of 64 cases were evaluated. Network-targeted surgery, followed by postoperative rs-fMRI normalization was significantly (p < 0.001) correlated with seizure reduction, with a Spearman rank correlation coefficient of 0.83. Of 39 cases with postoperative rs-fMRI SOZ normalization, 38 (97%) became completely seizure free. In contrast, of the 25 cases without complete rs-fMRI SOZ normalization, only 3 (5%) became seizure free. The accuracy of rs-fMRI as a biomarker predicting seizure freedom is 94%, with 96% sensitivity and 93% specificity. INTERPRETATION: Among seizure localization techniques in pediatric epilepsy, network-targeted surgery, followed by postoperative rs-fMRI normalization, has high correlation with seizure freedom. This study shows that rs-fMRI SOZ can be used as a biomarker of the epileptogenic zone, and postoperative rs-fMRI normalization is a biomarker for SOZ quiescence. ANN NEUROL 2019;86:344-356.

Stereotactic laser ablation for subependymal giant cell astrocytomas: personal experience and review of the literature.

PURPOSE: Subependymal giant cell astrocytomas (SEGAs) are rare tumors typically found in tuberous sclerosis patients. They typically grow in the region of the foramen of Monro and can occlude it, leading to hydrocephalus. Currently, gross total resection is the standard of care, with low rates of recurrence but high rates of complication, especially with larger lesions. Laser interstitial thermal therapy (LITT) is a newly emerging treatment modality for a variety of pathologies. Here, we present a case series of SEGAs managed via LITT and endoscopic, stereotactic septostomy. METHODS: A retrospective chart review was performed to identify three cases in which SEGAs were treated via LITT and septostomy. Stereotactic ablation was performed via magnetic resonance (MR) thermometry with laser output set to 69% for 2.5 min, with post-ablation scans for visualization of treatment area. RESULTS: Average age at surgery was 8.2 years. Pre-operative tumor volumes were 0.43, 1.51, and 3.88 cm3. Post-operative tumor volumes were 0.25, 0.21, and 0.68 cm3. Mean tumor volume reduction was 70%. No complications occurred. CONCLUSION: LITT with septostomy should be considered a viable primary or adjunct treatment modality for SEGAs.

Subcentimeter epilepsy surgery targets by resting state functional magnetic resonance imaging can improve outcomes in hypothalamic hamartoma.

OBJECTIVE: The purpose of this study is to investigate the outcomes of epilepsy surgery targeting the subcentimeter-sized resting state functional magnetic resonance imaging (rs-fMRI) epileptogenic onset zone (EZ) in hypothalamic hamartoma (HH). METHODS: Fifty-one children with HH-related intractable epilepsy received anatomical MRI-guided stereotactic laser ablation (SLA) procedures. Fifteen of these children were control subjects (CS) not guided by rs-fMRI. Thirty-six had been preoperatively guided by rs-fMRI (RS) to determine EZs, which were subsequently targeted by SLA. The primary outcome measure for the study was a predetermined goal of 30% reduction in seizure frequency and improvement in class I Engel outcomes 1 year postoperatively. Quantitative and qualitative volumetric analyses of total HH and ablated tissue were also assessed. RESULTS: In the RS group, the EZ target within the HH was ablated with high accuracy (>87.5% of target ablated in 83% of subjects). There was no difference between the groups in percentage of ablated hamartoma volume (P = 0.137). Overall seizure reduction was higher in the rs-fMRI group: 85% RS versus 49% CS (P = 0.0006, adjusted). The Engel Epilepsy Surgery Outcome Scale demonstrated significant differences in those with freedom from disabling seizures (class I), 92% RS versus 47% CS, a 45% improvement (P = 0.001). Compared to prior studies, there was improvement in class I outcomes (92% vs 76%-81%). No postoperative morbidity or mortality occurred. SIGNIFICANCE: For the first time, surgical SLA targeting of subcentimeter-sized EZs, located by rs-fMRI, guided surgery for intractable epilepsy. Our outcomes demonstrated the highest seizure freedom rate without surgical complications and are a significant improvement over prior reports. The approach improved freedom from seizures by 45% compared to conventional ablation, regardless of hamartoma size or anatomical classification. This technique showed the same or reduced morbidity (0%) compared to recent non-rs-fMRI-guided SLA studies with as high as 20% permanent significant morbidity.

Immediate Abdominal Pain after Placement of Thoracic Paddle Leads for Spinal Cord Stimulation: A Case Series.

BACKGROUND: Spinal cord stimulation (SCS) is a well-established treatment modality for chronic pain. Thoracic radiculopathy has been reported as a complication of SCS paddle lead implantation by several authors and commonly presents as abdominal pain. METHODS: We performed a search of all patients who underwent either placement of a new epidural paddle lead electrode or revision of an epidural paddle lead electrode for SCS in the thoracic region from January 2017 to January 2018. We then investigated all cases of immediate postoperative abdominal pain. RESULTS: We identified 7 patients who had immediate postoperative abdominal pain among 86 cases of epidural SCS procedures. Most patients were discharged on postoperative days 1-3. No patients required revisions or removals of their SCS for any reason. CONCLUSIONS: We conclude that the etiology of immediate postoperative abdominal pain after thoracic paddle lead implantation for SCS is most likely thoracic radiculopathy. We hypothesize that small, transient epidural hematomas could be the cause of this thoracic radiculopathy. We argue that all patients with immediate postoperative abdominal pain and no other neurologic deficits after thoracic paddle lead implantation for SCS should first be treated conservatively with observation and pain management.

Pediatric National Surgical Quality Improvement Program: Useful for Quality Improvement in Craniosynostosis Surgery?

The American College of Surgeons and the American Pediatric Surgical Association collaborate to provide pediatric hospitals with multispeciality surgical outcomes data through the Pediatric National Surgical Quality Improvement Program (NSQIP Peds). The authors used this national multicenter database to describe 30-day outcomes from craniosynostosis surgery and identify associations with perioperative events and blood transfusion.Data from NSQIP Peds were used to describe children undergoing craniosynostosis surgery. The authors examined statistical association of clinical risk factors with the defined end point outcomes of perioperative complications and blood transfusion.Five hundred seventy-two surgeries were included. By Common Procedural Terminology codes, 93 identified as single suture synostosis, the remainder as multiple or unknown suture involvement. Location of the affected suture is not captured. Mean surgical time was 196.84 minutes (SD 113.46). Mean length of stay was 4.22 days (SD 5.04). Sixty-seven percent of patients received blood transfusions. 3.15% were other perioperative occurrences, including infection, wound disruption, unplanned reintubation, stroke/hemorrhage, cardiac arrest, seizures, thromboembolism. 2.8% were readmitted; 2.45% underwent reoperation within 30 days. Duration of surgery and length of hospital stay significantly differed in the presence of blood transfusion versus none. On multivariate analysis, duration from anesthesia start to surgery start, duration from surgery end to anesthesia end, and duration of operation were risk factors for blood transfusion.Pediatric NSQIP gives a national overview of 30-day outcome metrics in craniosynostosis surgery. Perioperative adverse event rate was 3.15%. Duration of surgery and duration of anesthesia were significantly associated with blood transfusion. The authors identified opportunities for pediatric NSQIP database improvement.

Learning Curve in Robotic Stereoelectroencephalography: Single Platform Experience.

BACKGROUND: Learning curve, training, and cost impede widespread implementation of new technology. Neurosurgical robotic technology introduces challenges to visuospatial reasoning and requires the acquisition of new fine motor skills. Studies detailing operative workflow, learning curve, and patient outcomes are needed to describe the utility and cost-effectiveness of new robotic technology. METHODS: A retrospective analysis was performed of pediatric patients who underwent robotic stereoelectroencephalography (sEEG) with the Medtronic Stealth Autoguide. Workflow, total operative time, and time per electrode were evaluated alongside target accuracy assessed via error measurements and root sum square. Patient demographics and clinical outcomes related to sEEG were also assessed. RESULTS: Robot-assisted sEEG was performed in 12 pediatric patients. Comparison of cases over time demonstrated a mean operative time of 363.3 ± 109.5 minutes for the first 6 cases and 256.3 ± 59.1 minutes for the second 6 cases, with reduced operative time per electrode (P = 0.037). Mean entry point error, target point error, and depth point error were 1.82 ± 0.77 mm, 2.26 ± 0.71 mm, and 1.27 ± 0.53 mm, respectively, with mean root sum square of 3.23 ± 0.97 mm. Error measurements between magnetic resonance imaging and computed tomography angiography found computed tomography angiography to be more accurate with significant differences in mean entry point error (P = 0.043) and mean target point error (P = 0.035). The epileptogenic zone was identified in 11 patients, with therapeutic surgeries following in 9 patients, of whom 78% achieved an Engel class I. CONCLUSIONS: This study demonstrated institutional workflow evolution and learning curve for the Autoguide in pediatric sEEG, resulting in reduced operative times and increased accuracy over a small number of cases. The platform may seamlessly and quickly be incorporated into clinical practice, and the provided workflow can facilitate a smooth transition.