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Jeremy Steinberger, Dominic A. Nistal and Saadi Ghatan

Infantile hemangiomas (IHs) are the most common benign neoplasm of the neonatal and newborn period, affecting approximately 5% of infants. However, true IHs presenting in the neuraxis are quite rare with only 15 documented cases in the literature. Management of IH consists of utilizing steroids and immunomodulatory therapies to reduce the size of the tumor and surgery to remove the tumor to decrease symptoms and the risk of bleeding. Operative management of epidural and intradural extramedullary spinal hemangiomas has been described; however, management of intradural intramedullary IH has not been detailed in the literature. In this report, the authors describe the case of a 3-year-old girl who presented with multiple hemangiomas involving the liver, lung, and spine, with one component of the tumor involving the posterior intramedullary aspect of the spinal cord at the level of T3. After medical therapies had failed, the patient underwent endovascular embolization of the spinal hemangioma followed by resection of the tumor. While there is extensive literature on IH throughout many organ systems, only a handful of cases involving the neuraxis have been described. Operative management of refractory IH seems to allow for the reduction of tumor burden and the prevention of hemorrhagic injury.

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Kiril Mladenov, Lena Braunschweig, Jennifer Behrend, Heiko M. Lorenz, Urs von Deimling and Anna K. Hell

OBJECTIVE

During childhood, early-onset scoliosis (EOS) may show severe progressive deformity, which consequently leads to aggressive treatment strategies, such as serial casting, long-term bracing, or surgical interventions. The latter usually includes repeated surgeries for implant lengthening every 6 months in order to allow sufficient growth of the thorax and spine. In 2011, the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24) was introduced to measure health-related quality of life for this patient group and their families. Since then, cross-culturally adapted versions of the EOSQ-24 have been published in Spanish, Turkish, traditional Chinese, and Norwegian. The purpose of the study was to transculturally adapt the original English version of the EOSQ-24 into the German language and evaluate the reliability of the German version.

METHODS

After adaptation and forward/backward translation, the German version of the EOSQ-24 was given to the parents or caregivers of 67 EOS patients (33 male, 34 female) Data quality was evaluated by mean, standard deviation, percentage of data missing, and extent of ceiling and floor effects. Reliability was estimated by internal consistency using Cronbach α and item-total correlations.

RESULTS

In the study group (n = 67), 12 children were either observed (n = 7) or treated with a brace (n = 5). The other 55 patients were treated surgically with growth-friendly implants. The item response to the German EOSQ-24 was high with a minimum of missing data (1.7%). All items showed very good to excellent internal consistencies (0.879–0.903). Floor effects for the 24 items were between 0% and 31% and ceiling effects between 9% and 78%. The calculated Cronbach α for the 24-item scale was 0.9003, indicating excellent reliability.

CONCLUSIONS

The German adaptation of the EOSQ-24 shows excellent reliability and therefore is a valid tool to measure objective health-related quality of life in children with EOS.

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Ian F. Pollack, Sameer Agnihotri and Alberto Broniscer

Brain tumors are the most common solid tumors in children, and, unfortunately, many subtypes continue to have a suboptimal long-term outcome. During the last several years, however, remarkable advances in our understanding of the molecular underpinnings of these tumors have occurred as a result of high-resolution genomic, epigenetic, and transcriptomic profiling, which have provided insights for improved tumor categorization and molecularly directed therapies. While tumors such as medulloblastomas have been historically grouped into standard- and high-risk categories, it is now recognized that these tumors encompass four or more molecular subsets with distinct clinical and molecular characteristics. Likewise, high-grade glioma, which for decades was considered a single high-risk entity, is now known to comprise multiple subsets of tumors that differ in terms of patient age, tumor location, and prognosis. The situation is even more complex for ependymoma, for which at least nine subsets of tumors have been described. Conversely, the majority of pilocytic astrocytomas appear to result from genetic changes that alter a single, therapeutically targetable molecular pathway. Accordingly, the present era is one in which treatment is evolving from the historical standard of radiation and conventional chemotherapy to a more nuanced approach in which these modalities are applied in a risk-adapted framework and molecularly targeted therapies are implemented to augment or, in some cases, replace conventional therapy. Herein, the authors review advances in the categorization and treatment of several of the more common pediatric brain tumors and discuss current and future directions in tumor management that hold significant promise for patients with these challenging tumors.

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Angela G. Viers, Khoi D. Nguyen, Perounsack X. Moon, Scott E. Forseen and Ian M. Heger

OBJECTIVE

Occipitocervical fusions in the pediatric population are rare but can be challenging because of the smaller anatomy. The procedure is even more exacting in patients with prior suboccipital craniectomy. A proposed method for occipitocervical fusion in such patients is the use of occipital condyle screws. There is very limited literature evaluating the pediatric occipital condyle for screw placement. The authors examined the occipital condyle in pediatric patients to determine if there was an age cutoff at which condylar screw placement is contraindicated.

METHODS

The authors performed a retrospective morphometric analysis of the occipital condyle in 518 pediatric patients aged 1 week–9 years old. Patients in their first decade of life whose occipital condyle was demonstrated on CT imaging in the period from 2009 to 2013 at the Augusta University Medical Center and Children’s Hospital of Georgia were eligible for inclusion in this study. Exclusion criteria were an age older than 10 years; traumatic, inflammatory, congenital, or neoplastic lesions of the occipital condyles; and any previous surgery of the occipitocervical junction. Descriptive statistical analysis was performed including calculation of the mean, standard deviation, and confidence intervals for all measurements. Probability values were calculated using the Student t-test with statistical significance determined by p < 0.05.

RESULTS

Overall, male patients had statistically significantly larger occipital condyles than the female patients, but this difference was not clinically significant. There was no significant difference in left versus right occipital condyles. There were statistically significant differences between age groups with a general trend toward older children having larger occipital condyles. Overall, 20.65% of all patients evaluated had at least one measurement that would prevent occipital condyle screw placement including at least one patient in every age group.

CONCLUSIONS

Occipital condyle screw fixation is feasible in pediatric patients younger than 10 years. More importantly, all pediatric patients should undergo critical evaluation of the occipital condyle in the axial, sagittal, and coronal planes preoperatively to determine individual suitability for occipital condyle screw placement.

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Coleman P. Riordan, Armide Storey, David J. Cote, Edward R. Smith and R. Michael Scott

OBJECTIVE

There are limited data on the long-term outcomes for children undergoing surgical revascularization for moyamoya disease (MMD) in North America. The authors present a series of pediatric MMD patients who underwent a standard revascularization procedure, pial synangiosis, more than 20 years previously at a single institution by a single surgical team.

METHODS

This study is a retrospective review of all patients aged 21 years or younger treated for MMD at Boston Children’s Hospital who were operated on more than 20 years previously by the senior author (R.M.S.). Radiographic and operative reports, outpatient clinical records, and communications with patients and families were reviewed to document current clinical status, ability to perform daily activities, and concurrent or new medical conditions.

RESULTS

A total of 59 patients (38 female [64.4%], 21 male [35.6%]; median age at surgery 6.2 years [IQR 0.5–21 years]) were identified who were diagnosed with MMD and underwent surgical revascularization procedures more than 20 years previously. Clinically, all but 2 patients (96.6%) presented with the following symptoms alone or in combination: 43 (73%) presented with stroke, 22 (37%) with transient ischemic attack, 12 (20%) with seizures, 7 (12%) with headache, 3 (5%) with choreiform movements, and 2 (3%) with hemorrhage; MMD was incidentally detected in 2 patients (3%). Five patients had unilateral MMD at presentation, but 3 of these ultimately progressed to develop bilateral MMD after an average of 16 months; therefore, pial synangiosis was ultimately performed in a total of 116 hemispheres during the study period. Clinical follow-up was available at a median interval of 20.6 years (IQR 16.1–23.2 years). Modified Rankin Scale scores were stable or improved in 43 of 50 patients with evaluable data; 45 of 55 are currently independent. There were 6 patient deaths (10.2%; 3 due to intracranial hemorrhage, 2 due to tumor-related complications, and 1 due to pulmonary artery stenosis), 4 of whom had a history of previous cranial radiation. One patient (1.7%) experienced a late stroke. Synangiosis vessels remained patent on all available late MRI and MRA studies. Four patients reported uneventful pregnancies and vaginal deliveries years following their revascularization procedures.

CONCLUSIONS

Revascularization for MMD by pial synangiosis appears to confer protection from stroke for pediatric patients over long-term follow-up. A history of cranial radiation was present in 4 of the 5 patients who died and in the lone patient with late stroke. Most patients can expect productive, independent lives following revascularization surgery in the absence of significant preoperative neurological deficits and comorbidities.

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Michael M. H. Yang, Walter Hader, Kelly Bullivant, Mary Brindle and Jay Riva-Cambrin

OBJECTIVE

The shunt protocol developed by the Hydrocephalus Clinical Research Network (HCRN) was shown to significantly reduce shunt infections in children. However, its effectiveness had not been validated in a non-HCRN, small- to medium-volume pediatric neurosurgery center. The present study evaluated whether the 9-step Calgary Shunt Protocol, closely adapted from the HCRN shunt protocol, reduced shunt infections in children.

METHODS

The Calgary Shunt Protocol was prospectively applied at Alberta Children’s Hospital from May 23, 2013, to all children undergoing any shunt procedure. The control cohort consisted of children undergoing shunt surgery between January 1, 2009, and the implementation of the Calgary Shunt Protocol. The primary outcome was the strict HCRN definition of shunt infection. Univariate analyses of the protocol, individual elements within, and known confounders were performed using Student t-test for measured variables and chi-square tests for categorical variables. Multivariable logistic regression was performed using stepwise analysis.

RESULTS

Two-hundred sixty-eight shunt procedures were performed. The median age of patients was 14 months (IQR 3–61), and 148 (55.2%) were male. There was a significant absolute risk reduction of 10.0% (95% CI 3.9%–15.9%) in shunt infections (12.7% vs 2.7%, p = 0.004) after implementation of the Calgary Shunt Protocol. In univariate analyses, chlorhexidine was associated with fewer shunt infections than iodine-based skin preparation solution (4.1% vs 12.3%, p = 0.02). Waiting ≥ 20 minutes between receiving preoperative antibiotics and skin incision was also associated with a reduction in shunt infection (4.5% vs 14.2%, p = 0.007). In the multivariable analysis, only the overall protocol independently reduced shunt infections (OR 0.19 [95% CI 0.06–0.67], p = 0.009), while age, etiology, procedure type, ventricular catheter type, skin preparation solution, and time from preoperative antibiotics to skin incision were not significant.

CONCLUSIONS

This study externally validates the published HCRN protocol for reducing shunt infection in an independent, non-HCRN, and small- to medium-volume pediatric neurosurgery setting. Implementation of the Calgary Shunt Protocol independently reduced shunt infection risk. Chlorhexidine skin preparation and waiting ≥ 20 minutes between administration of preoperative antibiotic and skin incision may have contributed to the protocol’s quality improvement success.

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Francesco Puccinelli, Minh Ngoc Thien Kim Tran Dong, Marta Iacobucci, Jean-Xavier Mazoit, Philippe Durand, Pierre Tissieres and Guillaume Saliou

OBJECTIVE

Endovascular treatment in children, especially neonates, can be more challenging than analogous procedures in adults. This study aimed to describe the clinical and radiological findings, type and timing of endovascular treatment, and early outcomes in children who present with neurovascular malformations, who are treated with embolization, and who weigh less than 5 kg.

METHODS

The authors carried out a retrospective review of all consecutively treated children weighing less than 5 kg with neurovascular arteriovenous malformations (AVMs) at a single institution over a 10-year period.

RESULTS

Fifty-two patients were included in the study. Thirty-eight had a vein of Galen aneurysmal malformation, 3 a pial AVM, 6 a pial arteriovenous fistula, and 5 a dural sinus malformation. The endovascular treatment goals were control of cardiac failure or hydrocephalus in cases of nonhemorrhagic malformations or to prevent new bleeding in cases of previous hemorrhage. A hemorrhagic complication occurred in 12 procedures and an ischemic complication in 2. Both complication types were correlated with the age of the infant (age cutoff at 3 months) (p = of 0.015 and 0.049, respectively). No correlation was found with the weight of the infant or the duration of the procedure.

CONCLUSIONS

The embolization of AVMs in these patients prevented adverse cardiac effects, hydrovenous disorders, and rebleeding. The risk of major cerebral complications seems mainly correlated with age, with a threshold at 3 months. A multidisciplinary team involved in the treatment of these children may help to improve treatment success and management.

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Michael Ragheb, Ashish H. Shah, Sarah Jernigan, Tulay Koru-Sengul and John Ragheb

OBJECTIVE

Hydrocephalus is recognized as a common disabling pediatric disease afflicting infants and children disproportionately in the developing world, where access to neurosurgical care is limited and risk of perinatal infection is high. This surgical case series describes the Project Medishare Hydrocephalus Specialty Surgery (PMHSS) program experience treating hydrocephalus in Haiti between 2008 and 2015.

METHODS

The authors conducted a retrospective review of all cases involving children treated for hydrocephalus within the PMHSS program in Port-au-Prince, Haiti, from 2008 through 2015. All relevant epidemiological information of children treated were prospectively collected including relevant demographics, birth history, hydrocephalus etiology, head circumference, and operative notes. All appropriate associations and statistical tests were performed using univariate and multivariate logistic regression analyses.

RESULTS

Among the 401 children treated within PMHSS, postinfectious hydrocephalus (PIH) accounted for 39.4% (n = 158) of cases based on clinical, radiographic, and endoscopic findings. The majority of children with hydrocephalus in Haiti were male (54.8%, n = 197), born in the rainy season (59.7%, n = 233), and born in a coastal/inland location (43.3%, n = 61). The most common surgical intervention was endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) (45.7%, n = 175). Multivariate logistic regression analysis yielded coastal birth location (OR 3.76, 95% CI 1.16–12.18) as a statistically significant predictor of PIH. Increasing head circumference (adjusted OR 1.06, 95% CI 0.99–1.13) demonstrated a slight trend toward significance with the incidence of PIH.

CONCLUSIONS

This information will provide the foundation for future clinical and public health studies to better understand hydrocephalus in Haiti. The 39.4% prevalence of PIH falls within observed rates in Africa as does the apparently higher prevalence for those born during the rainy season. Although PIH was the most frequent etiology seen in almost all birth locations, the potential relationship with geography noted in this series will be the focus of further research in an effort to understand the link between climate and PIH in Haiti. The ultimate goal will be to develop an appropriate public health strategy to reduce the burden of PIH on the children of Haiti.