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Andrew C. Vivas, Nir Shimony, Eric M. Jackson, Risheng Xu, George I. Jallo, Luis Rodriguez, Gerald F. Tuite and Carolyn M. Carey

OBJECTIVE

Hydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively.

METHODS

A retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review.

RESULTS

Over the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population.

CONCLUSIONS

De novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.

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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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David S. Hersh, Rajiv R. Iyer, Tomas Garzon-Muvdi, Ann Liu, George I. Jallo and Mari L. Groves

OBJECTIVE

Spinal deformity has become a well-recognized complication of intramedullary spinal cord tumor (IMSCT) resection. In particular, laminectomy can result in biomechanical instability caused by loss of the posterior tension band. Therefore, laminoplasty has been proposed as an alternative to laminectomy. Here, the authors describe the largest current series of pediatric patients who have undergone laminoplasty for IMSCT resection and investigate the need for surgical fusion after both laminectomy and laminoplasty.

METHODS

The medical records of pediatric patients who underwent resection of an IMSCT at a single institution between November 2003 and May 2014 were reviewed retrospectively. Demographic, clinical, radiological, surgical, histopathological, and follow-up data were collected.

RESULTS

Sixty-six consecutive patients underwent resection of an IMSCT during the study period. Forty-three (65%) patients were male. The patients had a median age of 12.9 years (interquartile range [IQR] 7.2–16.5 years) at the time of surgery. Patients typically presented with a tumor that involved the cervical and/or thoracic spine. Nineteen (29%) patients underwent laminectomy, and 47 (71%) patients underwent laminoplasty. Patients in each cohort had a similar rate of postoperative deformity. Overall, 10 (15%) patients required instrumented spinal fusion for spinal deformity. Four patients required revision of the primary fusion.

CONCLUSIONS

These findings show that among pediatric patients with an IMSCT, postoperative surgical fusion rates remain high, even after laminoplasty. Known risk factors, such as the age of the patient, location of the tumor, and the number of involved levels, might play a larger role than replacement of the laminae in determining the rate of surgical fusion after IMSCT resection.

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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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Courtney Pendleton, Jordina Rincon-Torroella, Ziya L. Gokaslan, George I. Jallo and Alfredo Quinones-Hinojosa

Although Harvey Cushing was mostly known for his contributions to brain tumor surgery, he was also a pioneer in the development of spinal cord surgery. This lesser known facet of Cushing’s career can provide a fresh and unique perspective into how the founders of neurosurgery surmounted early challenges in the field. The authors bring to light and examine for the first time Cushing’s unpublished writing “Technique of Laminectomy” along with his first 3 documented intramedullary spinal cord tumor (IMSCT) cases at the Johns Hopkins Hospital. The authors draw lessons from the challenges in pathological classification, preoperative diagnosis, tumor localization, and surgical technique ofthat time. Although Cushing’s attempts at exploration and resection of IMSCT as described here were of limited success, his ability to adapt his clinical and surgical technique to the challenges of the time, as well as develop skills to successfully manipulate the spinal cord during these exploratory procedures without the patients incurring neurological damage, postoperative infection, or complications, is a testament to his determination to advance the field and his meticulous operative technique. In spite of the limitations imposed on the pioneer neurosurgeons, Harvey Cushing and his contemporaries persevered through many of the challenges and built an essential part of neurosurgery’s common story.

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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.