BACKGROUND Intraoperative electrocorticography (ECoG) has been utilized in patients with tumor-as... more BACKGROUND Intraoperative electrocorticography (ECoG) has been utilized in patients with tumor-associated seizures; however, its effectiveness for seizure control remains controversial. OBJECTIVE To evaluate clinical outcomes in pediatric patients undergoing lesionectomy with or without ECoG. METHODS Patients undergoing brain tumor resection at Boston Children's Hospital were examined retrospectively (2005-2014). Inclusion criteria involved diagnosis of a supratentorial tumor, ≥2 unequivocal seizures, and ≥6 mo follow-up. Patients with isolated cortical dysplasia or posterior fossa tumors were excluded. Logistic regression models evaluated predictors of ECoG use, and the impact of ECoG, gross total resection, and focal cortical dysplasia with tumors on seizure freedom by Engel Class and anti-epileptic drug use (AED). RESULTS A total of 119 pediatric patients were included (n = 69 males, 58%; median age, 11.3 yr). Forty-one patients (34.5%) had ECoG-guided surgery. Preoperative s...
Complications of Le Fort III midfacial advancement include CSF rhinorrhea, meningitis, and ocular... more Complications of Le Fort III midfacial advancement include CSF rhinorrhea, meningitis, and ocular and cerebral injury. This report reviews the anatomy of the Le Fort III osteotomies and their relevance to the unusual complication of meningoencephalocele. In this report, a young male patient with Crouzon syndrome underwent subcranial midfacial advancement at the age of 10 years for obstructive sleep apnea and ocular exposure. He presented 4 years later complaining of nasal obstruction. On physical examination, a mucous-covered mass was noted in the left upper nasal vault medial to the turbinates. Computed tomography scanning and MR imaging confirmed the diagnosis of frontoethmoidal meningoencephalocele. Repair of the meningoencephalocele was accomplished using a combined neurosurgery and plastic surgery approach. Meningoencephalocele is a rare complication of subcranial midfacial advancement. The abnormal anatomy of the anterior cranial base in patients with syndromic craniosynostosi...
Volume 1: Additive Manufacturing; Bio and Sustainable Manufacturing, 2018
Precise and firm fixation of the cranium is critical during craniotomy and delicate brain neurosu... more Precise and firm fixation of the cranium is critical during craniotomy and delicate brain neurosurgery making head immobilization devices (HIDs) a staple instrument in brain neurosurgical operations today. However, despite their popularity, there is no standard procedure for their use and many complications arise from using HIDs in pediatric neurosurgery. In this paper, we identify biomechanical causes of complications and quantify risks in pin-type HIDs including clamping force selection, positioning and age effects. Based on our root cause analysis, we develop a framework to address the biomechanical factors that influence complications and understand the biomechanics of the clamping process. We develop an age-dependent finite element model (FEM) of a single pin on a cranial bone disc with the representative properties and skull thickness depending on age. This model can be utilized to reduce risk of complications by design as well as to provide recommendations for current practices.
While a noninvasive flow determination would be desirable in the diagnosis of cerebrospinal fluid... more While a noninvasive flow determination would be desirable in the diagnosis of cerebrospinal fluid shunt malfunction, existing studies have not yet defined a role for thermal flow detection. To evaluate a revised test protocol using a micropumper designed to transiently enhance flow during thermal testing to determine whether thermal detection of flow is associated with progression to shunt revision surgery. Eighty-two unique tests were performed in 71 shunts. The primary outcome, need for revision within 7 d of testing, was compared with results of micropumper-augmented thermal flow detection. Statistical analysis was based on blind interpretation of test results and raw temperature data recorded during testing. The test was sensitive (73%) and specific (68%) in predicting need for revision, with 5.6-fold higher probability of revision when flow was not detected. Negative predictive value in our sample was 94.2%. The probability of not requiring revision increased with increasing to...
Disseminated glioneuronal tumors of childhood are rare. We present a retrospective IRB-approved r... more Disseminated glioneuronal tumors of childhood are rare. We present a retrospective IRB-approved review of the clinical course and frequency of BRAF mutations in disseminated glioneuronal tumors at two institutions. Defining features of our cohort include diffuse leptomeningeal-spread, often with a discrete spinal cord nodule and oligodendroglioma-like histologic features. Patients were identified through a pathology database search of all cases with disseminated low-grade neoplasms with an oligodendroglioma-like component. De-identified clinical information was collected by chart review and all imaging was reviewed. We retrieved the results of targeted genomic analyses for alterations in BRAF. Ten patients (aged 2-14 years) were identified from the Dana-Farber/Boston Children's Hospital and the Royal Children's Hospital, Melbourne pathology databases. Nine patients received chemotherapy. Eight patients are alive, although three have had episodes of progressive disease. We identified genomic alterations affecting the MAPK pathway in six patients. One patient had a germline RAF1 mutation and a clinical diagnosis of cardio-facio-cutaneous syndrome. BRAF duplications were identified in four and BRAF V600E mutation was identified in one. These data support the presence of targetable genomic alterations in this disease.
To describe our experience and current management approach for congenital nasal neuroglial hetero... more To describe our experience and current management approach for congenital nasal neuroglial heterotopia (NGH) and encephaloceles. Retrospective chart review at a tertiary pediatric hospital from 1970 to 2013. Thirty patients met inclusion criteria: 21 NGH and nine encephaloceles. Data including demographics, pathology, imaging modality, surgical approach, resection extent, outcomes, and complications were analyzed. Fourteen NGH patients (67%) presented with an internal nasal mass and nasal obstruction. Three patients (14%) presented with an external nasal mass and four (19%) had a mixed lesion. Median age at surgery was 0.51 years (interquartile range 1.32 years). Thirteen (62%) had an intranasal endoscopic approach. Median operative time was 1.6 hours (interquartile range 1.2 hours), and there were no major complications. Nine patients with encephalocele were identified: six (67%) presented with transethmoidal encephaloceles, two (22%) presented with nasoethmoidal encephaloceles, an...
Despite clinical impressions that cognitive complaints are prominent in patients with a history o... more Despite clinical impressions that cognitive complaints are prominent in patients with a history of craniopharyngioma, formal neuropsychologic documentation is inconsistent. This study assessed everyday cognitive complaints and neuropsychologic test performance to evaluate the prevalence of problems and the relationship of these domains to one another in patients treated for craniopharyngioma in childhood or adolescence. Ten patients treated for craniopharyngioma completed measures of everyday cognitive function (Cognitive Failures Questionnaire, Rivermead Behavioural Memory Test) and a battery of standard neuropsychologic tests. The prevalence of problems was ascertained for each measure. Most patients demonstrated significant deficits in everyday memory (Cognitive Failures Questionnaire, 9/10 patients; Rivermead Behavioural Memory Test, 7/10 patients). Scores were within normal limits, however, for intelligence quotient, achievement, attention, verbal memory, and spatial working me...
Journal of nuclear medicine : official publication, Society of Nuclear Medicine, 1993
We previously found that 201TI SPECT is a highly specific agent for detection of metabolic activi... more We previously found that 201TI SPECT is a highly specific agent for detection of metabolic activity of childhood brain tumors. To compare the relative diagnostic accuracy of 201TI and a technetium-based tumor-avid agent, we have obtained SPECT in 19 children using 201TI (37-111 MBq) followed immediately by 99mTc-methoxyisobutylisonitrile (MIBI) (370-740 MBq) intravenously. Moderate to intense focal uptake of both tracers characterized true positive cases (n = 6). Lesion boundaries were better defined by MIBI. Uptake of MIBI by choroid plexus occurred despite pretreatment with potassium perchlorate (6 mg/kg) and complicated interpretation of deep/paraventricular lesions. Preliminary assessment indicated sensitivity approximately 67% (TI and MIBI); specificity approximately 91% (TI); approximately 100% (MIBI). Two tumors (medulloblastoma, dysgerminoma) were TI/MIBI nonavid. Semi-quantitative assessment of tracer uptake was made using a ratio of radioactivity in tumor-containing areas ...
Object Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric ... more Object Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric population and can be effectively treated by resection. The authors aimed to use imaging to evaluate how often tumors recurred and to determine if recurrences were associated with any clinical symptoms, along with the financial costs of imaging, in patients with radiographically proven gross-total resection (GTR) at Boston Children's Hospital. These data were assessed to propose guidelines regarding postoperative surveillance. Methods The authors performed a retrospective cohort analysis of the Pediatric Brain Tumor Program database from 1993 to 2003 to identify patients with glial or glioneuronal tumors initially evaluated at Boston Children's Hospital. Among the 888 patients evaluated for any type of brain tumor during this period, 67 patients had WHO Grade I glial or glioneuronal lesions with radiographically proven GTR and available follow-up data. The frequency and timing of...
Complications of Le Fort III midfacial advancement include cerebrospinal fluid (CSF) rhinorrhea, ... more Complications of Le Fort III midfacial advancement include cerebrospinal fluid (CSF) rhinorrhea, meningitis, and ocular and cerebral injury. This report reviews our experience with Le Fort III distraction, highlighting complications of dural disruption, and correlates occurrences with the anatomy of the cranial base and prior cranial procedures. This was a retrospective chart review of all patients who had Le Fort III subcranial osteotomies and midfacial advancement with distraction. Complications related to dural disruption were documented. The anatomy of the anterior cranial fossa was assessed with preoperative computed tomographic (CT) scans and compared with age- and sex-matched normal control scans, with particular attention paid to the anterior cranial fossa and fovea ethmoidalis (FE). On reconstructed midline sagittal images, the anterior cranial fossa was characterized as normal, sagging, or slanting. On reconstructed coronal images, immediately posterior to the plane of the lacrimal sac, each FE was characterized as normal, flattened, or depressed, relative to the adjacent cribriform plates. Thirty-one patients have had Le Fort III midfacial advancement with distraction at Children's Hospital Boston since 1995. Two patients underwent a second Le Fort III distraction. Two patients (6.5%) had postoperative CSF rhinorrhea, 2 had CSF leak at a pin site, and 1 patient had a late complication of meningoencephalocele. Twenty-six patients had 27 available preoperative three-dimensional reformatted CT scans. Seven of these had a normal sagittal anterior cranial fossa and normal coronal FE morphology. One of these 7 patients had a second CT at an older age showing development of bilateral FE flattening. Eleven patients had a sagging midline anterior cranial fossa including both patients who developed CSF rhinorrhea. Of these 11 patients, all had unilateral or bilateral flattening or depression of the FE, and 5 had abnormal slanting of the anterior cranial fossa. Eight patients had normal sagittal morphology, but bilateral or unilateral depression of the FE, including the patient who developed a meningoencephalocele. All patients with CSF leak had previously had a fronto-orbital advancement (FOA). Three of 4 patients with CSF leak did not have prior ventriculoperitoneal shunt placement. The patient with postoperative meningoencephalocele had prior FOA and shunt. We studied the abnormal position of the sagging or slanted anterior cranial base and depressed FE in patients with syndromic coronal synostosis. These findings may explain the risk for dural tear during osteotomies at the nasofrontal suture and superior-medial orbital wall. Attention to the morphology of the anterior cranial base, as seen on sagittal and coronal CT images, aids in preventing these complications. Patients who have a shunt are at lower risk for CSF leak; however, patients who have had an FOA are at higher risk.
Purpose Atypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primari... more Purpose Atypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primarily affecting young children, with a historic median survival ranging from 6 to 11 months. Based on a previous pilot series, a prospective multi-institutional trial was conducted for patients with newly diagnosed CNS ATRT. Patients and Methods Treatment was divided into five phases: preirradiation, chemoradiation, consolidation, maintenance, and continuation therapy. Intrathecal chemotherapy was administered, alternating intralumbar and intraventricular routes. Radiation therapy (RT) was prescribed, either focal (54 Gy) or craniospinal (36 Gy, plus primary boost), depending on age and extent of disease at diagnosis. Results Between 2004 and 2006, 25 patients were enrolled; 20 were eligible for evaluation. Median age at diagnosis was 26 months (range, 2.4 months to 19.5 years). Gross total resection of the primary tumor was achieved in 11 patients. Fourteen patients had M0 disease at diagn...
BACKGROUND Intraoperative electrocorticography (ECoG) has been utilized in patients with tumor-as... more BACKGROUND Intraoperative electrocorticography (ECoG) has been utilized in patients with tumor-associated seizures; however, its effectiveness for seizure control remains controversial. OBJECTIVE To evaluate clinical outcomes in pediatric patients undergoing lesionectomy with or without ECoG. METHODS Patients undergoing brain tumor resection at Boston Children's Hospital were examined retrospectively (2005-2014). Inclusion criteria involved diagnosis of a supratentorial tumor, ≥2 unequivocal seizures, and ≥6 mo follow-up. Patients with isolated cortical dysplasia or posterior fossa tumors were excluded. Logistic regression models evaluated predictors of ECoG use, and the impact of ECoG, gross total resection, and focal cortical dysplasia with tumors on seizure freedom by Engel Class and anti-epileptic drug use (AED). RESULTS A total of 119 pediatric patients were included (n = 69 males, 58%; median age, 11.3 yr). Forty-one patients (34.5%) had ECoG-guided surgery. Preoperative s...
Complications of Le Fort III midfacial advancement include CSF rhinorrhea, meningitis, and ocular... more Complications of Le Fort III midfacial advancement include CSF rhinorrhea, meningitis, and ocular and cerebral injury. This report reviews the anatomy of the Le Fort III osteotomies and their relevance to the unusual complication of meningoencephalocele. In this report, a young male patient with Crouzon syndrome underwent subcranial midfacial advancement at the age of 10 years for obstructive sleep apnea and ocular exposure. He presented 4 years later complaining of nasal obstruction. On physical examination, a mucous-covered mass was noted in the left upper nasal vault medial to the turbinates. Computed tomography scanning and MR imaging confirmed the diagnosis of frontoethmoidal meningoencephalocele. Repair of the meningoencephalocele was accomplished using a combined neurosurgery and plastic surgery approach. Meningoencephalocele is a rare complication of subcranial midfacial advancement. The abnormal anatomy of the anterior cranial base in patients with syndromic craniosynostosi...
Volume 1: Additive Manufacturing; Bio and Sustainable Manufacturing, 2018
Precise and firm fixation of the cranium is critical during craniotomy and delicate brain neurosu... more Precise and firm fixation of the cranium is critical during craniotomy and delicate brain neurosurgery making head immobilization devices (HIDs) a staple instrument in brain neurosurgical operations today. However, despite their popularity, there is no standard procedure for their use and many complications arise from using HIDs in pediatric neurosurgery. In this paper, we identify biomechanical causes of complications and quantify risks in pin-type HIDs including clamping force selection, positioning and age effects. Based on our root cause analysis, we develop a framework to address the biomechanical factors that influence complications and understand the biomechanics of the clamping process. We develop an age-dependent finite element model (FEM) of a single pin on a cranial bone disc with the representative properties and skull thickness depending on age. This model can be utilized to reduce risk of complications by design as well as to provide recommendations for current practices.
While a noninvasive flow determination would be desirable in the diagnosis of cerebrospinal fluid... more While a noninvasive flow determination would be desirable in the diagnosis of cerebrospinal fluid shunt malfunction, existing studies have not yet defined a role for thermal flow detection. To evaluate a revised test protocol using a micropumper designed to transiently enhance flow during thermal testing to determine whether thermal detection of flow is associated with progression to shunt revision surgery. Eighty-two unique tests were performed in 71 shunts. The primary outcome, need for revision within 7 d of testing, was compared with results of micropumper-augmented thermal flow detection. Statistical analysis was based on blind interpretation of test results and raw temperature data recorded during testing. The test was sensitive (73%) and specific (68%) in predicting need for revision, with 5.6-fold higher probability of revision when flow was not detected. Negative predictive value in our sample was 94.2%. The probability of not requiring revision increased with increasing to...
Disseminated glioneuronal tumors of childhood are rare. We present a retrospective IRB-approved r... more Disseminated glioneuronal tumors of childhood are rare. We present a retrospective IRB-approved review of the clinical course and frequency of BRAF mutations in disseminated glioneuronal tumors at two institutions. Defining features of our cohort include diffuse leptomeningeal-spread, often with a discrete spinal cord nodule and oligodendroglioma-like histologic features. Patients were identified through a pathology database search of all cases with disseminated low-grade neoplasms with an oligodendroglioma-like component. De-identified clinical information was collected by chart review and all imaging was reviewed. We retrieved the results of targeted genomic analyses for alterations in BRAF. Ten patients (aged 2-14 years) were identified from the Dana-Farber/Boston Children's Hospital and the Royal Children's Hospital, Melbourne pathology databases. Nine patients received chemotherapy. Eight patients are alive, although three have had episodes of progressive disease. We identified genomic alterations affecting the MAPK pathway in six patients. One patient had a germline RAF1 mutation and a clinical diagnosis of cardio-facio-cutaneous syndrome. BRAF duplications were identified in four and BRAF V600E mutation was identified in one. These data support the presence of targetable genomic alterations in this disease.
To describe our experience and current management approach for congenital nasal neuroglial hetero... more To describe our experience and current management approach for congenital nasal neuroglial heterotopia (NGH) and encephaloceles. Retrospective chart review at a tertiary pediatric hospital from 1970 to 2013. Thirty patients met inclusion criteria: 21 NGH and nine encephaloceles. Data including demographics, pathology, imaging modality, surgical approach, resection extent, outcomes, and complications were analyzed. Fourteen NGH patients (67%) presented with an internal nasal mass and nasal obstruction. Three patients (14%) presented with an external nasal mass and four (19%) had a mixed lesion. Median age at surgery was 0.51 years (interquartile range 1.32 years). Thirteen (62%) had an intranasal endoscopic approach. Median operative time was 1.6 hours (interquartile range 1.2 hours), and there were no major complications. Nine patients with encephalocele were identified: six (67%) presented with transethmoidal encephaloceles, two (22%) presented with nasoethmoidal encephaloceles, an...
Despite clinical impressions that cognitive complaints are prominent in patients with a history o... more Despite clinical impressions that cognitive complaints are prominent in patients with a history of craniopharyngioma, formal neuropsychologic documentation is inconsistent. This study assessed everyday cognitive complaints and neuropsychologic test performance to evaluate the prevalence of problems and the relationship of these domains to one another in patients treated for craniopharyngioma in childhood or adolescence. Ten patients treated for craniopharyngioma completed measures of everyday cognitive function (Cognitive Failures Questionnaire, Rivermead Behavioural Memory Test) and a battery of standard neuropsychologic tests. The prevalence of problems was ascertained for each measure. Most patients demonstrated significant deficits in everyday memory (Cognitive Failures Questionnaire, 9/10 patients; Rivermead Behavioural Memory Test, 7/10 patients). Scores were within normal limits, however, for intelligence quotient, achievement, attention, verbal memory, and spatial working me...
Journal of nuclear medicine : official publication, Society of Nuclear Medicine, 1993
We previously found that 201TI SPECT is a highly specific agent for detection of metabolic activi... more We previously found that 201TI SPECT is a highly specific agent for detection of metabolic activity of childhood brain tumors. To compare the relative diagnostic accuracy of 201TI and a technetium-based tumor-avid agent, we have obtained SPECT in 19 children using 201TI (37-111 MBq) followed immediately by 99mTc-methoxyisobutylisonitrile (MIBI) (370-740 MBq) intravenously. Moderate to intense focal uptake of both tracers characterized true positive cases (n = 6). Lesion boundaries were better defined by MIBI. Uptake of MIBI by choroid plexus occurred despite pretreatment with potassium perchlorate (6 mg/kg) and complicated interpretation of deep/paraventricular lesions. Preliminary assessment indicated sensitivity approximately 67% (TI and MIBI); specificity approximately 91% (TI); approximately 100% (MIBI). Two tumors (medulloblastoma, dysgerminoma) were TI/MIBI nonavid. Semi-quantitative assessment of tracer uptake was made using a ratio of radioactivity in tumor-containing areas ...
Object Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric ... more Object Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric population and can be effectively treated by resection. The authors aimed to use imaging to evaluate how often tumors recurred and to determine if recurrences were associated with any clinical symptoms, along with the financial costs of imaging, in patients with radiographically proven gross-total resection (GTR) at Boston Children's Hospital. These data were assessed to propose guidelines regarding postoperative surveillance. Methods The authors performed a retrospective cohort analysis of the Pediatric Brain Tumor Program database from 1993 to 2003 to identify patients with glial or glioneuronal tumors initially evaluated at Boston Children's Hospital. Among the 888 patients evaluated for any type of brain tumor during this period, 67 patients had WHO Grade I glial or glioneuronal lesions with radiographically proven GTR and available follow-up data. The frequency and timing of...
Complications of Le Fort III midfacial advancement include cerebrospinal fluid (CSF) rhinorrhea, ... more Complications of Le Fort III midfacial advancement include cerebrospinal fluid (CSF) rhinorrhea, meningitis, and ocular and cerebral injury. This report reviews our experience with Le Fort III distraction, highlighting complications of dural disruption, and correlates occurrences with the anatomy of the cranial base and prior cranial procedures. This was a retrospective chart review of all patients who had Le Fort III subcranial osteotomies and midfacial advancement with distraction. Complications related to dural disruption were documented. The anatomy of the anterior cranial fossa was assessed with preoperative computed tomographic (CT) scans and compared with age- and sex-matched normal control scans, with particular attention paid to the anterior cranial fossa and fovea ethmoidalis (FE). On reconstructed midline sagittal images, the anterior cranial fossa was characterized as normal, sagging, or slanting. On reconstructed coronal images, immediately posterior to the plane of the lacrimal sac, each FE was characterized as normal, flattened, or depressed, relative to the adjacent cribriform plates. Thirty-one patients have had Le Fort III midfacial advancement with distraction at Children's Hospital Boston since 1995. Two patients underwent a second Le Fort III distraction. Two patients (6.5%) had postoperative CSF rhinorrhea, 2 had CSF leak at a pin site, and 1 patient had a late complication of meningoencephalocele. Twenty-six patients had 27 available preoperative three-dimensional reformatted CT scans. Seven of these had a normal sagittal anterior cranial fossa and normal coronal FE morphology. One of these 7 patients had a second CT at an older age showing development of bilateral FE flattening. Eleven patients had a sagging midline anterior cranial fossa including both patients who developed CSF rhinorrhea. Of these 11 patients, all had unilateral or bilateral flattening or depression of the FE, and 5 had abnormal slanting of the anterior cranial fossa. Eight patients had normal sagittal morphology, but bilateral or unilateral depression of the FE, including the patient who developed a meningoencephalocele. All patients with CSF leak had previously had a fronto-orbital advancement (FOA). Three of 4 patients with CSF leak did not have prior ventriculoperitoneal shunt placement. The patient with postoperative meningoencephalocele had prior FOA and shunt. We studied the abnormal position of the sagging or slanted anterior cranial base and depressed FE in patients with syndromic coronal synostosis. These findings may explain the risk for dural tear during osteotomies at the nasofrontal suture and superior-medial orbital wall. Attention to the morphology of the anterior cranial base, as seen on sagittal and coronal CT images, aids in preventing these complications. Patients who have a shunt are at lower risk for CSF leak; however, patients who have had an FOA are at higher risk.
Purpose Atypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primari... more Purpose Atypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primarily affecting young children, with a historic median survival ranging from 6 to 11 months. Based on a previous pilot series, a prospective multi-institutional trial was conducted for patients with newly diagnosed CNS ATRT. Patients and Methods Treatment was divided into five phases: preirradiation, chemoradiation, consolidation, maintenance, and continuation therapy. Intrathecal chemotherapy was administered, alternating intralumbar and intraventricular routes. Radiation therapy (RT) was prescribed, either focal (54 Gy) or craniospinal (36 Gy, plus primary boost), depending on age and extent of disease at diagnosis. Results Between 2004 and 2006, 25 patients were enrolled; 20 were eligible for evaluation. Median age at diagnosis was 26 months (range, 2.4 months to 19.5 years). Gross total resection of the primary tumor was achieved in 11 patients. Fourteen patients had M0 disease at diagn...
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