Surgery versus endovascular treatment for spinal dural arteriovenous fistulas: a multicenter experience and systematic literature review.

Surgery and endovascular therapy are the primary treatment options for spinal dural arteriovenous fistula (SDAVF). Due to the absence of a consensus regarding which therapy yields a superior outcome, we conducted a comparative analysis of the surgical and endovascular treatment of SDAVF through a multicenter case series and a systematic literature review. Patients with SDAVF, surgically or endovascularly treated at four neurosurgical centers from January 2001 to December 2021, were included in this study. Level of SDAVF, primary treatment modality, baseline and post-procedural neurological status were collected. The primary outcomes were failure, complication rates, and a newly introduced parameter named as therapeutic delay. A systematic review of the literature was performed according to PRISMA-P guidelines. The systematic review identified 511 papers, of which 18 were eligible for analysis, for a total of 814 patients, predominantly male (72%) with a median age of 61 and mainly thoracic SDAVFs (65%). The failure rate was significantly higher for endovascular therapy (20%) compared to surgery (4%) (p < 0.01). Neurological complications were generally rare, with similar rates among the two groups (endovascular 2.9%; surgery 2.6%). Endovascular treatment showed a statistically significantly higher rate of persistent neurological complications than surgical treatment (2.9% versus 0.2%; p < 0.01). Both treatments showed similar rates of clinical improvement based on Aminoff Logue scale score. The multicenter, retrospective study involved 131 patients. The thoracic region was the most frequent location (58%), followed by lumbar (37%). Paraparesis (45%) and back pain (41%) were the most common presenting symptoms, followed by bladder dysfunction (34%) and sensory disturbances (21%). The mean clinical follow-up was 21 months, with all patients followed for at least 12 months. No statistically significant differences were found in demographic and clinical data, lesion characteristics, or outcomes between the two treatment groups. Median pre-treatment Aminoff-Logue score was 2.6, decreasing to 1.4 post-treatment with both treatments. The mean therapeutic delay for surgery and endovascular treatment showed no statistically significant difference. Surgical treatment demonstrated significantly lower failure rates (5% vs. 46%, p < 0.01). In the surgical group, 2 transient neurological (1 epidural hematoma, 1 CSF leak) and 3 non-neurological (3 wound infections) complications were recorded; while 2 permanent neurological (spinal infarcts), and 5 non-neurological (inguinal hematomas) were reported in the endovascular group. According to the literature review and this multicenter clinical series, surgical treatment has a significantly lower failure rate than endovascular treatment. Although the two treatments have similar complication rates, endovascular treatment seems to have a higher rate of persistent neurological complications.

Embolization of the middle meningeal artery for the prevention of chronic subdural hematoma recurrence in high-risk patients: a randomized controlled trial-the EMPROTECT study protocol.

BACKGROUND: Middle meningeal artery (MMA) embolization has been proposed as a treatment of chronic subdural hematoma (CSDH). The benefit of the procedure has yet to be demonstrated in a randomized controlled trial. We aim to assess the efficacy of MMA embolization in reducing the risk of CSDH recurrence 6 months after burr-hole surgery compared with standard medical treatment in patients at high risk of postoperative recurrence. METHODS: The EMPROTECT trial is a multicenter open label randomized controlled trial (RCT) involving 12 French centers. Adult patients (≥18 years) operated for CSDH recurrence or for a first episode with a predefined recurrence risk factor are randomized 1:1 to receive either MMA embolization within 7 days of the burr-hole surgery (experimental group) or standard medical care (control group). The number of patients to be included is 342. RESULTS: The primary outcome is the rate of CSDH recurrence at 6 months. Secondary outcomes include the rate of repeated surgery for a homolateral CSDH recurrence during the 6-month follow-up period, the rate of disability and dependency at 1 and 6 months, defined by a modified Rankin Scale (mRS) score ≥4, mortality at 1 and 6 months, total cumulative duration of hospital stay during the 6-month follow-up period, directly or indirectly related to the CSDH and embolization procedure-related complication rates. CONCLUSIONS: The EMPROTECT trial is the first RCT evaluating the benefit of MMA embolization as a surgical adjunct for the prevention of CSDH recurrence. If positive, this trial will have a significant impact on patient care. TRIAL REGISTRATION NUMBER: NCT04372147.

Pachymeningitis associated with IgG4-related disease and ANCA positivity: Case report and review of the literature.

OBJECTIVE: Hypertrophic pachymeningitis is a rare clinical disorder involving localized or diffuse thickening of the dura mater. Considering pachymeningitis is both in the clinical spectrum of IgG4-RD and ANCA vasculitis (specifically granulomatosis with polyangiitis), an overlap syndrome is discussed. METHODS: We report a case of hypertrophic pachymeningitis revealed by headache and cranial nerve dysfunction, and coexistence of biopsy-proven IgG4-RD pachymeningitis and MPO-ANCA positivity. Furthermore, all cases previously reported in the literature of pachymeningitis with IgG4-RD and presence of ANCA were analyzed. RESULTS: Thirteen patients with pachymeningitis, IgG4-RD and ANCA were analyzed. Patients with HP-related IgG4 and ANCA are mainly male (8, 62%). Median age at diagnosis was 64 years. Main clinical manifestations at diagnosis were localized to the head and neck with headaches (10, 77%), cranial nerve dysfunction (7, 54%), hearing impairment (6, 46%) and vertigo (4, 31%). Except 1 patient with diffuse aortitis, no other systemic manifestation was observed at diagnosis and during follow-up. Serum IgG4 was often elevated (11, 85%) and ANCA was mainly with myeloperoxidase specificity (11, 85%). Seven patients had cerebrospinal fluid analyse with lymphocytic pleocytosis in 5 cases (71%), elevated proteins in 4 cases (57%), positive oligoclonal bands in 3 cases (42%) and decreased glucose in one case (14%). On the MRI, the thickening of the dura mater concerned most often the posterior fossa, in 7 cases (54%). Among 10 cases with histological findings, all showed increased IgG4-positivity of plasma cells, 50% lymphocytic infiltrate but none presented the three major histological criteria of IgG4-related disease. Three (30%) showed histological signs of vasculitis with vascular wall damage and/or giant cells. Among the 12 patients treated with steroid therapy, a clinical improvement was noted in 11 cases (92%). Relapse occurred during tapering in 4 patients (33%). An immunosuppressive drug was added in 2nd line for 7 cases (54%), with a clinical improvement in all. CONCLUSION: Pachymeningitis with IgG4 and ANCA seems a localized disease to the head and neck. Leptomeningeal biopsy commonly found IgG4 criteria and no vasculitis. All patients responded well to steroid therapy and immunosuppressive drugs, especially rituximab, with clinical and radiological improvement but relapse and/or sequelae are not uncommon.

DNA methylation-based prognostic subtypes of chordoma tumors in tissue and plasma.

BACKGROUND: Chordomas are rare malignant bone cancers of the skull-base and spine. Patient survival is variable and not reliably predicted using clinical factors or molecular features. This study identifies prognostic epigenetic chordoma subtypes that are detected noninvasively using plasma methylomes. METHODS: Methylation profiles of 68 chordoma surgical samples were obtained between 1996 and 2018 across three international centers along with matched plasma methylomes where available. RESULTS: Consensus clustering identified two stable tissue clusters with a disease-specific survival difference that was independent of clinical factors in a multivariate Cox analysis (HR = 14.2, 95%CI: 2.1-94.8, P = 0.0063). Immune-related pathways with genes hypomethylated at promoters and increased immune cell abundance were observed in the poor-performing "Immune-infiltrated" subtype. Cell-to-cell interaction plus extracellular matrix pathway hypomethylation and higher tumor purity were observed in the better-performing "Cellular" subtype. The findings were validated in additional DNA methylation and RNA sequencing datasets as well as with immunohistochemical staining. Plasma methylomes distinguished chordomas from other clinical differential diagnoses by applying fifty chordoma-versus-other binomial generalized linear models in random 20% testing sets (mean AUROC = 0.84, 95%CI: 0.52-1.00). Tissue-based and plasma-based methylation signals were highly correlated in both prognostic clusters. Additionally, leave-one-out models accurately classified all tumors into their correct cluster based on plasma methylation data. CONCLUSIONS: Here, we show the first identification of prognostic epigenetic chordoma subtypes and first use of plasma methylome-based biomarkers to noninvasively diagnose and subtype chordomas. These results may transform patient management by allowing treatment aggressiveness to be balanced with patient risk according to prognosis.

A new simple and free tubular device for microscopic transcortical approach to deep-seated lesions: technical note and case example.

BACKGROUND: Surgery for deep-seated brain tumors remains challenging. Transcortical approaches often require brain retraction to ensure an adequate surgical corridor, thus possibly leading to brain damage. Various techniques have been developed to minimize brain retraction such as self-retaining retractors, endoscopic approaches, or tubular retractor systems. Even if they evenly distribute the mechanical pressure over the parenchyma, rigid retractors can also cause some degree of brain damage and have significant disadvantages. We propose here a soft cottonoid retractor for microscopic resection of deep-seated and ventricular lesions. METHODS: Through a small corticectomy, a channel route with a blunt cannula is developed until the lesion is reached. Then, a "balloon-like system" made with a surgical glove is progressively inflated, dilatating the surgical corridor. A mini-tubular device, handmade by suturing a surgical cottonoid, is positioned into the corridor, unfolded, and sutured to the edge of the dura, to prevent it from being progressively expelled from the working channel. This allows a good visualization of the lesion and surrounding structures under the microscope. RESULTS: Advantages of this technique are the softness of the tube walls, the absence of rigid arm to hold the tube, and the possibility for the tube to follow the movements of the instruments and to modify its orientation according to the working area. CONCLUSION: This simple and inexpensive tubular working channel for microscopic transcortical approach is a valuable alternative technique to traditional self-retaining retractor and rigid tube for the microsurgical resection of deep-seated brain tumors.

Atypical evolution of meningiomatosis after discontinuation of cyproterone acetate: clinical cases and histomolecular characterization.

PURPOSE: The long-term use of cyproterone acetate (CPA) is associated with an increased risk of developing intracranial meningiomas. CPA discontinuation most often induces a stabilization or regression of the tumor. The underlying biological mechanisms as well as the reasons why some meningiomas still grow after CPA discontinuation remain unknown. We reported a series of patients presenting CPA-induced meningiomatosis with opposed tumor evolutions following CPA discontinuation, highlighting the underlying histological and genetic features. METHODS: Patients presenting several meningiomas with opposite tumor evolution (coexistence of growing and shrinking tumors) following CPA discontinuation were identified. Clinical and radiological data were reviewed. A retrospective volumetric analysis of the meningiomas was performed. All the growing meningiomas were operated. Each operated tumor was characterized by histological and genetic analyses. RESULTS: Four women with multiple meningiomas and opposite tumor volume evolutions after CPA discontinuation were identified. Histopathological analysis characterized the convexity and tentorial tumors which continued to grow after CPA discontinuation as fibroblastic meningiomas. The decreasing skull base tumor was characterized as a fibroblastic meningioma with increased fibrosis and a widespread collagen formation. The two growing skull base meningiomas were identified as meningothelial and transitional meningiomas. The molecular characterization found two NF2 mutations among the growing meningiomas and a PIK3CA mutation in the skull base tumor which decreased. CONCLUSION: To our knowledge, this is the first report describing an atypical tumor evolution of CPA-associated meningiomas after CPA discontinuation. The underlying biological mechanisms explaining this observation and especially the close relationship between mutational landscapes and embryologic origins of the meninges in CPA-related meningiomas as well as their clonal origin require further research.

Intracranial venous sinus stenting for the treatment of lateral sinus stenoses: An analysis of 200 patients.

PURPOSE: The purpose of this study was to analyze the long-term efficacy and safety of intracranial venous sinus stenting in a large cohort of patients with any type of presentation of primary lateral venous sinus stenosis (VSS). MATERIALS AND METHODS: A retrospective cohort study was performed including patients treated by venous sinus stenting for symptomatic VSS from 2012 to 2019. Successful primary resolution of symptoms without adjunctive treatment or recurrence, and complications after stenting were analyzed at the last follow-up time point. RESULTS: Two-hundred patients were included. There were 14 men and 186 women with a mean age of 39±14 (SD) years (age range: 13-75 years). Presenting symptoms included venous pulsatile tinnitus in 168 patients (168/200; 84%), idiopathic intracranial hypertension in 100 patients (100/200; 50%) and/or spontaneous cerebrospinal fluid leak in 35 patients (35/200; 17%). The overall rate of successful primary resolution of any typical presenting symptoms was 79% (95% CI: 73-85%). This rate ranged from 74% to 93% depending on the symptom with no significant difference between patients with and those without idiopathic intracranial hypertension (P=0.08). Recurrence rate was 10% (95% CI: 6-14%). No death or permanent morbidity were observed during a median follow-up of 2.2 years (Q1, Q3: 1.4, 3.3; range: 1-7.7 years). CONCLUSION: Our study shows that venous sinus stenting has a low morbidity and high success rate at long-term follow-up for the treatment of idiopathic intracranial hypertension, venous pulsatile tinnitus or spontaneous cerebrospinal fluid leak associated with VSS. The excellent safety suggests considering this treatment as first-line treatment when medical management is ineffective or poorly tolerated.

Intracranial Meningiomas Decrease in Volume on Magnetic Resonance Imaging After Discontinuing Progestin.

BACKGROUND: The behavior of meningiomas under influence of progestin therapy remains unclear. OBJECTIVE: To investigate the relationship between growth kinetics of intracranial meningiomas and usage of the progestin cyproterone acetate (PCA). METHODS: This study prospectively followed 108 women with 262 intracranial meningiomas and documented PCA use. A per-meningioma analysis was conducted. Changes in meningioma volumes over time, and meningioma growth velocities, were measured on magnetic resonance imaging (MRI) after stopping PCA treatment. RESULTS: Mean follow-up time was 30 (standard deviation [SD] 29) mo. Ten (4%) meningiomas were treated surgically at presentation. The other 252 meningiomas were followed after stopping PCA treatment. Overall, followed meningiomas decreased their volumes by 33% on average (SD 28%). A total of 188 (72%) meningiomas decreased, 51 (20%) meningiomas remained stable, and 13 (4%) increased in volume of which 3 (1%) were surgically treated because of radiological progression during follow-up after PCA withdrawal. In total, 239 of 262 (91%) meningiomas regressed or stabilized during follow-up. Subgroup analysis in 7 women with 19 meningiomas with follow-up before and after PCA withdrawal demonstrated that meningioma growth velocity changed statistically significantly (P = .02). Meningiomas grew (average velocity of 0.25 mm3/day) while patients were using PCA and shrank (average velocity of -0.54 mm3/day) after discontinuation of PCA. CONCLUSION: Ninety-one percent of intracranial meningiomas in female patients with long-term PCA use decrease or stabilize on MRI after stopping PCA treatment. Meningioma growth kinetics change significantly from growth during PCA usage to shrinkage after PCA withdrawal.

The Rostral Mucosa: The Door to Open and Close for Targeted Endoscopic Endonasal Approaches to the Clivus.

BACKGROUND: Extended endoscopic endonasal approaches (EEAs) have progressively widened the armamentarium of skull base surgeons. In order to reduce approach-related morbidity of EEAs and closure techniques, the development of alternative strategies that minimize the resection of normal tissue and alleviate the use of naso-septal flap (NSF) is needed. We report on a novel targeted approach to the clivus, with incision and closure of the mucosa of the rostrum, as the initial and final step of the approach. OBJECTIVE: To present an alternative minimally invasive approach and reconstruction technique for selected clival chordomas. METHODS: Three cases of clival chordomas illustrating this technique are provided, together with an operative video. RESULTS: The mucosa of the rostrum is incised and elevated from the underlying bone, as first step of surgery. Following tumor resection with angled scope and instruments, the mucosa of the sphenoid sinus (SS) is removed and the tumor cavity and SS are filled with abdominal fat. The mucosal incision of the rostrum is then sutured. A hangman knot is prepared outside the nasal cavity and tightened after the first stitch and a running suture is performed. CONCLUSION: We propose, in this preliminary report, a new targeted approach and reconstruction strategy, applying to EEAs the classic concept of skin incision and closure for transcranial approaches. With further development in the instrumentations and visualization tools, this technique may become a valuable minimally invasive endonasal approach for selected lesions.

Tumor Growth Rate as a New Predictor of Progression-Free Survival After Chordoma Surgery.

BACKGROUND: Currently, different postoperative predictors of chordoma recurrence have been identified. Tumor growth rate (TGR) is an image-based calculation that provides quantitative information of tumor's volume changing over time and has been shown to predict progression-free survival (PFS) in other tumor types. OBJECTIVE: To explore the usefulness of TGR as a new preoperative radiological marker for chordoma recurrence. METHODS: A retrospective single-institution study was carried out including patients reflecting these criteria: confirmed diagnosis of chordoma on pathological analysis, no history of previous radiation, and at least 2 preoperative thin-slice magnetic resonance images available to measure TGR. TGR was calculated for all patients, showing the percentage change in tumor size over 1 mo. RESULTS: A total of 32 patients were retained for analysis. Patients with a TGR ≥ 10.12%/m had a statistically significantly lower mean PFS (P < .0001). TGR ≥ 10.12%/m (odds ratio = 26, P = .001) was observed more frequently in recurrent chordoma. In a subgroup analysis, we found that the association of Ki-67 labeling index ≥ 6% and TGR ≥ 10.12%/m was correlated with recurrence (P = .0008). CONCLUSION: TGR may be considered as a preoperative radiological indicator of tumor proliferation and seems to preoperatively identify more aggressive tumors with a higher tendency to recur. Our findings suggest that the therapeutic strategy and clinical-radiological follow-up of patients with chordoma can be adapted also according to this new parameter.

Endoscope-assisted far-lateral transcondylar approach for craniocervical junction chordomas: a retrospective case series and cadaveric dissection.

OBJECTIVE: Craniocervical junction (CCJ) chordomas are a neurosurgical challenge because of their deep localization, lateral extension, bone destruction, and tight relationship with the vertebral artery and lower cranial nerves. In this study, the authors present their surgical experience with the endoscope-assisted far-lateral transcondylar approach (EA-FLTA) for the treatment of CCJ chordomas, highlighting the advantages of this corridor and the integration of the endoscope to reach the anterior aspect and contralateral side of the CCJ and the possibility of performing occipitocervical fusion (OCF) during the same stage of surgery. METHODS: Nine consecutive cases of CCJ chordomas treated with the EA-FLTA between 2013 and 2020 were retrospectively reviewed. Preoperative characteristics, surgical technique, postoperative results, and clinical outcome were analyzed. A cadaveric dissection was also performed to clarify the anatomical landmarks. RESULTS: The male/female ratio was 1.25, and the median age was 36 years (range 14-53 years). In 6 patients (66.7%), the lesion showed a bilateral extension, and 7 patients (77.8%) had an intradural extension. The vertebral artery was encased in 5 patients. Gross-total resection was achieved in 5 patients (55.6%), near-total resection in 3 (33.3%), and subtotal resection 1 (11.1%). In 5 cases, the OCF was performed in the same stage after tumor removal. Neither approach-related complications nor complications related to tumor resection occurred. During follow-up (median 18 months, range 5-48 months), 1 patient, who had already undergone treatment and radiotherapy at another institution and had an aggressive tumor (Ki-67 index of 20%), showed tumor recurrence at 12 months. CONCLUSIONS: The EA-FLTA provides a safe and effective corridor to resect extensive and complex CCJ chordomas, allowing the surgeon to reach the anterior, lateral, and posterior portions of the tumor, and to treat CCJ instability in a single stage.

Use of high dose cyproterone acetate and risk of intracranial meningioma in women: cohort study.

OBJECTIVE: To assess the risk of meningioma associated with use of high dose cyproterone acetate, a progestogen indicated for clinical hyperandrogenism. DESIGN: Observational cohort study. SETTING: Data from SNDS, the French administrative healthcare database, between 2007 and 2015. PARTICIPANTS: 253 777 girls and women aged 7-70 years living in France who started cyproterone acetate between 2007 and 2014. Participants had at least one reimbursement for high dose cyproterone acetate and no history of meningioma or benign brain tumour, or long term disease status. Participants were considered to be exposed when they had received a cumulative dose of at least 3 g during the first six months (139 222 participants) and very slightly exposed (control group) when they had received a cumulative dose of less than 3 g (114 555 participants). 10 876 transgender participants (male to female) were included in an additional analysis. MAIN OUTCOME MEASURE: Surgery (resection or decompression) or radiotherapy for one or more intracranial meningiomas. RESULTS: Overall, 69 meningiomas in the exposed group (during 289 544 person years of follow-up) and 20 meningiomas in the control group (during 439 949 person years of follow-up) were treated by surgery or radiotherapy. The incidence of meningioma in the two groups was 23.8 and 4.5 per 100 000 person years, respectively (crude relative risk 5.2, 95% confidence interval 3.2 to 8.6; adjusted hazard ratio 6.6, 95% confidence interval 4.0 to 11.1). The adjusted hazard ratio for a cumulative dose of cyproterone acetate of more than 60 g was 21.7 (10.8 to 43.5). After discontinuation of cyproterone acetate for one year, the risk of meningioma in the exposed group was 1.8-fold higher (1.0 to 3.2) than in the control group. In a complementary analysis, 463 women with meningioma were observed among 123 997 already using cyproterone acetate in 2006 (risk of 383 per 100 000 person years in the group with the highest exposure in terms of cumulative dose). Meningiomas located in the anterior skull base and middle skull base, particularly the medial third of the middle skull base, involving the spheno-orbital region, appeared to be specific to cyproterone acetate. An additional analysis of transgender participants showed a high risk of meningioma (three per 14 460 person years; 20.7 per 100 000 person years). CONCLUSIONS: A strong dose-effect relation was observed between use of cyproterone acetate and risk of intracranial meningiomas. A noticeable reduction in risk was observed after discontinuation of treatment.

The sellar barrier on preoperative imaging predicts intraoperative cerebrospinal fluid leak: a prospective multicenter cohort study.

BACKGROUND: The type of sellar barrier observed between a pituitary tumor and cerebrospinal fluid (CSF) on preoperative magnetic resonance imaging (MRI) may predict intraoperative CSF leak during endonasal pituitary surgery. This is the first multicentric prospective cohort trial to study the sellar barrier concept and CSF leak rate during endoscopic pituitary surgery. METHODS: This multi-center, international study enrolled patients operated for pituitary adenomas via fully endoscopic endonasal surgery over a period of 4 months. The independent variable was the subtype of sellar barrier observed on preoperative MRI (strong, mixed or weak); the dependent variable was the presence of an intraoperative CSF leak. The primary goal was to determine the association between a particular type of sellar barrier and the risk of intraoperative CSF leak. Appropriate statistical methods were then applied for data analysis. RESULTS: Over the study period, 310 patients underwent endoscopic endonasal surgery for pituitary tumor. Preoperative imaging revealed a weak sellar barrier in 73 (23.55%), a mixed sellar barrier in 75 (24.19%), and a strong sellar barrier in 162 (52.26%) patients. The overall rate of intraoperative CSF leak among all patients was 69 (22.26%). A strong sellar-type barrier was associated with significantly reduced rate of intraoperative CSF leak (RR = 0.08; 95% CI 0.03-0.19; p < 0.0001), while a weak sellar barrier associated with higher rates of CSF leak (RR = 8.54; 95% CI 5.4-13.5; p < 0.0001). CONCLUSIONS: The preoperative MRI of pituitary patients can suggest intraoperative CSF leak rates, utilizing the concept of the sellar barrier. Patients with a weak sellar barrier carry a higher risk for an intraoperative CSF leak, whereas a strong sellar barrier on MRI seems to mitigate intraoperative CSF leak. We propose that preoperatively assessment of the sellar barrier can prepare surgeons for intraoperative CSF leak repair.

Endoscopic Endonasal Pituitary Surgery For Nonfunctioning Pituitary Adenomas: Long-Term Outcomes and Management of Recurrent Tumors.

INTRODUCTION: Endoscopic endonasal approaches (EEAs) provide improved access and operative visualization for resection of pituitary adenomas. Although the technique has gained wide acceptance, there is a paucity of data regarding late recurrence. OBJECTIVE: We aim to assess long-term outcomes of patients with nonfunctioning pituitary adenomas (NFPAs) who underwent EEA. METHODS: We reviewed 269 patients operated on for an NFPA between 2005 and 2015. Clinical and radiologic factors including those potentially related to higher chances of recurrence were analyzed. Progression-free survival was analyzed using the Kaplan-Meier method, and univariate and multivariate survival were analyzed using a Cox regression model. RESULTS: The study included 269 patients. The gross total resection rate was 46.0% (n = 124) but cavernous sinus involvement was present in almost half the patients (n = 115). The probability of recurrence at 5 years and 10 years was 22.0% and 47.2%, respectively. The median time to recurrence was 10 years for patients without cavernous sinus involvement and 6 years for those with cavernous sinus involvement. Univariate and multivariate analysis showed that tumor size, cavernous sinus invasion, anterior skull base extensions, and residual tumor were significantly associated with recurrence. CONCLUSIONS: Recurrence rate of NFPA remains high despite the better visualization offered by EEA, especially in those tumors involving the cavernous sinus and/or previously operated on. Repeat surgery is adequate for tumor debulking and decompression of the optic apparatus but is unlikely to achieve gross total resection if a successful previous EEA has been performed. Radiation therapy is an effective option for management of recurrent tumors.

Chiasmapexy for secondary empty sella syndrome: diagnostic and therapeutic considerations.

PURPOSE: Secondary empty sella syndrome (SESS) following pituitary surgery remains a diagnostic and therapeutic challenge. The aim of this study was to specify the diagnostic criteria, surgical indications and results of chiasmapexy in the SESS. METHODS: Three cases from two experienced neurosurgical centers were collected and the available literature was reviewed. RESULTS: The 3 patients were operated for a giant non-functioning pituitary adenoma, a cystic macroprolactinoma, and an arachnoid cyst respectively. Postoperative visual outcome was initially improved, and then worsened progressively. At the time of SESS diagnosis, visual field defect was severe in all cases with optic nerve (ON) atrophy in 2 cases. Patients were operated via an endoscopic endonasal extradural approach. One patient was re-operated because of early fat reabsorption. Visual outcome improved in 1 case and stabilized in 2 cases. Statistical analyses performed on 24 cases from the literature review highlighted that patient age and severity of the preoperative visual defect were respectively significant and nearly significant prognostic factors for visual outcome, unlike the surgical technique. CONCLUSION: T2-weighted or CISS/FIESTA sequence MRI is mandatory to visualize adhesions, ON kinking and neurovascular conflict. TS approach is the most commonly used approach. The literature review could not conclude on the need for an intra or extradural approach suggesting case by case adapted strategy. Intrasellar packing with non-absorbable material such as bone should be considered. Severity of the visual loss clearly decreases the visual outcome suggesting early chiasmapexy. In case of severe and long standing symptoms before surgery, benefits and surgical risks should be carefully balanced.

Delayed Instrumentation Following Removal of Cranio-Vertebral Junction Chordomas: A Technical Note.

Objective To investigate on the feasibility and safety of a new approach which consists of delaying instrumentation after destabilizing craniovertebral junction (CVJ) chordoma surgery, allowing proton beam radiotherapy to be performed in a metal-free tumoral cavity. Design This is a retrospective series of a prospectively maintained database. Participants Five consecutive patients operated on for a CVJ chordomas for which instrumentation after tumor resection was deferred to after radiotherapy treatment. Main Outcome Measures The main outcome consisted of measurements of the following parameters: C0-C2 angle, atlanto-dens interval (ADI), condylar gap, and the position of the dens relative to McGregor's line and coronal inclination, performed at 3 different times for all patients: before tumor surgery (baseline), before instrumentation surgery, and after instrumentation surgery. Results For all patients, CVJ parameters deteriorated during the delay period, but stayed within normal limits for most. Because of radiological instability, one patient necessitated instrumentation before receiving radiotherapy. All parameters except condylar gap were partially corrected after instrumentation. No new neurological symptom or evolving neck pain occurred during the delay period. Conclusion Delayed instrumentation of CVJ chordomas can be a safe alternative that might lead to improved subsequent radiotherapeutical treatment. Patient's selection and close clinical and radiological follow-up are mandatory for the success of this approach.

The orbitopterygoid corridor as a deep keyhole for endoscopic access to the paranasal sinuses and clivus.

OBJECTIVE: The anteromedial triangle (AMT) is the triangle formed by the ophthalmic (V1) and maxillary (V2) nerves. Opening of this bony space offers a limited access to the sphenoid sinus (SphS). This study aims to demonstrate the utility of the orbitopterygopalatine corridor (OPC), obtained by enlarging the AMT and transposing the contents of the pterygopalatine fossa (PPF) and V2, as an entrance to the SphS, maxillary sinus (MaxS), and nasal cavity. METHODS: Five formalin-injected cadaveric specimens were used for this study (10 approaches). A classic pterional approach was performed. An OPC was created through the inferior orbital fissure, between the orbit and the PPF, by transposing the PPF inferiorly. The extent of the OPC was measured using neuronavigation and manual measurements. Two illustrative cases using the OPC to access skull base tumors are presented in the body of the article. RESULTS: Via the OPC, the SphS, MaxS, ethmoid sinus (EthS), and nasal cavity could be accessed. The use of endoscopic assistance through the OPC achieved better visualization of the EthS, SphS, MaxS, clivus, and nasal cavity. A significant gain in the area of exposure could be achieved using the OPC compared to the AMT (22.4 mm2 vs 504.1 mm2). CONCLUSIONS: Opening of the AMT and transposition of V2 and the contents of the PPF creates the OPC, a potentially useful deep keyhole to access the paranasal sinuses and clival region through a middle fossa approach. It is a valuable alternative approach to reach deep-seated skull base lesions infiltrating the cavernous sinus and middle cranial fossa and extending into the paranasal sinus.

Vertebrobasilar Artery Encasement by Skull Base Chordomas: Surgical Outcome and Management Strategies.

BACKGROUND: Vascular encasement by skull base chordomas can increase surgical risk and hinder completeness of resection. However, the evidence behind this remains anecdotic. OBJECTIVE: To give a better portrayal of chordomas encasing vertebrobasilar arteries mainly in regard of surgical vascular risk and its impact on extent of resection. METHODS: A retrospective cohort study comparing skull base chordomas with encasement (≥180o encirclement) of the vertebrobasilar arteries to a control group of skull base chordomas with intradural extension. Data gathered involved pre- and postoperative volumetric analysis of the tumor, degree of encasement of involved vessel, occurrence of complication, and survival data including progression-free survival (PFS) and overall survival (OS). RESULTS: A total of 24 patients with vertebrobasilar encasement were included in the study and an equal number of control cases were randomly selected from the same time period, totalizing 48 patients. Lower clival tumors with condyle involvement were more likely to have encasement. Gross total resection (GTR) rate was significantly lower in the encasement group (13% vs 42%, P = .023). Rates of postoperative new neurological deficit, CFS leak and 30 d postoperative mortality were not statistically different between groups. There was no statistically significant difference in mean PFS (P = .608) and OS (P = .958). CONCLUSION: Skull base chordomas encasing vertebrobasilar arteries are highly challenging tumors. This study demonstrates that although safe resection is possible, GTR is hindered by the presence of encasement. We advocate letting the tumor's adherence to vessels lead the resection, leaving a small piece of tumor behind if adherent to the vessels.

Hybrid antero-lateral transcondylar approach to the clivus: a laboratory investigation and case illustration.

BACKGROUND: Surgical treatment of lesions involving the ventral craniovertebral junction (CVJ) and the lower clivus, traditionally involved complex lateral or transoral approaches to the skull base. However, mid or upper clivus involvement requires more extensive lateral approaches. Recently, the endoscopic endonasal approach (EEA) has become the standard for upper CVJ lesions and medial clival, and a valuable alternative for those tumors extending in its upper third as well as laterally. However, the EEA is associated with an increased risk of post-operative CSF leakage and infection when the tumor is characterized by an intradural extension. Furthermore, whenever the tumor has significant lateral and/or inferior extension below the odontoid process, the chances for a complete resection decrease. METHOD: To analyze the extent of exposure of a hybrid microscopic-endoscopic transcondylar antero-lateral approach to the CVJ and clival region, and to verify its effectiveness in terms of mid and upper clival access. Five silicone-injected cadaver heads were used. Following a standard antero-lateral approach, condylectomy and jugular tubercle drilling were performed, after which angled endoscopes were utilized to extend the bone resection to the clivus. A volumetric assessment of the amount of clival removal was carried out. A case of CVJ chordoma operated through this approach is presented. RESULTS: The hybrid antero-lateral transcondylar approach provides adequate exposure of the ventral CVJ, up to the dorsum sellae and the sphenoid sinus, the contralateral petrous apex, and the contralateral paraclival internal carotid artery (ICA). Approximately 60% of the total clival volume can be removed with this approach. The main limitation is the limited visualization of the ipsilateral paraclival ICA and petrous apex. CONCLUSION: The hybrid antero-lateral transcondylar approach is a valuable surgical option for CVJ tumor extending from C2 to the mid and upper clivus.