Peter P. Sun

Seizure outcomes after temporal lobectomy in pediatric patients

Temporal lobe epilepsy (TLE) is the most common form of epilepsy in adults and is responsible for 15%-20% of epilepsy cases in children. Class I evidence strongly supports the use of temporal lobectomy for intractable TLE in adults, but fewer studies have examined seizure outcomes and predictors of seizure freedom after temporal lobectomy in pediatric patients. The authors performed a systematic review and meta-analysis of studies including 10 or more pediatric patients (age ≤ 19 years) published over the last 20 years examining seizure outcomes after temporal lobectomy for TLE. Thirty-six studies met their inclusion criteria. These 36 studies included 1318 pediatric patients with a mean age (± SEM) of 10.7 ± 0.3 years. Overall, seizure freedom (Engel Class I outcome) was achieved in 1002 cases (76%); 316 patients (24%) continued to have seizures (Engel Class II-IV outcome). All patients had at least 1 year of follow-up. Statistically significant predictors of seizure freedom after surgery included lesional epilepsy etiology (odds ratio [OR] 1.08, 95% confidence interval [CI] 1.02-1.15), abnormal findings on preoperative MRI (OR 1.27, 95% CI 1.16-1.40), and lack of generalized seizures (OR 1.36, 95% CI 1.20-1.56). Among lesional epilepsy cases, there was a trend toward better outcome with gross-total lesionectomy than with subtotal resection. Approximately three-fourths of pediatric patients with TLE attain seizure freedom after temporal lobectomy. Favorable outcomes may be predicted by lesional epilepsy etiology, abnormal MRI, and lack of generalized seizures. Pediatric patients with medically refractory TLE should be referred to a comprehensive pediatric epilepsy center for surgical evaluation.

A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas

OBJECT Ependymoma is the third most common primary brain tumor in children. Tumors are classified according to the WHO pathological grading system. Prior studies have shown high levels of variability in patient outcomes within and across pathological grades. The authors reviewed the results from the published literature on intracranial ependymomas in children to describe clinical outcomes as they relate to treatment modality, associated mortality, and associated progression-free survival (PFS). METHODS A search of English language peer-reviewed articles describing patients 18 years of age or younger with intracranial ependymomas yielded data on 182 patients. These patients had undergone treatment for ependymoma with 1 of 5 modalities: 1) gross-total resection (GTR), 2) GTR as well as external beam radiation therapy (EBRT), 3) subtotal resection (STR), 4) STR as well as EBRT, or 5) radiosurgery. Mortality and outcome data were analyzed for time to tumor progression in patients treated with 1 of these 5 treatment modalities. RESULTS Of these 182 patients, 69% had supratentorial ependymomas and 31% presented with infratentorial lesions. Regardless of tumor location or pathological grade, STR was associated with the highest rates of mortality. In contrast, GTR was associated with the lowest rates of mortality, the best overall survival, and the longest PFS. Children with WHO Grade II ependymomas had lower mortality rates when treated more aggressively with GTR. However, patients with WHO Grade III tumors had slightly better survival outcomes after a less aggressive surgical debulking (STR+EBRT) when compared with GTR. CONCLUSIONS Mortality, PFS, and overall survival vary in pediatric patients with intracranial ependymomas. Pathological classification, tumor location, and method of treatment play a role in outcomes. In this study, GTR was associated with the best overall and PFS rates. Patients with WHO Grade II tumors had better overall survival after GTR+EBRT and better PFS after GTR alone. Patients with WHO Grade III tumors had better overall survival after STR+EBRT. Patients with infratentorial tumors had improved overall survival compared with those with supratentorial tumors. Progression-free survival was best in those patients with infratentorial tumors following STR+EBRT. Consideration of all of these factors is important when counseling families on treatment options.

In utero surgery for hydrocephalus

IntroductionNeonatal hydrocephalus is one of the most common congenital anomalies affecting the nervous system.Discussion Currently, ultrasonography allows for early detection of fetal ventriculomegaly and presents the family with several treatment options: termination of pregnancy, early delivery and neonatal shunting, and delivery at term followed by shunting. Despite ventricular decompression after birth, the cognitive outcome is variable as prolonged in utero hydrocephalus has a detrimental effect. In the early 1980s, fetal intervention was explored with the intention of improving outcome. However, patient selection was poor. Fetal ventriculomegaly from other conditions was not adequately distinguished from fetal hydrocephalus. In addition, fetal surgical techniques were not advanced. Consequently, the results were poor and a de facto moratorium on fetal shunting was imposed. However, recent improvements in fetal imaging, such as magnetic resonance imaging, and advances in fetal surgical techniques offer the possibility that properly selected fetuses with hydrocephalus can benefit from an in utero intervention.

Cervical cord compression in mucopolysaccharidosis VI (MPS VI): Findings from the MPS VI Clinical Surveillance Program (CSP)

OBJECTIVES To gain insight into the frequency, age of onset, and management of cervical cord compression in mucopolysaccharidosis VI (MPS VI). METHODS Cervical spine magnetic resonance imaging (MRI) data and/or cervical decompression surgery data collected between 30 June 2005 and 1 September 2015 were analyzed from subjects enrolled in the MPS VI Clinical Surveillance Program (CSP) (ClinicalTrials.gov: NCT00214773), an ongoing multicenter, observational, retrospective and prospective registry. RESULTS Of 213 subjects enrolled in the CSP, 134 (62.9%) had at least one documented cervical spine MRI assessment. An additional four subjects were identified through surgery records alone to yield a study population comprising 138 subjects (mean age at enrollment =15.1years; age range=0.80-65.0years). Cervical cord compression was documented in 101 (75.4%) of the 134 subjects with ≥1 MRI assessment, the majority (95.0%) by the time of the first recorded MRI. In general, subjects with cervical cord compression had significantly lower height Z-scores compared to those without cervical cord compression (p<0.0001); nevertheless, a few subjects of taller stature had documented cervical cord compression at a young age. Most subjects >20years of age (31/33, 93.9%) presented with cervical cord compression. There was an insufficient number of subjects with both pre- and post-enzyme replacement therapy (ERT) MRI data to determine any association between ERT and cervical cord compression. Surgical decompression was performed on 58 subjects (42.0%), with mean age at first surgery of 13.1years. Decompression plus stabilization procedures accounted for 12.1% of surgeries. Eight subjects (13.8%) underwent reoperation. Complications during or following surgery were reported in 3 subjects, with anesthesia-related complications resulting in two deaths. CONCLUSIONS All individuals with MPS VI are at high risk of developing cervical cord compression at an early age. Routine MRI assessments should be initiated from the time of MPS VI diagnosis. The perioperative management of MPS VI patients can be challenging. This study contributes to the understanding of the natural history of MPS VI.

Cervical spinal stenosis and sports-related cervical cord neurapraxia

Cervical cord neurapraxia is a common sports-related injury. It is defined as a transient neurological deficit following trauma localizing to the cervical spinal cord and can be caused by hyperextension, hyperflexion, or axial load mechanisms. Symptoms usually last less than 15 minutes, but can persist up to 48 hours in adults and as long as 5 days in children. While a strong causal relationship exists between cervical spine stenosis and cervical cord neurapraxia in adult patients, this association has not been observed in children. Likewise, while repeated episodes of neurapraxia can be commonplace in adult patients, recurrences have not been reported in the pediatric population. Treatment is usually supportive, but in adults with focal cervical lesions or instability, surgery is an option. Surgery for neurapraxia in children is rarely indicated.

Intractable episodic bradycardia resulting from progressive lead traction in an epileptic child with a vagus nerve stimulator: a delayed complication.

Vagus nerve stimulation (VNS) is used as palliation for adult and pediatric patients with intractable epilepsy who are not candidates for curative resection. Although the treatment is generally safe, complications can occur intraoperatively, perioperatively, and in a delayed time frame. In the literature, there are 2 reports of pediatric patients with implanted VNS units who had refractory bradycardia that resolved after the stimulation was turned off. The authors report the case of a 13-year-old boy with a history of vagus nerve stimulator placement at 2 years of age, who developed intractable episodic bradycardia that persisted despite the cessation of VNS and whose imaging results suggested vagus nerve tethering by the leads. He was subsequently taken to the operating room for exploration, where it was confirmed that the stimulator lead was exerting traction on the vagus nerve, which was displaced from the carotid sheath. After the vagus nerve was untethered and the leads were replaced, the bradycardia eventually resolved with continual effective VNS therapy. When placing a VNS unit in a very young child, accommodations must be made for years of expected growth. Delayed intractable bradycardia can result from a vagus nerve under traction by tethered stimulator leads.

Fast dynamic imaging technique to identify obstructive lesions in the CSF space: report of 2 cases

Disorders of CSF dynamics such as syringomyelia and obstructive hydrocephalus can be caused by thin mobile obstructive lesions not visible on traditional MRI sequences. New imaging techniques with balanced steady-state free precession (bSSFP) and dynamic imaging with bSSFP cine allow visualization of these pulsatile structures within the CSF space. The authors present 2 cases involving pediatric patients-one who developed presumed idiopathic syringomyelia and one with presumed communicating hydrocephalus in association with Pfeiffer syndrome-who harbored thin dynamic obstructive lesions seen on bSSFP cine studies using 1.5-T MRI. In combination with traditional CSF cine studies and bSSFP, bSSFP cine sequence was able to detect dynamic membranous adhesions not seen on traditional MRI sequences. These previously undetectable lesions on traditional MRI sequences were the etiology of CSF obstruction, and tailored surgical approaches were performed to avoid shunting in both patients. These reports demonstrate the clinical utility for using these novel imaging tools for the detection of thin adhesions and dynamic lesions in the central nervous system. Balanced SSFP cine sequences can supplement conventional MR modalities to identify these otherwise poorly visualized lesions responsible for presumed communicating hydrocephalus or idiopathic syringomyelia.

Myxoid glioneuronal tumor of the septum pellucidum and lateral ventricle is defined by a recurrent PDGFRA p.K385 mutation and DNT-like methylation profile

David A. Solomon1,2 · Andrey Korshunov3,4 · Martin Sill5,6,7 · David T. W. Jones5,6 · Marcel Kool5,6 · Stefan M. Pfister5,6,7 · Xuemo Fan8 · Serguei Bannykh8 · Jethro Hu9 · Moise Danielpour10 · Rong Li11 · James Johnston12 · Elaine Cham13 · Tabitha Cooney14 · Peter P. Sun15 · Nancy Ann Oberheim Bush16 · Michael McDermott17 · Jessica Van Ziffle1,2 · Courtney Onodera1,2 · James P. Grenert1,2 · Boris C. Bastian1,2 · Javier E. Villanueva‐Meyer18 · Melike Pekmezci1 · Andrew W. Bollen1 · Arie Perry1,17

Spinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report

Choroid plexus papillomas (CPPs) are typically benign tumors that can occur in any age group but are more commonly found in pediatric patients. Although these tumors are benign, there are several reports in adult patients of distant metastases present either at the time of diagnosis or occurring months to years after initial resection. Here, the authors report the case of a 14-year-old boy who presented with symptoms of elevated intracranial pressure due to obstructive hydrocephalus that was caused by a large fourth ventricular mass. Preoperative imaging included a full MRI of the spine, which revealed an intradural lesion that encased the distal sacral nerve roots at the tip of the thecal sac and was concerning for a drop metastasis. The patient underwent gross-total resection of both the fourth ventricular and sacral tumors with histology of both lesions consistent with benign CPP (WHO Grade I). In addition, the authors review prior reports of both pediatric and adult patients in whom benign CPPs have metastasized with either benign or atypical pathology found at a distant site. Taking into account this unusual case and reports in the literature, patients with even benign CPPs may warrant initial and routine follow-up imaging of the total neural axis in search of the rare, but possible, occurrence of drop metastasis.

Encephaloduroarteriosynangiosis (EDAS) in young patients with cerebrovascular complications of sickle cell disease: Single-institution experience

ABSTRACT Moyamoya syndrome occurs in sickle cell disease (SCD) as a secondary complication of large-artery stenosis. Moyamoya increases the risk of stroke, but its optimal management in SCD is not established.  Encephaloduroarteriosynangiosis (EDAS) is a neurosurgical revascularization procedure for moyamoya whose use has been reported in SCD patients. We report the outcomes of 11 patients with SCD systematically evaluated for EDAS by a multidisciplinary team and compare the rate of stroke in patients who received EDAS to those who did not. Moyamoya syndrome was diagnosed by flow abnormalities on magnetic resonance angiography at median age of 8.2 years. Four patients deferred surgery. Seven patients underwent EDAS at median age of 19 years. There were no intraoperative complications, perioperative strokes, or deaths. Transient postoperative complications occurred in six cases (86%). On follow-up, three patients (43%) had no evidence of flow in their EDAS grafts, and one later developed a hemorrhagic stroke. Five EDAS patients (71%) had radiographically stable vasculopathy. Compared to the four patients who deferred surgery, the incidence of stroke in EDAS group was no different. The optimal use of EDAS in patients with SCD-associated moyamoya syndrome requires further investigation by a prospective, controlled clinical trial.