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Konstantina Svokos, Lígia Batista-Silverman, Sarah J. Graber, Brent R. O’Neill, and Michael H. Handler

OBJECTIVE

Occult spinal dysraphism (OSD) is a common pediatric neurosurgical diagnosis rife with controversy surrounding both the screening of asymptomatic infants and the threshold to offer a prophylactic detethering operation. The authors sought to clarify international practice patterns with a survey of pediatric neurosurgeons.

METHODS

A survey asked pediatric neurosurgeons whether they would perform imaging in patients with a variety of cutaneous stigmata associated with OSD and whether they would offer prophylactic detethering surgery for asymptomatic patients with a variety of imaging findings on the OSD spectrum.

RESULTS

Completed surveys were received from 141 pediatric neurosurgeons. Broad consensus was demonstrated on the need for obtaining images in sample patients with more severe stigmata ranging from large lipoma with a skin appendage to focal dysplastic skin in the lumbar midline. Ninety percent of respondents would perform MRI for these patients. In contrast, for patients with a low-sacral dimple, flat hemangioma, and symmetric (Y-shaped) splaying of the intergluteal cleft, opinion on the need for imaging varied considerably (between 57% and 89% recommended imaging). Respondents differed on the type of imaging that they would perform, with 31% to 38% recommending ultrasound screening. The responses reflected less consensus on when to offer surgery to patients with simple spinal tethering (low-lying conus medullaris and fatty filum terminale). Both a lower level of the conus and increased thickness of the filum terminale affected decision-making.

CONCLUSIONS

The results of this survey showed significant consensus on the recommendation for screening imaging in patients with more dramatic cutaneous stigmata, although these stigmata are the rarest. A significant variance in opinions was reflected in the recommendation for imaging of the most common cutaneous stigmata. Consensus was also lacking on which lesions deserve prophylactic detethering surgery. Significant equipoise exists for future study of screening imaging and of surgical decision-making in patients with asymptomatic OSD and associated cutaneous stigmata.

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Elsa V. Arocho-Quinones, Sean M. Lew, Michael H. Handler, Zulma Tovar-Spinoza, Matthew Smyth, Robert Bollo, David Donahue, M. Scott Perry, Michael L. Levy, David Gonda, Francesco T. Mangano, Phillip B. Storm, Angela V. Price, Daniel E. Couture, Chima Oluigbo, Ann-Christine Duhaime, Gene H. Barnett, Carrie R. Muh, Michael D. Sather, Aria Fallah, Anthony C. Wang, Sanjiv Bhatia, Kadam Patel, Sergey Tarima, Sarah Graber, Sean Huckins, Daniel M. Hafez, Kavelin Rumalla, Laurie Bailey, Sabrina Shandley, Ashton Roach, Erin Alexander, Wendy Jenkins, Deki Tsering, George Price, Antonio Meola, Wendi Evanoff, Eric M. Thompson, Nicholas Brandmeir, and the Pediatric Stereotactic Laser Ablation Workgroup

OBJECTIVE

This study aimed to assess the safety and efficacy of MR-guided stereotactic laser ablation (SLA) therapy in the treatment of pediatric brain tumors.

METHODS

Data from 17 North American centers were retrospectively reviewed. Clinical, technical, and radiographic data for pediatric patients treated with SLA for a diagnosis of brain tumor from 2008 to 2016 were collected and analyzed.

RESULTS

A total of 86 patients (mean age 12.2 ± 4.5 years) with 76 low-grade (I or II) and 10 high-grade (III or IV) tumors were included. Tumor location included lobar (38.4%), deep (45.3%), and cerebellar (16.3%) compartments. The mean follow-up time was 24 months (median 18 months, range 3–72 months). At the last follow-up, the volume of SLA-treated tumors had decreased in 80.6% of patients with follow-up data. Patients with high-grade tumors were more likely to have an unchanged or larger tumor size after SLA treatment than those with low-grade tumors (OR 7.49, p = 0.0364). Subsequent surgery and adjuvant treatment were not required after SLA treatment in 90.4% and 86.7% of patients, respectively. Patients with high-grade tumors were more likely to receive subsequent surgery (OR 2.25, p = 0.4957) and adjuvant treatment (OR 3.77, p = 0.1711) after SLA therapy, without reaching significance. A total of 29 acute complications in 23 patients were reported and included malpositioned catheters (n = 3), intracranial hemorrhages (n = 2), transient neurological deficits (n = 11), permanent neurological deficits (n = 5), symptomatic perilesional edema (n = 2), hydrocephalus (n = 4), and death (n = 2). On long-term follow-up, 3 patients were reported to have worsened neuropsychological test results. Pre-SLA tumor volume, tumor location, number of laser trajectories, and number of lesions created did not result in a significantly increased risk of complications; however, the odds of complications increased by 14% (OR 1.14, p = 0.0159) with every 1-cm increase in the volume of the lesion created.

CONCLUSIONS

SLA is an effective, minimally invasive treatment option for pediatric brain tumors, although it is not without risks. Limiting the volume of the generated thermal lesion may help decrease the incidence of complications.

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Soliman Oushy, Jonathon J. Parker, Kristen Campbell, Claire Palmer, Corbett Wilkinson, Nicholas V. Stence, Michael H. Handler, and David M. Mirsky

OBJECTIVE

Placement of a cerebrospinal fluid diversion device (i.e., shunt) is a routine pediatric neurosurgical procedure, often performed in the first weeks of life for treatment of congenital hydrocephalus. In the postoperative period, shunt placement may be complicated by subdural, catheter tract, parenchymal, and intraventricular hemorrhages. The authors observed a subset of infants and neonates who developed multifocal intraparenchymal hemorrhages (MIPH) following shunt placement and sought to determine any predisposing perioperative variables.

METHODS

A retrospective review of the electronic medical record at a tertiary-care children’s hospital was performed for the period 1998–2015. Inclusion criteria consisted of shunt placement, age < 30 days, and available pre- and postoperative brain imaging. The following data were collected and analyzed for each case: ventricular size ratios, laboratory values, clinical presentation, shunt and valve type, and operative timing and approach.

RESULTS

A total of 121 neonates met the inclusion criteria for the study, and 11 patients (9.1%) had MIPH following shunt placement. The preoperative frontal and occipital horn ratio (FOR) was significantly higher in the patients with MIPH than in those without (0.65 vs 0.57, p < 0.001). The change in FOR (∆FOR) after shunt placement was significantly greater in the MIPH group (0.14 vs 0.08, p = 0.04). Among neonates who developed MIPH, aqueductal stenosis was the most common etiology (45%). The type of shunt valve was associated with incidence of MIPH (p < 0.001). Preoperative clinical parameters, including head circumference, bulging fontanelle, and coagulopathy, were not significantly associated with development of MIPH.

CONCLUSIONS

MIPH represents an underrecognized complication of neonatal shunted hydrocephalus. Markers of severity of ventriculomegaly (FOR) and ventricular response to CSF diversion (∆FOR) were significantly associated with occurrence of MIPH. Choice of shunt and etiology of hydrocephalus were also significantly associated with MIPH. After adjusting for corrected age, etiology of hydrocephalus, and shunt setting, the authors found that ∆FOR after shunting was still associated with MIPH. A prospective study of MIPH prevention strategies and assessment of possible implications for patient outcomes is needed.

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Brent R. O'Neill, Danielle Gallegos, Alex Herron, Claire Palmer, Nicholas V. Stence, Todd C. Hankinson, C. Corbett Wilkinson, and Michael H. Handler

OBJECTIVE

Cutaneous stigmata or congenital anomalies often prompt screening for occult spinal dysraphism (OSD) in asymptomatic infants. While a number of studies have examined the results of ultrasonography (US) screening, less is known about the findings when MRI is used as the primary imaging modality. The object of this study was to assess the results of MRI screening for OSD in infants.

METHODS

The authors undertook a retrospective review of all infants who had undergone MRI of the lumbar spine to screen for OSD over a 6-year period (September 2006–September 2012). All images had been obtained on modern MRI scanners using sequences optimized to detect OSD, which was defined as any fibrolipoma of the filum terminale (FFT), a conus medullaris ending at or below the L2–3 disc space, as well as more complex lesions such as lipomyelomeningocele (LMM).

RESULTS

Five hundred twenty-two patients with a mean age of 6.2 months at imaging were included in the study. Indications for imaging included isolated dimple in 235 patients (45%), asymmetrically deviated gluteal cleft in 43 (8%), symmetrically deviated (Y-shaped) gluteal cleft in 38 (7%), hemangioma in 28 (5%), other isolated cutaneous stigmata (subcutaneous lipoma, vestigial tail, hairy patch, and dysplastic skin) in 31 (6%), several of the above stigmata in 97 (18%), and congenital anomalies in 50 (10%).

Twenty-three percent (122 patients) of the study population had OSD. Lesions in 19% of these 122 patients were complex OSD consisting of LMM, dermal sinus tract extending to the thecal sac, and lipomeningocele. The majority of OSD lesions (99 patients [81%]) were filar abnormalities, a group including FFT and low-lying conus.

The rate of OSD ranged from 12% for patients with asymmetrically deviated gluteal crease to 55% for those with other isolated cutaneous stigmata.

Isolated midline dimple was the most common indication for imaging. Among this group, 20% (46 of 235) had OSD. There was no difference in the rate of OSD based on dimple location. Those with OSD had a mean dimple position of 15 mm (SD 11.8) above the coccyx. Those without OSD had a mean dimple position of 12.2 mm (SD 19) above the coccyx (p = 0.25).

CONCLUSIONS

The prevalence of OSD identified with modern high-resolution MRI screening is significantly higher than that reported with US screening, particularly in patients with dimples. The majority of OSD lesions identified are FFT and low conus. The clinical significance of such lesions remains unclear.

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Seerat Poonia, Sarah Graber, C. Corbett Wilkinson, Brent R. O'neill, Michael H. Handler, and Todd C. Hankinson

OBJECTIVE

Postoperative management following the release of simple spinal cord–tethering lesions is highly variable. As a quality improvement initiative, the authors aimed to determine whether an institutional protocol of discharging patients on postoperative day (POD) 1 was associated with a higher rate of postoperative CSF leaks than the prior protocol of discharge on POD 2.

METHODS

This was a single-center retrospective review of all children who underwent release of a spinal cord–tethering lesion that was not associated with a substantial fascial or dural defect (i.e., simple spinal cord detethering) during 2 epochs: prior to and following the institution of a protocol for discharge on POD 1. Outcomes included the need for and timing of nonroutine care of the surgical site, including return to the operating room, wound suturing, and nonsurgical evaluation and management.

RESULTS

Of 169 patients identified, none presented with CSF-related complications prior to discharge. In the preintervention group (n = 113), the postoperative CSF leak rate was 4.4% (5/113). The mean length of stay was 2.3 days. In the postintervention group, the postoperative CSF leak rate was 1.9% (1/53) in the patients with postdischarge follow-up. The mean length of stay in that group was 1.3 days.

CONCLUSIONS

At a single academic children's hospital, a protocol of discharging patients on POD 1 following uncomplicated release of a simple spinal cord–tethering lesion was not associated with an increased rate of postoperative CSF leaks, relative to the previous protocol. The rates identified are consistent with the existing literature. The authors' practice has changed to discharge on POD 1 in most cases.

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Todd C. Hankinson, Roy W. R. Dudley, Michelle R. Torok, Mohana Rao Patibandla, Kathleen Dorris, Seerat Poonia, C. Corbett Wilkinson, Jennifer L. Bruny, Michael H. Handler, and Arthur K. Liu

OBJECT

Thirty-day mortality is increasingly a reference metric regarding surgical outcomes. Recent data estimate a 30-day mortality rate of 1.4−2.7% after craniotomy for tumors in children. No detailed analysis of short-term mortality following a diagnostic neurosurgical procedure (e.g., resection or tissue biopsy) for tumor in the US pediatric population has been conducted.

METHODS

The Surveillance, Epidemiology and End Results (SEER) data sets identified patients ≤ 21 years who underwent a diagnostic neurosurgical procedure for primary intracranial tumor from 2004 to 2011. One- and two-month mortality was estimated. Standard statistical methods estimated associations between independent variables and mortality.

RESULTS

A total of 5533 patients met criteria for inclusion. Death occurred within the calendar month of surgery in 64 patients (1.16%) and by the conclusion of the calendar month following surgery in 95 patients (1.72%). Within the first calendar month, patients < 1 year of age (n = 318) had a risk of death of 5.66%, while those from 1 to 21 years (n = 5215) had a risk of 0.88% (p < 0.0001). By the end of the calendar month following surgery, patients < 1 year (n = 318) had a risk of death of 7.23%, while those from 1 to 21 years (n = 5215) had a risk of 1.38% (p < 0.0001). Children < 1 year at diagnosis were more likely to harbor a high-grade lesion than older children (OR 1.9, 95% CI 1.5–2.4).

CONCLUSIONS

In the SEER data sets, the risk of death within 30 days of a diagnostic neurosurgical procedure for a primary pediatric brain tumor is between 1.16% and 1.72%, consistent with contemporary data from European populations. The risk of mortality in infants is considerably higher, between 5.66% and 7.23%, and they harbor more aggressive lesions.

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Brent R. O’Neill, Michael H. Handler, Suhong Tong, and Kevin E. Chapman

OBJECT

Seizures may cause diagnostic confusion and be a source of metabolic stress after traumatic brain injury (TBI) in children. The incidence of electroencephalography (EEG)-confirmed seizures and of subclinical seizures in the pediatric population with TBI is not well known.

METHODS

A routine protocol for continuous EEG (cEEG) monitoring was initiated for all patients with moderate or severe TBI at a Level 1 pediatric trauma center. Over a 3.5-year period, all patients with TBI who underwent cEEG monitoring, both according to protocol and those with mild head injuries who underwent cEEG monitoring at the discretion of the treating team, were identified prospectively. Clinical data were collected and analyzed.

RESULTS

Over the study period, 594 children were admitted with TBI, and 144 of these children underwent cEEG monitoring. One hundred two (71%) of these 144 children had moderate or severe TBI. Abusive head trauma (AHT) was the most common mechanism of injury (65 patients, 45%) in children with cEEG monitoring. Seizures were identified on cEEG in 43 patients (30%). Forty (93%) of these 43 patients had subclinical seizures, including 17 (40%) with only subclinical seizures and 23 (53%) with both clinical and subclinical seizures. Fifty-three percent of patients with seizures experienced status epilepticus. Age less than 2.4 years and AHT mechanism were strongly correlated with presence of seizures (odds ratios 8.7 and 6.0, respectively). Those patients with only subclinical seizures had the same risk factors as the other groups. The presence of seizures did not correlate with discharge disposition but was correlated with longer hospital stay and intensive care unit stay.

CONCLUSIONS

Continuous EEG monitoring identifies a significant number of subclinical seizures acutely after TBI. Children younger than 2.4 years of age and victims of AHT are particularly vulnerable to subclinical seizures, and seizures in general. Continuous EEG monitoring allows for accurate diagnosis and timely treatment of posttraumatic seizures, and may mitigate secondary injury to the traumatized brain.