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Manish Kuchakulla, Ashish H. Shah, Valerie Armstrong, Sarah Jernigan, Sanjiv Bhatia, and Toba N. Niazi

OBJECTIVE

Carotid body tumors (CBTs), extraadrenal paragangliomas, are extremely rare neoplasms in children that often require multimodal surgical treatment, including preoperative anesthesia workup, embolization, and resection. With only a few cases reported in the pediatric literature, treatment paradigms and surgical morbidity are loosely defined, especially when carotid artery infiltration is noted. Here, the authors report two cases of pediatric CBT and provide the results of a systematic review of the literature.

METHODS

The study was divided into two sections. First, the authors conducted a retrospective review of our series of pediatric CBT patients and screened for patients with evidence of a CBT over the last 10 years (2007–2017) at a single tertiary referral pediatric hospital. Second, they conducted a systematic review, according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, of all reported cases of pediatric CBTs to determine the characteristics (tumor size, vascularity, symptomatology), treatment paradigms, and complications.

RESULTS

In the systematic review (n = 21 patients [includes 19 cases found in the literature and 2 from the authors’ series]), the mean age at diagnosis was 11.8 years. The most common presenting symptoms were palpable neck mass (62%), cranial nerve palsies (33%), cough or dysphagia (14%), and neck pain (19%). Metastasis occurred only in 5% of patients, and 19% of cases were recurrent lesions. Only 10% of patients presented with elevated catecholamines and associated sympathetic involvement. Preoperative embolization was utilized in 24% of patients (external carotid artery in 4 and external carotid artery and vertebral artery in 1). Cranial nerve palsies (cranial nerve VII [n = 1], IX [n = 1], X [n = 4], XI [n = 1], and XII [n = 3]) were the most common cause of surgical morbidity (33% of cases). The patients in the authors’ illustrative cases underwent preoperative embolization and balloon test occlusion followed by resection, and both patients suffered from transient Horner’s syndrome after embolization.

CONCLUSIONS

Surgical management of CBTs requires an extensive preoperative workup, anesthesia, and multimodal surgical management. Due to a potentially high rate of surgical morbidity and vascularity, balloon test occlusion with embolization may be necessary in select patients prior to resection. Careful thorough preoperative counseling is vital to preparing families for the intensive management of these children.

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Ashish H. Shah, George M. Ibrahim, Jun Sasaki, John Ragheb, Sanjiv Bhatia, and Toba N. Niazi

OBJECTIVE

Although endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has gained increasing prominence in the management of hydrocephalus caused by intraventricular hemorrhage of prematurity, the rates of long-term shunt independence remain low. Furthermore, limited evidence is available to identify infants who might benefit from the procedure. The authors tested the hypothesis that elevated venous pressure that results from comorbid cardiac disease might predispose patients to ETV/CPC failure and shunt dependence.

METHODS

A retrospective analysis was performed on a consecutive series of 48 infants with hydrocephalus who underwent ETV/CPC and also underwent preoperative echocardiography between 2007 and 2014. Comorbid cardiac abnormalities that are known to result in elevated right heart pressure were reviewed. Associations between ETV/CPC success and the presence of pulmonary hypertension, right ventricular hypertrophy, left-to-right shunting, ventricular septal defect, or patent ductus arteriosus were determined using multivariate logistic regression analysis.

RESULTS

Of the 48 children who met the inclusion criteria, ETV/CPC failed in 31 (65%). In univariate analysis, no single echocardiogram abnormality was associated with shunt failure, but the presence of 2 or more concurrent echocardiogram abnormalities was associated with ETV/CPC failure (17 [85%] of 20 vs 14 [50%] of 28, respectively; p = 0.018). In multivariate logistic regression analysis, when the authors adjusted for the child’s ETV success score, the presence of 2 abnormalities remained independently associated with poor outcome (2 or more echocardiogram abnormalities, OR 0.13, 95% CI 0.01–0.7, p = 0.032; ETV success score, OR 1.1, 95% CI 1–1.2, p = 0.05).

CONCLUSIONS

In this study, cardiac abnormalities were inversely associated with the success of ETV/CPC in infants with hydrocephalus of prematurity. ETV/CPC might not be as efficacious in patients with significant cardiac anomalies. These results provide a basis for future efforts to stratify surgical candidacy for ETV/CPC on the basis of comorbid abnormalities. Proper cardiac physiological pressure monitoring might help elucidate the relationship between cardiac abnormalities and hydrocephalus.

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Aria Fallah, Alexander G. Weil, Kyle Juraschka, George M. Ibrahim, Anthony C. Wang, Louis Crevier, Chi-hong Tseng, Abhaya V. Kulkarni, John Ragheb, and Sanjiv Bhatia

OBJECTIVE

Combined endoscopic third ventriculostomy (ETC) and choroid plexus cauterization (CPC)—ETV/CPC— is being investigated to increase the rate of shunt independence in infants with hydrocephalus. The degree of CPC necessary to achieve improved rates of shunt independence is currently unknown.

METHODS

Using data from a single-center, retrospective, observational cohort study involving patients who underwent ETV/CPC for treatment of infantile hydrocephalus, comparative statistical analyses were performed to detect a difference in need for subsequent CSF diversion procedure in patients undergoing partial CPC (describes unilateral CPC or bilateral CPC that only extended from the foramen of Monro [FM] to the atrium on one side) or subtotal CPC (describes CPC extending from the FM to the posterior temporal horn bilaterally) using a rigid neuroendoscope. Propensity scores for extent of CPC were calculated using age and etiology. Propensity scores were used to perform 1) case-matching comparisons and 2) Cox multivariable regression, adjusting for propensity score in the unmatched cohort. Cox multivariable regression adjusting for age and etiology, but not propensity score was also performed as a third statistical technique.

RESULTS

Eighty-four patients who underwent ETV/CPC had sufficient data to be included in the analysis. Subtotal CPC was performed in 58 patients (69%) and partial CPC in 26 (31%). The ETV/CPC success rates at 6 and 12 months, respectively, were 49% and 41% for patients undergoing subtotal CPC and 35% and 31% for those undergoing partial CPC. Cox multivariate regression in a 48-patient cohort case-matched by propensity score demonstrated no added effect of increased extent of CPC on ETV/CPC survival (HR 0.868, 95% CI 0.422–1.789, p = 0.702). Cox multivariate regression including all patients, with adjustment for propensity score, demonstrated no effect of extent of CPC on ETV/CPC survival (HR 0.845, 95% CI 0.462–1.548, p = 0.586). Cox multivariate regression including all patients, with adjustment for age and etiology, but not propensity score, demonstrated no effect of extent of CPC on ETV/CPC survival (HR 0.908, 95% CI 0.495–1.664, p = 0.755).

CONCLUSIONS

Using multiple comparative statistical analyses, no difference in need for subsequent CSF diversion procedure was detected between patients in this cohort who underwent partial versus subtotal CPC. Further investigation regarding whether there is truly no difference between partial versus subtotal extent of CPC in larger patient populations and whether further gain in CPC success can be achieved with complete CPC is warranted.

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Walter J. Jermakowicz, Alexander G. Weil, Artyom Vlasenko, Sanjiv Bhatia, and Toba N. Niazi

Cognard Type V dural arteriovenous fistulas (dAVFs) are a unique type of cranial vascular malformation characterized by congestion of the perimedullary venous system that may lead to devastating spinal cord pathology if left untreated. The authors present the first known case of a pediatric patient diagnosed with a Type V dAVF. A 14-year-old girl presented with a 3-week history of slowly progressive unilateral leg weakness that quickly progressed to bilateral leg paralysis, sphincter dysfunction, and complete sensory loss the day of her presentation. MRI revealed an extensive T2 signal change in the cervical spine and tortuous perimedullary veins along the entire length of the cord. An emergency cranial angiogram showed a Type V dAVF fed by the posterior meningeal artery with drainage into the perimedullary veins of the cervical spine. The fistula was not amenable to embolization because vascular access was difficult; therefore, the patient underwent urgent suboccipital craniotomy and ligation of the arterialized venous drainage from the fistula. The patient’s clinical course immediately reversed; she had a complete recovery over the course of a year, and she remains asymptomatic at the 2-year follow-up. This report adds to a growing body of evidence that describes the diverse and unpredictable nature of Type V dAVFs and highlights the need to obtain a cranial angiogram in pediatric patients with unexplained myelopathy and cervical cord T2 signal change on MRI.

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Alexander G. Weil, Aria Fallah, Evan C. Lewis, and Sanjiv Bhatia

OBJECTIVE

Insular lobe epilepsy (ILE) is an under-recognized cause of extratemporal epilepsy and explains some epilepsy surgery failures in children with drug-resistant epilepsy. The diagnosis of ILE usually requires invasive investigation with insular sampling; however, the location of the insula below the opercula and the dense middle cerebral artery vasculature renders its sampling challenging. Several techniques have been described, ranging from open direct placement of orthogonal subpial depth and strip electrodes through a craniotomy to frame-based stereotactic placement of orthogonal or oblique electrodes using stereo-electroencephalography principles. The authors describe an alternative method for sampling the insula, which involves placing insular depth electrodes along the long axis of the insula through the insular apex following dissection of the sylvian fissure in conjunction with subdural electrodes over the lateral hemispheric/opercular region. The authors report the feasibility, advantages, disadvantages, and role of this approach in investigating pediatric insular-opercular refractory epilepsy.

METHODS

The authors performed a retrospective analysis of all children (< 18 years old) who underwent invasive intracranial studies involving the insula between 2002 and 2015.

RESULTS

Eleven patients were included in the study (5 boys). The mean age at surgery was 7.6 years (range 0.5–16 years). All patients had drug-resistant epilepsy as defined by the International League Against Epilepsy and underwent comprehensive noninvasive epilepsy surgery workup. Intracranial monitoring was performed in all patients using 1 parasagittal insular electrode (1 patient had 2 electrodes) in addition to subdural grids and strips tailored to the suspected epileptogenic zone. In 10 patients, extraoperative monitoring was used; in 1 patient, intraoperative electrocorticography was used alone without extraoperative monitoring. The mean number of insular contacts was 6.8 (range 4–8), and the mean number of fronto-parieto-temporal hemispheric contacts was 61.7 (range 40–92). There were no complications related to placement of these depth electrodes. All 11 patients underwent subsequent resective surgery involving the insula.

CONCLUSIONS

Parasagittal transinsular apex depth electrode placement is a feasible alternative to orthogonally placed open or oblique-placed stereotactic methodologies. This method is safe and best suited for suspected unilateral cases with a possible extensive insular-opercular epileptogenic zone.

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Alexander G. Weil, Ngoc Minh D. Le, Prasanna Jayakar, Trevor Resnick, Ian Miller, Aria Fallah, Michael Duchowny, and Sanjiv Bhatia

OBJECTIVE

Seizure onset in the insular cortex as a cause of refractory epilepsy is underrepresented in the pediatric population, possibly due to difficulties localizing seizure onset in deep anatomical structures and limited surgical access to the insula, a complex anatomical structure with a rich overlying vascular network. Insular seizure semiology may mimic frontal, temporal, or parietal lobe semiology, resulting in false localization, incomplete resection, and poor outcome.

METHODS

The authors retrospectively reviewed the records of all pediatric patients who underwent insular cortical resections for intractable epilepsy at Miami Children's Hospital from 2009 to 2015. Presurgical evaluation included video electroencephalography monitoring and anatomical/functional neuroimaging. All patients underwent excisional procedures utilizing intraoperative electrocorticography or extraoperative subdural/depth electrode recording.

RESULTS

Thirteen children (age range 6 months–16 years) with intractable focal epilepsy underwent insular-opercular resection. Seven children described symptoms that were suggestive of insular seizure origin. Discharges on scalp EEG revealed wide fields. Four patients were MRI negative (i.e., there were no insular or brain abnormalities on MRI), 4 demonstrated insular signal abnormalities, and 5 had extrainsular abnormalities. Ten patients had insular involvement on PET/SPECT. All patients underwent invasive investigation with insular sampling; in 2 patients resection was based on intraoperative electrocorticography, whereas 11 underwent surgery after invasive EEG monitoring with extraoperative monitoring. Four patients required an extended insular resection after a failed initial surgery. Postoperatively, 2 patients had transient hemiplegia. No patients had new permanent neurological deficits. At the most recent follow-up (mean 43.8 months), 9 (69%) children were seizure free and 1 had greater than 90% seizure reduction.

CONCLUSIONS

Primary insular seizure origin should be considered in children with treatment-resistant focal seizures that are believed to arise within the perisylvian region based on semiology, widespread electrical field on scalp EEG, or insular abnormality on anatomical/functional neuroimaging. There is a reasonable chance of seizure freedom in this group of patients, and the surgical risks are low.

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Alexander G. Weil, Aria Fallah, Parthasarathi Chamiraju, John Ragheb, and Sanjiv Bhatia

OBJECT

Combining endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has been shown to improve the success rate compared with ETV alone in infants (less than 24 months) with hydrocephalus who were treated in developing countries. The authors sought to evaluate the safety and efficacy of this procedure, using a rigid neuroendoscope, in a single North American center, and to assess whether the ETV success score (ETVSS), the CURE Children’s Hospital of Uganda ETVSS (CCHU ETVSS), and other pre- and intraoperative variables could predict success.

METHODS

The authors performed a retrospective review of consecutive ETV/CPC procedures performed using a rigid neuroendoscope to treat infantile hydrocephalus. The infants underwent the procedure at Miami Children’s Hospital between January 2007 and 2014, with at least one postoperative follow-up. Duration of follow-up or time to failure of ETV/CPC, the primary outcome measure, was documented. A repeat CSF diversion procedure or death was considered as a failure of ETV/CPC. The time to event was measured using a Kaplan-Meier analysis. The authors analyzed ETVSS, CCHU ETVSS, and pre- and intraoperative variables to determine their suitability to predict success.

RESULTS

Eighty-five patients (45 boys) with a mean age of 4.3 months (range 1 day to 20 months) underwent ETV/CPC. Etiology included intraventricular hemorrhage of prematurity in 44 patients (51.7%), myelomeningocele (MMC) in 7 (8.2%), congenital aqueductal stenosis in 12 (14.1%), congenital communicating hydrocephalus in 6 (7.1%), Dandy-Walker complex in 6 (7.1%), postinfectious hydrocephalus in 5 (5.8%), and other cause in 5 (5.8%). Six procedure-related complications occurred in 5 (5.8%) patients, including 2 hygromas, 1 CSF leak, and 3 infections. There were 3 mortalities in this cohort. ETV/CPC was successful in 42.1%, 37.7%, and 36.8% of patients at 6, 12, and 24 months follow-up, respectively. The median (95% CI) time to ETV/CPC failure was 4.0 months (0.9–7.1 months). In univariate analyses, both the ETVSS (hazard ratio [HR] 1.03; 95% CI 1.01–1.05; p = 0.004) and CCHU ETVSS (HR 1.48; 95% CI 1.04–2.09; p = .028) were predictive of outcome following ETV/CPC. In multivariate analysis, the presence of prepontine scarring was associated with ETV/CPC failure (HR 0.34; 95% CI 0.19–0.63; p < 0.001). Other variables, such as radiological criteria (prepontine interval, prepontine space, aqueductal stenosis, Third Ventricular Morphology Index) and intraoperative findings (ventriculostomy pulsations, extent of CPC), did not predict outcome.

CONCLUSIONS

ETV/CPC is a feasible alternative to ETV and ventriculoperitoneal shunt in infants with hydrocephalus. Both the ETVSS and CCHU ETVSS predicted success following ETV/CPC in this single-center North American cohort of patients.

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Aria Fallah, Alexander G. Weil, Samir Sur, Ian Miller, Prasanna Jayakar, Glenn Morrison, Sanjiv Bhatia, and John Ragheb

OBJECT

Pediatric brain tumors may be associated with medically intractable epilepsy for which surgery is indicated. The authors sought to evaluate the efficacy of epilepsy surgery for seizure control in pediatric patients with brain tumors.

METHODS

The authors performed a retrospective review of consecutive patients undergoing resective epilepsy surgery related to pediatric brain tumors at Miami Children’s Hospital between June 1986 and June 2014. Time-to-event analysis for seizure recurrence was performed; an “event” was defined as any seizures that occurred following resective epilepsy surgery, not including seizures and auras in the 1st postoperative week. The authors analyzed several preoperative variables to determine their suitability to predict seizure recurrence following surgery.

RESULTS

Eighty-four patients (47 males) with a mean age (± standard deviation) of 8.7 ± 5.5 years (range 0.5–21.6 years) were included. The study included 39 (46%) patients with gliomas, 20 (24%) with dysembryoplastic neuroepithelial tumors (DNETs), 14 (17%) with gangliogliomas, and 11 (13%) with other etiologies. Among the patients with gliomas, 18 were classified with low-grade glioma, 5 had oligodendroglioma, 6 had uncategorized astrocytoma, 3 had pilocytic astrocytoma, 3 had pleomorphic xanthoastrocytoma, 3 had glioblastoma, and 1 had gliomatosis cerebri. Seventy-nine (94.0%) resections were guided by intraoperative electrocorticography (ECoG). The mean time (± standard deviation) to seizure recurrence was 81.8 ± 6.3 months. Engel Class I outcome was achieved in 66 (78%) and 63 (75%) patients at 1 and 2 years’ follow-up, respectively. Patients with ganglioglioma demonstrated the highest probability of long-term seizure freedom, followed by patients with DNETs and gliomas. In univariate analyses, temporal location (HR 1.75, 95% CI 0.26–1.27, p = 0.171) and completeness of resection (HR 1.69, 95% CI 0.77–3.74, p = 0.191) demonstrated a trend toward a longer duration of seizure freedom.

CONCLUSIONS

ECoG-guided epilepsy surgery for pediatric patients with brain tumors is highly effective. Tumors located in the temporal lobe and those in which a complete ECoG-guided resection is performed may result in a greater likelihood of long-term seizure freedom.

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Anna Lo Presti, Alexander G. Weil, Aria Fallah, Eric C. Peterson, Toba N. Niazi, and Sanjiv Bhatia

Sickle cell disease (SCD) is an autosomal recessive hematological disorder, characterized by sickling of the red blood cells in response to a hypoxic stress and vaso-occlusive crises. It is associated with moyamoya-like changes on cerebral angiographic imaging in 43% of patients. Cerebral aneurysms, arteriovenous malformations, and dural arteriovenous fistulas (AVFs) have been described in association with SCD and moyamoya disease. However, the description of a pial AVF (pAVF) in a patient with SCD and/or moyamoya formation has not yet been reported. The authors present the case of a 15-year-old boy with SCD-associated moyamoya disease harboring a pAVF who developed a de novo venous aneurysm 8 months after undergoing indirect superficial temporal artery-middle cerebral artery (MCA) bypass that was complicated by bilateral ischemia of the MCA territory. The pAVF was successfully treated with transarterial embolization using Onyx. The authors describe the possible pathophysiological mechanisms and management strategies for this rare occurrence.

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

OBJECT

Operations on tumors of the posteromedial temporal (PMT) region, that is, on those arising from the posterior parahippocampal, fusiform, and lingual gyri, are challenging to perform because of the deep-seated location of these tumors between critical cisternal neurovascular structures and the adjacent temporal and occipital cortexes. Traditional surgical approaches require temporal or occipital transgression, retraction, or venous sacrifice. These approaches may result in unintended complications that should be avoided. To avoid these complications, the supracerebellar-transtentorial (SCTT) approach to this region has been used as an effective alternative treatment in adult patients. The SCTT approach uses a sitting position that offers a direct route to the posterior fusiform and lingual gyri of the temporal lobe. The authors report the feasibility, safety, and efficacy of this approach, using a modified lateral park-bench position in a small cohort of pediatric patients.

METHODS

The authors carried out a retrospective case review of 5 consecutive patients undergoing a paramedian SCTT approach between 2009 and 2014 at the authors' institution.

RESULTS

The SCTT approach in the park-bench position was used in 3 boys and 2 girls with a mean age of 7.8 years (range 13 months to 16 years). All patients presented with a seizure disorder related to a tumor in a PMT region involving the parahippocampal and fusiform gyri of the left (n = 3) or right (n = 2) temporal lobe. No procedure-related complications were observed. Gross-total resection and control of seizures were achieved in all cases. Tumor classes and types included 1 Grade II astrocytoma, 1 pleomorphic xanthoastrocytoma, 1 ganglioglioma, and 2 glioneural tumors. None of the tumors had recurred by the mean follow-up of 22 months (range 1–48 months). Outcomes of epileptic seizures were excellent, with seizure symptoms in all 5 patients scoring in Engel Class IA.

CONCLUSIONS

The SCTT approach represents a viable option when resecting tumors in this region, providing a reasonable working corridor and low morbidity. The authors' experience in a cohort of pediatric patients demonstrates that complete resection of the lesions in this location is feasible and is safe when involving an approach that involves using a park-bench lateral positioning.