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Recent advances in the neurosurgical treatment of pediatric epilepsy

JNSPG 75th Anniversary Invited Review Article

Jarod L. Roland and Matthew D. Smyth

The field of epilepsy surgery has seen tremendous growth in recent years. Innovative new devices have driven much of this growth, but some has been driven by revisions of existing products. Devices have also helped to rejuvenate existing procedures, as in the case of robotic assistance for electrode placement for stereo-electroencephalography, and these devices have brought significant attention along with their introduction. Other devices, such as responsive neurostimulators or laser interstitial thermal therapy systems, have introduced novel treatment modalities and broadened the surgical indications. Collectively, these advances are rapidly changing much of the landscape in the world of pediatric neurosurgery for medically refractory epilepsy. The foundations for indications for neurosurgical intervention are well supported in strong research data, which has also been expanded in recent years. In this article, the authors review advances in the neurosurgical treatment of pediatric epilepsy, beginning with trials that have repeatedly demonstrated the value of neurosurgical procedures for medically refractory epilepsy and following with several recent advances that are largely focused on less-invasive intervention.

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Syed Hassan A. Akbari, Christine E. Averill, Jarod L. Roland, Rachel Orscheln, and Jennifer Strahle

Bartonella henselaeis a gram-negative bacillus implicated in cat-scratch disease. Cat-scratch disease is usually self-limiting and results in local lymphadenopathy. In rare circumstances, patients may develop endocarditis, neuroretinitis, or osteomyelitis. Osteomyelitis of the cervical spine is exceedingly rare, especially in the pediatric population, and to date there have been only 4 previously reported cases of cervical spine osteomyelitis caused byB. henselae, all of which were treated surgically. In this article, the authors report the case of a 7-year-old boy who presented with neck swelling and was found to have a C2–4 paravertebralB. henselaeabscess with osteomyelitis of C-3 and epidural extension. To the authors’ knowledge, this represents the first case in the literature of a cervical spineB. henselaeinfection managed conservatively.

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Jarod L. Roland, Syed Hassan A. Akbari, Afshin Salehi, and Matthew D. Smyth

OBJECTIVE

Corpus callosotomy is a palliative procedure that is effective at reducing seizure burden in patients with medically refractory epilepsy. The procedure is traditionally performed via open craniotomy with interhemispheric microdissection to divide the corpus callosum. Concerns for morbidity associated with craniotomy can be a deterrent to patients, families, and referring physicians for surgical treatment of epilepsy. Laser interstitial thermal therapy (LITT) is a less invasive procedure that has been widely adopted in neurosurgery for the treatment of tumors. In this study, the authors investigated LITT as a less invasive approach for corpus callosotomy.

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The authors retrospectively reviewed all patients treated for medically refractory epilepsy by corpus callosotomy, either partial or completion, with LITT. Chart records were analyzed to summarize procedural metrics, length of stay, adverse events, seizure outcomes, and time to follow-up. In select cases, resting-state functional MRI was performed to qualitatively support effective functional disconnection of the cerebral hemispheres.

RESULTS

Ten patients underwent 11 LITT procedures. Five patients received an anterior two-thirds LITT callosotomy as their first procedure. One patient returned after LITT partial callosotomy for completion of callosotomy by LITT. The median hospital stay was 2 days (IQR 1.5–3 days), and the mean follow-up time was 1.0 year (range 1 month to 2.86 years). Functional outcomes are similar to those of open callosotomy, with the greatest effect in patients with a significant component of drop attacks in their seizure semiology. One patient achieved an Engel class II outcome after anterior two-thirds callosotomy resulting in only rare seizures at the 18-month follow-up. Four others were in Engel class III and 5 were Engel class IV. Hemorrhage occurred in 1 patient at the time of removal of the laser fiber, which was placed through the bone flap of a prior open partial callosotomy.

CONCLUSIONS

LITT appears to be a safe and effective means for performing corpus callosotomy. Additional data are needed to confirm equipoise between open craniotomy and LITT for corpus callosotomy.

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Matthew R. Reynolds, Jarod L. Roland, Ashwin A. Kamath, DeWitte T. Cross III, and Ralph G. Dacey Jr.

Perforating arteries rarely project from the fundus of an aneurysm. We present the case of a 35-year-old woman who was found to have a right posterior communicating artery (PCOM) aneurysm via catheter angiography. Superselective microcatheter angiography revealed that perforating arteries arose from the aneurysm fundus that supplied the anterolateral thalamus. Microsurgical exploration confirmed several small perforating arteries arising from the aneurysm dome as well as an atretic distal PCOM artery. Given the complex anatomy, the lesion was unsuitable for clipping. We propose that this aneurysm represents a developmental variant whereby the proximal PCOM artery becomes atretic and terminates in PCOM perforators.

The video can be found here:http://youtu.be/iDcp9fsDjq4.

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Carl D. Hacker, Jarod L. Roland, Albert H. Kim, Joshua S. Shimony, and Eric C. Leuthardt

Resting-state functional MRI (rs-fMRI) is a well-established method for studying intrinsic connectivity and mapping the topography of functional networks in the human brain. In the clinical setting, rs-fMRI has been used to define functional topography, typically language and motor systems, in the context of preoperative planning for neurosurgery. Intraoperative mapping of critical speech and motor areas with electrocortical stimulation (ECS) remains standard practice, but preoperative noninvasive mapping has the potential to reduce operative time and provide functional localization when awake mapping is not feasible. Task-based fMRI has historically been used for this purpose, but it can be limited by the young age of the patient, cognitive impairment, poor cooperation, and need for sedation. Resting-state fMRI allows reliable analysis of all functional networks with a single study and is inherently independent of factors affecting task performance. In this review, the authors provide a summary of the theory and methods for resting-state network mapping. They provide case examples illustrating clinical implementation and discuss limitations of rs-fMRI and review available data regarding performance in comparison to ECS. Finally, they discuss novel opportunities for future clinical applications and prospects for rs-fMRI beyond mapping of regions to avoid during surgery but, instead, as a tool to guide novel network-based therapies.

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Jarod L. Roland, Richard L. Price, Ashwin A. Kamath, S. Hassan Akbari, Eric C. Leuthardt, Brandon A. Miller, and Matthew D. Smyth

作者描述2例triventricular海德拉巴rocephalus initially presenting as aqueductal stenosis that subsequently developed tumors of the pineal and tectal region. The first case resembled late-onset idiopathic aqueductal stenosis on serial imaging. Subsequent imaging revealed a new tumor in the pineal region causing mass effect on the midbrain. The second case presented in a more typical pattern of aqueductal stenosis during infancy. On delayed follow-up imaging, an enlarging tectal mass was discovered. In both cases hydrocephalus was successfully treated by cerebrospinal fluid diversion prior to tumor presentation. The differential diagnoses, diagnostic testing, and treatment course for these unusual cases are discussed. The importance of follow-up MRI in cases of idiopathic aqueductal stenosis is emphasized by these exemplar cases.

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Rebecca A. Reynolds, Diane J. Aum, Ignacio Gonzalez-Gomez, Michael Wong, Kaleigh Roberts, Sonika Dahiya, Luis F. Rodriguez, Jarod L. Roland, and Matthew D. Smyth

OBJECTIVE

Tuberous sclerosis is a rare genetic condition caused byTSC1orTSC2变异可以遗传的,零星的或result of somatic mosaicism. Subependymal giant-cell astrocytoma (SEGA) is a major diagnostic feature of tuberous sclerosis complex (TSC). This study aimed to present a series of cases in which a pathological diagnosis of SEGA was not diagnostic of tuberous sclerosis.

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The authors retrospectively reviewed a clinical case series of 5 children who presented with a SEGA tumor to Johns Hopkins All Children’s Hospital and St. Louis Children’s Hospital between 2010 and 2022 and whose initial genetic workup was negative for tuberous sclerosis. All patients were treated with craniotomy for SEGA resection.TSCgenetic testing was performed on all SEGA specimens.

RESULTS

The children underwent open frontal craniotomy for SEGA resection from the ages of 10 months to 14 years. All cases demonstrated the classic imaging features of SEGA. Four were centered at the foramen of Monro and 1 in the occipital horn. One patient presented with hydrocephalus, 1 with headaches, 1 with hand weakness, 1 with seizures, and 1 with tumor hemorrhage. SomaticTSC1mutation was present in the SEGA tumors of 2 patients andTSC2mutation in 1 patient. Germline TSC mutation testing was negative for all 5 cases. No patient had other systemic findings of tuberous sclerosis on ophthalmological, dermatological, neurological, renal, or cardiopulmonary assessments and thus did not meet the clinical criteria for tuberous sclerosis. The average follow-up was 6.7 years. Recurrence was noted in 2 cases, in which 1 patient underwent radiosurgery and 1 was started on a mammalian target of rapamycin (mTOR) inhibitor (rapamycin).

CONCLUSIONS

There may be intracranial implications of somatic mosaicism associated with tuberous sclerosis. Children who are diagnosed with SEGA do not necessarily have a diagnosis of tuberous sclerosis. Tumors may carry aTSC1orTSC2mutation, but germline testing can be negative. These children should continue to be followed with serial cranial imaging for tumor progression, but they may not require the same long-term monitoring as patients who are diagnosed with germlineTSC1orTSC2mutations.

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Jarod L. Roland, Natalie Griffin, Carl D. Hacker, Ananth K. Vellimana, S. Hassan Akbari, Joshua S. Shimony, Matthew D. Smyth, Eric C. Leuthardt, and David D. Limbrick Jr.

OBJECTIVE

Cerebral mapping for surgical planning and operative guidance is a challenging task in neurosurgery. Pediatric patients are often poor candidates for many modern mapping techniques because of inability to cooperate due to their immature age, cognitive deficits, or other factors. Resting-state functional MRI (rs-fMRI) is uniquely suited to benefit pediatric patients because it is inherently noninvasive and does not require task performance or significant cooperation. Recent advances in the field have made mapping cerebral networks possible on an individual basis for use in clinical decision making. The authors present their initial experience translating rs-fMRI into clinical practice for surgical planning in pediatric patients.

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作者回顾性综述病例摘要h the rs-fMRI analysis technique was used prior to craniotomy in pediatric patients undergoing surgery in their institution. Resting-state analysis was performed using a previously trained machine-learning algorithm for identification of resting-state networks on an individual basis. Network maps were uploaded to the clinical imaging and surgical navigation systems. Patient demographic and clinical characteristics, including need for sedation during imaging and use of task-based fMRI, were also recorded.

RESULTS

Twenty patients underwent rs-fMRI prior to craniotomy between December 2013 and June 2016. Their ages ranged from 1.9 to 18.4 years, and 12 were male. Five of the 20 patients also underwent task-based fMRI and one underwent awake craniotomy. Six patients required sedation to tolerate MRI acquisition, including resting-state sequences. Exemplar cases are presented including anatomical and resting-state functional imaging.

CONCLUSIONS

Resting-state fMRI is a rapidly advancing field of study allowing for whole brain analysis by a noninvasive modality. It is applicable to a wide range of patients and effective even under general anesthesia. The nature of resting-state analysis precludes any need for task cooperation. These features make rs-fMRI an ideal technology for cerebral mapping in pediatric neurosurgical patients. This review of the use of rs-fMRI mapping in an initial pediatric case series demonstrates the feasibility of utilizing this technique in pediatric neurosurgical patients. The preliminary experience presented here is a first step in translating this technique to a broader clinical practice.

Open access

M. Travis Caton, Kazim Narsinh, Amanda Baker, Adib A. Abla, Jarod L. Roland, Van V. Halbach, Christine K. Fox, Heather J. Fullerton, and Steven W. Hetts

BACKGROUND

The authors recently reported a series of children with vertebral artery (VA) compression during head turning who presented with recurrent posterior circulation stroke. Whether VA compression occurs during head positioning for cranial surgery is unknown.

OBSERVATIONS

The authors report a case of a child with incidental rotational occlusion of the VA observed during surgical head positioning for treatment of an intracranial arteriovenous fistula. Intraoperative angiography showed dynamic V3occlusion at the level of C2 with distal reconstitution via a muscular branch “jump” collateral, supplying reduced flow to the V4segment. She had no clinical history or imaging suggesting acute or prior stroke. Sequential postoperative magnetic resonance imaging scans demonstrated signal abnormality of the left rectus capitus muscle, suggesting ischemic edema.

LESSONS

This report demonstrates that rotational VA compression during neurosurgical head positioning can occur in children but may be asymptomatic due to the presence of muscular VA–VA “jump” collaterals and contralateral VA flow. Although unilateral VA compression may be tolerated by children with codominant VAs, diligence when rotating the head away from a dominant VA is prudent during patient positioning to avoid posterior circulation ischemia or thromboembolism.

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