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David S. Hersh, Nir Shimony, Mari L. Groves, Gerald F. Tuite, George I. Jallo, Ann Liu, Tomas Garzon-Muvdi, Thierry A. G. M. Huisman, Ryan J. Felling, Joseph A. Kufera, and Edward S. Ahn

OBJECTIVE

Pediatric cerebral venous sinus thrombosis has been previously described in the setting of blunt head trauma; however, the population demographics, risk factors for thrombosis, and the risks and benefits of detection and treatment in this patient population are poorly defined. Furthermore, few reports differentiate between different forms of sinus pathology. A series of pediatric patients with skull fractures who underwent venous imaging and were diagnosed with intrinsic cerebral venous sinus thrombosis or extrinsic sinus compression is presented.

METHODS

The medical records of patients at 2 pediatric trauma centers were retrospectively reviewed. Patients who were evaluated for blunt head trauma from January 2003 to December 2013, diagnosed with a skull fracture, and underwent venous imaging were included.

RESULTS

Of 2224 pediatric patients with skull fractures following blunt trauma, 41 patients (2%) underwent venous imaging. Of these, 8 patients (20%) had intrinsic sinus thrombosis and 14 patients (34%) displayed extrinsic compression of a venous sinus. Three patients with intrinsic sinus thrombosis developed venous infarcts, and 2 of these patients were treated with anticoagulation. One patient with extrinsic sinus compression by a depressed skull fracture underwent surgical elevation of the fracture. All patients with sinus pathology were discharged to home or inpatient rehabilitation. Among patients who underwent follow-up imaging, the sinus pathology had resolved by 6 months postinjury in 80% of patients with intrinsic thrombosis as well as 80% of patients with extrinsic compression. All patients with intrinsic thrombosis or extrinsic compression had a Glasgow Outcome Scale score of 4 or 5 at their last follow-up.

CONCLUSIONS

In this series of pediatric trauma patients who underwent venous imaging for suspected thrombosis, the yield of detecting intrinsic thrombosis and/or extrinsic compression of a venous sinus was high. However, few patients developed venous hypertension or infarction and were subsequently treated with anticoagulation or surgical decompression of the sinus. Most had spontaneous resolution and good neurological outcomes without treatment. Therefore, in the setting of pediatric skull fractures after blunt injury, venous imaging is recommended when venous hypertension or infarction is suspected and anticoagulation is being considered. However, there is little indication for pervasive venous imaging after pediatric skull fractures, especially in light of the potential risks of CT venography or MR venography in the pediatric population and the unclear benefits of anticoagulation.

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Andrew S. Luksik, Tomas Garzon-Muvdi, Wuyang Yang, Judy Huang, and George I. Jallo

OBJECTIVE

Intramedullary spinal cord tumors comprise 1%–10% of all childhood central nervous system neoplasms, with astrocytomas representing the most common subtype. Due to their rarity and poor prognosis, large population-based studies are needed to assess the epidemiology and survival risk factors associated with these tumors in the hope of improving outcome. The authors undertook this retrospective study to explore factors that may influence survival in pediatric patients with spinal cord astrocytomas.

METHODS

Utilizing the Surveillance, Epidemiology, and End Results (SEER) database, a prospective cancer registry, the authors retrospectively assessed survival in histologically confirmed, primary spinal cord astrocytomas in patients 21 years of age and younger. Survival was described with Kaplan-Meyer curves, and a multivariate regression analysis was used to assess the association of several variables with survival while controlling for confounding variables.

RESULTS

This analysis of 348 cases showed that age (hazard ratio [HR] 1.05, 95% CI 1.01–1.09, p = 0.017), nonwhite race (HR 1.74, 95% CI 1.11–2.74, p = 0.014), high-grade tumor status (HR 14.67, 95% CI 6.69–32.14, p < 0.001), distant or invasive extension of the tumor (HR 2.37, 95% CI 1.02–5.49, p = 0.046), and radiation therapy (HR 3.74, 95% CI 2.18–6.41, p < 0.001) were associated with decreased survival. Partial resection (HR 0.37, 95% CI 0.16–0.83, p = 0.017) and gross-total resection (HR 0.39, 95% CI 0.16–0.95, p = 0.039) were associated with improved survival.

CONCLUSIONS

Younger age appears to be protective, while high-grade tumors have a much worse prognosis. Early diagnosis and access to surgery appears necessary for improving outcomes, while radiation therapy has an unclear role. There is still much to learn about this disease in the hope of curing children with the misfortune of having one of these rare tumors.

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Miguel Gelabert-González, Eduardo Arán-Echabe, and José María Santín-Amo

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Alan F. Utria, Joseph Lopez, Regina S. Cho, Gerhard S. Mundinger, George I. Jallo, Edward S. Ahn, Craig Vander Kolk, and Amir H. Dorafshar

OBJECTIVE

Due to the changing properties of the infant skull, there is still no clear consensus on the ideal time to surgically intervene in cases of nonsyndromic craniosynostosis (NSC). This study aims to shed light on how patient age at the time of surgery may affect surgical outcomes and the subsequent need for reoperation.

METHODS

A retrospective cohort review was conducted for patients with NSC who underwent primary cranial vault remodeling between 1990 and 2013. Patients' demographic and clinical characteristics and surgical interventions were recorded. Postoperative outcomes were assessed by assigning each procedure to a Whitaker category. Multivariate logistic regression analysis was performed to determine the relationship between age at surgery and need for minor (Whitaker I or II) versus major (Whitaker III or IV) reoperation. Odds ratios (ORs) for Whitaker category by age at surgery were assigned.

RESULTS

A total of 413 unique patients underwent cranial vault remodeling procedures for NSC during the study period. Multivariate logistic regression demonstrated increased odds of requiring major surgical revisions (Whitaker III or IV) in patients younger than 6 months of age (OR 2.49, 95% CI 1.05–5.93), and increased odds of requiring minimal surgical revisions (Whitaker I or II) in patients older than 6 months of age (OR 2.72, 95% CI 1.16–6.41).

CONCLUSIONS

Timing, as a proxy for the changing properties of the infant skull, is an important factor to consider when planning vault reconstruction in NSC. The data presented in this study demonstrate that patients operated on before 6 months of age had increased odds of requiring major surgical revisions.

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Kevin M. Stanko, Young M. Lee, Jennifer Rios, Adela Wu, Giovanna W. Sobrinho, Jon D. Weingart, Eric M. Jackson, Edward S. Ahn, Kaisorn L. Chaichana, and George I. Jallo

OBJECT

Chiari Type I malformation involves caudal displacement of the cerebellar tonsils below the foramen magnum, which obstructs normal cerebrospinal fluid flow and increases intracranial pressure. Certain aspects of its surgical treatment remain controversial. A retrospective study was conducted to assess the efficacy of tonsillar cautery on syrinx resolution among pediatric Chiari patients undergoing cervicomedullary decompression.

METHODS

A retrospective cohort study was performed for patients 0–18 years of age who underwent surgical correction for Chiari Type I malformation with syrinx between 1995 and 2013. Basic demographic information was collected as well as data for preoperative symptoms, prior surgical history, perioperative characteristics, and postsurgical outcomes. Descriptive statistics were performed in addition to bivariate analyses. Candidate predictor variables were identified based on an association with tonsillar cautery with p < 0.10. Forward stepwise likelihood ratio was used to select candidate predictors in a binary logistic regression model (Pin = 0.05, Pout = 0.10) most strongly associated with the outcome.

RESULTS

A total of 171 patients with Chiari Type I malformation with syrinx were identified, and 43 underwent tonsillar cautery. Patients who underwent tonsillar cautery had 6.11 times greater odds of improvement in their syrinx (95% CI 2.57–14.49, p < 0.001). There was no effect of tonsillar cautery on increased perioperative complications as well as the need for repeat decompressions.

CONCLUSIONS

Tonsillar cautery is safe and effective in the treatment of Chiari Type I malformation with syrinx and may decrease time to syrinx resolution after cervicomedullary decompression. Tonsillar cautery does not increase postoperative complications in pediatric Chiari Type I malformation patients.

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Gunes Orman, Matthias W. Wagner, Daniel Seeburg, Carlos A. Zamora, Alexander Oshmyansky, Aylin Tekes, Andrea Poretti, George I. Jallo, Thierry A. G. M. Huisman, and Thangamadhan Bosemani

OBJECT

The authors compared the efficacy of combining 2D+3D CT reconstructions with standard 2D CT images in the diagnosis of linear skull fractures in children with head trauma.

METHODS

This was a retrospective evaluation of consecutive head CT studies of children presenting with head trauma. Two experienced pediatric neuroradiologists in consensus created the standard of reference. Three readers independently evaluated the 2D CT images alone and then in combination with the 3D reconstructions for the diagnosis of linear skull fractures. Sensitivity and specificity in the diagnosis of linear skull fractures utilizing 2D and 2D+3D CT in combination were measured for children less than 2 years of age and for all children for analysis by the 3 readers.

RESULTS

Included in the study were 250 consecutive CT studies of 250 patients (167 boys and 83 girls). The mean age of the children was 7.82 years (range 4 days to 17.4 years). 2D+3D CT combined had a higher sensitivity and specificity (83.9% and 97.1%, respectively) compared with 2D alone (78.2% and 92.8%, respectively) with statistical significance for specificity (p < 0.05) in children less than 2 years of age. 2D+3D CT combined had a higher sensitivity and specificity (81.3% and 90.5%, respectively) compared with 2D alone (74.5% and 89.1%, respectively) with statistical significance for sensitivity (p < 0.05) in all children.

CONCLUSIONS

In this study, 2D+3D CT in combination showed increased sensitivity in the diagnosis of linear skull fractures in all children and increased specificity in children less than 2 years of age. In children less than 2 years of age, added confidence in the interpretation of fractures by distinguishing them from sutures may have a significant implication in the setting of nonaccidental trauma. Furthermore, 3D CT is available at no added cost, scan time, or radiation exposure, providing trainees and clinicians with limited experience an additional valuable tool for routine imaging of pediatric head trauma.

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Xin Zhou, C. Rory Goodwin, Pablo F. Recinos, Jesse X. Yang, and George I. Jallo

Object

Polyethylene glycol (PEG) sealant in conjunction with standard closure techniques is effective in preventing CSF leaks after cranial procedures in adult patients, but the safety of PEG sealant in the pediatric population has not been shown.

Methods

The authors performed a retrospective analysis of pediatric neurosurgery patients (0–18 years of age) treated from 2005 to 2010 at The Johns Hopkins Hospital. There were 163 patients who underwent cranial surgery with the use of PEG sealant as an adjunct to standard closure techniques. There were 92 males and 71 females with an average age of 10.2 years. The incidences of revision surgery, CSF leak, meningitis, and neurological deficit were recorded.

Results

In the cohort's 90-day postoperative clinical course, the authors found that 4 patients (2.5%) required revision surgery, 2 patients (1.2%) developed a CSF leak, 4 patients (2.5%) developed a superficial skin infection, and 1 patient developed meningitis (0.6%) with no deaths or neurological deficits observed.

Conclusions

PEG sealant appears to be a safe adjunct to standard dural closure in pediatric cranial surgery patients to augment dural closure.

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Joanna Y. Wang, Anubhav G. Amin, George I. Jallo, and Edward S. Ahn

Object

The most common neurosurgical condition observed in preterm infants is intraventricular hemorrhage (IVH), which often results in posthemorrhagic hydrocephalus (PHH). These conditions portend an unfavorable prognosis; therefore, the potential for poor neurodevelopmental outcomes necessitates a better understanding of the comparative effectiveness of 2 temporary devices commonly used before the permanent insertion of a ventriculoperitoneal (VP) shunt: the ventricular reservoir and the ventriculosubgaleal shunt (VSGS).

Methods

The authors analyzed retrospectively collected information for 90 patients with IVH and PHH who were treated with insertion of a ventricular reservoir (n = 44) or VSGS (n = 46) at their institution over a 14-year period.

Results

The mean gestational age and weight at device insertion were lower for VSGS patients (30.1 ± 1.9 weeks, 1.12 ± 0.31 kg) than for reservoir patients (31.8 ± 2.9 weeks, 1.33 ± 0.37 kg; p = 0.002 and p = 0.004, respectively). Ventricular reservoir insertion was predictive of more CSF taps prior to VP shunt placement compared with VSGS placement (10 ± 8.7 taps vs 1.6 ± 1.7 taps, p < 0.001). VSGS patients experienced a longer time interval prior to VP shunt placement than reservoir patients (80.8 ± 67.5 days vs 48.8 ± 26.4 days, p = 0.012), which corresponded to VSGS patients gaining more weight by the time of shunt placement than reservoir patients (3.31 ± 2.0 kg vs 2.42 ± 0.63 kg, p = 0.016). Reservoir patients demonstrated a trend toward more positive CSF cultures compared with VSGS patients (n = 9 [20.5%] vs n = 5 [10.9%], p = 0.21). There were no significant differences in the rates of overt device infection requiring removal (reservoir, 6.8%; VSGS, 6.5%), VP shunt insertion (reservoir, 77.3%; VSGS, 76.1%), or early VP shunt infection (reservoir, 11.4%; VSGS, 13.0%) between the 2 cohorts.

Conclusions

Although the rates of VP shunt requirement and device infection were similar between patients treated with the reservoir versus the VSGS, VSGS patients were significantly older and had achieved greater weights at the time of VP shunt insertion. The authors' results suggest that the VSGS requires less labor-intensive management by ventricular tapping; the VSGS patients also attained higher weights and more optimal surgical candidacy at the time of VP shunt insertion. The potential differences in long-term developmental and neurological outcomes between VSGS and reservoir placement warrant further study.

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Gunes Orman, Thangamadhan Bosemani, George I. Jallo, Thierry A. G. M. Huisman, and Andrea Poretti

Hypertrophic olivary degeneration (HOD) is a dynamic process caused by disruptive lesions affecting components of the Guillain-Mollaret triangle (GMT). The authors applied diffusion tensor imaging (DTI) to investigate longitudinal changes of the GMT components in a child with HOD after neurosurgery for a midbrain tumor. Diffusion tensor imaging data were acquired on a 1.5-T MRI scanner using a balanced pair of diffusion gradients along 20 noncollinear directions 1 day and 3, 6, and 9 months after surgery. Measurements from regions of interest (ROIs) were sampled in the affected inferior olivary nucleus, ipsilateral red nucleus, and contralateral superior and inferior cerebellar peduncles and dentate nucleus. For each ROI, fractional anisotropy and the mean, axial, and radial diffusivities were calculated. In the affected inferior olivary nucleus, the authors found a decrease in fractional anisotropy and an increase in mean, axial, and radial diffusivities 3 months after surgery, while 3 months later fractional anisotropy increased and diffusivities decreased. For all other GMT components, changes in DTI scalars were less pronounced, and fractional anisotropy mildly decreased over time. A detailed analysis of longitudinal DTI scalars in the various GMT components may shed light on a better understanding of the dynamic complex histopathological processes occurring in pediatric HOD over time.

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C. Rory Goodwin, Pablo F. Recinos, Xin Zhou, Jesse X. Yang, and George I. Jallo

Object

Cerebrospinal fluid leakage following durotomy in spinal surgery can lead to significant patient morbidity and mortality, including meningitis and even death. Usage of a polyethylene glycol (PEG) sealant in combination with standard closure techniques has been shown to be effective in preventing CSF leaks in animal models and adult patients, but the results of its use have not been reported in the pediatric population.

Methods

A retrospective analysis was performed of pediatric neurosurgery patients (0–18 years of age) treated at The Johns Hopkins Hospital from 2003 to 2010. There were 93 spinal surgery patients identified in whom PEG was applied. The incidence of CSF leakage, meningitis, and neurological injury was recorded. There were 54 males and 39 females in this study with an average age of 8.7 years. Of the identified patients, 16.1%, 28%, and 55.9% underwent surgery in the cervical region, thoracic region, and lumbar region, respectively.

Results

At 90-day follow-up, 5 patients (5.4%) had a CSF leak, 4 patients (4.3%) required a reoperation, and 1 patient (1.1%) had meningitis within this time period. No deaths or associated neurological deficits were observed.

Conclusions

The use of a PEG sealant to augment dural closure in pediatric spine surgery appears to be a safe adjunct to standard dural closure in pediatric spine patients.