Archives of Pediatric Neurosurgery

Vagal Nerve Stimulation for Refractory Epileptic Encephalopathy in Patient with Lissencephaly: Case Report from Indonesia

Arie Khairani — 2025-09-13

Introduction: Lissencephaly is a rare brain malformation that causes severe developmental delays and intractable seizures. Traditional anti-epileptic drugs (AEDs) often fail to control seizures in these patients. Vagal nerve stimulation (VNS) has shown promise as an adjunctive treatment for refractory epilepsy.

Case Presentation: A 19-year-old male with lissencephaly and epileptic encephalopathy was referred for VNS implantation after experiencing poorly controlled seizures despite maximum doses of Levetiracetam and Valproic Acid. The patient had daily seizures and frequent episodes of convulsive status epilepticus. EEG revealed multifocal spikes, primarily in the frontal regions. VNS implantation was performed, initially set at 0.2 mA and gradually increased to 1.5 mA over two months. Follow-up results showed a progressive reduction in seizure frequency. By two months, the patient experienced 5-7 seizure-free days each month, with further improvements noted at six months. At ten months, the seizures were brief and infrequent, and the patient no longer required rescue medication.

Conclusion: VNS therapy significantly improved seizure control and the patient’s quality of life. Regular follow-up and tailored adjustments to the VNS settings were essential for optimizing seizure management. This case highlights VNS as a valuable option for managing refractory epilepsy in patients with severe neurological conditions.

Intraventricular baclofen and bilateral pallidotomy in a pediatric patient with acquired generalized dystonia and refractory status dystonicus: illustrative case

Juan Camilo Arias-Angulo — 2025-09-13

Background: Status dystonicus (SD) is a life-threatening emergency in pediatric patients with severe dystonia, often requiring intensive care. Surgical interventions, such as deep brain stimulation, intrathecal baclofen (ITB), or pallidotomy, are considered for refractory cases, but evidence for intraventricular baclofen (IVB) and bilateral pallidotomy (BP) in children is limited. We report a novel combined approach using IVB followed by BP in a 3-year-old boy with refractory SD secondary to hypoxic-ischemic encephalopathy.

Case Description: A 3-year-old male developed SD and spastic quadriparesis following cardiorespiratory arrest due to airway obstruction. Despite aggressive medical management (levodopa, clonazepam, tetrabenazine, oral baclofen, sedatives), SD persisted, requiring prolonged mechanical ventilation. An ITB test (100 mcg) reduced the modified Ashworth scale from 5 to 3 and dystonic episodes from 8 to 2/day. IVB was placed via a third ventricle catheter with a pump delivering 100-1050 mcg/day, achieving spasticity control but fluctuating dystonia response. Due to recurrent SD, poor prognosis, and malnutrition, BP was performed using stereotactic microelectrode guidance and radiofrequency lesions (80°C, 60s). Postoperative MRI confirmed precise pallidal lesions. The patient achieved full SD resolution, was weaned from ventilation, and stabilized at home with tracheostomy and gastrostomy.

Conclusions: Bilateral pallidotomy and IVB should be considered in the treatment of refractory SD, particularly for patients with contraindications for DBS and concurrent severe spasticity. The literature review support this findings, providing an overview that can guide palliative management in similar cases. Further studies are needed to establish comprehensive guidelines for surgical management of SD in children.

Combined Anteromedial and Posteroventral Radiofrequency Pallidotomy in the Management of Severe Generalized Dystonia and Aggressivity in Lesch-Nyhan Syndrome

Luciano Furlanetti — 2025-09-13

Background: Lesch-Nyhan syndrome (LNS) is a rare genetic disorder caused by a deficiency of the enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT). It typically presents in infancy with compulsive self-injurious behavior accompanied by severe generalized dystonia and dyskinesia. The clinical management of affected individuals is particularly complex and poses significant challenges for both healthcare providers and caregivers.

Objectives: Pallidal deep brain stimulation (DBS) has been described as an adjuvant surgical treatment in LNS. We report the use of combined anteromedial and posterolateral radiofrequency pallidotomy in a pediatric patient with LNS, targeting both the limbic and sensorimotor networks.

Methods: Preoperative and postoperative functional assessment data prospectively collected by a multidisciplinary motor disorders team, including neuropsychology and neurophysiology evaluations were analyzed with regards to motor and behavioral control. Additionally, advanced image processing was conducted to analyze network connectivity patterns.

Results: An adolescent male with LNS was submitted to a right-sided combined anteromedial and posteroventral RF pallidotomy and followed-up (FU) for 48 months. Functional improvement was observed right after the surgery and remained stable throughout the FU time. Structural connectivity profile revealed completely distinct networks targeted within the GPi in the management of his motor and behavioral manifestations.

Conclusion: Combined anteromedial and posteroventral RF pallidotomy may be considered as an option for managing refractory dystonia and self-harm behavior in LNS patients, especially in cases where invasive neuromodulation with deep brain stimulation is not available or desirable. A multi-disciplinary team-based approach is essential to achieve functional improvement and alleviate the overall disease burden for patients and caregivers.

Intraventricular Baclofen via neuronavegated Ommaya reservoir as Bridge Therapy in Refractory Status Dystonicus: A Case Report

Antônio Jorge Barbosa de Oliveira — 2025-09-13

Introduction:
Status dystonicus (SD) is a life-threatening neurologic emergency that requires prompt recognition and intervention. Intraventricular baclofen (IVB) has been shown to be an alternative therapy with promising results in refractory cases.

Case Presentation:

We present a 12-year-old male with Wilson's disease and acquired generalized dystonia who developed status dystonicus after a gastrostomy procedure. Due to refractory, his dystonic symptoms despite of optimized intensive medical management, including intravenous sedation and invasive ventilatory assistance, we implemented intraventricular baclofen therapy via a temporary Ommaya reservoir. This allowed for rapid stabilization and resolution of the SD, prior to attempting to a more definitive surgical solution.

Conclusion:
Temporary Ommaya-based IVB is a feasible and cost-effective bridge therapy for SD management. It provides rapid symptomatic control and may enable strategic planning of definitive interventions, when applicable

Selective Ventral and Dorsal Rhizotomy for Refractory Status Dystonicus Cerebral Palsy: A Case Report

Antônio Jorge Barbosa de Oliveira — 2025-09-13

Introduction/Background: Status dystonicus (SD) is a life-threatening neurological emergency, particularly in children with mixed-type cerebral palsy (CP), where dystonia and spasticity coexist. Although deep brain stimulation (DBS) is a recognized therapeutic option, its use in acute settings and resource-limited environments can be challenging.

Case Presentation: We report the case of a 4-year-old boy with mixed CP secondary to traumatic brain injury, who developed refractory SD. The patient presented with severe dystonia, spasticity (Ashworth grade 3) and required intensive care due to rhabdomyolysis and autonomic instability. After failure of clinical management, a mixed selective rhizotomy (MVR) was performed as an emergency intervention.

Conclusions: MVR resulted in immediate motor relief, full resolution of SD, and functional improvement of the upper limbs. This case highlights MVR as an effective, low-cost and viable alternative for controlling refractory SD in patients with mixed motor phenotypes, especially in resource-limited settings.

Intracerebroventricular Baclofen Therapy for Chronic Pelvic Pain in Cerebral Palsy: A Case Report

Guilherme Corsaletti Gregório — 2025-09-13

Introduction: Although pain is frequent among patients with cerebral palsy (CP), vulvodynia is an uncommon finding. Its diagnosis is underrated in this population and its treatment is challenging, due to uncertainty in pathophysiological mechanisms of the symptom. The management of this rare combination of relevant sensorial and motor components requires a skilled multidisciplinary team capable of offering and testing all available forms of treatment, since this pain carries high levels of suffering and incapacity, and patients with CP usually have many other chronic and severe comorbidities.

Case presentation: This is a 14-year-old girl with mixed (hyperkinetic and spastic) CP, with minimal cognitive impairment, and classified as Gross Motor Function Classification System (GMFCS) level V. She failed all available conservative treatments for a longstanding and disabling vulvodynia. Local injections, including toxin botulinum failed to provide relief. After responding to an intrathecal baclofen trial, a baclofen pump was implanted with the catheter positioned within the third ventricle through navigation guidance. She experienced complete pain relief, and spasticity improvement as well. During the 6 months of follow-up, patient was pain free. Her quality of life also improved with the therapy during the follow-up period.

Conclusion: Intracerebroventricular baclofen therapy may be a viable option for managing chronic pain associated with spastic-dystonic symptoms in cerebral palsy.

; vulvodynia; ; dystonia; spasticity

Neurectomy for treatment of pediatric spasticity: a review of literature

Stephanie Oliveira Fernandes de Bulhões — 2025-09-13

Introduction: Spasticity in the pediatric population presents significant management challenges. The aim of this study was to review the current literature on the use of selective neurectomy for treating pediatric spasticity. It is important to analyze children as a distinct group, as the selection criteria differ from those used in adults, and the procedure affects not only spasticity but also the overall development of the child.

Methods: After a PubMed database search and applying inclusion criteria, we identified 14 relevant studies for this review. The following parameters were analyzed: patient age, number of participants, follow-up duration, Modified Ashworth Scale (MAS) scores, goniometry/passive range of motion (PROM), and motor function.

Results: There was considerable variability in the first three parameters (age, sample size, and follow-up period). All included studies reported improvements in spasticity, range of motion, and motor function when these outcomes were assessed. However, the methods used to evaluate these outcomes varied, limiting direct comparisons across studies.

Conclusion: Although few studies on selective neurectomy for pediatric spasticity were found, all demonstrated significant improvements in parameters associated with enhanced quality of life. These findings highlight the need for further research focused specifically on this population.

In Memory of Professor M. Gazi Yaşargil (1925–2025). Honoring the Legacy of a Master of Microneurosurgery

Ricardo Santos de Oliveira — 2025-06-20

Abstracts - X Congreso Latino-Americano de Neurocirurgia Pediátrica

Ricardo Santos de Oliveira — 2025-09-13

The city of São Paulo hosted the 10th Latin American Congress of Pediatric Neurosurgery – CLANPED, from August 12 to 16, 2025.

Simultaneously, we held the 3rd Latin American Symposium on Instrumentation and Neurosurgical Nursing and the Symposium of Academic Medical Leagues.

CLANPED featured a program of high scientific rigor. This edition included two pre-congress “hands-on” courses, as well as lectures and discussions on highly relevant topics. On August 12 and 13, we had the ISPN (International Society for Pediatric Neurosurgery) theoretical-practical course and the dissection course at the UNIFESP microsurgery laboratory, coordinated by the renowned Prof. Dr. Feres Chaddad.

The event was carefully organized to provide high-quality and relevant knowledge to colleagues across Latin America, ensuring a congress of outstanding scientific level and great practical application.

We were pleased to welcome everyone to share experiences and disseminate knowledge.

Sincerely,

Dr. Ricardo Santos de Oliveira
President of the 10th CLANPED

Bilateral pallidotomy to status dystonicus in children from public health system: case series and technical notes

Antônio Jorge Barbosa de Oliveira — 2025-09-13

Background: Dystonia is the second most common pediatric movement disorder after spasticity and may occasionally present as status dystonicus (SD), a life-threatening neurological emergency. Medical management of SD remains challenging and sometimes ineffective, therefore neurosurgical alternatives such as intrathecal/intraventricular baclofen administration, deep brain stimulation and pallidotomy may be needed in severe refractory cases.
Objective: To evaluate the safety and effectiveness of bilateral pallidotomy in children with severe, drug-resistant dystonia, including cases of status dystonicus.
Methods: This retrospective study analyzed five pediatric patients (mean age: 8.23 years) treated between January 2024 and February 2025 at a tertiary public hospital in Brazil. All had a Dystonia Severity Score (DSS) ≥3 and underwent bilateral pallidotomy. Clinical outcomes, DSS changes, medication use, and postoperative medications were assessed.
Results: All patients showed clinical improvement, including resolution of status dystonicus. The average time to symptom stabilization was 6.8 days. The number of medications decreased by 22.86% postoperatively. No major complications were observed. Two patients had minor postoperative radiologic findings without clinical repercussions.
Conclusion: Bilateral pallidotomy appears to be a safe and effective option in the management of severe, drug-resistant dystonia in children, particularly status dystonicus. These preliminary results highlight its potential role in selected pediatric cases. Larger studies are needed to confirm long-term efficiency and safety of bilateral pallidotomy in this context

Special Issue Pediatric Functional Neurosurgery

Luciano Furlanetti — 2025-09-13

Dear Colleagues,

Pediatric functional neurosurgery encompasses a range of surgical interventions aimed at addressing complex neurological disorders in children, with the goal of improving function and quality of life. This field has made significant advancements in recent decades, with innovative techniques and technologies broadening its scope and efficacy. While conditions such as epilepsy, spasticity and movement disorders are well-established indications for functional neurosurgical interventions in children, emerging applications in neuropsychiatric and developmental disorders are under active investigation.

This Special Issue aimed to collect clinical and pre-clinical studies exploring the application of functional neurosurgical techniques in pediatric populations. We have received works focusing on novel indications, upcoming technologies, refined methodologies, and long-term outcomes in the management of neurological and neuropsychiatric disorders, including well documented case reports.

Barbosa et al have contributed with their experience with pallidotomies, intraventricular baclofen and combined selective dorsal and ventral rhizotomies in the management of severe spasticity and dystonia in children. Furlanetti et al have also contributed with an interesting case report and review of the literature on the application of combined limbic and sensorimotor pallidotomies in the management of Lesch-Nyhan syndrome. In line with this, Gregorio et al presented an interesting report and review on the application of intraventricular baclofen for the management of pelvic pain associated with spasticity in the context of cerebral palsy. Furlanetti et al and Arias et al discussed safety and efficacy of deep brain stimulation or pallidotomy combined with intrathecal baclofen in the management of severe refractory spasticity and dystonia in cerebral palsy, showing that in such cases multimodal neurosurgical techniques may be required to provide better quality of life for patients and caregivers. Bulhoes et al addressed the current utility of selective peripheral neurectomies in the management of spasticity and dystonia should still be considered in cases of focal or segmental clinical manifestations. Khairani et al closed-up the special issue reporting their experience with vagal nerve stimulation in the treatment of severe refractory status epilepticus.

We hope this very interesting articles will contribute to a deeper understanding of the mechanisms, challenges, and future directions in pediatric functional neurosurgery and help define its evolving role in treating the most challenging conditions affecting the developing brain.

Dr. Luciano Furlanetti

Dr. Ricardo Gepp

Guest Editors