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Ryan P. Lee, Kimberly A. Foster, Jock C. Lillard, Paul Klimo Jr., David W. Ellison, Brent Orr, and Frederick A. Boop

OBJECTIVE

Thalamopeduncular tumors are a group of pediatric low-grade gliomas that arise at the interface of the thalamus and brainstem peduncle. They typically occur within the first 2 decades of life, presenting with progressive spastic hemiparesis. Treatment strategies, including surgical intervention, have varied significantly. The authors present their experience in the treatment of 13 children, ages 2–15 years, with non-neurofibromatosis–related pilocytic astrocytomas located in the thalamopeduncular region.

METHODS

Between 2003 and 2016, 13 children presenting with progressive spastic hemiparesis due to a pilocytic astrocytoma at the interface of the thalamus and cerebral peduncles were identified. Medical records were reviewed retrospectively for clinical, radiological, pathological, and surgical data. Formalin-fixed, paraffin-embedded tissue was obtained for 12 cases and tested for KIAA1549-BRAF fusion and BRAF V600E point mutation.

RESULTS

On preoperative diffusion tensor imaging tractography (performed in 12 patients), the ipsilateral corticospinal tract was displaced laterally in 1 case (8.3%), medially in 1 case (8.3%), anterolaterally in 10 cases (83%), and posteriorly in no cases. Ten patients underwent resection via a transtemporal, transchoroidal approach, which was chosen to avoid further damage to motor function in cases of tumors that caused anterolateral or medial corticospinal tract displacement. With this approach, complications included hemianopia, oculomotor palsy, and tremor at a rate of 50%. Among the 12 patients with obtainable follow-up (mean 50.9 months), none received adjuvant therapy, and only 2 (17%) experienced recurrence or progression. KIAA1549-BRAF fusions were present in 10 cases (83%), while BRAF V600E was absent (0%). The 2 fusion-negative tumors had clinical features atypical for the series, including multi-focality and infiltration.

CONCLUSIONS

Transcortical, transchoroidal resection of thalamopeduncular tumors through the middle temporal gyrus allows for a high rate of gross-total resection and cure. Diffuse tensor tractography is a critical component of the preoperative planning process to determine the location of white matter tracts in proximity. Molecular status may correlate with clinical features, and the presence of BRAF lesions offers an additional target for future novel therapeutics.

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Jesús A. Morales-Gómez, Vicente V. Garza-Oyervides, José A. Arenas-Ruiz, Mariana Mercado-Flores, C. Guillermo Elizondo-Riojas, Frederick A. Boop, and Ángel Martínez-Ponce de León

Intracranial pial arteriovenous fistulas, also known as nongalenic fistulas, are rare vascular malformations affecting predominantly the pediatric population. Hydrocephalus is an unusual presentation in which the exact pathophysiology is not fully understood. The aim of treatment in these cases is occlusion of the fistula prior to considering ventricular shunting. Here, the authors describe the hydrodynamic considerations of the paravascular pathway and the resolution of hydrocephalus with endovascular treatment of the fistula.

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Michael DeCuypere, Michael S. Muhlbauer, Frederick A. Boop, and Paul Klimo Jr.

OBJECTIVE

Penetrating brain injury in civilians is much less common than blunt brain injury but is more severe overall. Gunshot wounds (GSWs) cause high morbidity and mortality related to penetrating brain injury; however, there are few reports on the management and outcome of intracranial GSWs in children. The goals of this study were to identify clinical and radiological factors predictive for death in children and to externally validate a recently proposed pediatric prognostic scale.

METHODS

The authors conducted a retrospective review of penetrating, isolated GSWs sustained in children whose ages ranged from birth to 18 years and who were treated at 2 major metropolitan Level 1 trauma centers from 1996 through 2013. Several standard clinical, laboratory, and radiological factors were analyzed for their ability to predict death in these patients. The authors then applied the St. Louis Scale for Pediatric Gunshot Wounds to the Head, a scoring algorithm that was designed to provide rapid prognostic information for emergency management decisions. The scale's sensitivity, specificity, and positive and negative predictability were determined, with death as the primary outcome.

RESULTS

Seventy-one children (57 male, 14 female) had a mean age of 14 years (range 19 months to 18 years). Overall mortality among these children was 47.9%, with 81% of survivors attaining a favorable clinical outcome (Glasgow Outcome Scale score ≥ 4). A number of predictors of mortality were identified (all p < 0.05): 1) bilateral fixed pupils; 2) deep nuclear injury; 3) transventricular projectile trajectory; 4) bihemispheric injury; 5) injury to ≥ 3 lobes; 6) systolic blood pressure < 100 mm Hg; 7) anemia (hematocrit < 30%); 8) Glasgow Coma Scale score ≤ 5; and 9) a blood base deficit < −5 mEq/L. Patient age, when converted to a categorical variable (0–9 or 10–18 years), was not predictive. Based on data from the 71 patients in this study, the positive predictive value of the St. Louis scale in predicting death (score ≥ 5) was 78%.

CONCLUSIONS

This series of pediatric cranial GSWs underscores the importance of the initial clinical exam and CT studies along with adequate resuscitation to make the appropriate management decision(s). Based on our population, the St. Louis Scale seems to be more useful as a predictor of who will survive than who will succumb to their injury.

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Chiaho Hua, Barry L. Shulkin, Daniel J. Indelicato, Yimei Li, Xingyu Li, Frederick A. Boop, and Thomas E. Merchant

OBJECT

The aim of the study was to document postoperative cerebral glucose distribution before proton therapy using F-18 fluorodeoxyglucose positron emission tomography (FDG PET) in children with craniopharyngioma.

METHODS

Between August 2011 and April 2014, 50 patients (20 males, 30 females) enrolled in a prospective trial for craniopharyngioma underwent FDG PET imaging before proton therapy. Proton therapy was delivered using doublescattered beams with a total prescribed dose of 54 cobalt gray equivalent. Tracer uptake in each of 63 anatomical regions was computed after warping PET images to a 3D reference template in Talairach coordinates. Regional uptake was deemed significantly low or high if it exceeded age-corresponding 95% prediction intervals of the normal population. The reference group included 132 children with non-CNS-related diseases and normal-appearing cerebral FDG PET scans.

RESULTS

Median patient age at diagnosis was 8.5 years (range 2–18 years). Forty-eight patients underwent 1–4 tumor-related surgeries before proton therapy, including placement of an Ommaya reservoir in 14 patients. Sixteen patients had symptomatic hydrocephalus that was treated with temporary (external ventricular drain, n = 16) or permanent CSF shunting (ventriculoperitoneal shunt, n = 1). The most commonly seen PET abnormalities in patients before proton therapy were significantly reduced uptake in subregions of the frontal lobe (often involving more than 1 gyrus), medial and ventral portions of the temporal lobe, cingulate gyrus, and caudate nucleus. A significantly high uptake was frequently observed on the contralateral side, including the superior, medial, and inferior temporal gyri and a large portion of the parietal lobe. Statistically significant predictor variables identified in the multivariate analysis for the extent of hypometabolism were sex (p = 0.005), hydrocephalus (p = 0.026), and the number of tumor-related surgeries (p = 0.017).

CONCLUSIONS

Postoperative FDG PET of patients with craniopharyngioma revealed metabolic abnormalities in specific regions of the brain. The ability to identify anatomical metabolic defects in individual patients facilitates the investigation of brain injury in children with craniopharyngioma.

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Joseph H. McAbee, Joseph Modica, Clinton J. Thompson, Alberto Broniscer, Brent Orr, Asim F. Choudhri, Frederick A. Boop, and Paul Klimo Jr.

OBJECT

Cervicomedullary tumors (CMTs) represent a heterogeneous group of intrinsic neoplasms that are typically low grade and generally carry a good prognosis. This single-institution study was undertaken to document the outcomes and current treatment philosophy for these challenging neoplasms.

METHODS

The charts of all pediatric patients with CMTs who received treatment at St. Jude Children’s Research Hospital between January 1988 and May 2013 were retrospectively reviewed. Demographic, surgical, clinical, radiological, pathological, and survival data were collected. Treatment-free survival and overall survival were estimated, and predictors of recurrence were analyzed.

RESULTS

Thirty-one children (16 boys, 15 girls) with at least 12 months of follow-up data were identified. The median age at diagnosis was 6 years (range 7 months-17 years) and the median follow-up was 4.3 years. Low-grade tumors (Grade I or II) were present in 26 (84%) patients. Thirty patients underwent either a biopsy alone or resection, with the majority of patients undergoing biopsy only (n = 12, 39%) or subtotal resection (n = 14, 45%). Only 4 patients were treated solely with resection; 21 patients received radiotherapy alone or in combination with other treatments. Recurrent tumor developed in 14 children (45%) and 4 died as a result of their malignancy. A high-grade pathological type was the only independent variable that predicted recurrence. The 5- and 10-year treatment-free survival estimates are 64.7% and 45.3%, respectively. The 5- and 10-year overall survival estimate is 86.7%.

CONCLUSIONS

Children with CMTs typically have low-grade neoplasms and consequently long-term survival, but high risk of recurrence. Therapy should be directed at achieving local tumor control while preserving and even restoring neurological function.

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Frederick A. Boop, Berkeley Bate, Asim F. Choudhri, Brian Burkholder, and Paul Klimo Jr.

The development of high-quality intraoperative MRI (iMRI) capability has offered a major advance in the care of patients with complex intracranial disease. To date, this technology has been limited by the need for pin fixation of the calvaria. The authors report their preliminary experience with an MRI-compatible horseshoe headrest that allows for the following: 1) iMRI in patients too young for pin fixation; 2) iMRI in patients with large calvarial defects; 3) the ability to move the head during iMRI surgery; and 4) the use of neuronavigation in such cases. The authors report 2 cases of infants in whom the Visius Surgical Theatre horseshoe headrest (IMRIS Inc.) was used. Image quality was equivalent to that of pin fixation. The infants suffered no skin issues. The use of neuronavigation with the system remained accurate and could be updated with the new iMRI information. The Visius horseshoe headrest offers a technical advance in iMRI technology for infants, for patients with cranial defects or prior craniotomies in whom pin fixation may not be safe, or for patients in whom the need to move the head during surgery is required. The image quality of the system remains excellent, and the ability to merge new images to the neuronavigation system is helpful.

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Paul Klimo Jr., Garrett T. Venable, Frederick A. Boop, and Thomas E. Merchant

OBJECT

In this paper the authors present their experience treating children with recurrent craniopharyngioma who were initially managed with surgery followed by conformal radiation therapy (CRT).

METHODS

A departmental oncology information system was queried to identify all children (< 18 years old) who received CRT for a craniopharyngioma between 1998 and 2010 (inclusive) and specifically those who experienced tumor progression. For each patient, the authors recorded the type of recurrence (solid, cystic, or both), the time interval to first progression and each subsequent progression, the associated treatment complications, and disease status at last follow-up evaluation.

RESULTS

Among the 97 patients that met criteria for entry into this study, 18 (18.6%) experienced tumor progression (9 cystic, 3 solid, 6 cystic and solid). The median time to first recurrence was 4.62 years (range 1.81–9.11 years). The subgroup included 6 female and 12 male patients with a median age of 7.54 years (range 3.61–13.83 years). Ten patients experienced first progression within 5 years of CRT. The 5- and 10-year treatment-free survival rates for the entire cohort were 89.0% (95% confidence interval [CI] 80.5%–93.9%) and 76.2% (95% CI 64%–85%), respectively. Seven patients had a single episode of progression and 11 had more than 1. The time interval between each subsequent progression was progressively shorter. The 18 patients underwent 38 procedures. The median follow-up duration for this group was 9.32 years (range 4.04–19.0 years). Three patients died, including 1 from perioperative complications.

CONCLUSIONS

Craniopharyngioma progression after prior irradiation is exceedingly difficult to treat and local control is challenging despite repeated surgical procedures. Given our results, gross-total resection may need to be the surgical goal at the time of first recurrence, if possible. Decompressing new cyst formation alone has a low rate of long-term success.

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Aska Arnautovic, Bruno Splavski, Frederick A. Boop, and Kenan I. Arnautovic

OBJECT

Chiari malformation Type I (CM-I) is a hindbrain disorder associated with elongation of the cerebellar tonsils, which descend below the foramen magnum into the spinal canal. It occurs in children and adults. Clinical symptoms mainly develop from alterations in CSF flow at the foramen magnum and the common subsequent development of syringomyelia.

METHODS

The authors reviewed English-language reports of pediatric, adult, and combined (adult and pediatric) surgical series of patients with CM-I published from 1965 through August 31, 2013, to investigate the following: 1) geographical distribution of reports; 2) demographics of patients; 3) follow-up lengths; 4) study durations; 5) spectrum and frequency of surgical techniques; 6) outcomes for neurological status, syrinx, and headache; 7) frequency and scope of complications; 8) mortality rates; and 9) differences between pediatric and adult populations. Research and inclusion criteria were defined, and all series that contained at least 4 cases and all publications with sufficient data for analysis were included.

RESULTS

The authors identified 145 operative series of patients with CM-I, primarily from the United States and Europe, and divided patient ages into 1 of 3 categories: adult (> 18 years of age; 27% of the cases), pediatric (≤ 18 years of age; 30%), or unknown (43%). Most series (76%) were published in the previous 21 years. The median number of patients in the series was 31. The mean duration of the studies was 10 years, and the mean follow-up time was 43 months. The peak ages of presentation in the pediatric studies were 8 years, followed by 9 years, and in the adult series, 41 years, followed by 46 years. The incidence of syringomyelia was 65%. Most of the studies (99%) reported the use of posterior fossa/foramen magnum decompression. In 92%, the dura was opened, and in 65% of these cases, the arachnoid was opened and dissected; tonsillar resection was performed in 27% of these patients. Postoperatively, syringomyelia improved or resolved in 78% of the patients. Most series (80%) reported postoperative neurological outcomes as follows: 75% improved, 17% showed no change, and 9% experienced worsening. Postoperative headaches improved or resolved in 81% of the patients, with a statistical difference in favor of the pediatric series. Postoperative complications were reported for 41% of the series, most commonly with CSF leak, pseudomeningocele, aseptic meningitis, wound infection, meningitis, and neurological deficit, with a mean complication rate of 4.5%. Complications were reported for 37% of pediatric, 20% of adult, and 43% of combined series. Mortality was reported for 11% of the series. No difference in mortality rates was seen between the pediatric and adult series.

CONCLUSIONS

Before undergoing surgical treatment for CM-I, symptomatic patients and their families should be given clear information about the success of treatment and potential complications. Furthermore, surgeons may benefit from comparing published data with their own. In the future, operative CM-I reports should provide all details of each case for the purpose of comparison.