Journal of Korean Neurosurgical Society最新文献

Objective: This study aimed to assess the safety and efficacy of using the PEAK Plasmablade TMX (PBX) in deep brain stimulation (DBS) of implantable pulse generator (IPG) replacement surgeries, alongside identifying potential risk factors for postoperative complications.

Methods: A prospective study was conducted on 50 patients undergoing IPG replacement with PBX, compared to 150 historical controls. Demographic data, surgical characteristics, and postoperative outcomes were analyzed. Logistic regression was used to identify predictors of surgical complications.

Results: The demographic and clinical profiles of patients in the PBX group were comparable to those in the control group. PBX significantly reduced surgical duration (p=0.005) and did not result in impedance abnormalities. While wound complications did not significantly differ between groups, logistic regression identified diabetes as a significant predictor of wound dehiscence or delayed healing (p=0.012).

Conclusion: The findings support the safety and efficacy of PBX in DBS IPG replacement surgeries, offering advantages such as reduced surgical duration and minimized risk of impedance abnormalities. However, diabetes emerged as a significant predictor of adverse wound outcomes, highlighting the need for tailored preoperative assessment and management strategies.

Objective: This study aims to evaluate the efficacy of the multiplanar deformity reducer (MDR) and direct vertebral rotation (DVR) techniques in surgically treating adolescent idiopathic scoliosis (AIS), focusing on surgical and radiographic outcomes.

Methods: A retrospective analysis was conducted on AIS patients who underwent surgery between December 2017 and August 2019, comparing the outcomes of those treated with MDR system and DVR technique. Data on demographics and surgical characteristics were collected, while radiographic parameters were measured manually by three spine surgeons and by EOS 3D analysis (EOS imaging, Paris, France).

Results: Nine patients were surgically corrected with the MDR system and 13 with DVR, who were predominantly females with an average age in their late teens. Despite more fusion levels in the MDR group (12.3±1.0) compared to the DVR group (10.0±3.2), operation time, blood loss, overall radiographic correction, and patient-reported outcome was comparable. Moreover, only the interrater reliability for manual apical vertebral rotation measurements was below good, which necessitates the use of EOS 3D analysis.

Conclusion: The MDR technique is effective and safe for AIS surgery, offering comparable corrective efficacy to the DVR technique. Furthermore, EOS 3D imaging was more reliable for assessing rotational deformities, which incorporates pelvic position.

Objective: Partially thrombosed intracranial aneurysms (PTIAs) are known to occur for both large and small aneurysms; however, standardized treatment guidelines remain undefined. This study aimed to evaluate and compare the efficacy of surgical and endovascular strategies for managing PTIAs to identify the optimal treatment approach.

Methods: A retrospective analysis was performed on patients diagnosed with PTIAs who underwent surgical or endovascular treatment at our institution from January 2005 to December 2022. Patients with intraluminal thrombi confirmed via brain imaging were categorized based on the treatment modality. A total of 45 patients were included. Clinical outcomes, including preoperative and postoperative modified Rankin Scale (mRS) scores, complication rates, and recurrent or remnant aneurysm rates, were reviewed to analyze the treatment results.

Results: Of the 45 enrolled patients, 31 patients (68.9%) underwent surgical treatment, and 14 patients (31.1%) received endovascular treatment. Surgical approaches included direct clipping, trapping with bypass, and aneurysm wrapping. Multivariate analysis revealed a statistically significant association between the treatment modality and recurrent or remnant aneurysms (P < 0.001). However, no significant differences were identified between the two treatment groups in terms of complication rates or functional outcomes.

Conclusion: In comparison to endovascular treatment, surgical management of PTIAs demonstrated superior efficacy in minimizing recurrent and remnant aneurysms. Considering the comparable rates of postoperative complications and functional outcomes, surgical treatment may be the preferred treatment strategy, particularly for younger patients with longer follow-up periods or for cases requiring decompression.

Rhabdoid tumor predisposition syndrome (RTPS) is a rare autosomal dominant disorder characterized by an increased risk of developing malignant rhabdoid tumors in early childhood. This syndrome is primarily caused by germline heterozygous loss-of-function pathogenic variants in the SMARCB1 gene (RTPS1) and rarely in the SMARCA4 gene (RTPS2). RTPS is characterized by the development of atypical teratoid rhabdoid tumors (ATRT) of the central nervous system, malignant rhabdoid tumors of the kidney (RTK), and/or extrarenal extracranial rhabdoid tumors (EERT). The syndrome demonstrates high penetrance, with most tumors developing before age 3 years, and carries a poor prognosis despite intensive multimodal therapy. Early diagnosis through genetic testing, implementation of surveillance protocols, and aggressive treatment approaches are crucial for improving outcomes. This review comprehensively examines the genetic basis, clinical manifestations, surveillance strategies, and current management approaches for RTPS, with particular emphasis on emerging therapeutic options and the importance of multidisciplinary care.

Objective: This study aimed to evaluate the clinical and radiographic results of patients with adolescent idiopathic scoliosis (AIS) operated by a single neurosurgeon.

Methods: A total of 139 consecutive patients with AIS who underwent corrective surgery by a single neurosurgeon were investigated between January 2011 and January 2024. Segmental instrumentation with pedicle screws was utilized for deformity correction. Demographic information, structural type of curve using Lenke classification, degree of correction achieved, total numbers of fusion levels, complications, and clinical/radiographic results were evaluated.

Results: Most patients were adolescent girls (105 vs. 34) with an average age of 15.9 vs. 16.0, respectively. The average duration of follow-up was 4.5 years (0.1-13.0 years). Nine (6.6%) patients had levoscoliosis, and the remaining 130 had dextroscoliosis. Chiari malformation with syringomyelia was incidentally diagnosed in four (2.8%) patients. Of the patients having Chiari malformation, two (50%) had levoscoliosis. The mean number of fused levels was 11.2. The mean value of the Cobb angle at the major structural curvature was 63.4° (43°-125°). The major structural curve's average percentage of correction was 78.8%. The final postoperative SRS-22 average score significantly improved to 4.3±0.4 compared with the preoperative score of 3.8±0.5 (P < 0.001). After the surgery, there were no observed cases of neurological deficits or need for screw repositioning. Revision fusion extension surgery was performed on one patient to treat postoperative shoulder imbalance.

Conclusion: Patients with AIS treated by a neurosurgeon had acceptable clinical and radiographic results. However, AIS surgery necessitates a fundamental comprehension of pediatric spinal deformities, which is crucial. Furthermore, spine surgeons should be careful with patients who have levoscoliosis.

Von Hippel-Lindau (VHL) disease is a genetic condition predisposing individuals to the development of benign and malignant tumors across various organs. This review explores the intricate genetic underpinnings of VHL disease, its clinical manifestations, and the associated malignancy risks. The latest diagnostic criteria, surveillance guidelines, and advancements in therapeutic strategies, including the Food and Drug Administration-approved hypoxia-inducible factor-2α inhibitor, Belzutifan, are focused on. Through a multidisciplinary approach, tailored surveillance programs aim to improve patient outcomes while balancing intervention risks. Emerging technologies such as whole-body magnetic resonance imaging and liquid biopsies hold promises for enhancing non-invasive surveillance. This review underscores the significance of ongoing research and interdisciplinary care in managing this complex syndrome.

Neurofibromatosis type 2 (NF2) is an autosomal dominant disease characterized by bilateral vestibular schwannomas and other central nervous tumors such as meningiomas and spinal ependymomas. Symptoms vary according to the age at diagnosis and the location of these tumors. The diagnostic criteria of NF2 have been regularly revised and recently updated in 2022 with a new nomenclature "NF2-related schwannomatosis" to differentiate NF2 from other schwannoma predisposing disorders, such as SMARCB1-, LZTR1-, and 22q-related schwannomatosis. Addition to the clinical features, genetic testing for pathogenic variants in these genes became an important essence to support diagnosis of NF2 and other schwannomatosis, including mosaic conditions.

Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder caused by pathogenic variants of TSC1 or TSC2 genes, leading to dysregulation of the mammalian target of rapamycin (mTOR) pathway. This dysregulation results in the formation of organ-specific tumors and neurological manifestations such as seizures, intellectual disability, and developmental delays. These characteristic clinical features are crucial for diagnosis, and genetic testing is playing an increasingly significant role. Long-term disease monitoring and appropriate interventions by multidisciplinary experts, including the use of mTOR inhibitors and promising therapeutic agents based on disease pathomechanisms, are essential for effective TSC management and improved clinical outcomes.

Schwannomatosis (SWN) is now recognized as a broad classification that includes neurofibromatosis (NF) type 2, reflecting their shared genetic and phenotypic characteristics. Previously, SWN and NF type 2 were considered distinct clinical entities; however, the 2022 classification revision has unified them under the umbrella of SWN, with NF type 2 now referred to as NF2-related SWN. SWN arises from mutations in NF2, SMARCB1 or LZTR1. Recent diagnostic criteria for SWN incorporate molecular classification, including "NF2-related SWN", "SMARCB1-related SWN", "LZTR1-related SWN", "22q-related SWN", "SWN-not otherwise specified", or "SWN-not elsewhere classified". NF2-related SWN is a genetic condition where all individuals with a germline or constitutional NF2 mutation are destined to develop the disease. The pathogenesis of SMARCB1- or LZTR1-related SWN follows a three-step, four-hit model. This involves retention of the mutated germline SMARCB1 or LZTR1 allele in the tumor, loss of the wild-type chromosome 22, and somatic mutation in the NF2 gene. Clinically, NF2-related SWN involves bilateral vestibular schwannomas, with treatment options including microsurgery, radiotherapy, and bevacizumab, each with specific benefits and limitations. Patients with SWN frequently present with chronic pain caused by schwannomas, which often does not correlate with tumor size, location, or burden. Management of SWN is primarily symptom-based. Surgical intervention is reserved for symptomatic lesions, particularly in cases of spinal cord compression or significant functional impairments. Multidisciplinary approaches to pain management are critical for enhancing quality of life. Although malignant transformation of schwannomas is a potential risk, the life expectancy of individuals with SWN is nearly normal. Despite advancements in understanding SWN, further research is necessary to elucidate the underlying genetic mechanisms and to develop targeted therapeutic strategies for this complex disorder.