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Virendra R. Desai, Jeffrey S. Raskin, Arvind Mohan, JoWinsyl Montojo, Valentina Briceño, Daniel J. Curry, and Sandi Lam

OBJECTIVE

Intrathecal baclofen pumps are generally placed in children for the treatment of spasticity and dystonia. Use of implants in this pediatric population with comorbidities is reported to have a high risk of complications and infections. With the aim of reducing baclofen pump–related infections, a quality improvement project was instituted at the authors’ institution.

METHODS

A workflow paradigm unique to baclofen pump implantation aimed at decreasing implant-related infections was implemented. All baclofen pump operations performed at the authors’ institution between August 2012 and June 2016 were reviewed. An infection prevention protocol was created and implemented in August 2014 based on a literature review and the consensus opinion of the pediatric neurosurgeons in the group. Compliance with the prevention bundle was tracked. Case outcomes before and after implementation of the protocol with a minimum of 3 months of follow-up were retrospectively reviewed. Univariate and multivariate analyses were performed to assess the association of the steps in the prevention bundle with infection and complication outcomes.

RESULTS

A total of 128 baclofen pump surgeries were performed (64 preprotocol and 64 postprotocol). The patient age range was 3 to 27 years. The overall compliance rate with the infection prevention bundle was 82%. The pre- and postimplementation infection rates were 12.5% and 6.3%, respectively (p = 0.225). The total pre- and postimplementation complication rates were 23.4% and 9.4%, respectively (p = 0.032). The absolute and relative risk reductions for infections were 6.3% (95% CI 3.8%–16.3%) and 50%, respectively; for complications, the absolute and relative risk reductions were 14.1% (95% CI 1.5%–26.7%) and 60%, respectively.

CONCLUSIONS

The total complication rate following intrathecal baclofen pump surgery was significantly lower after implementation of the quality improvement protocol. This study is an example of using checklist standardization to diminish special cause variability.

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Gavin W. Britz, Sandip S. Panesar, Peter Falb, Johnny Tomas, Virendra Desai, and Alan Lumsden

OBJECTIVE

The aim of this study was to evaluate new, neuroendovascular-specific engineering and software modifications to the CorPath GRX Robotic System for their ability to support safer and more effective cranial neurovascular interventions in a preclinical model.

METHODS

Active device fixation (ADF) control software, permitting automated manipulation of the guidewire relative to the microcatheter, and a modified drive cassette suitable for neuroendovascular instruments were the respective software and hardware modifications to the current CorPath GRX robot, which was cleared by the FDA for percutaneous coronary and peripheral vascular intervention. The authors then trialed the modified system in a live porcine model with simulated neuroendovascular pathology. Femoral access through the aortic arch to the common carotid artery was accomplished manually (without robotic assistance), and the remaining endovascular procedures were performed with robotic assistance. The system was tested for the enhanced ability to navigate and manipulate neurovascular-specific guidewires and microcatheters. The authors specifically evaluated the movement of the wire forward and backward during the advancement of the microcatheter.

RESULTS

Navigation of the rete mirabile and an induced aneurysm within the common carotid artery were successful. The active device fixation feature enabled independent advancement and retraction of the guidewire and working device relative to the microcatheter. When ADF was inactive, the mean forward motion of the guidewire was 5 mm and backward motion was 0 mm. When ADF was active, the mean forward motion of the guidewire was 0 mm and backward motion was 1.5 mm. The modifications made to the robotic cassette enabled the system to successfully manipulate the microcatheter and guidewire safely and in a manner more suited to neuroendovascular procedures than before. There were no occurrences of dissection, extravasation, or thrombosis.

CONCLUSIONS

The robotic system was originally designed to navigate and manipulate devices for cardiac and peripheral vascular intervention. The current modifications described here improved its utility for the more delicate and tortuous neurovascular environment. This will set the stage for the development of a neurovascular-specific robot.

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Virendra Desai, David Gonda, Sheila L. Ryan, Valentina Briceño, Sandi K. Lam, Thomas G. Luerssen, Sohail H. Syed, and Andrew Jea

OBJECT

Several studies have indicated that the 30-day morbidity and mortality risks are higher among pediatric and adult patients who are admitted on the weekends. This “weekend effect” has been observed among patients admitted with and fora variety of diagnoses and procedures, including myocardial infarction, pulmonary embolism, ruptured abdominal aortic aneurysm, stroke, peptic ulcer disease, and pediatric surgery. In this study, morbidity and mortality outcomes for emergency pediatric neurosurgical procedures carried out on the weekend or after hours are compared with emergency surgical procedures performed during regular weekday business hours.

METHODS

A retrospective analysis of operative data was conducted. Between December 1, 2011, and August 20, 2014, a total of 710 urgent or emergency neurosurgical procedures were performed at Texas Children’s Hospital in children younger than than 18 years of age. These procedures were then stratified into 3 groups: weekday regular hours, weekday after hours, and weekend hours. By cross-referencing these events with a prospectively collected morbidity and mortality database, the impact of the day and time on complication incidence was examined. Outcome metrics were compared using logistic regression models.

RESULTS

The weekday regular hours and after-hours (weekday after hours and weekends) surgery groups consisted of 341 and 239 patients and 434 and 276 procedures, respectively. There were no significant differences in the types of cases performed (p = 0.629) or baseline preoperative health status as determined by American Society of Anesthesiologists classifications (p = 0.220) between the 2 cohorts. After multivariate adjustment and regression, children undergoing emergency neurosurgical procedures during weekday after hours or weekends were more likely to experience complications (p = 0.0227).

CONCLUSIONS

Weekday after-hours and weekend emergency pediatric neurosurgical procedures are associated with significantly increased 30-day morbidity and mortality risk compared with procedures performed during weekday regular hours.

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Virendra R. Desai, Aditya Vedantam, Sandi K. Lam, Lucia Mirea, Stephen T. Foldes, Daniel J. Curry, P. David Adelson, Angus A. Wilfong, and Varina L. Boerwinkle

OBJECTIVE

Determining language laterality in patients with intractable epilepsy is important in operative planning. Wada testing is the gold standard, but it has a risk of stroke. Both Wada and task-based functional MRI (tb-fMRI) require patient cooperation. Recently, resting-state fMRI (rs-fMRI) has been explored for language lateralization. In the present study, the correlation between rs-fMRI and tb-fMRI in language lateralization is estimated in a pediatric population with intractable epilepsy.

METHODS

rs-fMRI and tb-fMRI language lateralization testing performed as part of epilepsy surgery evaluation was retrospectively reviewed.

RESULTS

Twenty-nine patients underwent rs-fMRI and tb-fMRI; a total of 38 rs-fMRI studies and 30 tb-fMRI studies were obtained. tb-fMRI suggested left dominance in 25 of 30 cases (83%), right in 3 (10%), and in 2 (7%) the studies were nondiagnostic. In rs-fMRI, 26 of 38 studies (68%) suggested left dominance, 3 (8%) right dominance, 6 (16%) bilateral, and 3 (8%) were nondiagnostic. When tb-fMRI lateralized to the left hemisphere (25 cases), rs-fMRI was lateralized to the left in 23 patients (92%) and it was bilateral/equal in 2 (8%). When tb-fMRI lateralized to the right (3 cases), rs-fMRI lateralized to the right in all cases (100%). The overall concordance rate was 0.93 (95% CI 0.76–0.99) when considering cases with tb-fMRI and rs-fMRI performed within 6 months of each other, and tb-fMRI results were not nondiagnostic.

CONCLUSIONS

rs-fMRI significantly correlated with tb-fMRI in lateralizing language and suggests the potential role for identifying hemispheric dominance via rs-fMRI. Further investigation and validation studies are warranted.

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Sandip S. Panesar, John J. Volpi, Alan Lumsden, Virendra Desai, Neal S. Kleiman, Trevis L. Sample, Eric Elkins, and Gavin W. Britz

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Audrey Grossen, Theresa Gavula, Deepti Chrusciel, Alexander Evans, Rene McNall-Knapp, Ashley Taylor, Benay Fossey, Margaret Brakefield, Carrick Carter, Nadine Schwartz, Naina Gross, Andrew Jea, and Virendra Desai

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.