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James M. Johnston, David D. Limbrick Jr., Wilson Z. Ray, Stephanie Brown, Joshua Shimony, and Tae Sung Park

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder that rarely involves the CNS. Rosai-Dorfman disease is exceedingly rare in the pediatric population and has never been observed in the cerebellum of a child. The authors present the case of a 14-year-old male with a cerebellar lesion having radiographic characteristics of Lhermitte-Duclos disease. After a period of observation with a presumptive diagnosis of Lhermitte-Duclos disease, the child underwent suboccipital craniotomy and resection of the lesion due to continuous suboccipital headaches. Histological examination of the tissue demonstrated RDD. The published literature on RDD is reviewed with an emphasis on differential diagnosis.

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James M. Johnston Jr., Francesco T. Mangano, Jeffrey G. Ojemann, Tae Sung Park, Edwin Trevathan, and Matthew D. Smyth

Object

The purpose of this study was to better define the incidence of complications associated with placement of subdural electrodes for localization of seizure foci and functional mapping in children.

Methods

The authors retrospectively reviewed the records of 112 consecutive patients (53 boys, 59 girls; mean age 10.9 years, range 10 months–21.7 years) with medically intractable epilepsy who underwent invasive monitoring at the Pediatric Epilepsy Center at St. Louis Children’s Hospital between January 1994 and July 2005. There were 122 implantation procedures (85 grids and strips, 32 strips only, five grids only, four with additional depth electrodes), with a mean monitoring period of 7.1 days (range 2–21 days). Operative complications included the need for repeated surgery for additional electrode placement (5.7%); wound infection (2.4%); cerebrospinal fluid leak (1.6%); and subdural hematoma, symptomatic pneumocephalus, bone flap osteomyelitis, and strip electrode fracture requiring operative retrieval (one patient [0.8%] each). There were four cases of transient neurological deficit (3.3%) and no permanent deficit or death associated with invasive monitoring.

Conclusions

Placement of subdural grid and strip electrodes for invasive video electroencephalographic monitoring is generally well tolerated in the pediatric population. The authors found that aggressive initial electrode coverage was not associated with higher rates of blood transfusion or perioperative complications, and reduced the frequency of repeated operations for placement of supplemental electrodes.

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Jacob Lepard, S. Hassan A. Akbari, James Mooney, Anastasia Arynchyna, Samuel G. McClugage III, René P. Myers, John Grant, and James M. Johnston Jr.

OBJECTIVE

In the last several decades, there has been much debate regarding the ideal treatment for sagittal synostosis. The purpose of this study was to compare perioperative, anthropometric, and subjective assessments of cosmetic outcomes between open and endoscopic management of isolated sagittal synostosis.

METHODS

At their routine postoperative follow-up, pediatric patients with sagittal craniosynostosis were recruited to undergo digital cranial measurement and standardized photography for objective and subjective assessments of perioperative outcomes. Age-normalized z-scores for cephalic index, head circumference, euryon-euryon diameter (Eu-Eu), and glabella-opisthocranion diameter (G-Op) were calculated for each patient. Faculty surgeons, surgical trainees, nurses, and laypersons were asked to rate the normalcy of craniofacial appearances using a 5-point Likert scale. Outcomes were compared between patients treated with endoscopic correction and those treated with open repair.

RESULTS

A total of 50 patients were included in the study. Thirty-one had undergone open surgical correction, and 19 had undergone endoscopic treatment. Endoscopic repair involved significantly lower operative time, blood loss, transfusion rate, and hospital length of stay than those with open repair (p < 0.001). There was no significant difference between groups in terms of z-scores for head circumference (p = 0.22), cephalic index (p = 0.25), or Eu-Eu (p = 0.38). Endoscopic treatment was associated with a significantly lower G-Op (p = 0.009). Additionally, the average subjective rating of head shape was higher for endoscopic treatment when corrected for age, gender, and ethnicity (p = 0.02).

CONCLUSIONS

The study findings suggest that patients who are treated endoscopically may have an overall more normal appearance in skull morphology and cosmesis, although these results are limited by poor reliability.

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Daxa M. Patel, R. Shane Tubbs, Gigi Pate, James M. Johnston Jr., and Jeffrey P. Blount

Object

Surveillance imaging of the cerebral ventricles can be valuable in following up children with shunt-treated hydrocephalus. There also, however, has been recent increased awareness and concern over the potential risk associated with imaging-related radiation exposure in children. Magnetic resonance imaging represents an imaging alternative that does not use ionizing radiation; however, its practical utility has been limited due to the near-uniform requirement for sedation or general anesthesia in children. Magnetic resonance imaging without sedation is often futile because of the movement artifact produced by the nonsedated pediatric patient. Some studies have demonstrated the feasibility of using fast-sequence MRI (fsMRI), but the reported experiences are limited. The authors have incorporated fsMRI into their routine shunt surveillance imaging paradigms and report here a 5-year experience with this modality.

Methods

The authors initially started using fsMRI for routine surveillance in a single clinic in 2008 and have gradually increased their institutional utilization of this modality as experience has accumulated and protocols have been refined. Imaging sequences obtained for each child include an axial T2-weighted half-Fourier acquisition single-shot turbo spin-echo (HASTE), coronal T2-weighted HASTE, and sagittal T2-weighted HASTE images. The authors conducted a retrospective chart and imaging review. They rated each fsMR image according to 5 visibility parameters: 1) ventricle size, 2) ventricle configuration, 3) presence or absence of transependymal flow, 4) presence or absence of motion artifact, and 5) visualization of the ventricular catheter. Each parameter was graded as 1 (present) or 0 (absent). Thus, the maximum value assigned to each scan could be 5 and the minimum value assigned to each scan could be 0. Interrater reliability between pairs of observers was calculated using the Kendall's tau-b and intraclass coefficients.

Results

Two hundred patients underwent fsMRI. No child required sedation. The average duration of examinations was approximately 3.37 minutes, and mean age of the patients was 5.7 years. Clinically useful images were attained in all cases. Overall quality of the fsMRI studies based on the 5 different visibility parameters showed that 169 images (84.5%) included 4 or 5 parameters (score ≥ 4) and had statistically significant excellent quality. The Kendall's tau-b for the overall fsMRI ratings was 0.82 (p = 0.002) and the intraclass coefficient was 0.87 (p < 0.0001).

Conclusions

In the present cohort of 200 patients, fsMRI studies were shown to have an excellent overall quality and a statistically significant high degree of interrater reliability. Consequently, the authors propose that fsMRI is a sufficiently effective modality that eliminates the need for sedation and the use of ionizing radiation and that it should supplant CT for routine surveillance imaging in hydrocephalic patients.

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Michael C. Dewan, Ronnie E. Baticulon, Abbas Rattani, James M. Johnston Jr., Benjamin C. Warf, and William Harkness

OBJECTIVE

The presence and capability of existing pediatric neurosurgical care worldwide is unknown. The objective of this study was to solicit the expertise of specialists to quantify the geographic representation of pediatric neurosurgeons, access to specialist care, and equipment and training needs globally.

METHODS

A mixed-question survey was sent to surgeon members of several international neurosurgical and general pediatric surgical societies via a web-based platform. Respondents answered questions on 5 categories: surgeon demographics and training, hospital and practice details, surgical workforce and access to neurosurgical care, training and equipment needs, and desire for international collaboration. Responses were anonymized and analyzed using Stata software.

RESULTS

A total of 459 surgeons from 76 countries responded. Pediatric neurosurgeons in high-income and upper-middle-income countries underwent formal pediatric training at a greater rate than surgeons in low- and lower-middle-income countries (89.5% vs 54.4%). There are an estimated 2297 pediatric neurosurgeons in practice globally, with 85.6% operating in high-income and upper-middle-income countries. In low- and lower-middle-income countries, roughly 330 pediatric neurosurgeons care for a total child population of 1.2 billion. In low-income countries in Africa, the density of pediatric neurosurgeons is roughly 1 per 30 million children. A higher proportion of patients in low- and lower-middle-income countries must travel > 2 hours to seek emergency neurosurgical care, relative to high-income countries (75.6% vs 33.6%, p < 0.001). Vast basic and essential training and equipment needs exist, particularly low- and lower-middle-income countries within Africa, South America, the Eastern Mediterranean, and South-East Asia. Eighty-nine percent of respondents demonstrated an interest in international collaboration for the purposes of pediatric neurosurgical capacity building.

CONCLUSIONS

Wide disparity in the access to pediatric neurosurgical care exists globally. In low- and lower-middle-income countries, wherein there exists the greatest burden of pediatric neurosurgical disease, there is a grossly insufficient presence of capable providers and equipped facilities. Neurosurgeons across income groups and geographic regions share a desire for collaboration and partnership.

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Matthew C. Davis, Brandon G. Rocque, Ash Singhal, Thomas Ridder, Jogi V. Pattisapu, and James M. Johnston Jr.

OBJECTIVE

Neurosurgical services are increasingly recognized as essential components of surgical care worldwide. The degree of interest among neurosurgeons regarding international work, and the barriers to involvement in global neurosurgical outreach, are largely unexplored. The authors distributed a survey to members of the American Association of Neurological Surgeons/Congress of Neurological Surgeons (AANS/CNS) Joint Section on Pediatric Neurosurgery to assess the state of global outreach among its members and to identify barriers to involvement.

METHODS

An internet-based questionnaire was developed by the International Education Subcommittee of the AANS/CNS Joint Section on Pediatric Neurosurgery and distributed to pediatric neurosurgeons via the AANS/CNS Joint Section email contact list. Participants were surveyed on their involvement in global neurosurgical outreach, geographic location, nature of the participation, and barriers to further involvement.

RESULTS

A 35.3% response rate was obtained, with 116 respondents completing the survey. Sixty-one percent have performed or taught neurosurgery in a developing country, and 49% travel at least annually. Africa was the most common region (54%), followed by South America (30%), through 29 separate organizing entities. Hydrocephalus was the most commonly treated condition (88%), followed by spinal dysraphism (74%), and tumor (68%). Most respondents obtained follow-up through communications from local surgeons (77%). Seventy-one percent believed the international experience improved their practice, and 74% were very or extremely interested in working elsewhere. Interference with current practice (61%), cost (44%), and difficulty identifying international partners (43%) were the most commonly cited barriers to participation.

CONCLUSIONS

Any coordinated effort to expand global neurosurgical capacity begins with appreciation for the current state of outreach efforts. Increasing participation in global outreach will require addressing both real and perceived barriers to involvement. Creation and curation of a centralized online database of ongoing projects to facilitate coordination and involvement may be beneficial.

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Editorial

Posthemorrhagic ventricular dilation

Joseph H. Piatt

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David D. Limbrick Jr., Amit Mathur, James M. Johnston, Rebecca Munro, James Sagar, Terrie Inder, Tae Sung Park, Jeffrey L. Leonard, and Matthew D. Smyth

Object

Intraventricular hemorrhage (IVH) and progressive posthemorrhagic ventricular dilation (PPHVD) may result in significant neurological morbidity in preterm infants. At present, there is no consensus regarding the optimal timing or type of neurosurgical procedure to best treat PPHVD. Conflicting data exist regarding the relative risks and benefits of two commonly used temporizing neurosurgical procedures (TNPs), ventricular access devices ([VADs] or ventricular reservoirs) versus ventriculosubgaleal (VSG) shunts. This study was designed to address this issue.

Methods

This is a single-center, 10-year retrospective review of all preterm infants admitted to the St. Louis Children's Hospital neonatal intensive care unit (NICU) with Papile Grade III–IV IVH. The development of PPHVD and the requirement for and type of TNP were recorded. Rates of TNP complication, ventriculoperitoneal (VP) shunt implantation, shunt infection, and mortality rates were used to compare the efficacy and limitations of each TNP type.

Results

Over this 10-year interval, 325 preterm infants with Grade III–IV IVH were identified, with trends showing an increasing number of affected infants annually, and an increasing number of TNPs were required annually. Ninety-five (29.2%) of the 325 infants underwent a TNP for PPHVD (65 VADs, 30 VSG shunts). The rate of permanent VP shunt implantation for all TNPs was 72.6% (69 of 95 infants). Forty-nine (75.4%) of the 65 infants treated with VADs and 20 (66.7%) of the 30 treated with VSG shunts required VP shunts (p = 0.38). There was no statistical difference between VAD or VSG shunt with regard to TNP-related infection (p = 0.57), need for TNP revision (p = 0.16), subsequent shunt infection (p = 0.77), shunt revision rate (p = 0.58), or mortality rate (p = 0.24).

Conclusions

Rates of IVH and PPHVD observed at the authors' center have increased over time. In contrast to recent literature, the results from the current study did not demonstrate a difference in complication rate or requirement for permanent VP shunt placement between VADs and VSG shunts. Definitive conclusions will require a larger, prospective trial.

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Sara Anne Wilkins, Chevis N. Shannon, Steven T. Brown, E. Haley Vance, Drew Ferguson, Kimberly Gran, Marshall Crowther, John C. Wellons III, and James M. Johnston Jr.

Object

Recent legislation and media coverage have heightened awareness of concussion in youth sports. Previous work by the authors' group defined significant variation of care in management of children with concussion. To address this variation, a multidisciplinary concussion program was established based on a uniform management protocol, with emphasis on community outreach via traditional media sources and the Internet. This retrospective study evaluates the impact of standardization of concussion care and resource utilization before and after standardization in a large regional pediatric hospital center.

Methods

This retrospective study included all patients younger than 18 years of age evaluated for sports-related concussion between January 1, 2007, and December 31, 2011. Emergency department, sports medicine, and neurosurgery records were reviewed. Data collected included demographics, injury details, clinical course, Sports Concussion Assessment Tool-2 (SCAT2) scores, imaging, discharge instructions, and referral for specialty care. The cohort was analyzed comparing patients evaluated before and after standardization of care.

Results

Five hundred eighty-nine patients were identified, including 270 before standardization (2007–2011) and 319 after standardization (2011–2012). Statistically significant differences (p < 0.0001) were observed between the 2 groups for multiple variables: there were more girls, more first-time concussions, fewer initial presentations to the emergency department, more consistent administration of the SCAT2, and more consistent supervision of return to play and return to think after adoption of the protocol.

Conclusions

A combination of increased public awareness and legislation has led to a 5-fold increase in the number of youth athletes presenting for concussion evaluation at the authors' center. Establishment of a multidisciplinary clinic with a standardized protocol resulted in significantly decreased institutional resource utilization and more consistent concussion care for this growing patient population.

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Robert P. Naftel, Nicole A. Safiano, Michael I. Falola, Chevis N. Shannon, John C. Wellons III, and James M. Johnston Jr.

Object

The Internet and social media are powerful disseminators of medical information, providing new portals for patient care. The authors of this study evaluated current technology hardware, Internet, and social media use and their socioeconomic relationships among caregivers of children with hydrocephalus.

Methods

A written survey was completed in the neurosurgical clinics at the University of Alabama at Birmingham by 300 parents of children with shunted hydrocephalus between October 26, 2010, and July 26, 2011.

Results

Computer use (94.6%), Internet use (91.7%), smartphone use (56.9%), and Internet research on hydrocephalus (81.9%) were prevalent. However, for each of these four utilizations there was significantly lower access by caregivers of minority races (p = 0.04, 0.03, 0.002, and < 0.0001, respectively), lower income (p = 0.02, 0.01, < 0.0001, and < 0.0001, respectively), and lower level of education (p = 0.001, 0.002, < 0.0001, and 0.001, respectively). Personal use of social media was prevalent (95.1% of all Internet users) with use being more prevalent among less-educated than higher-educated caregivers (p = 0.017). Hydrocephalus-related social media use (59.5% of Internet users) was not associated with socioeconomic factors. For hydrocephalus education on the Internet, caregivers chose information websites such as Wikipedia or the Hydrocephalus Association as preferred platforms; these preferences were followed by use of social media websites. Facebook and YouTube were the preferred social media platforms for personal and hydrocephalus-related use. Parents indicate moderate skepticism about the trustworthiness of the Internet; only 21.7% always trust the online sources. Most parents (89.8%) say that they would visit neurosurgeon-recommended websites. Of Internet-using caregivers, 28.6% use the Internet or social media to find hydrocephalus support groups, and 34.8% have used the Internet to communicate with other caregivers who have children with similar conditions.

Conclusions

Technology hardware, the Internet, and social media are widely used with some skepticism by parents of children with shunted hydrocephalus. Caregivers are interested in physician-recommended Internet resources. Socioeconomic factors including race, income, and level of education reveal a disparity in access to some of these resources, although all groups have relatively high use. Unlike typical technology use, social media use is breaking down the digital divide among ethnic and socioeconomic groups.