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Ryan P. Morton, Renee M. Reynolds, Rohan Ramakrishna, Michael R. Levitt, Richard A. Hopper, Amy Lee, and Samuel R. Browd

Object

In this study, the authors describe their experience with a low-dose head CT protocol for a preselected neurosurgical population at a dedicated pediatric hospital (Seattle Children's Hospital), the largest number of patients with this protocol reported to date.

Methods

All low-dose head CT scans between October 2011 and November 2012 were reviewed. Two different low-dose radiation dosages were used, at one-half or one-quarter the dose of a standard head CT scan, based on patient characteristics agreed upon by the neurosurgery and radiology departments. Patient information was also recorded, including diagnosis and indication for CT scan.

Results

Six hundred twenty-four low-dose head CT procedures were performed within the 12-month study period. Although indications for the CT scans varied, the most common reason was to evaluate the ventricles and catheter placement in hydrocephalic patients with shunts (70%), followed by postoperative craniosynostosis imaging (12%). These scans provided adequate diagnostic imaging, and no patient required a follow-up full-dose CT scan as a result of poor image quality on a low-dose CT scan. Overall physician comfort and satisfaction with interpretation of the images was high. An additional 2150 full-dose head CT scans were performed during the same 12-month time period, making the total number of CT scans 2774. This value compares to 3730 full-dose head CT scans obtained during the year prior to the study when low-dose CT and rapid-sequence MRI was not a reliable option at Seattle Children's Hospital. Thus, over a 1-year period, 22% of the total CT scans were able to be converted to low-dose scans, and full-dose CT scans were able to be reduced by 42%.

Conclusions

The implementation of a low-dose head CT protocol substantially reduced the amount of ionizing radiation exposure in a preselected population of pediatric neurosurgical patients. Image quality and diagnostic utility were not significantly compromised.

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Wilson Z. Ray, Amy Lee, Spiros L. Blackburn, Gregg T. Lueder, and Jeffrey R. Leonard

✓The authors report on an 8-month-old infant with an orbital capillary hemangioma. The patient had been treated with high-dose corticosteroid therapy and had had a recent decrease in dose. The patient presented to the emergency department with increased irritability and bulging fontanelles. On lumbar puncture the opening pressure was > 55 cm H2O. Ophthalmological examination revealed interval development of papilledema. The child was treated with high-volume lumbar puncture, subsequent drainage of 10 ml of cerebrospinal fluid, resumption of the previous steroid dose, and acetazolomide therapy. The patient's symptoms resolved and follow-up ophthalmological examination revealed interval resolution of papilledema. The authors present the youngest reported case of pseudotumor development after corticosteroid tapering.

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David I. Bass, Amy Lee, Samuel R. Browd, Richard G. Ellenbogen, and Jason S. Hauptman

The purpose of this article is to serve as a rational guide for the pediatric neurosurgeon in navigating common medicolegal issues that arise in the management of abusive head trauma (AHT). Many of these issues may be unfamiliar or unpleasant to surgeons focused on addressing disease. The authors begin with a brief history on the origins of the diagnosis of AHT and the controversy surrounding it, highlighting some of the facets of the diagnosis that make it particularly unique in pediatric neurosurgery. They then review some special medical considerations in these patients through the perspective of the neurosurgeon and provide several examples as illustration. The authors discuss how to appropriately document these cases in the medical record for expected legal review, and last, they provide an overview of the legal process through which the neurosurgeon may be called to provide testimony.

Restricted access

Amy Lee, Andrea E. Van Pelt, Alex A. Kane, Thomas K. Pilgram, Daniel P. Govier, Albert S. Woo, and Matthew D. Smyth

Object

Deformational plagiocephaly (DP) is the leading cause of head shape abnormalities in infants. Treatment options include conservative measures and cranial molding. Pediatric neurosurgeons and craniofacial plastic surgeons have yet to agree on an ideal therapy, and no definable standards exist for initiating treatment with helmets. Furthermore, there may be differences between specialties in their perceptions of DP severity and need for helmet therapy.

Methods

Requests to participate in a web-based questionnaire were sent to diplomates of the American Board of Pediatric Neurological Surgery and US and Canadian members of the Pediatric Joint Section of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons and the American Cleft Palate–Craniofacial Association. Questions focused on educational background; practice setting; volume of DP patients; preferences for evaluation, treatment, follow-up; and incentives or deterrents to treat with helmet therapy. Six examples of varying degrees of DP were presented to delineate treatment preferences.

Results

Requests were sent to 302 neurosurgeons and 470 plastic surgeons, and responses were received from 71 neurosurgeons (24%) and 64 plastic surgeons (14%). The following responses represented the greatest variations between specialties: 1) 8% of neurosurgeons and 26% of plastic surgeons strongly agreed with the statement that helmet therapy is more beneficial than conservative therapy (p < 0.01); and 2) 25% of neurosurgeons and 58% of plastic surgeons would treat moderate to severe DP with helmets (p < 0.01).

Conclusions

Survey responses suggest that neurosurgeons are less likely to prescribe helmet therapy for DP than plastic surgeons. Parents of children with DP are faced with a costly treatment decision that may be influenced more strongly by referral and physician bias than medical evidence.

Free access

Philippe De Vloo, Terhi J. Huttunen, Dalila Forte, Ivana Jankovic, Amy Lee, Mark Hair, Stephanie Cawker, Deepti Chugh, Lucinda Carr, Belinda H. A. Crowe, Matthew Pitt, and Kristian Aquilina

OBJECTIVE

Selective dorsal rhizotomy (SDR) is effective at permanently reducing spasticity in children with spastic cerebral palsy. The value of intraoperative neurophysiological monitoring in this procedure remains controversial, and its robustness has been questioned. This study describes the authors’ institutional electrophysiological technique (based on the technique of Park et al.), intraoperative findings, robustness, value to the procedure, and occurrence of new motor or sphincter deficits.

METHODS

The authors analyzed electrophysiological data of all children who underwent SDR at their center between September 2013 and February 2019. All patients underwent bilateral SDR through a single-level laminotomy at the conus and with transection of about 60% of the L2–S2 afferent rootlets (guided by intraoperative electrophysiology) and about 50% of L1 afferent roots (nonselectively).

RESULTS

One hundred forty-five patients underwent SDR (64% male, mean age 6 years and 7 months, range 2 years and 9 months to 14 years and 10 months). Dorsal roots were distinguished from ventral roots anatomically and electrophysiologically, by assessing responses on free-running electromyography (EMG) and determining stimulation thresholds (≥ 0.2 mA in all dorsal rootlets). Root level was determined anatomically and electrophysiologically by assessing electromyographic response to stimulation. Median stimulation threshold was lower in sacral compared to lumbar roots (p < 0.001), and 16% higher on the first operated (right) side (p = 0.023), but unrelated to age, sex, or functional status. Similarly, responses to tetanic stimulation were consistent: 87% were graded 3+ or 4+, with similar distributions between sides. This was also unrelated to age, sex, and functional status. The L2–S2 rootlets were divided (median 60%, range 50%–67%), guided by response to tetanic stimulation at threshold amplitude. No new motor or sphincter deficits were observed, suggesting sparing of ventral roots and sphincteric innervation, respectively.

CONCLUSIONS

This electrophysiological technique appears robust and reproducible, allowing reliable identification of afferent nerve roots, definition of root levels, and guidance for rootlet division. Only a direct comparative study will establish whether intraoperative electrophysiology during SDR minimizes risk of new motor or sphincter worsening and/or maximizes functional outcome.

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Alexander G. Weil, John Ragheb, Toba N. Niazi, and Sanjiv Bhatia

Restricted access

Mark S. Dias, Ming Wang, Elias B. Rizk, Robin Bowman, Michael D. Partington, Jeffrey P. Blount, Brandon G. Rocque, Betsy Hopson, Daria Ettinger, Amy Lee, William O. Walker, and on behalf of the National Spina Bifida Patient Registry Group

OBJECTIVE

The aims of this study were to review the National Spina Bifida Patient Registry (NSBPR) data set to study the rates of tethered spinal cord release (TCR) among patients with myelomeningocele and variability between centers, to compare TCR rates between males and females, and to study the relationships between TCR rates and other condition-specific characteristics.

METHODS

The NSBPR registry was queried to identify all patients with myelomeningocele. TCR rates were calculated over time using survival analyses; rates between centers and between males and females were compared. Cox proportional hazards models were constructed to identify relationships between TCR rates and sex, functional lesion level, ambulation status, treated hydrocephalus, and prior Chiari decompression.

RESULTS

Of 6339 patients with information about their operations, 1366 (21.5%) underwent TCR, with significant variability between centers. The majority (75.8%) underwent a single TCR. The annual TCR rate was linear between birth and 13 years (1.8%/year) but declined sharply from 14 to 21 years (0.7%/year). There was no period of time at which the TCR rate accelerated. There were no significant differences in TCR rates between males and females. TCR rate was not related to functional lesion level but was lower among nonambulators compared with community ambulators (p = 0.005) and among those with treated hydrocephalus (HR 0.30, p < 0.001), and higher among those having prior Chiari decompression (HR 1.71, p < 0.001).

CONCLUSIONS

These results extend the results of prior single-institution studies, demonstrate significant treatment variability between institutions, and challenge the traditional concept that tethering is related to spinal cord stretching due to spinal growth.

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John R. W. Kestle, Amy Lee, Richard C. E. Anderson, Barbu Gociman, Kamlesh B. Patel, Matthew D. Smyth, Craig Birgfeld, Ian F. Pollack, Jesse A. Goldstein, Mandeep Tamber, Thomas Imahiyerobo, Faizi A. Siddiqi, and for the Synostosis Research Group

OBJECTIVE

The authors created a collaborative network, the Synostosis Research Group (SynRG), to facilitate multicenter clinical research on craniosynostosis. To identify common and differing practice patterns within the network, they assessed the SynRG surgeons’ management preferences for sagittal synostosis. These results will be incorporated into planning cooperative studies.

METHODS

The SynRG consists of 12 surgeons at 5 clinical sites. An email survey was distributed to SynRG surgeons in late 2016, and responses were collected through early 2017. Responses were collated and analyzed descriptively.

RESULTS

All of the surgeons—7 plastic/craniofacial surgeons and 5 neurosurgeons—completed the survey. They varied in both experience (1–24 years) and sagittal synostosis case volume in the preceding year (5–45 cases). Three sites routinely perform preoperative CT scans. The preferred surgical technique for children younger than 3 months is strip craniectomy (10/12 surgeons), whereas children older than 6 months are all treated with open cranial vault surgery. Pre-incision cefazolin, preoperative complete blood count panels, and an arterial line were used by most surgeons, but tranexamic acid was used routinely at 3 sites and never at the other 2 sites. Among surgeons performing endoscopic strip craniectomy surgery (SCS), most create a 5-cm-wide craniectomy, whereas 2 surgeons create a 2-cm strip. Four surgeons routinely send endoscopic SCS patients to the intensive care unit after surgery. Two of the 5 sites routinely obtain a CT scan within the 1st year after surgery.

CONCLUSIONS

The SynRG surgeons vary substantially in the use of imaging, the choice of surgical procedure and technique, and follow-up. A collaborative network will provide the opportunity to study different practice patterns, reduce variation, and contribute multicenter data on the management of children with craniosynostosis.

Free access

Cordell M. Baker, Vijay M. Ravindra, Barbu Gociman, Faizi A. Siddiqi, Jesse A. Goldstein, Matthew D. Smyth, Amy Lee, Richard C. E. Anderson, Kamlesh B. Patel, Craig Birgfeld, Ian F. Pollack, Thomas Imahiyerobo, John R. W. Kestle, and for the Synostosis Research Group

OBJECTIVE

Sagittal synostosis is the most common form of isolated craniosynostosis. Although some centers have reported extensive experience with this condition, most reports have focused on a single center. In 2017, the Synostosis Research Group (SynRG), a multicenter collaborative network, was formed to study craniosynostosis. Here, the authors report their early experience with treating sagittal synostosis in the network. The goals were to describe practice patterns, identify variations, and generate hypotheses for future research.

METHODS

All patients with a clinical diagnosis of isolated sagittal synostosis who presented to a SynRG center between March 1, 2017, and October 31, 2019, were included. Follow-up information through October 31, 2020, was included. Data extracted from the prospectively maintained SynRG registry included baseline parameters, surgical adjuncts and techniques, complications prior to discharge, and indications for reoperation. Data analysis was descriptive, using frequencies for categorical variables and means and medians for continuous variables.

RESULTS

Two hundred five patients had treatment for sagittal synostosis at 5 different sites. One hundred twenty-six patients were treated with strip craniectomy and 79 patients with total cranial vault remodeling. The most common strip craniectomy was wide craniectomy with parietal wedge osteotomies (44%), and the most common cranial vault remodeling procedure was total vault remodeling without forehead remodeling (63%). Preoperative mean cephalic indices (CIs) were similar between treatment groups: 0.69 for strip craniectomy and 0.68 for cranial vault remodeling. Thirteen percent of patients had other health problems. In the cranial vault cohort, 81% of patients who received tranexamic acid required a transfusion compared with 94% of patients who did not receive tranexamic acid. The rates of complication were low in all treatment groups. Five patients (2%) had an unintended reoperation. The mean change in CI was 0.09 for strip craniectomy and 0.06 for cranial vault remodeling; wide craniectomy resulted in a greater change in CI in the strip craniectomy group.

CONCLUSIONS

The baseline severity of scaphocephaly was similar across procedures and sites. Treatment methods varied, but cranial vault remodeling and strip craniectomy both resulted in satisfactory postoperative CIs. Use of tranexamic acid may reduce the need for transfusion in cranial vault cases. The wide craniectomy technique for strip craniectomy seemed to be associated with change in CI. Both findings seem amenable to testing in a randomized controlled trial.

Free access

Anthony C. Wang, George M. Ibrahim, Andrew V. Poliakov, Page I. Wang, Aria Fallah, Gary W. Mathern, Robert T. Buckley, Kelly Collins, Alexander G. Weil, Hillary A. Shurtleff, Molly H. Warner, Francisco A. Perez, Dennis W. Shaw, Jason N. Wright, Russell P. Saneto, Edward J. Novotny, Amy Lee, Samuel R. Browd, and Jeffrey G. Ojemann

OBJECTIVE

The potential loss of motor function after cerebral hemispherectomy is a common cause of anguish for patients, their families, and their physicians. The deficits these patients face are individually unique, but as a whole they provide a framework to understand the mechanisms underlying cortical reorganization of motor function. This study investigated whether preoperative functional MRI (fMRI) and diffusion tensor imaging (DTI) could predict the postoperative preservation of hand motor function.

METHODS

Thirteen independent reviewers analyzed sensorimotor fMRI and colored fractional anisotropy (CoFA)–DTI maps in 25 patients undergoing functional hemispherectomy for treatment of intractable seizures. Pre- and postoperative gross hand motor function were categorized and correlated with fMRI and DTI findings, specifically, abnormally located motor activation on fMRI and corticospinal tract atrophy on DTI.

RESULTS

Normal sensorimotor cortical activation on preoperative fMRI was significantly associated with severe decline in postoperative motor function, demonstrating 92.9% sensitivity (95% CI 0.661–0.998) and 100% specificity (95% CI 0.715–1.00). Bilaterally robust, symmetric corticospinal tracts on CoFA-DTI maps were significantly associated with severe postoperative motor decline, demonstrating 85.7% sensitivity (95% CI 0.572–0.982) and 100% specificity (95% CI 0.715–1.00). Interpreting the fMR images, the reviewers achieved a Fleiss’ kappa coefficient (κ) for interrater agreement of κ = 0.69, indicating good agreement (p < 0.01). When interpreting the CoFA-DTI maps, the reviewers achieved κ = 0.64, again indicating good agreement (p < 0.01).

CONCLUSIONS

Functional hemispherectomy offers a high potential for seizure freedom without debilitating functional deficits in certain instances. Patients likely to retain preoperative motor function can be identified prior to hemispherectomy, where fMRI or DTI suggests that cortical reorganization of motor function has occurred prior to the operation.