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James T. Rutka

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Joshua K. Schaffzin, Katherine Simon, Beverly L. Connelly, and Francesco T. Mangano

OBJECTIVE

Surgical site infections (SSIs) are costly to patients and the health care system. Pediatric neurosurgery SSI risk factors are not well defined. Intraoperative protocols have reduced, but have not eliminated, SSIs. The effect of preoperative intervention is unknown. Using quality improvement methods, a preoperative SSI prevention protocol for pediatric neurosurgical patients was implemented to assess its effect on SSI rate.

METHODS

Patients who underwent a scheduled neurosurgical procedure between January 2014 and December 2015 were included. Published evidence and provider consensus were used to guide preoperative protocol development. The Model for Improvement was used to test interventions. Intraoperative and postoperative management was not standardized or modified systematically. Staff, family, and overall adherence was measured as all-or-nothing. In addition, SSI rates among eligible procedures were measured before and after protocol implementation.

RESULTS

Within 4 months, overall protocol adherence increased from 51.3% to a sustained 85.7%. SSI rates decreased from 2.9 per 100 procedures preintervention to 0.62 infections postintervention (p = 0.003). An approximate 79% reduction in SSI risk was identified (risk ratio 0.21, 95% CI 0.08–0.56; p = 0.001).

CONCLUSIONS

Clinical staff and families successfully collaborated on a standardized preoperative protocol for pediatric neurosurgical patients. Standardization of the preoperative phase of care alone reduced SSI rates. Attention to the preoperative in addition to the intraoperative and postoperative phases of care may lead to further reduction in SSI rates.

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Marci Madera, Alvin Crawford, and Francesco T. Mangano

✓ Larsen syndrome is a rare genetic disorder of the connective tissue that is characterized by multiple joint dislocations, distinctive deformities of the hands and feet, characteristic facial features, kyphoscoliosis, and segmentation anomalies of the vertebrae. Diverse treatment options, including conservative observation and surgical correction, have been reported for patients who present with cervical spine pathophysiology. Differences in surgical approaches, timing of the correction, and pre- or postoperative bracing have been reported. According to the authors, the present case is the first report of a pediatric patient with Larsen syndrome in whom an asymptomatic cervical instability was treated before neurological deterioration with synchronous anterior decompression and fixation, posterior fusion and fixation, and halo placement. A review of the literature on similar patients reveals the variety of practices associated with a diagnosis of Larsen syndrome.

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Dean A. Hertzler II, John J. DePowell, Charles B. Stevenson, and Francesco T. Mangano

Tethered cord syndrome (TCS) is a clinical condition of various origins that arises from tension on the spinal cord. Radiographic findings may include the conus medullaris in a lower than normal position, fatty infiltration of the filum terminale, lipomyelomeningocele, myelomeningocele, myelocystocele, meningocele, split cord malformations, dermal sinus, anorectal malformations, and intraspinal tumors. The clinical constellation of signs and symptoms associated with TCS may include dermatologic, urological, gastrointestinal, neurological, and orthopedic findings. The current review focuses on TCS by age group of the more common causes of the condition, including myelomeningocele, lipomyelomeningocele, as well as the adult presentation of occult TCS. Pertinent review of the neuroembryology and normal anatomical position of the conus medullaris is included.

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Abdullah S. Bdaiwi, Hansel M. Greiner, James Leach, Francesco T. Mangano, and Mark W. DiFrancesco

OBJECTIVE

Focal cortical dysplasia (FCD) is often associated with drug-resistant epilepsy, leading to a recommendation to surgically remove the seizure focus. Predicting outcome for resection of FCD is challenging, requiring a new approach. Lesion-symptom mapping is a powerful and broadly applicable method for linking neurological symptoms or outcomes to damage to particular brain regions. In this work, the authors applied lesion network mapping, an expansion of the traditional approach, to search for the association of lesion network connectivity with surgical outcomes. They hypothesized that connectivity of lesion volumes, preoperatively identified by MRI, would associate with seizure outcomes after surgery in a pediatric cohort with FCD.

METHODS

This retrospective study included 21 patients spanning the ages of 3 months to 17.7 years with FCD lesions who underwent surgery for drug-resistant epilepsy. The mean brain-wide functional connectivity map of each lesion volume was assessed across a database of resting-state functional MRI data from healthy children (spanning approximately 2.9 to 18.9 years old) compiled at the authors’ institution. Lesion connectivity maps were averaged across age and sex groupings from the database and matched to each patient. The authors sought to associate voxel-wise differences in these maps with subject-specific surgical outcome (seizure free vs persistent seizures).

RESULTS

Lesion volumes with persistent seizures after surgery tended to have stronger connectivity to attention and motor networks and weaker connectivity to the default mode network compared with lesion volumes with seizure-free surgical outcome.

CONCLUSIONS

Network connectivity–based lesion-outcome mapping may offer new insight for determining the impact of lesion volumes discerned according to both size and specific location. The results of this pilot study could be validated with a larger set of data, with the ultimate goal of allowing examination of lesions in patients with FCD and predicting their surgical outcomes.

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Abdullah S. Bdaiwi, Hansel M. Greiner, James Leach, Francesco T. Mangano, and Mark W. DiFrancesco

OBJECTIVE

Focal cortical dysplasia (FCD) is often associated with drug-resistant epilepsy, leading to a recommendation to surgically remove the seizure focus. Predicting outcome for resection of FCD is challenging, requiring a new approach. Lesion-symptom mapping is a powerful and broadly applicable method for linking neurological symptoms or outcomes to damage to particular brain regions. In this work, the authors applied lesion network mapping, an expansion of the traditional approach, to search for the association of lesion network connectivity with surgical outcomes. They hypothesized that connectivity of lesion volumes, preoperatively identified by MRI, would associate with seizure outcomes after surgery in a pediatric cohort with FCD.

METHODS

This retrospective study included 21 patients spanning the ages of 3 months to 17.7 years with FCD lesions who underwent surgery for drug-resistant epilepsy. The mean brain-wide functional connectivity map of each lesion volume was assessed across a database of resting-state functional MRI data from healthy children (spanning approximately 2.9 to 18.9 years old) compiled at the authors’ institution. Lesion connectivity maps were averaged across age and sex groupings from the database and matched to each patient. The authors sought to associate voxel-wise differences in these maps with subject-specific surgical outcome (seizure free vs persistent seizures).

RESULTS

Lesion volumes with persistent seizures after surgery tended to have stronger connectivity to attention and motor networks and weaker connectivity to the default mode network compared with lesion volumes with seizure-free surgical outcome.

CONCLUSIONS

Network connectivity–based lesion-outcome mapping may offer new insight for determining the impact of lesion volumes discerned according to both size and specific location. The results of this pilot study could be validated with a larger set of data, with the ultimate goal of allowing examination of lesions in patients with FCD and predicting their surgical outcomes.

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James L. Leach, Reem Awwad, Hansel M. Greiner, Jennifer J. Vannest, Lili Miles, and Francesco T. Mangano

OBJECTIVE

Diagnostic criteria for hippocampal malrotation (HIMAL) on brain MRI typically include a rounded hippocampus, vertical collateral sulcus, and architectural blurring. Relationship to epileptogenesis remains speculative, and usefulness for surgical guidance is unknown. The study was performed to determine the prevalence of hippocampal rotational anomalies in a cohort of pediatric patients with intractable epilepsy undergoing evaluation for surgery and to determine the significance of this finding in the context of surgical planning.

METHODS

Forty-eight surgically treated children with intractable epilepsy were compared with matched healthy subjects; reviewers were blinded to surgical side. Each temporal lobe was evaluated for rounded hippocampus, blurring, vertical collateral sulcus, wide choroidal fissure, enlarged temporal horn, low fornix, hippocampal signal, and findings of hippocampal sclerosis. A mesial temporal lobe (MTL) score was calculated by summing the number of features, and the collateral sulcus angle (CSA) was measured in each temporal lobe. Surgical side, pathological diagnosis, and imaging findings elsewhere in the brain were tabulated. Presence of HIMAL, associated imaging features, and MTL score were compared between sides, between epilepsy and control groups, in relationship to side of surgery, and in relationship to postoperative outcome.

RESULTS

Only 3 epilepsy patients (6.2%) and no controls exhibited all 3 features of HIMAL (p = 0.12). Eight of 48 (16.7%) epilepsy versus 2 of 48 (4.6%) control subjects had both a rounded hippocampus and vertical collateral sulcus (suggesting HIMAL) (p = 0.045). In control and epilepsy subjects, most findings were more prevalent on the left, and the left CSA was more vertical (p < 0.0001). Epilepsy subjects had higher MTL scores (z = −2.95, p = 0.002) and more acute CSAs (p = 0.04) than controls. Only lateralizing raw MTL score had a significant association with surgical side (p = 0.03, OR 7.33); however, this was not significant when hippocampal sclerosis cases were excluded. HIMAL findings were more prevalent and MTL scores were higher in patients with resections involving the temporal lobes. On group analysis, HIMAL findings did not predict eventual surgical side and did not predict outcome, although the numbers are small. In 4 patients the abnormally rotated hippocampus was resected and showed hippocampal sclerosis and/or dysplastic changes on histopathology. All of these patients had a good outcome after surgery.

CONCLUSIONS

While increased in prevalence in children with intractable epilepsy, imaging findings of HIMAL did not have preoperative lateralizing utility in this group. Findings of HIMAL (including round hippocampus, architectural blurring, and vertical collateral sulcus) did not predict outcome after surgery, although the small number of patients with these findings limits evaluation. In the small number of patients in which the malrotated hippocampus was removed, outcome was good. Further research is needed to continue to define this association in children with intractable epilepsy, focusing on a temporal lobe cohort.

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Francesco T. Mangano and Matthew D. Smyth

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A. Scott Emmert, Shawn M. Vuong, Crystal Shula, Diana Lindquist, Weihong Yuan, Yueh-Chiang Hu, Francesco T. Mangano, and June Goto

OBJECTIVE

Emergence of CRISPR/Cas9 genome editing provides a robust method for gene targeting in a variety of cell types, including fertilized rat embryos. The authors used this method to generate a transgenic rat L1cam knockout model of X-linked hydrocephalus (XLH) with human genetic etiology. The object of this study was to use diffusion tensor imaging (DTI) in studying perivascular white matter tract injury in the rat model and to characterize its pathological definition in histology.

METHODS

Two guide RNAs designed to disrupt exon 4 of the L1cam gene on the X chromosome were injected into Sprague-Dawley rat embryos. Following embryo transfer into pseudopregnant females, rats were born and their DNA was sequenced for evidence of L1cam mutation. The mutant and control wild-type rats were monitored for growth and hydrocephalus phenotypes. Their macro- and microbrain structures were studied with T2-weighted MRI, DTI, immunohistochemistry, and transmission electron microscopy (TEM).

RESULTS

The authors successfully obtained 2 independent L1cam knockout alleles and 1 missense mutant allele. Hemizygous male mutants from all 3 alleles developed hydrocephalus and delayed development. Significant reductions in fractional anisotropy and axial diffusivity were observed in the corpus callosum, external capsule, and internal capsule at 3 months of age. The mutant rats did not show reactive gliosis by then but exhibited hypomyelination and increased extracellular fluid in the corpus callosum.

CONCLUSIONS

The CRISPR/Cas9-mediated genome editing system can be harnessed to efficiently disrupt the L1cam gene in rats for creation of a larger XLH animal model than previously available. This study provides evidence that the early pathology of the periventricular white matter tracts in hydrocephalus can be detected in DTI. Furthermore, TEM-based morphometric analysis of the corpus callosum elucidates the underlying cytopathological changes accompanying hydrocephalus-derived variations in DTI. The CRISPR/Cas9 system offers opportunities to explore novel surgical and imaging techniques on larger mammalian models.

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Ellen L. Air, Yashar M. Ghomri, Rachana Tyagi, Andrew W. Grande, Kerry Crone, and Francesco T. Mangano

Object

Vagal nerve stimulators (VNSs) have been used successfully to treat medically refractory epilepsy. Although their efficacy is well established, appropriate management of infections is less clearly defined. In the authors' experience, patients who have gained a benefit from VNS implantation have been reluctant to have the device removed. The authors therefore sought conservative management options to salvage infected VNS systems.

Methods

The authors performed a retrospective review of 191 (93 female and 98 male) consecutive patients in whom VNS systems were placed between 2000 and 2007.

Results

They identified 10 infections (5.2%). In 9 of 10 patients the cultured organism was Staphylococcus aureus. Three (30%) of 10 patients underwent early removal (within 1 month) of the VNS as the initial treatment. The remaining 7 patients were initially treated with antibiotics. Two (28.6%) of these patients were successfully treated using antibiotics without VNS removal. Patients in whom conservative treatment failed were given cephalexin as first-line antibiotic treatment. All patients recovered completely regardless of treatment regimen.

Conclusions

This study confirms the low rate of infection associated with VNS placement and suggests that, in the case of infection, treatment without removal is a viable option. However, the authors' data suggest that oral antibiotics are not the best first-line therapy.