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Brain tumors represent the second most frequent etiology in patients with focal seizure onset before 18 years of age and submitted to epilepsy surgery. Hence, this category of brain tumors, herein defined as low-grade, developmental, epilepsy-associated brain tumors (LEAT) is different from those frequently encountered in adults as (A): 77% of LEAT occur in the temporal lobe; (B): the vast majority of LEAT are of low malignancy and classified as WHO I°; (C): LEAT are often composed of mixed glial and neuronal cell components and present with variable growth patterns including small cysts or nodules; (D): LEAT do not share common gene driving mutations, such as IDH1 or 1p/19q co-deletions. Characteristic entities comprise the ganglioglioma (GG), the dysembryoplastic neuroepithelial tumor (DNT), the angiocentric glioma (AG), the isomorphic diffuse glioma (IDG) and the papillary glio-neuronal tumor (PGNT), representing 73.2% of 1680 tumors collected in a large German series of 6747 patients submitted to epilepsy surgery. In the realm of exciting discoveries of genetic drivers of brain tumors new genes have been also reported for LEAT. BRAF V600E mutations were linked to GG with CD34 expression, FGFR1 mutations to DNT, MYB alterations to AG and also IDG and PRKCA fusions to PGNT, suggesting the possibility to also develop a genetically driven tumor classification scheme for LEAT. Rare availability of LEAT in a single center is a challenging obstacle, however, to systematically unravel the neurobiological nature and clinical behavior of LEAT. Other challenges in need of clarification include malignant tumor progression of LEAT entities, seizure relapse in patients following bulk tumor resection and the controversial issue of associated focal cortical dysplasia as additional pathomechanism. In order to advance our understanding and promote reliable diagnostic work-up of LEAT, we recommend, therefore, international collaboration to achieve our goals.

Background Spinal epidural arachnoid cysts (SEACs) are rare, non-neoplastic pathologies that can cause compressive myelopathy. Preoperative identification of the exact fistula location is crucial for minimally invasive management. Methods This single-center retrospective study included 27 patients with SEACs who underwent “double-needle puncture myelography” to precisely localize the fistula before minimally invasive surgery. Clinical presentations, radiological findings, and surgical outcomes were analyzed. Results Among the 27 patients, the most common clinical complaint was sensory dysfunction (14 patients, 51.9%). Motor dysfunction, manifested as lower limb weakness, was observed in 11 patients (40.7%), while 2 patients were asymptomatic at admission. Consistency analysis between radiographic localization and intraoperative findings demonstrated that the “double-needle puncture myelography” technique accurately identified the surgical segments and laterality of all fistulas except for one patient, who required repeat radiographic localization. Minimally invasive approaches, including hemilaminectomy or mini-hemilaminectomy, were successfully performed in 26 patients (96.3%) for fistula exposure. Conclusions The “double-needle puncture myelography” technique allows for the swift acquisition of precise anteroposterior and lateral projection dynamic X-ray images during the procedure. As a valuable preoperative diagnostic tool, it accurately pinpoints the location of the fistula, thereby minimizing surgical trauma and preserving spinal stability.

Background Spinal arachnoid cysts (SAC) and spinal arachnoid webs (SAW) are intradural pathologies, often presenting with pain and/or myelopathic symptoms. Reports on postoperative outcomes for SAC and SAW are scarce. This study aims to assess the surgical outcomes of SAC and SAW. Methods Patients who underwent surgical treatment for SAC or SAW at our institution between 2011 and 2023 were retrospectively reviewed. Demographic data, radiological findings, surgical management, and clinical outcomes were evaluated using the modified McCormick (mMC) scale. Outcomes were categorized as \"favorable\" (mMC I-II) or \"unfavorable\" (mMC III-V). Results A total of 35 patients (29 SAC, 6 SAW) were analyzed. Mean age was 50.9 (SD ± 9.8) years. Males comprised 70.3% of cases (SAC: 64.5%, SAW: 100%). Most common symptoms were local or radiating pain (SAC 77.4%; SAW 100%), gait-disturbance (SAC 45.2%; SAW 16.7%), sensory-deficits (SAC 32.3%; SAW 66.7%) and impaired motor function (SAC 20%; SAW 16.7%). Median preoperative mMC was 2 in both groups (SAC IQR: 1; SAW IQR: 1). Median postoperative mMC was 1 in both groups (SAC CI 1.07–1.34; SAW CI 0.74–1.60). Favorable outcome (postoperative mMC I-II) was achieved in 26 SAC patients (90%) and 5 SAW patients (83%) respectively. Surgical complications occurred in 14.7%, all in the SAC group, without lasting deficits. Conclusion Surgical treatment for SAC and SAW appears to have an overall favorable outcome, though larger cohort analyses are needed. Surgery should be considered in symptomatic patients.

Objective Spinal extradural arachnoid cysts (SEDC) are rare primary spinal lesions, accounting for less than 1% of all spinal epidural lesions. The literature contains only case reports of this pathology, and treatment remains controversial due to its rarity. Major reported SEDC cases are caused by leaking out of cerebrospinal fluid through a dural defect in the thecal sac forming an extradural cyst. Other reports describe non-communicating SEDC cases where the dural defect was not identified. We report a literature review on SEDC and the case of a 53 year‑old female who presented with type IA extradural cyst with subarachnoid space communication. Methods Literature review, preoperative imaging and surgical technique. Results The extradural cyst was excised completely and the dural defect was repaired. After surgical decompression, neurological symptoms gradually recovered. Conclusions The extradural arachnoid cyst is an uncommon entity. Preoperative imaging is one of the determining elements in orienting the therapeutic management of the SEDCs. The choice of the surgical technique must be the least invasive in order to avoid postoperative complications. Subtotal or complete excision of the cyst, followed by obliteration of the communication stalk and repair of the dural defect is the gold standard treatment.

No consensus stands regarding optimal treatment for pediatric middle fossa arachnoid cysts (MFACs). This study aims to compare interventions for management of pediatric MFACs. A systematic review was conducted per PRISMA guidelines using PubMed, Google Scholar, and Web of Science electronic databases to highlight management strategies of pediatric MFACs. Sixty two articles (22 retrospective studies, 40 case reports/series) were included. Across the retrospectives, 87.3 % patients were treated surgically and 24.0 % conservatively. Those treated surgically most underwent microscopic and endoscopic/keyhole fenestration. Microscopic fenestration was more frequently performed in ruptured cysts or cases with subdural hygroma (30.6 %, p = 0.044) while endoscopy was preferred for unruptured cysts (p = 0.005). Neurologic improvement was significantly greater with microscopic (99 %,) compared to endoscopic fenestration (91 %) (p = 0.01). Postoperative subdural fluid collections and mortality did not differ significantly between approaches. Conservative management primarily involved observation (24.6 %) and anticonvulsants (61 %). In case reports, 123 patients underwent surgery (102 open, 17 endoscopic), while 27 were managed conservatively. Microscopic fenestration correlated with preoperative cyst growth (OR: 37.63, p = 0.0004). Surgery carried greater complication risk (OR: Inf, p = 0.0014), while conservative management conferred higher cyst regression rates (OR: 20.84, p < 0.001). Common complications included subdural fluid and hygroma. Surgery is only warranted in severely symptomatic patients. Management of symptomatic, ruptured MFACs should target controlling acute subdural hygromas or hematomas. Microscopic and endoscopic surgeries yielded similar outcomes and complications. Most importantly, given the benign natural history of MFACs after the age of 4 years, most patients often require neither surgery nor follow-up imaging. •Most MFAC patients managed surgically; endoscopic and microscopic most common.•Surgery warranted only in severely symptomatic or ruptured MFACs.•Conservative management linked to higher cyst regression and fewer risks.•Microscopic fenestration may confer greater neurological improvement.•After age four, most MFACs remain stable without surgery or serial imaging.

Purpose Children diagnosed with suprasellar arachnoid cysts often concurrently have hydrocephalus. This study aims to classify the relationship between suprasellar arachnoid cysts and hydrocephalus, discussing surgical strategies—shunting or neuroendoscopic approaches—and their sequence, based on this classification. Methods A retrospective analysis was conducted on 14 patients diagnosed with suprasellar arachnoid cysts and hydrocephalus, treated surgically by the first author between January 2016 and December 2020. Clinical features, radiological findings, surgical strategies, and outcomes were reviewed. The classification of the relationship between the suprasellar arachnoid cysts and hydrocephalus was developed and illustrated with specific cases. Recommendations for future surgical management based on this classification are provided. Results We classified the relationship between suprasellar arachnoid cysts and hydrocephalus into three categories. SACH-R1, the direct type, represents cases where the cysts cause obstructive hydrocephalus. Here, neuroendoscopic ventriculocystocisternostomy (VCC) effectively treats both conditions. SACH-R2, the juxtaposed type, involves concurrent occurrences of cysts and hydrocephalus without a causative link. This is further subdivided into SACH-R2a, where acute progressive communicating hydrocephalus coexists with the cyst, initially managed with a ventriculoperitoneal shunt, followed by VCC upon stabilization of hydrocephalus; and SACH-R2b, where the cyst coexists with chronic stable communicating hydrocephalus, first addressed with VCC, followed by monitoring and potential secondary shunting if needed. Key factors differentiating SACH-R2a from SACH-R2b include the patient's age, imaging signs of fourth ventricle and cisterna magna enlargement, and the rapid progression or chronic stability and severity of hydrocephalus symptoms. SACH-R3, the reverse type, describes scenarios where shunting for hydrocephalus leads to the development or enlargement of the cyst, managed via neuroendoscopic VCC with precautions to prevent infections in existing shunt systems. Conclusion The simultaneous presence of suprasellar arachnoid cysts and hydrocephalus requires a nuanced understanding of their complex relationship for optimal surgical intervention. The analysis and classification of their relationship are crucial for determining appropriate surgical approaches, including the choice and sequence of shunting and neuroendoscopic techniques. Treatment should be tailored to the specific type identified, rather than blindly opting for neuroendoscopy. Particularly for SACH-R2a cases, we recommend initial ventriculoperitoneal shunting.

Background A retained medullary cord (RMC) is an uncommon, closed spinal dysraphism characterized by a robust extended cord-like structure that extends continuously from the conus medullaris to the dural cul-de-sac. There have been six reports of RMC extending to a related sacral subcutaneous meningocele. To the best of authors’ knowledge, the combination of retained medullary cord with thoracic arachnoid cyst has never been reported in the literature. Case presentation We present a case of the above combination in a 2-year-old child who underwent resection of RMC and fenestration of associated anteriorly placed arachnoid cyst under neuromonitoring. It is prudent to delineate functional and nonfunctional cord segment with neuromonitoring before truncating. The child improved significantly after surgery, and at 1-year follow-up, the child was able to walk with support. Conclusion Surgery for RMC involves delineation with neuromonitoring and resection of the nonfunctional cord. Fenestration of the arachnoid cyst must be included in surgical planning, which can be performed as a staged procedure or at same setting.

Arachnoid cysts are relatively common, but rarely require intervention. While most arachnoid cysts in typical middle or posterior cranial fossa locations are seldom symptomatic, suprasellar cysts may become symptomatic due to the potential for ventricular outflow obstruction and hydrocephalus. Typical standard of care for the treatment of these lesions is endoscopic fenestration with third ventriculostomy, or the placement of ventriculoperitoneal or cystoperitoneal shunts. The surgical and anaesthetic risks of traditional interventions may be higher in the early neonatal period, including leak of cerebrospinal fluid, infection, and premature failure of ventriculostomy or shunts. This note describes a novel bedside ultrasound-guided technique to percutaneously fenestrate large suprasellar arachnoid cysts under local anaesthesia. The technique involves insertion of a 25-g spinal needle until contact with the membrane of the arachnoid cyst medially, followed by a lateral sweeping to widely incise/fenestrate the lesion into the ventricular space under continuous ultrasound visualisation. This note describes an example case which demonstrates durable radiological and clinical improvement after 2 years of follow-up. This may represent a management option to temporise, or perhaps definitively manage suprasellar arachnoid cysts in the neonatal period.

Objective The best treatment strategies for cerebral arachnoid cysts (CAC) are still up for debate. In this study, we present CAC management, outcome data, and risk factors for recurrence after surgical treatment, focusing on microscopic/endoscopic approaches as compared to minimally invasive stereotactic procedures in children and adults. Methods In our single-institution retrospective database, we identified all patients treated surgically for newly diagnosed CAC between 2000 and 2022. Microscopic/endoscopic surgery (ME) aimed for safe cyst wall fenestration. Stereotactic implantation of an internal shunt catheter (STX) to drain CAC into the ventricles and/or cisterns was used as an alternative procedure in patients aged ≥ 3 years. Treatment decisions in favor of ME vs. STX were made by interdisciplinary consensus. The primary study endpoint was time to CAC recurrence (TTR). Secondary endpoints were outcome metrics including clinical symptoms and MR-morphological analyses. Data analysis included subdivision of the total cohort into three distinct age groups (AG1, < 6 years; AG2, 6–18 years; AG3, ≥ 18 years). Results Sixty-two patients (median age 26.5 years, range 0–82 years) were analyzed. AG1 included 15, AG2 10, and AG3 37 patients, respectively. The main presenting symptoms were headache and vertigo. In AG1 hygromas, an increase in head circumference and thinning of cranial calvaria were most frequent. Thirty-five patients underwent ME and 27 STX, respectively; frequency did not differ between AGs. There were two (22.2%) periprocedural venous complications in infants (4- and 10-month-old) during an attempt at prepontine fenestration of a complex CAC, one with fatal outcome in a 10-month-old boy. Other complications included postoperative bleeding (2, 22.2%), CSF leaks (4, 44.4%), and meningitis (1, 11.1%). Overall, clinical improvement and significant volume reduction ( p  = 0.008) were seen in all other patients; this did not differ between AGs. Median follow-up for all patients was 25.4 months (range, 3.1–87.1 months). Recurrent cysts were seen in 16.1%, independent of surgical procedure used ( p  = 0.7). In cases of recurrence, TTR was 7.9 ± 12.7 months. Preoperative ventricular expansion ( p  = 0.03), paresis ( p  = 0.008), and age under 6 years ( p  = 0.03) were significant risk factors for CAC recurrence in multivariate analysis. Conclusions In patients suffering from CAC, both ME and STX can improve clinical symptoms at low procedural risk, with equal extent of CAC volume reduction. However, in infants and young children, CAC are more often associated with severe clinical symptoms, stereotactic procedures have limited use, and microsurgery in the posterior fossa may bear the risk of severe venous bleeding.

Introduction Multiple synchronous spinal extradural arachnoid cysts are extremely rare in children and can lead to cord compression and disability. Multiple spinal extradural arachnoid cysts (SEDACs) in children with immature and growing spine need to be studied separately from multiple SEDACs in adults with mature and fully grown spine because of multiple surgical limitations in children (blood loss, surgery duration, long-term spinal stability after long segment exposure, etc.). Material and methods We planned a non-systematic literature review of similar cases described in literature to analyse the pattern of presentation, management, and outcome of this surgically curable disease. Results A total of 28 cases of paediatric multiple SEDACs in the age range of 5 months to 17 years and mean age of 11.54 years were analysed. Exposure was achieved by laminectomy in 9, laminoplastic laminotomy in 13, laminectomy in first surgery followed by laminoplasty in second surgery (re-exploration) in 1, hemilaminectomy in 2, and technique “not reported” in 3 children. Complete/near-complete recovery was seen in 27 (96.42%) children. Postoperative spine deformity was reported in 7 (25%) children and was progressive in 2 children during serial follow-up. Conclusions Symptomatic multiple synchronous SEDACs in children is a rare surgically curable condition. Sincere attempt to find out a dural defect in all cysts is a very important step in multiple SEDAC surgery. Total cyst wall excision with closure of all dural defects is the gold standard treatment for symptomatic cases. Laminoplasty is preferred for excision of multiple SEDACs in children to prevent postoperative spine deformity during long-term follow-up. Long serial follow-up for postoperative spine deformity is necessary.