Added value of corpus callosotomy following vagus nerve stimulation in children with Lennox-Gastaut syndrome: A multicenter, multinational study.

OBJECTIVE: Lennox-Gastaut syndrome (LGS) is a severe form of epileptic encephalopathy, presenting during the first years of life, and is very resistant to treatment. Once medical therapy has failed, palliative surgeries such as vagus nerve stimulation (VNS) or corpus callosotomy (CC) are considered. Although CC is more effective than VNS as the primary neurosurgical treatment for LGS-associated drop attacks, there are limited data regarding the added value of CC following VNS. This study aimed to assess the effectiveness of CC preceded by VNS. METHODS: This multinational, multicenter retrospective study focuses on LGS children who underwent CC before the age of 18 years, following prior VNS, which failed to achieve satisfactory seizure control. Collected data included epilepsy characteristics, surgical details, epilepsy outcomes, and complications. The primary outcome of this study was a 50% reduction in drop attacks. RESULTS: A total of 127 cases were reviewed (80 males). The median age at epilepsy onset was 6 months (interquartile range [IQR] = 3.12-22.75). The median age at VNS surgery was 7 years (IQR = 4-10), and CC was performed at a median age of 11 years (IQR = 8.76-15). The dominant seizure type was drop attacks (tonic or atonic) in 102 patients. Eighty-six patients underwent a single-stage complete CC, and 41 an anterior callosotomy. Ten patients who did not initially have a complete CC underwent a second surgery for completion of CC due to seizure persistence. Overall, there was at least a 50% reduction in drop attacks and other seizures in 83% and 60%, respectively. Permanent morbidity occurred in 1.5%, with no mortality. SIGNIFICANCE: CC is vital in seizure control in children with LGS in whom VNS has failed. Surgical risks are low. A complete CC has a tendency toward better effectiveness than anterior CC for some seizure types.

Novel "T-Dimension" Therapies for Pediatric Optic Pathway Glioma: A Timely, Targeted, and Tailored Treatment Trend.

INTRODUCTION: Novel targeted and tailored therapies can substantially improve the prognosis for optic pathway glioma (OPG), especially when implemented in a timely manner. However, their tremendous potential remains underestimated. Therefore, in this study, we provide an updated overview of the clinical trials, current trends, and future perspectives for OPG's novel therapeutic strategies. METHODS: We completed an extensive literature review using the PubMed, MEDLINE, and ClinicalTrials.gov databases. We analyzed and reported the data following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. RESULTS: Thioguanine, procarbazine, lomustine, and vincristine/vinblastine, as well as cisplatin-etoposide, provided excellent results in advanced-phase trials. Selumetinib and trametinib, two oral MEK inhibitors, have been approved for recurrent or refractory OPGs in association with the angiogenetic inhibitor bevacizumab. Among the mTOR inhibitors, everolimus and sirolimus showed the best results. Stereotactic radiosurgery and proton beam radiation therapy have advantages over conventional radiotherapy regimens. Timely treatment is imperative for acute visual symptoms with evidence of tumor progression. This latest evidence can help define a novel "T-Dimension" for pediatric OPG therapies. CONCLUSION: The novel "T-Dimension" for pediatric OPGs is based on recent evidence-based treatments, including combination chemotherapy regimens, molecular targeted therapies, stereotactic radiosurgery, and proton beam radiation therapy. Additional clinical trials are essential for validating each of these new therapies.

Shunt technology for infants and a lifetime.

The use of cerebrospinal fluid (CSF) shunts remains a fundamental therapeutic modality in the management of hydrocephalus. Nowadays, neurosurgeons have an arsenal of different shunt technologies on their hands, with several companies producing many different configurations of them. The greatest difficulty of treating a child with hydrocephalus is to deal with a brain that will enormously change its size and hydrodynamic conditions and a body that will multiply its height and weight in a short time. Detailed knowledge of the hydrodynamic properties of shunts is mandatory for any neurosurgeon and much more for those taking care of pediatric patients. It is necessary to know that these properties of the valve may influence the evolution of the patient after shunting and it is recognized that a patient physiology-specific valve selection may yield better outcomes and decrease complications. This article provides a summary of the most common available CSF valves and overdrainage control devices, their technology, and possible combinations. The objective is to offer a quick overview of the armamentarium to facilitate the recognition of the implanted device and improve the selection of the most suitable valve for each patient.

Pediatric cerebellar pilocytic astrocytoma presenting with spontaneous intratumoral hemorrhage.

Pilocytic astrocytomas (PAs) are benign glial tumors and one of the most common childhood posterior fossa tumors. Spontaneous intratumoral hemorrhage in PAs occurs occasionally, in about 8-20% of cases. Cerebellar hemorrhages in pediatric population are rare and mainly due to head injuries, rupture of vascular malformations, infections, or hematological diseases. We have investigated the still controversial and unclear pathophysiology underlying intratumoral hemorrhage in PAs. Bleeding in low-grade tumors might be related to structural abnormalities and specific angio-architecture of tumor vessels, such as degenerative mural hyalinization, "glomeruloid" endothelial proliferation, presence of encased micro-aneurysms, and glioma-induced neoangiogenesis. The acute hemorrhagic presentation of cerebellar PA in childhood although extremely uncommon is of critical clinical importance and necessitates promptly treatment. We described a case of hemorrhagic cerebellar PA in a 9-year-old child and reviewed the English-language literature that reported spontaneous hemorrhagic histologically proven cerebellar PA in pediatric patients (0-18 years). According to our analysis, the mortality was not related to symptom onset, tumor location, hemorrhage distribution, presence of acute hydrocephalous, and timing of surgery, while the GCS at hospital admission resulted to be the only statistically significant prognostic factor affecting survival outcome. The abrupt onset of signs and symptoms of acute hydrocephalous and consequent raised intracranial pressure are life-threatening conditions, which need emergent medical and neurosurgical treatments. At a later time, the identification of posterior fossa hemorrhage etiology is crucial to select the appropriate treatment and address the surgical strategy, optimizing the postoperative results.

Surgical management of craniopharyngiomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section.

BACKGROUND AND OBJECTIVE: Craniopharyngiomas are locally aggressive neuroepithelial tumors infiltrating nearby critical neurovascular structures. The majority of published surgical series deal with childhood-onset craniopharyngiomas, while the optimal surgical management for adult-onset tumors remains unclear. The aim of this paper is to summarize the main principles defining the surgical strategy for the management of craniopharyngiomas in adult patients through an extensive systematic literature review in order to formulate a series of recommendations. MATERIAL AND METHODS: The MEDLINE database was systematically reviewed (January 1970-February 2019) to identify pertinent articles dealing with the surgical management of adult-onset craniopharyngiomas. A summary of literature evidence was proposed after discussion within the EANS skull base section. RESULTS: The EANS task force formulated 13 recommendations and 4 suggestions. Treatment of these patients should be performed in tertiary referral centers. The endonasal approach is presently recommended for midline craniopharyngiomas because of the improved GTR and superior endocrinological and visual outcomes. The rate of CSF leak has strongly diminished with the use of the multilayer reconstruction technique. Transcranial approaches are recommended for tumors presenting lateral extensions or purely intraventricular. Independent of the technique, a maximal but hypothalamic-sparing resection should be performed to limit the occurrence of postoperative hypothalamic syndromes and metabolic complications. Similar principles should also be applied for tumor recurrences. Radiotherapy or intracystic agents are alternative treatments when no further surgery is possible. A multidisciplinary long-term follow-up is necessary.

Postoperative Immobilization following Occipitocervical Fusion in the Pediatric Population: Outcome Evaluation and Review of Literature.

The scientific literature does not have a consensus about the role and method of postoperative immobilization after occipitocervical fusion in the pediatric population. The primary goal of this study is to review the medical literature and evaluate different immobilization methods and their impact on fusion, following the surgical management of craniocervical instability in children. It started with an extensive research of randomized controlled trials, series of cases and case reports, describing occipitocervical junction pathologies, clinical, epidemiological characteristics, and treatment. The search was performed using the Pubmed database evaluating all the literature involving postoperative immobilization after occipitocervical fusion in pediatric patients. The results showed that most cases of occipitocervical stabilization were due to congenital spinal instability followed by trauma in most series. The most common type of surgery performed was occipitocervical fusion using screw and rod constructs. The different methods of postoperative immobilization did not affect outcomes. Then, we can conclude that screw-and-rod constructions in occipitocervical fusion augment the rates of fusion, independently from which immobilization was used, even when none was used at all.

First 60 fetal in-utero myelomeningocele repairs at Saint Louis Fetal Care Institute in the post-MOMS trial era: hydrocephalus treatment outcomes (endoscopic third ventriculostomy versus ventriculo-peritoneal shunt).

INTRODUCTION: The published results of the Management of Myelomeningocele Study (MOMS) trial in 2011 showed improved outcomes (reduced need for shunting, decreased incidence of Chiari II malformation, and improved scores of mental development and motor function) in the fetal prenatal repair group compared to the postnatal group. Historically, endoscopic third ventriculostomy (ETV) remains as a controversial hydrocephalus treatment option with high failure rates in pediatric patients with a history of myelomeningocele (MMC). We report hydrocephalus treatment outcomes in the fetal in-utero myelomeningocele repair patients who underwent repair at our Saint Louis Fetal Care Institute following the MOMS trial. We looked carefully at ETV outcomes in this patient population and we identified risk factors for failure. METHODS: At our Saint Louis Fetal Care Institute, we followed the maternal and fetal inclusion and exclusion criteria used by the MOMS trial. The records of our first 60 fetal MMC repairs performed at our institute between 2011 and 2017 were examined. We retrospectively reviewed the charts, prenatal fetal magnetic resonance imaging (MRI) and ultrasound (US) imaging findings, postnatal brain MRI, and Bayley neurodevelopment testing results for infants and children who underwent surgical treatment of symptomatic hydrocephalus (VP shunt versus ETV). Multiple variables possibly related to ETV failure were considered for identifying risk factors for ETV failure. RESULTS: Between May 2011 and March 2017, 60 pregnant female patients underwent the prenatal MMC repair for their fetuses between 20 and 26 weeks' gestational age (GA) utilizing the standard hysterotomy for exposure of the fetus, and microsurgical repair of the MMC defect. All MMC defects underwent successful in-utero repair, with subsequent progression of the pregnancy. At the time of this study, 58 babies have been born, 56 are alive since there were 2 mortalities in the neonatal period due to prematurity. One patient was excluded given lack of consent for research purposes. From the remaining 55 patient included in this study, a total of 30 infants and toddlers underwent treatment of hydrocephalus (ETV and VPS groups). Twenty-five patients underwent ETV (24 primary ETV and 1 after shunt failure). Nineteen patients underwent shunt placements (6 primary/13 after ETV failure). Mean GA at time of MMC repair for the ETV group was 24 + 6/7 weeks (range 22 + 4/7 to 25 + 6/7). Mean follow up for patients who had a successful ETV was 17.25 months (range 4-57 months). Bayley neurodevelopmental testing results were examined pre- and post-ETV. Overall ETV success rate was 11/24 (45.8%) at the time of this study. The total number of patients who underwent shunt placement was 19/55 (34.5%), while shunting rate was 40% in the MOMS trial. Using a simple logistic regression analysis to identify predictors of ETV failure, ETV age ≤6 months and gestational age ≥23 weeks at repair of myelomeningocele were significant predictors for ETV failure while in-utero ventricular stability ≤4 mm and in-utero ventricular size post-repair ≤15.5 mm were significant predictors for ETV success. None of the listed variables independently predicted classification into ETV success versus ETV failure groups when entered into multiple logistic regression analysis. CONCLUSIONS: ETV, as an alternative to initial shunting, may continue to show promising results for treating fetal MMC repair patient population who present with symptomatic hydrocephalus during infancy and early childhood. Although our overall CSF diversion rate (ETV and VPS groups) in our fetal MMC group is higher than the MOMS trial, our shunting rate is lower given our higher incidence of patients with successful ETV. To our knowledge, this is the largest reported ETV series in patients who underwent fetal MMC repair. ETV deserves a closer look in the setting of improved hindbrain herniation in fetal in-utero MMC repair patients. In our series, young age (less than 6 months) and late GA at time of fetal MMC repair (after 23 weeks GA) were predictors for ETV failure, while in-utero stability of ventricular size (less than 4 mm) and in-utero ventricular size post-repair ≤15.5 mm were predictors for ETV success. Larger series and potential prospective randomized studies are required for further evaluation of risk factors for ETV failure in the fetal MMC patient population.

Management of concomitant scoliosis and tethered cord syndrome in non-spina bifida pediatric population.

BACKGROUND: The management of concomitant scoliosis and tethered cord syndrome in the non-spina bifida pediatric population is challenging. In the present study, we evaluate the efficacy of different treatment modalities and propose a treatment plan for the management of affected patients. METHODS: A systematic literature review was conducted by querying the MEDLINE, PubMed, Cochrane, EMBASE, Scopus, and Web of Science databases for papers published between January 1996 and June 2016 and reporting on concomitant scoliosis and tethered cord. We excluded animal studies, non-English papers as well as papers reporting on patients with multiple concomitant intraspinal anomalies such as spina bifida. RESULTS: Out of 1993 articles, only 13 met our inclusion criteria. These 13 articles described six main management approaches: Observation, cord untethering only, cord untethering followed by deformity correction, simultaneous cord untethering and deformity correction, and deformity correction without untethering. Selection of the best approach is a function of the patient's symptomatology and Cobb angle. CONCLUSION: We propose treatment plan for the management of patients with concomitant tethered cord syndrome and spinal deformity. Asymptomatic patients can be followed conservatively and managed as scoliosis patients with no need for untethering. Surgical management in a staged fashion seems appropriate in symptomatic patients with a Cobb angle less than 35°. In these patients, deformity can improve following untethering, thus sparing the patient the risks of surgical correction of scoliosis. Staged or non-staged cord untethering and curve correction seem to be adequate in symptomatic patients with Cobb angle >35° as these patients are likely to require both untethering and deformity correction.

H3 K27M mutations are extremely rare in posterior fossa group A ependymoma.

BACKGROUND: Mutations in the tail of histone H3 (K27M) are frequently found in pediatric midline high-grade glioma's but have rarely been reported in other malignancies. Recently, recurrent somatic nucleotide variants in histone H3 (H3 K27M) have been reported in group A posterior fossa ependymoma (EPN_PFA), an entity previously described to have no recurrent mutations. However, the true incidence of H3 K27M mutations in EPN_PFA is unknown. METHODS: In order to discern the frequency of K27M mutations in histone H3 in EPN_PFA, we analyzed 151 EPN_PFA previously profiled with genome-wide methylation arrays using a validated droplet digital PCR assay. RESULTS: We identified only 1 case out of 151 EPN_PFA harboring the K27M mutation indicating that histone mutations are extremely rare in EPN_PFA. Morphologically, this single mutated case is clearly consistent with an ependymoma, and the presence of the K27M mutation was confirmed using immunohistochemistry. DISCUSSION: K27M mutations are extremely rare in EPN_PFA. Routine evaluation of K27M mutations in EPN_PFA is of limited utility, and is unlikely to have any bearing on prognosis and/or future risk stratification.

The paramedian supracerebellar transtentorial approach to the posterior fusiform gyrus.

BACKGROUND: The posterior fusiform gyrus lies in a surgically challenging region. Several approaches have been described to access this anatomical area. The paramedian supracerebellar transtentorial (SCTT) approach benefits from minimal disruption of normal neurovascular tissue. The aim of this study was to demonstrate its application to access the posterior fusiform gyrus. METHODS: Three brains and six cadaveric heads were examined. A stepwise dissection of the SCTT approach to the posterior fusiform gyrus was performed. Local cortical anatomy was studied. The operability score was applied for comparative analysis on surgical anatomy. RESULTS: The major posterior landmark used to identify the fusiform gyrus with respect to the medial occipitotemporal gyrus was the collateral sulcus, which commonly bifurcated at its caudal extent. Compared with other surgical approaches addressed to access the region, SCTT demonstrated the best operability in terms of maneuverability arc. Favorable tentorial anatomy is the only limiting factor. CONCLUSIONS: The supracerebellar transtentorial approach is able to provide access to the posterior fusiform gyrus via a minimally disruptive, anatomic, microsurgical corridor.

A systematic review of neuropsychological outcomes following posterior fossa tumor surgery in children.

PURPOSE: Central nervous system tumors are the most common solid tumors in the pediatric population. As children with central nervous system (CNS) tumors are surviving into adolescence and adulthood, more research is being focused on the long-term cognitive outcomes of the survivors. This review examines the literature on different cognitive outcomes of survivors of different childhood posterior fossa CNS tumor types. METHODS: The authors reviewed the literature for articles published from 2000 to 2012 about long-term neuropsychological outcomes of children diagnosed with posterior fossa brain tumors before the age of 18, which distinguished between histological tumor types, and had a minimum follow-up of 3 years. RESULTS: The literature search returned 13 articles, and a descriptive analysis was performed comparing intelligence quotient (IQ), attention/executive function, and memory components of 456 survivors of childhood posterior fossa tumors. Four articles directly compared astrocytoma and medulloblastoma survivors and showed medulloblastoma survivors fared worse in IQ, attention/executive function, and memory measurements. Five articles reporting medulloblastomas found IQ, attention, and memory scores to be significantly below the standardized means. Articles examining astrocytoma survivors found IQ scores within the normal range for the population. Survivors of ependymomas reported 2/23 survivors impaired on IQ scores, while a second study reported a significant number of ependymoma survivors lower than the expected population norm. CONCLUSIONS: Tumor histopathology and the type of postoperative adjuvant therapy seem to have a significant impact on the long-term neuropsychological complications of pediatric posterior fossa CNS tumor survivors. Age at diagnosis and treatment factors are important variables that affect the outcomes of the survivors.

Impact of DTI tractography on surgical planning for resection of a pediatric pre-pontine neurenteric cyst: a case discussion and literature review.

We report a case of a four-year-old male who presented with symptoms of brainstem compression and lower cranial nerve neuropathies. MRI revealed a large, pre-pontine mass causing brainstem compression with an uncertain intra-axial component. Using diffusion tensor imaging (DTI) tractography and other imaging modalities, we were able to confirm that the lesion was extra-axial and did not involve the corticospinal tracts. In addition, DTI tractography illustrated that corticospinal tracts were displaced to the right obligating a left-sided approach. Upon resection, the mass was identified as a pre-pontine, extra-axial neurenteric cyst (NEC), which represents a rare finding in the pediatric population. The patient ultimately did well following the drainage and resection of the cyst wall and had excellent recovery. In this paper, we discuss the pathophysiology of and treatment options for NECs and explain how DTI tractography in our case assisted in planning the surgical approach.

Holocord syringomyelia secondary to tethered spinal cord associated with anterior sacral meningocele and tailgut cyst: case report and review of literature.

BACKGROUND AND IMPORTANCE: Anterior sacral meningoceles are lesions that are uncommonly reported and can be associated with other pathology including presacral masses, tethered spinal cord, and syringomyelia. Tethered spinal cord and syringomyelia can result in neurologic deficits, while large meningoceles and presacral masses can have gastroenterologic, urologic, reproductive, and oncologic consequences. CLINICAL PRESENTATION: The authors report a case of a 14-year-old girl with an anterior sacral meningocele, tailgut cyst, and tethered cord with holocord syringomyelia who presented with a tethered cord syndrome, manifested by constipation, urinary retention, bilateral lower extremity weakness, and sensory deficits. After extensive radiographic and urodynamic workups were performed, the patient was treated by the neurosurgery and pediatric surgery teams with a posterior sagittal approach for cord detethering, resection of an intradural cystic mass, resection of the anterior sacral meningocele, and resection of the adjacent presacral mass. After surgical treatment, motor weakness and sensory deficits were resolved, though urinary symptoms persisted. The syrinx resolved after detethering alone. Pathology of the intradural cystic mass and the presacral mass inferior to the anterior sacral meningocele were consistent with tailgut cyst. CONCLUSION: The patient's clinical and surgical management are discussed, and a literature review related to anterior sacral meningoceles and their related pathologies is presented. An interdisciplinary approach is required for the best treatment of this constellation of findings.

Cranio-Orbito-Zygomatic Approach: Core Techniques for Tailoring Target Exposure and Surgical Freedom.

BACKGROUND: The cranio-orbito-zygomatic (COZ) approach is a workhorse of skull base surgery, and each of its steps has a precise effect on target exposure and surgical freedom. The present study overviews the key techniques for execution and tailoring of the COZ approach, focusing on the quantitative effects resulting from removal of the orbitozygomatic (OZ) bar, orbital rim, and zygomatic arch. METHODS: A PRISMA-based literature review was performed on the PubMed/Medline and Web of Science databases using the main keywords associated with the COZ approach. Articles in English without temporal restriction were included. Eligibility was limited to neurosurgical relevance. RESULTS: A total of 78 articles were selected. The range of variants of the COZ approach involves a one-piece, two-piece, and three-piece technique, with a decreasing level of complexity and risk of complications. The two-piece technique includes an OZ and orbitopterional variant. Superolateral orbitotomy expands the subfrontal and transsylvian corridors, increasing surgical freedom to the basal forebrain, hypothalamic region, interpeduncular fossa, and basilar apex. Zygomatic osteotomy shortens the working distance of the pretemporal and subtemporal routes. CONCLUSION: Subtraction of the OZ bar causes a tremendous increase in angular exposure of the subfrontal, transsylvian, pretemporal, and subtemporal perspectives avoiding brain retraction, allowing for multiangled trajectories, and shortening the working distance. The COZ approach can be tailored based on the location of the lesion, thus optimizing the target exposure and surgical freedom and decreasing the risk of complications.

Future Directions for Global Clinical Neurosurgical Training: Challenges and Opportunities.

OBJECTIVE: Expanded access to training opportunities is necessary to address 5 million essential neurosurgical cases not performed annually, nearly all in low- and middle-income countries. To target this critical neurosurgical workforce issue and advance positive collaborations, a summit (Global Neurosurgery 2019: A Practical Symposium) was designed to assemble stakeholders in global neurosurgical clinical education to discuss innovative platforms for clinical neurosurgery fellowships. METHODS: The Global Neurosurgery Education Summit was held in November 2021, with 30 presentations from directors and trainees in existing global neurosurgical clinical fellowships. Presenters were selected based on chain referral sampling from suggestions made primarily from young neurosurgeons in low- and middle-income countries. Presentations focused on the perspectives of hosts, local champions, and trainees on clinical global neurosurgery fellowships and virtual learning resources. This conference sought to identify factors for success in overcoming barriers to improving access, equity, throughput, and quality of clinical global neurosurgery fellowships. A preconference survey was disseminated to attendees. RESULTS: Presentations included in-country training courses, twinning programs, provision of surgical laboratories and resources, existing virtual educational resources, and virtual teaching technologies, with reference to their applicability to hybrid training fellowships. Virtual learning resources developed during the coronavirus disease 2019 pandemic and high-fidelity surgical simulators were presented, some for the first time to this audience. CONCLUSIONS: The summit provided a forum for discussion of challenges and opportunities for developing a collaborative consortium capable of designing a pilot program for efficient, sustainable, accessible, and affordable clinical neurosurgery fellowship models for the future.

Three-Dimensional Volumetric Assessment of Resected Gliomas Assisted by Horos Imaging Software: Video Case Series of Postoperative Tumor Analyses.

Horos (LGPL 3.0; GNU Lesser General Public License, Version 3) is a free, open-source medical image viewer with a user-friendly interface and three-dimensional (3D) volumetric rendering capabilities. We present the use of Horos software as a postoperative tool for residual tumor volume analysis in children with high-grade gliomas (HGG). This is a case series of two pediatric patients with histologically confirmed high-grade gliomas who underwent tumor resection as definitive treatment from June 2011 to June 2019. Volumetric data and extent of resection were obtained via region of interest-based 3D analysis using Horos image-processing software. Horos software provides increased accuracy and confidence in determining the postoperative volume and is useful in assessing the impact of residual volume on outcomes in patients with high-grade gliomas. Horos software is a highly effective means of volumetric analysis for the postoperative analysis of residual volume after maximal safe resection of high-grade gliomas in pediatric patients.

Pulsatile cerebrospinal fluid dynamics in Chiari I malformation syringomyelia: Predictive value in posterior fossa decompression and insights into the syringogenesis.

BACKGROUND: Pathophysiological mechanisms underlying the syringomyelia associated with Chiari I malformation (CM-1) are still not completely understood, and reliable predictors of the outcome of posterior fossa decompression (PFD) are lacking accordingly. The reported prospective case-series study aimed to prove the existence of a pulsatile, biphasic systolic-diastolic cerebrospinal fluid (CSF) dynamics inside the syrinx associated with CM-1 and to assess its predictive value of patients' outcome after PFD. Insights into the syringogenesis are also reported. METHODS: Fourteen patients with symptomatic CM-1 syringomyelia underwent to a preoperative neuroimaging study protocol involving conventional T1/T2 and cardiac-gated cine phase-contrast magnetic resonance imaging sequences. Peak systolic and diastolic velocities were acquired at four regions of interest (ROIs): syrinx, ventral, and dorsal cervical subarachnoid space and foramen magnum region. Data were reported as mean ± standard deviation. After PFD, the patients underwent a scheduled follow-up lasting 3 years. One-way analysis of variance with Bonferroni Post hoc test of multiple comparisons was performed P was <0.001. RESULTS: All symptoms but atrophy and spasticity improved. PFD caused a significant velocity changing of each ROI. Syrinx and premedullary cistern velocities were found to be decreased within the 1st month after PFD (<0.001). A caudad and cephalad CSF jet flow was found inside the syrinx during systole and diastole, respectively. CONCLUSION: Syrinx and premedullary cistern velocities are related to an early improvement of symptoms in patients with CM-1 syringomyelia who underwent PFD. The existence of a biphasic pulsatile systolic-diastolic CSF pattern inside the syrinx validates the "transmedullary" theory about the syringogenesis.

Anterolateral approach for subaxial vertebral artery decompression in the treatment of rotational occlusion syndrome: results of a personal series and technical note.

OBJECTIVES: To report the results of a retrospective series and a technical note about the anterolateral approach for the treatment of the rotational occlusion syndrome (ROS) involving the subaxial V2 segment of the vertebral artery (VA). METHODS: We retrospectively reviewed the data of a cohort of patients that underwent an anterolateral approach to decompress the VA as they suffered from ROS secondary to a subaxial compression. A dynamic study with ultrasonography, CT, MRI, and catheter-based angiography were obtained in all cases. Severe symptomatology and cerebellar-brainstem strokes were indications for surgery. The anterolateral approach involved a pre-sternocleidomastoid precarotid exposure. The retro-longus colli and pre-scalenic corridors were used to access the C5-C6 and C3-C4 segment, respectively, and to perform the decompression. RESULTS: Twelve patients were treated. Recurrent drop attacks were present in all cases. Osteophytes at C5 and C6 were the most common causes of subaxial VA compression. Anterior decompression stand-alone was performed in all but 1 patient. A recurrent laryngeal nerve palsy and a numbness of the C5 nerve root were the only complications observed, both transient. A satisfactory untethering of the VA with a complete recovery was achieved in all patients, apart from those with severe infratentorial strokes. DISCUSSION READ: Anterolateral approach allows for an effective and safe treatment of the ROS involving the subaxial portion of the VA. Retro-longus colli and pre-scalenic corridors, developed through a precarotid exposure, have an anatomical rationale in decreasing the risks of complications. Decompression stand-alone is adequate in almost the totality of cases.

Extreme Lateral Supracerebellar Infratentorial Approach: Surgical Anatomy and Review of the Literature.

OBJECTIVE: The extreme lateral supracerebellar infratentorial (ELSI) approach has the potential to access several distinct anatomical regions that are otherwise difficult to reach. We have illustrated the surgical anatomy through cadaveric dissections and provided an extensive review of the literature to highlight the versatility of this approach, its limits, and comparisons with alternative approaches. METHODS: The surgical anatomy of the ELSI has been described using 1 adult-injected cadaveric head. Formalized noninjected brain specimens were also dissected to describe the brain parenchymal anatomy of the region. An extensive review of the literature was performed according to each targeted anatomical region. Illustrative cases are also presented. RESULTS: The ELSI approach allows for wide exposure of the middle and posterolateral incisural spaces with direct access to centrally located intra-axial structures such as the splenium, pulvinar, brainstem, and mesial temporal lobe. In addition, for skull base extra-axial tumors such as petroclival meningiomas, the ELSI approach represents a rapid and adequate method of access without the use of extensive skull base approaches. CONCLUSIONS: The ELSI approach represents one of the most versatile approaches with respect to its ability to address several anatomical regions centered at the posterior and middle incisural spaces. For intra-axial pathologies, the approach allows for access to the central core of the brain with several advantages compared with alternate approaches that frequently involve significant brain retraction and cortical incisions. In specific cases of skull base lesions, the ELSI approach is an elegant alternative to traditionally used skull base approaches, thereby avoiding approach-related morbidity.