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Sarah T. Garber and Jay K. Riva-Cambrin

Aneurysmal bone cysts (ABCs) are benign, expansile, osteolytic lesions that represent 1%–2% of primary bone tumors. Cranial ABCs are even more rare and represent 3%–6% of these unique lesions. The authors describe the case of a 3-year-old girl who presented with an acute posterior fossa epidural hematoma after minor trauma. Imaging workup revealed a previously undiagnosed suboccipital ABC that appeared to have ruptured as a result of her trauma, leading to a life-threatening hemorrhage. To the authors' knowledge, a ruptured ABC has never before been presented in the pediatric literature. In this case report, the authors review the imaging findings, natural history, clinical course, and treatment of these rare lesions.

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Cydni N. Williams, Jay Riva-Cambrin and Susan L. Bratton

OBJECT

Cerebral salt wasting (CSW) and the syndrome of inappropriate antidiuretic hormone secretion (SIADH) cause postoperative hyponatremia in neurosurgery patients, can be difficult to distinguish clinically, and are associated with increased morbidity. The authors aimed to determine risk factors associated with CSW and SIADH among children undergoing surgery for intracranial tumors.

METHODS

This retrospective cohort study included children 0–19 years of age who underwent a first intracranial tumor surgery with postoperative hyponatremia (sodium ≤ 130 mEq/L). CSW was differentiated from SIADH by urine output and fluid balance, exclusive of other causes of hyponatremia. The CSW and SIADH groups were compared with basic bivariate analysis and recursive partitioning.

RESULTS

Of 39 hyponatremic patients, 17 (44%) had CSW and 10 (26%) had SIADH. Patients with CSW had significantly greater natriuresis compared with those with SIADH (median urine sodium 211 vs 28 mEq/L, p = 0.01). Age ≤ 7 years and female sex were significant risk factors for CSW (p = 0.03 and 0.04, respectively). Both patient groups had hyponatremia onset within the first postoperative week. Children with CSW had trends toward increased sodium variability and symptomatic hyponatremia compared with those with SIADH. Most received treatment, but inappropriate treatment was noted to worsen hyponatremia.

CONCLUSIONS

The authors found that CSW was more common following intracranial tumor surgery and was associated with younger age and female sex. Careful assessment of fluid balance and urine output can separate patients with CSW from those who have SIADH, and high urine sodium concentrations (> 100 mEq/L) support a CSW diagnosis. Patients with CSW and SIADH had similar clinical courses, but responded to different interventions, making appropriate diagnosis and treatment imperative to prevent morbidity.

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Jian Guan, Jay Riva-Cambrin and Douglas L. Brockmeyer

OBJECTIVE

Patients treated for Chiari I malformation (CM-I) with posterior fossa decompression (PFD) may occasionally and unpredictably develop postoperative hydrocephalus. The clinical risk factors predictive of this type of Chiari-related hydrocephalus (CRH) are unknown. The authors' objective was to evaluate their experience to identify risk factors that may predict which of these patients undergoing PFD will develop CRH after surgery.

METHODS

The authors performed a retrospective clinical chart review of all patients who underwent PFD surgery and duraplasty for CM-I at the Primary Children's Hospital in Utah from June 1, 2005, through May 31, 2015. Patients were dichotomized based on the need for long-term CSF diversion after PFD. Analysis included both univariate and multivariable logistic regression analyses.

RESULTS

The authors identified 297 decompressive surgeries over the period of the study, 22 of which required long-term postoperative CSF diversion. On multivariable analysis, age < 6 years old (OR 3.342, 95% CI 1.282–8.713), higher intraoperative blood loss (OR 1.003, 95% CI 1.001–1.006), and the presence of a fourth ventricular web (OR 3.752, 95% CI 1.306–10.783) were significantly associated with the need for long-term CSF diversion after decompressive surgery.

CONCLUSIONS

Younger patients, those with extensive intraoperative blood loss, and those found during surgery to have a fourth ventricular web were at higher risk for the development of CRH. Clinicians should be alert to evidence of CRH in this patient population after PFD surgery.

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Abhaya V. Kulkarni, Jay Riva-Cambrin and Samuel R. Browd

Object

Published case series of endoscopic third ventriculostomy (ETV) for childhood hydrocephalus have reported widely varying success rates. The authors recently developed and internally validated the ETV Success Score (ETVSS); this is a simplified means of predicting the 6-month success rate of ETV for a child with hydrocephalus, based on age, etiology of hydrocephalus, and presence of a previous shunt. The authors hypothesized that the ETVSS would be able to predict with reasonable accuracy the actual ETV success rate reported among published case series.

Methods

A literature search was performed to identify published pediatric ETV papers that contained enough information with which to calculate an aggregate, mean predicted ETVSS for the cohort. This was then compared with the actual ETV success rate in the cohort. Data were extracted independently in triplicate, including by 2 individuals who were not involved with the development of the ETVSS.

Results

Fifteen papers reporting on 322 patients were included. Interrater reliability was very high in determining the predicted ETVSS (intraclass correlation coefficient 0.99). The predicted ETVSS for each paper agreed strongly with the actual ETV success rate reported in each paper (reliability intraclass correlation coefficient 0.81). There was no significant difference in the magnitude of the predicted ETVSS and the actual ETV success (p = 0.98, paired t-test). In a linear regression model, the predicted ETVSS explained 62% of the variation in actual ETV success. When the entire cohort was combined and analyzed together, the overall mean predicted ETVSS was 57.9%, which was nearly identical to the actual ETV success rate of 59.2%.

Conclusions

The ETVSS closely predicts the actual ETV success rate reported in selected papers published over the last 20 years and explains much of the variation.

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Sarah T. Garber, Robert J. Bollo and Jay K. Riva-Cambrin

Pediatric spinal pilomyxoid astrocytoma (PMA) is an extremely rare tumor that merits recognition as a specific, unique entity. The authors present the case of an intramedullary PMA in the thoracic spinal cord of an 11-year-old boy who presented with back pain, scoliosis, and multiple lung nodules. The patient underwent T5–11 laminoplasty and near-total resection of the spinal tumor. The final pathological diagnosis was WHO Grade II PMA. The patient did well for 14 months until the tumor progressed both clinically and radiographically. A literature review focusing on the clinical characteristics, histology, and treatment of PMAs provides a better understanding of these rare lesions. Because of the small number of cases optimal treatment guidelines have not been established, but gross-total resection and adjuvant chemotherapy with alkylating agents appear to confer a better long-term prognosis. Pediatric patients with PMAs can remain recurrence free at least 5 years after surgery, although these tumors may disseminate or dedifferentiate into more malignant gliomas. Recognition of intramedullary PMA as a unique entity in children is vital to the development of specific surgical and adjuvant treatment regimens.

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Prospective multicenter studies in pediatric hydrocephalus

JNSPG 75th Anniversary Invited Review Article

John R. W. Kestle and Jay Riva-Cambrin

Prospective multicenter clinical research studies in pediatric hydrocephalus are relatively rare. They cover a broad spectrum of hydrocephalus topics, including management of intraventricular hemorrhage in premature infants, shunt techniques and equipment, shunt outcomes, endoscopic treatment of hydrocephalus, and prevention and treatment of infection. The research methodologies include randomized trials, cohort studies, and registry-based studies. This review describes prospective multicenter studies in pediatric hydrocephalus since 1990. Many studies have included all forms of hydrocephalus and used device or procedure failure as the primary outcome. Although such studies have yielded useful findings, they might miss important treatment effects in specific subgroups. As multicenter study networks grow, larger patient numbers will allow studies with more focused entry criteria based on known and evolving prognostic factors. In addition, increased use of patient-centered outcomes such as neurodevelopmental assessment and quality of life should be measured and emphasized in study results. Well-planned multicenter clinical studies can significantly affect the care of children with hydrocephalus and will continue to have an important role in improving care for these children and their families.

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Heather S. Spader, Robert J. Bollo, Christian A. Bowers and Jay Riva-Cambrin

OBJECTIVE

Intrathecal baclofen infusion systems to manage severe spasticity and dystonia are associated with higher infection rates in children than in adults. Factors unique to this population, such as poor nutrition and physical limitations for pump placement, have been hypothesized as the reasons for this disparity. The authors assessed potential risk factors for infection in a multivariate analysis.

METHODS

Patients who underwent implantation of a programmable pump and intrathecal catheter for baclofen infusion at a single center between January 1, 2000, and March 1, 2012, were identified in this retrospective cohort study. The primary end point was infection. Potential risk factors investigated included preoperative (i.e., demographics, body mass index [BMI], gastrostomy tube, tracheostomy, previous spinal fusion), intraoperative (i.e., surgeon, antibiotics, pump size, catheter location), and postoperative (i.e., wound dehiscence, CSF leak, and number of revisions) factors. Univariate analysis was performed, and a multivariate logistic regression model was created to identify independent risk factors for infection.

RESULTS

A total of 254 patients were evaluated. The overall infection rate was 9.8%. Univariate analysis identified young age, shorter height, lower weight, dehiscence, CSF leak, and number of revisions within 6 months of pump placement as significantly associated with infection. Multivariate analysis identified young age, dehiscence, and number of revisions as independent risk factors for infection.

CONCLUSIONS

Young age, wound dehiscence, and number of revisions were independent risk factors for infection in this pediatric cohort. A low BMI and the presence of either a gastrostomy or tracheostomy were not associated with infection and may not be contraindications for this procedure.

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Lucy He, Stephen Gannon, Chevis N. Shannon, Brandon G. Rocque, Jay Riva-Cambrin and Robert P. Naftel

OBJECTIVE

The success of endoscopic third ventriculostomy with choroid plexus cauterization may have associations with age, etiology of hydrocephalus, previous shunting, cisternal scarring, and possibly aqueduct patency. This study aimed to measure interrater reliability among surgeons in identifying cisternal scarring and aqueduct patency.

METHODS

Using published definitions of cistern scarring and aqueduct patency, 7 neuroendoscopists with training from Dr. Warf in Uganda and 7 neuroendoscopists who were not trained by Dr. Warf rated cistern status from 30 operative videos and aqueduct patency from 26 operative videos. Interrater agreement was calculated using Fleiss' kappa coefficient (κ). Fisher's 2-tailed exact test was used to identify differences in the rates of agreement between the Warf-trained and nontrained groups compared with Dr. Warf's reference answer.

RESULTS

Aqueduct status, among all raters, showed substantial agreement with κ = 0.663 (confidence interval [CI] 0.626–0.701); within the trained group and nontrained groups, there was substantial agreement with κ = 0.677 (CI 0.593–0.761) and κ = 0.631 (CI 0.547–0.715), respectively. The identification of cistern scarring was less reliable, with moderate agreement among all raters with κ = 0.536 (CI 0.501–0.571); within the trained group and nontrained groups, there was moderate agreement with κ = 0.555 (CI 0.477–0.633) and κ = 0.542 (CI 0.464–0.620), respectively. There was no statistically significant difference in the amount of agreement between groups compared with Dr. Warf's reference.

CONCLUSIONS

Regardless of training with Dr. Warf, all neuroendoscopists could identify scarred cisterns and aqueduct patency with similar reliability, emphasizing the strength of the published definitions. This makes the identification of this risk factor for failure generalizable for surgical decision making and research studies.

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Vijay M. Ravindra, Jay Riva-Cambrin, Walavan Sivakumar, Ryan R. Metzger and Robert J. Bollo

OBJECT

Computed tomography angiography (CTA) is frequently used to examine patients for blunt cerebrovascular injury (BCVI) after cranial trauma, but the pediatric population at risk for BCVI is poorly defined. Although CTA is effective for BCVI screening in adults, the increased lifetime risk for malignant tumors associated with this screening modality warrants efforts to reduce its use in children. The authors' objective was to evaluate the incidence of BCVI diagnosed by CTA in a pediatric patient cohort and to create a prediction model to identify children at high risk for BCVI.

METHODS

Demographic, clinical, and radiographic data were collected retrospectively for pediatric patients who underwent CTA during examination for traumatic cranial injury from 2003 through 2013. The primary outcome was injury to the carotid or vertebral artery diagnosed by CTA.

RESULTS

The authors identified 234 patients (mean age 8.3 years, range 0.04–17 years, 150 [64%] boys) who underwent CTA screening for BCVI. Of these, 24 (10.3%) had a focal neurological deficit, and 153 (65.4%) had intracranial hemorrhage on a head CTA. Thirty-seven BCVIs were observed in 36 patients (15.4%), and 16 patients (6.8%) died. Multivariate regression analysis identified fracture through the carotid canal, petrous temporal bone fracture, Glasgow Coma Scale (GCS) score of < 8, focal neurological deficit, and stroke on initial CT scan as independent risk factors for BCVI. A prediction model for identifying children at high risk for BCVI was created. A score of ≤ 2 yielded a 7.9% probability of BCVI and a score of ≥ 3 a risk of 39.3% for BCVI.

CONCLUSIONS

For cranial trauma in children, fracture of the petrous temporal bone or through the carotid canal, focal neurological deficit, stroke, and a GCS score of < 8 are independent risk factors for BCVI.

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Cydni N. Williams, Jay Riva-Cambrin, Angela P. Presson and Susan L. Bratton

OBJECT

Pediatric intracranial neoplasms are common and cause substantial neurological morbidity. Postoperative hyponatremia is also common and may exacerbate neurological injury. The authors performed an exploratory analysis to evaluate an exposure-response relationship between hyponatremia severity and cognitive function at discharge.

METHODS

A retrospective cohort of patients 0–19 years old who underwent a first intracranial neoplasm surgery at a pediatric tertiary care hospital was reviewed. Outcome was assessed by Pediatric Cerebral Performance Category (PCPC) score of 1–6 at hospital discharge. Poor outcome was defined as PCPC score 3–6, corresponding to moderate or worse disability.

RESULTS

Of 319 total children, 80 (25%) had poor outcomes. One hundred thirty-seven children (43%) had serum sodium concentrations ≤ 131–135 mEq/L and 39 (12%) had serum sodium concentrations ≤ 130 mEq/L. Lower nadir sodium concentration and longer duration of hyponatremia were significantly associated with worsening PCPC score (p < 0.001). Rapid sodium decreases and more hyponatremic episodes were also significantly associated with worsening PCPC score (p < 0.001). After adjustment for patient factors, tumor characteristics, and measures of sodium disruption, multivariable analysis revealed noncortical tumor locations and lower nadir sodium concentration (adjusted odds ratio 0.86, 95% confidence interval 0.78–0.95) were important independent risk factors for poor cognitive outcome.

CONCLUSIONS

Neurocognitive disability and hyponatremia are common in children undergoing surgery for intracranial neoplasms. This study found a significant association between severity of hyponatremia and worsened cognitive outcome, with an apparent exposure-response relationship. These data support the need for careful postoperative monitoring of serum sodium. Further research is needed to determine if prevention and treatment of hyponatremia can improve outcomes in these children.