scholarly journals Responsive neurostimulation for the treatment of medically refractory epilepsy in pediatric patients: strategies, outcomes, and technical considerations

Author(s):  
Armin Mortazavi ◽  
Ross-Jordon S. Elliott ◽  
Tiffany N. Phan ◽  
John Schreiber ◽  
William D. Gaillard ◽  
...  

OBJECTIVE Children with medically refractory partial-onset epilepsy arising from eloquent cortex present a therapeutic challenge, as many are not suitable for resective surgery. For these patients, responsive neurostimulation may prove to be a potential tool. Although responsive neurostimulation has demonstrated utility in adults, little has been discussed regarding its utility in the pediatric population. In this study, the authors present their institution’s experience with responsive neurostimulation via the RNS System through a case series of 5 pediatric patients. METHODS A single-center retrospective study of patients who underwent RNS System implantation at Children’s National Hospital was performed. RESULTS Five patients underwent RNS System implantation. The mean patient age at treatment was 16.8 years, and the average follow-up was 11.2 months. All patients were considered responders, with a seizure frequency reduction of 64.2% without adverse events. CONCLUSIONS All 5 patients experienced medium-term improvements in seizure control after RNS System implantation with decreases in seizure frequency > 50% from baseline preoperative seizure frequency. The authors demonstrated two primary configurations of electrode placement: hippocampal or amygdala placement via an occipitotemporal trajectory, as well as infratemporal surface electrodes and surface electrodes on the primary motor cortex. No adverse events were experienced in this case series.

2010 ◽  
Vol 5 (1) ◽  
pp. 49-60 ◽  
Author(s):  
John A. Jane ◽  
Daniel M. Prevedello ◽  
Tord D. Alden ◽  
Edward R. Laws

Object The majority of pediatric craniopharyngiomas are treated using a transcranial approach. Although there is an increasing acceptance of transsphenoidal resection in adults, there are few reports describing this approach in the pediatric population. The purpose of this study is to report the outcomes after transsphenoidal surgery in a consecutive series of pediatric patients with craniopharyngiomas treated at a single institution with the goal of gross-total resection (GTR). Methods Twenty-three patients with pathologically proven craniopharyngiomas were identified who were 18 years of age or less at the time of surgery. The medical records and imaging studies of the patients were retrospectively reviewed. One patient who was lost to follow-up after surgery was excluded. Results Among the 22 patients included in the study, 11 underwent transsphenoidal surgery as the primary procedure and 11 underwent transsphenoidal surgery as a secondary procedure after a previous procedure. All patients had at least some sellar component to their tumor and all had either anterior or posterior pituitary dysfunction at presentation. In the entire cohort, a GTR was achieved in 15 (68%) of 22 patients, a radical subtotal resection in 4 (18%) of 22 patients, a subtotal resection in 1 patient, and a partial resection in 2 patients. The degree of resection was higher in the primary transsphenoidal group. After a mean follow-up of 82 months, 4 patients (18%) experienced recurrence. Recurrence occurred in 13% after GTR compared with 28.5% after all other degrees of resection. Tumor recurred in 9% of the primary transsphenoidal group and in 30% of patients who had undergone other therapies prior to the transsphenoidal operation. No patient who had panhypopituitarism experienced a gain of function postoperatively, 67% developed new panhypopituitarism, and 56% experienced new diabetes insipidus. Vision improved or normalized in 9 (64%) of 14 patients presenting with visual loss. Complications included 1 death 3 weeks postoperatively, 2 CSF leaks, and new obesity in 37%. Conclusions Transsphenoidal resection of pediatric craniopharyngiomas results in a high rate of both visual improvement and GTR with a low associated risk of recurrence. The transsphenoidal approach should be considered in selected pediatric patients with craniopharyngioma, especially those with infradiaphragmatic origin.


2018 ◽  
Vol 6 (3) ◽  
pp. 232596711876311 ◽  
Author(s):  
S. Clifton Willimon ◽  
Tim Schrader ◽  
Crystal A. Perkins

Background: Pigmented villonodular synovitis (PVNS) is a benign proliferative synovial disorder most commonly described to affect the knee in adults. Literature describing PVNS in the pediatric population is limited to 2 small case series and a handful of single-patient case reports. Within these studies, only 2 patients with PVNS of the hip are described. Purpose: To describe the presentation, management, and outcomes of a single-center series of pediatric patients with PVNS of the hip treated with arthroscopic synovectomy. Study Design: Case series; Level of evidence, 4. Methods: A retrospective review of consecutive pediatric patients treated for PVNS at a single institution was performed. Inclusion criteria consisted of patients younger than 19 years with surgically treated PVNS of the hip. Results: Five pediatric patients with a mean age of 11.0 years were treated for PVNS of the hip from 2011 to 2016. The mean duration of symptoms from onset to surgical treatment was 247 days (range, 3-933 days). Upon review of magnetic resonance imaging (MRI) results, radiologists included PVNS in their differential in 3 patients. Seven surgeries were performed in 5 patients. All therapeutic procedures were arthroscopic synovectomies. Nodular PVNS was present in 4 patients, and diffuse disease was present in 1 patient. At a mean 32-month follow-up (range, 12-63 months), all patients were considered to be free of recurrence based on clinical examination and/or follow-up MRI. Four patients were asymptomatic and returned to all of their previous sports activities. Conclusion: Young age at the time of diagnosis is a point to be highlighted in this cohort, and symptoms may be present for many months prior to diagnosis due to the failure to consider PVNS in children. Therefore, for patients with “atypical” presentations or lack of improvement with treatment for rheumatologic, bleeding, or infectious disorders, PVNS should be strongly considered. MRI with gradient echo sequences is the diagnostic imaging study of choice. One patient with diffuse involvement and preoperative degenerative changes showed progressive changes postoperatively. This type of PVNS may have a worse prognosis, but more diffuse cases are needed before the prognosis can be determined. Arthroscopic synovectomy following a timely diagnosis of PVNS produces good outcomes in nodular cases, with no evidence of symptomatic or radiographic disease persistence among these patients.


2020 ◽  
pp. neurintsurg-2020-016183
Author(s):  
Katriel E Lee ◽  
Aqib Zehri ◽  
Sauson Soldozy ◽  
Hasan Syed ◽  
Joshua S Catapano ◽  
...  

BackgroundDural venous sinus stenting (VSS) is an effective treatment for idiopathic intracranial hypertension (IIH) in adult patients. There are no published series to date evaluating safety and efficacy of VSS in pediatric patients.ObjectiveTo report on procedural device selection and technique as well as safety and efficacy of VSS for pediatric patients with medically refractory IIH due to underlying venous sinus stenosis.MethodsA multi-institutional retrospective case series identified patients with medically refractory IIH aged less than 18 years who underwent VSS.Results14 patients were identified at four participating centers. Patient ages ranged from 10 to 17 years, and 10 patients (71.4%) were female. Mean body mass index was 25.7 kg/m2 (range 15.8–34.6 kg/m2). Stenting was performed under general endotracheal anesthesia in all except two patients. The average trans-stenotic gradient during diagnostic venography was 10.6 mm Hg. Patients had stents placed in the superior sagittal sinus, transverse sinus, sigmoid sinus, occipital sinus, and a combination. Average follow-up was 1.7 years after stenting. Six patients out of 10 (60%) had reduced medication dosing, 12 of 14 patients (85.7%) had improvements in headaches, two patients (100%) with pre-stent tinnitus had resolution of symptoms, and four (80%) of five patients with papilledema had improvement on follow-up ophthalmological examinations. Two patients (14.3%) developed postprocedural groin hematomas, one patient (7.1%) developed a groin pseudoaneurysm, and one patient (7.1%) had postprocedural groin bleeding. No other procedural complications occurred. Four patients (28.6%) required further surgical treatment (cerebrospinal shunting and/or stenting) after their first stenting procedure.ConclusionsThis series suggests that VSS is feasible in a pediatric population with IIH and has a low complication rate and good clinical outcomes.


2019 ◽  
Vol 24 (6) ◽  
pp. 534-537
Author(s):  
Shane C. Rainey ◽  
Nadia Shaikh ◽  
Keith A. Hanson

Gadolinium-based compounds are frequently used in contrast-enhanced magnetic resonance imaging studies. Rarely, adverse events have been reported with administration of these compounds, of which the most common are nausea and vomiting. Although well established in the adult literature, these adverse effects are less well described in the pediatric population, who often need sedation to complete imaging studies. In this case series, we present 3 children who experienced vomiting shortly after contrast administration while under sedation with propofol, which is known to have antiemetic properties. Although all 3 children recovered without complication, this case series illustrates the serious potential consequences of vomiting while sedated, and providers should be aware of these possible adverse events as pediatric sedation becomes more common outside the operating room.


2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.


2011 ◽  
Vol 8 (1) ◽  
pp. 57-62 ◽  
Author(s):  
Kyle M. Fargen ◽  
Richard C. E. Anderson ◽  
David H. Harter ◽  
Peter D. Angevine ◽  
Valerie C. Coon ◽  
...  

Object Although rarely encountered, pediatric patients with severe cervical spine deformities and instability may occasionally require occipitocervicothoracic instrumentation and fusion. This case series reports the experience of 4 pediatric centers in managing this condition. Occipitocervical fixation is the treatment of choice for craniocervical instability that is symptomatic or threatens neurological function. In children, the most common distal fixation level with modern techniques is C-2. Treated patients maintain a significant amount of neck motion due to the flexibility of the subaxial cervical spine. Distal fixation to the thoracic spine has been reported in adult case series. This procedure is to be avoided due to the morbidity of complete loss of head and neck motion. Unfortunately, in rare cases, the pathological condition or highly aberrant anatomy may require occipitocervical constructs to include the thoracic spine. Methods The authors identified 13 patients who underwent occipitocervicothoracic fixation. Demographic, radiological, and clinical data were gathered through retrospective review of patient records from 4 institutions. Results Patients ranged from 1 to 14 years of age. There were 7 girls and 6 boys. Diagnoses included Klippel-Feil, Larsen, Morquio, and VATER syndromes as well as postlaminectomy kyphosis and severe skeletal dysplasia. Four patients were neurologically intact and 9 had myelopathy. Five children were treated with preoperative traction prior to instrumentation; 5 underwent both anterior and posterior spinal reconstruction. Two patients underwent instrumentation beyond the thoracic spine. Allograft was used anteriorly, and autologous rib grafts were used in the majority for posterior arthrodesis. Follow-up ranged from 0 to 43 months. Computed tomography confirmed fusion in 9 patients; the remaining patients were lost to follow-up or had not undergone repeat imaging at the time of writing. Patients with myelopathy either improved or stabilized. One child had mild postoperative unilateral upper-extremity weakness, and a second child died due to a tracheostomy infection. All patients had severe movement restriction as expected. Conclusions Occipitocervicothoracic stabilization may be employed to stabilize and reconstruct complex pediatric spinal deformities. Neurological function can be maintained or improved. The long-term morbidity of loss of cervical motion remains to be elucidated.


Author(s):  
Ilkka Rauma ◽  
Tiina Mustonen ◽  
Juha Matti Seppä ◽  
Maritta Ukkonen ◽  
Marianne Männikkö ◽  
...  

Abstract Background Alemtuzumab is an effective disease-modifying therapy (DMT) for highly active multiple sclerosis (MS). However, safety concerns limit its use in clinical practice. Objectives To evaluate the safety of alemtuzumab in a nationwide cohort of Finnish MS patients. Methods In this retrospective case series study, we analyzed the data of all but two MS patients who had received alemtuzumab in Finland until 2019. Data were systematically collected from patient files. Results Altogether 121 patients were identified, most of whom had received previous DMTs (82.6%). Median follow-up time after treatment initiation was 30.3 months and exceeded 24 months in 78 patients. Infusion-associated reactions (IARs) were observed in 84.3%, 57.3%, and 57.1% of patients during alemtuzumab courses 1–3, respectively. Serious adverse events (SAEs) were observed in 32.2% of patients, serious IARs in 12.4% of patients, and SAEs other than IARs in 23.1% of patients. Autoimmune adverse events were observed in 30.6% of patients. One patient died of hemophagocytic lymphohistiocytosis, and one patient died of pneumonia. A previously unreported case of thrombotic thrombocytopenic purpura was documented. Conclusions SAEs were more frequent in the present cohort than in previous studies. Even though alemtuzumab is a highly effective therapy for MS, vigorous monitoring with a long enough follow-up time is advised.


2021 ◽  
pp. 112067212110206
Author(s):  
Iliya Simantov ◽  
Lior Or ◽  
Inbal Gazit ◽  
Biana Dubinsky-Pertzov ◽  
David Zadok ◽  
...  

Background: Retrospective cohort study evaluating long term keratoconus progression amongst cross-linking (CXL) treated pediatric patients in the treated and the fellow untreated eyes. Methods: Data on 60 eyes of 30 patients, 18 years old or younger, who underwent CXL in at least one eye was collected and analyzed. Follow-up measurements taken from the treated and untreated eye up to 7 years after CXL treatment, were compared to baseline measurements. Parameters included uncorrected distance visual acuity (UCDVA), best-corrected spectacle visual acuity (BCSVA), manifest refraction, pachymetry, corneal tomography, and topography. Results: Mean age of patients was 16 ± 2.1 years. For the treated eyes, during follow-up period mean UCDVA had improved (from 0.78 ± 0.22 at baseline to 0.58 ± 0.26 logMAR at 7 years; p = 0.13), as well as mean BCSVA (from 0.23 ± 0.107 at baseline to 0.172 ± 0.05 logMAR at 7 years; p = 0.37). The mean average keratometry showed a significant flattening (from 49.95 ± 4.04 to 47.94 ± 3.3 diopters (D); p < 0.001), However there was no change in the mean maximal keratometry. The mean minimal corneal thickness (MCT) showed a significant mild reduction of 26 µm ( p = 0.006). Although statistically insignificant, the mean manifest cylinder was also reduced to 2D ( p = 0.15). During the follow-up period, eight untreated eyes (26.6%) deteriorated and underwent CXL, while only one treated eye (3.33%) required an additional CXL. Conclusion: CXL is a safe and efficient procedure in halting keratoconus progression in the pediatric population, the fellow eye needs to be carefully monitored but only a 25% of the patients will require CXL in that eye during a period of 7 years.


2008 ◽  
Vol 1 (4) ◽  
pp. 337-342 ◽  
Author(s):  
Matthew J. McGirt ◽  
Frank J. Attenello ◽  
Daniel M. Sciubba ◽  
Ziya L. Gokaslan ◽  
Jean-Paul Wolinsky

✓ Pediatric basilar invagination and cranial settling have traditionally been approached through a transoral–transpharyngeal route with or without extended maxillotomy or mandibulotomy for resection of the anterior portion of C-1 and the odontoid. The authors hypothesize that application of a recently described endoscopic transcervical odontoidectomy (ETO) technique would allow an alternative approach for the treatment of ventral pathological entities at the craniocervical junction in pediatric patients. The authors performed ETO in a consecutive series of pediatric patients presenting with myelopathy or bulbar dysfunction resulting from basilar invagination or cranial settling. All clinical, radiographic, surgical, and follow-up data were prospectively collected. The initial experience with ETO in the pediatric population is analyzed and outcomes are reported. Three patients required ETO for basilar invagination and 1 required ETO with anterior C-1 arch and distal clivus resection for cranial settling. All patients presented with myelopathy. One patient was wheelchair bound with severe quadriparesis. The mean age was 14 ± 3 years (mean ± standard deviation [SD]) in the 2 male and 2 female patients. The ETO and posterior fusion were performed as a 2-stage procedure in 2 (50%) and as a single-stage procedure in 2 (50%) cases. Prolonged intubation or postoperative placement of a gastrostomy tube was not needed in any case. The postoperative hospitalization lasted 9 ± 4 days (mean ± SD). At last follow-up (mean 5 months), head and neck pain had resolved and motor strength had improved or stabilized in all cases. All 4 children were independently functioning and ambulatory at the last follow-up. In the authors' initial experience, ETO has allowed ventral brainstem decompression without the need for prolonged intubation, worsening dysphagia requiring enteral tube feeding, or prolonged hospitalization, and has resulted in cosmetically appealing results. The ETO technique allows an alternative approach for the treatment of ventral pathological entities at the craniocervical junction in pediatric patients.


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