Surgical evolution in spinal dural arteriovenous fistula treatment-a 7 years monocentric experience.

Accounting for 70% of all spinal vascular malformations, spinal dural arteriovenous fistulas (SDAVF) are the most common type of malformation. Interruption of the fistulous arterialized vein point is the goal of surgical treatment. The aim of the study was to compare open surgery (laminectomy) versus minimal invasive surgery (MIS) in SDAVF treatment. Between March 2013 and March 2020, we retrospectively collected 21 consecutive adult patients with SDAVF. Since March 2017, MIS has been routinely used for surgical treatment. Pre- and post-operative clinical evaluations used Aminoff-Logue score (ALS). Complication rate was noted. Post-operative occlusion of the malformation was confirmed by digital subtraction angiography (DSA) in all patients. MIS was compared to open surgery in terms of efficacy and complications with statistical evaluation. Standard laminectomy was performed in 12 patients and MIS technique in 9 patients. No difference was noted on pre-operative parameters. ALS and MRI signs of myelopathy were improved in all cases except for 1 patient in each group. All SDAVFs were excluded based on post-operative DSA. Significant differences were noted between the 2 groups in terms of perioperative blood loss (p<0.001), post-operative pain visual analog scale values (p<0.001), and first time out of bed (p<0.001). Wrong level surgery occurred in one patient in each group; patients were re-operated using the same technique. No infection or cerebrospinal fluid (CSF) leak was noted. In our experience, MIS is a safe alternative to open laminectomy for SDAVF treatment. MIS contributes to patient comfort and minimizes blood loss without increasing complication rate.

Progestin-related WHO grade II meningiomas behavior-a single-institution comparative case series.

WHO grade II progestin-related meningiomas have been reported in recent series but we found no previous study describing their long-term outcome. Our study aimed to evaluate patients operated on for high-grade intracranial meningioma and who underwent long-term exposure to high dose of cyproterone acetate, nomegestrol acetate, and chlormadinone acetate. Our study retrospectively included 9 patients with high-grade progestin-related intracranial meningioma between December 2006 and September 2021. In each patient, clinico-radiological follow-up was performed every 6 months after diagnosis and treatment withdrawal recommendation. The mean progestative exposure was 11.4 years. Edema existence or absence of cleft sign on MRI were the key factors for surgical indication. All patients underwent surgery. Adjuvant radiotherapy was indicated in 1 patient, and Gamma Knife radiosurgery was proposed in 2 other patients for a second location of meningioma. Six patients harbored a grade II chordoid meningioma subtype with 100% PR expression and 3 patients a grade II atypical meningioma subtype with lower PR expression. The mean follow-up was 8.1 years and none of the 9 patients presented with a recurrence. Patients with grade II progestin-related meningiomas have less tumor recurrence after surgery than patients with sporadic grade II meningiomas, especially after progestin withdrawal. The presence/appearance of peri-meningioma edema and the absence of cleft sign before volumetric change should suggest the existence of an underlying WHO grade II meningiomas. In these cases, surgical resection may immediately be considered and adjuvant radiotherapy should be reserved for proven recurrence cases.

Fusiform dilatation of internal carotid artery after pterional but not subfrontal craniotomy in 6 patients.

PURPOSE: Our study aimed to evaluate potential risk factors for the development of FDICA after suprasellar tumor resection. MATERIALS AND METHOD: After reviewing all cases of pediatric patients who benefited from a suprasellar lesion resection in our two medical institutions, we found 6 patients with a FDICA. Surgical approach strategy (pterional or subfrontal approaches) was noted. Postoperative cranial MRI was performed in each patient 3 months after surgery and every year. When a FDICA occurred, MRI was performed 6 months after the diagnosis and 1 year later to detect any progression. RESULTS: There were 6 males with a mean age at treatment of 11 years (6 to 15). Pterional approach was performed in these 6 patients. At the 2 institutions, we have done at least 50 pterional craniotomies for suprasellar lesion resection. No FDICA was reported after subfrontal approach in 27 consecutive pediatric patients operated on from a craniopharyngioma. The delay between the surgery and the diagnosis of the FDICA was 9 months (3 to 17 months). No symptoms related to the FDICA were recorded. The mean maximal diameter of the aneurysm was 14 mm (10 to 21). ICA bifurcation was involved in 2 cases. Asymptomatic FDICA progression was noted in 2 cases but no treatment was proposed. CONCLUSION: The pathogenesis of FDICA is unclear, and might involve arterial wall necrosis caused by postoperative arachnoid fibrosis which might be worsened by the pterional approach.

Ruptured blood blister like aneurysm: does the best therapeutic option really exist?

Our study aimed to evaluate the outcome of patients with ruptured blood-blister like aneurysm (BBLA) in our institution by comparing microsurgical selective treatment to endovascular treatment using flow-diverter stent (FD). Our study included 18 consecutive patients treated for BBLA between 2004 and 2020. Until 2014, microsurgery was preferred in all patients with BBLA (n = 10). Significant postoperative morbi-mortality was recorded at this time and led us to change therapeutic strategy and to favor FD as first-line treatment in all patients (n = 8). Postprocedural complications and BBLA occlusion were recorded. High WFNS score (> 2) was noted in 6 patients of microsurgical group and in 2 of endovascular group. In microsurgical group, ischemic lesions were noted in 6 patients and led to death in 3 patients. Immediate BBLA occlusion was obtained in all patients. Favorable outcome after 3 months (mRS < 3) was recorded in 4 of the 7 survivors. In endovascular group, ischemic lesions were noted in 4 patients. One patient died from early postprocedural BBLA rebleeding. Scarpa hematoma was noted in 3 patients with surgical evacuation in 1. Persistent BBLA at 3 months was recorded in 4 patients without rebleeding, but further FD was required in 1 with growing BBLA. Favorable outcome was noted in 6 of the 7 survivors. Although, rate of morbi-mortality appear lower in patients treated with FD, neurological presentation was better and BBLA diagnosis remains questionable in this group. Moreover, persistent BBLA imaging with potential risk of rebleeding after FD deserves to be discussed.

Protective STA-MCA bypass to prevent brain ischemia during high-flow bypass surgery: case series of 10 patients.

BACKGROUND: High-flow extracranial-intracranial bypass is associated with a significant risk of ischemic stroke. The goal of this study is to evaluate the effectiveness of STA-MCA bypass preceding a high-flow bypass as a means of protecting the brain from ischemia during the high-flow bypass anastomosis in patients with otherwise untreatable aneurysms. MATERIALS AND METHOD: This prospective study included 10 consecutive patients treated for complex/giant aneurysm using a previous combined STA-MCA bypass and high-flow EC-IC bypass between June 2016 and January 2018 when classical endovascular or microsurgical exclusion was estimated too risky. Early cranial Doppler, MRI, CT scan, and conventional angiography were performed in each patient to confirm patency of bypasses, measure flow in the anastomoses, detect any ischemic lesions, and evaluate exclusion of the aneurysm. RESULTS: The mean age at treatment was 55 years (range 34 to 67). The mean time of microsurgical procedure was 11 h (range 9 to 12). In all patients, the high-flow bypass was patent intraoperatively and complete occlusion of aneurysm was obtained. No ischemic lesions were noted on early MRI. One patient died from a large hemispheric infarction related to a common carotid artery dissection 10 days after the microsurgical procedure and immediate postoperative epidural hematoma was noted in one other patient. CONCLUSION: In this study, we described the use of a protective STA-MCA bypass, performed prior to the high-flow bypass, in order to reduce the risk of perioperative ischemic lesions without increasing the morbidity of the surgical procedure. This treatment paradigm was feasible in all ten patients without complications related to the STA-MCA anastomosis.

Gamma Knife Radiosurgery of Brainstem Cavernous Malformations.

BACKGROUND/AIMS: Our study aimed to evaluate the efficiency and morbidity of Gamma Knife radiosurgery (GKS) in the treatment of hemorrhagic brainstem cavernous malformations (CMs). METHODS: We included in this study all patients who underwent GKS for the treatment of a hemorrhagic brainstem CM(s) in our institution between January 2007 and December 2012. The GKS was privileged when the surgical procedure was evaluated as very risky. The mean dose of radiation was 14.8 Gy, and the mean target volume was 0.282 cm3. All patients participated in a scheduled clinical follow-up. The posttreatment MRI was performed after 6 months and after 1 year, and then all patients had an annual MRI follow-up. RESULTS: There were 19 patients with a mean age of 36.7 years. The mean follow-up period was 51.2 months. The annual hemorrhage rate (AHR) was 27.31% before GKS, 2.46% during the first 2 years following the GKS, and 2.46% after the first 2 years following the GKS. The decrease in AHR after GKS was significant (p < 0.001). CONCLUSION: GKS should be suggested when the surgical procedure harbors a high risk of neurological morbidity in patients with brainstem CM. Compared to prior literature results, a lower dose than applied in this study could be discussed.

Pediatric neurofibromatosis type 2: clinical and molecular presentation, management of vestibular schwannomas, and hearing rehabilitation.

OBJECTIVE: This study aims to describe the clinical and molecular presentation of pediatric neurofibromatosis type 2 (NF2) and the subsequent management of vestibular schwannomas (VS) and hearing rehabilitation. METHODS: This is a single-center retrospective study of neurofibromatosis type 2 diagnosed before the age of 18 years old from 1997. Natural history of vestibular schwannomas and surgical outcomes were evaluated using volumetric MRI, hearing, and facial nerve assessment. Patients included in chemotherapy protocols were excluded. RESULTS: From a database of 80 patients followed up for NF2 on a regular basis, 25 patients were eligible (11 sporadic cases, 14 inherited in five families). The mean age at diagnosis was 11.6 years old. The average clinical follow-up was 6.5 years. NF2 mutation was identified in 81 % of the probands. The average growth rate based on the maximum linear diameter (DGR) was 1.68 mm/year (n = 33, average follow-up 4.22 years) and 545 mm3/year in volumetric assessment (VGR) for VS larger than 1 cm (n = 21, average follow-up 3.4 years). In unoperated ears, hearing was stable in about 50 % of ears. The mean change in dB HL was 9.5 dB/year for pure-tone average and 3.5 for speech-recognition threshold (n = 34, 5.5 years 1-12). Eight children required removal through a translabyrinthine approach (mean follow-up was 4.5 years), six patients were operated on for hearing preservation (mean postoperative follow-up 4.3 years). Six patients were eligible for hearing rehabilitation with cochlear implantation (I), and five received placement of an auditory brainstem implant. CONCLUSION: Early diagnosis and treatment of small growing VS should be carefully discussed considering familial history and possible rehabilitation with a CI.

Complications related to the use of an intraventricular access device for the treatment of leptomeningeal metastases from solid tumor: a single centre experience in 112 patients.

Ventricular access devices (VAD) offer several advantages compared to intralumbar injections for the administration of intra-CSF agents in the treatment of leptomeningeal metastases (LM). However, there are few prospective studies reporting on complications with the use of VADs. All complications were prospectively collected that pertained to the implantation and use of a VAD in consecutive patients with solid tumor-related LM from June 2006 to December 2013. Clinical follow-up was every 2 weeks during the initial 2 months of treatment and then once monthly. Complete neuraxis MRI was performed at baseline and then every 2-3 months. A total of 112 patients (88 women) with a mean age of 51.1 years (range 26-73) were included. Primary cancers included breast (79 patients), lung (12) and melanoma (6). All patients were treated with intra-CSF liposomal cytarabine. 72 % of the patients received concomitant systemic and intra-CSF chemotherapy. The placement of the VAD was performed under local anesthesia in all cases. The mean operative time was 15 min and no perioperative complications were reported. The mean number of intraventricular injections per patient was 9.34 (range 1-47). A total of 11 complications in 11 patients were seen including 7 infections, 1 intracranial hemorrhage, 2 instances of symptomatic leukoencephalopathy and 1 catheter malpositioning. 8 complications required an operation and 1 complication was fatal. The use of a VAD is safe and may improve patients' comfort and compliance with LM-directed therapy.

Causes of mortality in neurofibromatosis type 2.

OBJECT: The causes of mortality in neurofibromatosis type 2 (NF2) patients are poorly studied in the literature. Our study aimed to fit this gap by analyzing the main causes of death in this population. METHODS: This study is the retrospective review of prospectively collected data of 80 patients with NF2 disease followed in Lille University Hospital between 1987 and 2011. Demographical data, diagnosis criteria, and cause of death were recorded. RESULTS: There were 45 men and 35 women, with a mean age at diagnosis of 27.2 years (range: 6-73 years; SD: ± 15.4). Sixty-eight patients met Manchester criteria and the others had an identified mutation in the NF2 gene which confirmed the diagnosis. Of all patients, we noted 7 deaths. The mean age at diagnosis of dead patients was 26 years. The mean age of death was 38.9 years. The causes of death were suicide in 1 patient, hematoma after surgical removal of grade IV vestibular schwannoma in 1 patient, aspiration pneumonia after swallowing disturbances in 3 patients, intracranial hypertension related to growth of multiple meningiomas in 1 patient, and brachial plexus sarcoma grade 3 in the last patient. CONCLUSION: NF2 is a serious disease that can quickly be life-threatening. The presence of lower cranial nerves schwannomas is a poor prognostic factor, and radiosurgery should be considered for their treatment, as surgical removal often worsens the swallowing disturbances. A psychological support should also be provided.

Glioblastoma occurring after the surgical resection of a craniopharyngioma.

The authors report the case of a man who underwent cranial surgery for the removal of a craniopharyngioma using a right pterional approach. Three months later, he developed a right-sided temporal glioblastoma. The presentation is suggestive of a causal relationship between surgery and the development of a malignant glial tumor.

Posterior Cerebral Artery-to-Superior Cerebellar Artery Side-to-Side Bypass via Extreme Lateral Supracerebellar Infratentorial Approach: Technical Note.

BACKGROUND: Difficult-to-treat aneurysms of the distal posterior cerebral artery (PCA) can often be treated by parent artery occlusion. A cerebrovascular bypass can complement PCA occlusion to curb the risk of ischemic complications. An in situ bypass may be considered when the occipital artery or superficial temporal artery cannot serve as a bypass donor. This article describes the use of a side-to-side bypass of superior cerebellar artery as a donor to the PCA via an extreme lateral supracerebellar infratentorial approach (ELSCIT). This bypass approach can be a useful surgical strategy for PCA revascularization. METHODS: A 40-year-old woman underwent a side-to-side PCA-superior cerebellar artery bypass via the ELSCIT approach for to treat a complex and previously coiled PCA aneurysm. The bypass was followed by endovascular aneurysm and parent artery occlusion. RESULTS: Postoperatively, the patient experienced transient, partial trochlear nerve palsy of the left eye without ischemic lesions on magnetic resonance imaging. The clinical condition was stable, and angiography showed a patent bypass and complete aneurysm occlusion 12 months after surgery. CONCLUSIONS: The ELSCIT approach offers access to the medial and distal PCA that is suitable for a side-to-side PCA-superior cerebellar artery bypass. This type of approach and bypass may be of value when revascularization of a P2-P3 portion of the PCA is needed, but a suitable occipital artery or superficial temporal artery is not available.

Impact of preaneurysmal M1 length in unruptured middle cerebral artery aneurysm: mid-term outcome and single-center experience.

OBJECTIVE: This study was design to investigate the surgical and functional outcome based on the preaneurysmal M1 length for unruptured MCA aneurysm. METHODS: Among 250 consecutive patients with unruptured aneurysms operated in our institution between 2015 and 2017, 72 were MCA aneurysms. Risk factors for IR (i.e., intraoperative rupture) were investigated including age, sex, preaneurysmal M1 length, maximal MCA aneurysm diameter, neck size, aneurysm shape, sphenoid ridge proximation sign. Outcome was measured at discharge, 1 yr and last follow-up. Outcome was compared according to the preaneurysmal M1 length. RESULTS: Among 68 patients included, five patients (7.3%) suffered IR. Mean maximal diameter of MCA aneurysm (7.9 mm ± 3.4 vs. 4.5 ± 1.8; p = 0.01) was significantly associated with IR risk. Mean M1 length seemed to be shorter in the IR group although not statistically significant (16.2 mm ± 5.1 vs. 11.5 mm ± 4.8; p = 0.053). Mid-term outcome was favorable for all patients at last follow-up but was worsen in case of short preaneurysmal M1 segment (10.7 mm ± 4.8 vs. 16.4 mm ± 5.3, p = 0.02). Complete aneurysm occlusion was achieved for sixty-nine patients (95.5%) with 6.9% of early postoperative complications. CONCLUSIONS: The microsurgical treatment of unruptured MCA aneurysm was associated with favorable mid-term outcome in all patients and high rates of complete occlusion. Aneurysm size was significantly associated with the intraoperative rupture risk for unruptured MCA aneurysm and patients with a short preaneurysmal M1 segment seemed to have a greater risk of intraoperative rupture although not statistically significant. Short preaneurysmal M1 patients had worsen mid-term outcome.

Microsurgical management of recurrent intracranial aneurysm after endovascular treatment: a series of 60 consecutive patients.

OBJECTIVE: The aim of this study was to evaluate the morbidity associated with microsurgical treatment in patients with a recurrent aneurysm to improve their surgical management. METHODS: From 2012 to 2022, among the 3128 patients with ruptured or unruptured intracranial aneurysms managed at the authors' institution, 954 patients were treated by a microsurgical procedure. Of these 3128 patients, 60 consecutive patients (6.3%) who had a recurrent microsurgically treated aneurysm after previous endovascular treatment were included in this study. Additional microsurgical treatment was considered in case of progressive remnant growth or significant aneurysm recurrence. Intraoperative and postoperative complications were noted. Early (< 7 days) and long-term clinical and radiological monitoring were performed. Good functional outcome was considered as a modified Rankin Scale score < 3. RESULTS: The mean age at initial treatment was 45 years (range 26-65 years). The mean delay between the first treatment and microsurgical treatment of the recurrence was 64 months (range 2 days-296 months). The mean size of the fundus recurrence was 5 mm, and the mean size of the neck recurrence was 4.6 mm. Five patients (8.3%) presented with subarachnoid hemorrhage associated with rupture of the recurrent aneurysm. Three patients died (6%) of aneurysm rupture and/or intensive care complications. The total morbidity rate associated with the microsurgical procedure was 14.5% (8/55) in patients with unruptured recurrent aneurysms. Among these patients, postoperative definitive complications (ischemic lesions) directly related to the microsurgical procedure were present in 3 patients (5.5%). Intraoperative rupture was recorded in these 3 patients. In the 54 surviving patients with unruptured recurrent aneurysms, good functional outcome was noted in 49 (91%). Poor functional outcome was significantly associated with intraoperative rupture. CONCLUSIONS: Microsurgery remains an effective therapeutic option for recurrent intracranial aneurysms. However, in the authors' experience, postoperative morbidity is higher than in patients with nonrecurrent aneurysms. Therefore, a pretherapeutic multidisciplinary evaluation is mandatory to reduce the potential morbidity associated with the retreatment as much as possible. When endovascular occlusion of the aneurysm requires both stenting and coiling, alternative microsurgical treatment should be carefully evaluated, as microsurgical clipping will become much more challenging in cases of aneurysm recurrence.

Hearing Outcomes from Gamma Knife Treatment for Intracanalicular Vestibular Schwannomas with Good Initial Hearing.

Background: The objective of this study was to describe the long-term hearing outcomes of gamma knife treatment for unilateral progressing vestibular schwannomas (VS) presenting with good initial hearing using audiologic data. Methods: A retrospective review was performed between 2010 and 2020 to select patients with progressing unilateral VS and good hearing (AAO-HNS class A) treated with stereotactic gamma knife surgery (GKS). Their audiograms were analyzed along with treatment metrics and patient data. Results: Hearing outcomes with a median follow-up of 5 years post-treatment showed statistically significant loss of serviceable hearing: 34.1% of patients maintained good hearing (AAO-HNS class A), and 56.1% maintained serviceable hearing (AAO-HNS class A and B). Non-hearing outcomes are favorable with excellent tumor control and low facial nerve morbidity. Conclusions: Hearing declines over time in intracanalicular VS treated with GKS, with a significant loss of serviceable hearing after 5 years. The mean cochlear dose and the presence of cochlear aperture obliteration by the tumor are the main statistically significant factors involved in the hearing outcomes.

A Cohort Study of CNS Tumors in Multiple Endocrine Neoplasia Type 1.

PURPOSE: Multiple endocrine neoplasia type 1 (MEN1) is thought to increase the risk of meningioma and ependymoma. Thus, we aimed to describe the frequency, incidence, and specific clinical and histological features of central nervous system (CNS) tumors in the MEN1 population (except pituitary tumors). EXPERIMENTAL DESIGN: The study population included patients harboring CNS tumors diagnosed with MEN1 syndrome after 1990 and followed up in the French MEN1 national cohort. The standardized incidence ratio (SIR) was calculated based on the French Gironde CNS Tumor Registry. Genomic analyses were performed on somatic DNA from seven CNS tumors, including meningiomas and ependymomas from patients with MEN1, and then on 50 sporadic meningiomas and ependymomas. RESULTS: A total of 29 CNS tumors were found among the 1,498 symptomatic patients (2%; incidence = 47.4/100,000 person-years; SIR = 4.5), including 12 meningiomas (0.8%; incidence = 16.2/100,000; SIR = 2.5), 8 ependymomas (0.5%; incidence = 10.8/100,000; SIR = 17.6), 5 astrocytomas (0.3%; incidence = 6.7/100,000; SIR = 5.8), and 4 schwannomas (0.3%; incidence = 5.4/100,000; SIR = 12.7). Meningiomas in patients with MEN1 were benign, mostly meningothelial, with 11 years earlier onset compared with the sporadic population and an F/M ratio of 1/1. Spinal and cranial ependymomas were mostly classified as World Health Organization grade 2. A biallelic MEN1 inactivation was observed in 4/5 ependymomas and 1/2 meningiomas from patients with MEN1, whereas MEN1 deletion in one allele was present in 3/41 and 0/9 sporadic meningiomas and ependymomas, respectively. CONCLUSIONS: The incidence of each CNS tumor was higher in the MEN1 population than in the French general population. Meningiomas and ependymomas should be considered part of the MEN1 syndrome, but somatic molecular data are missing to conclude for astrocytomas and schwannomas.

Early postoperative radiotherapy improves progression free survival in patients with grade 2 meningioma.

BACKGROUND: Grade 2 meningiomas are a real problem in therapeutic management because of their tendency to reoccur. The most effective treatment is surgery. The role of adjuvant radiotherapy in this disease is still disputed due to its uncertain effect on progression-free survival. OBJECTIVE: To show that early adjuvant radiotherapy is an effective treatment in patients with grade 2 meningiomas. MATERIALS AND METHODS: A retrospective study was performed on all patients operated on for grade 2 meningioma in our center between 1994 and 2011. For every patient, we recorded the age at diagnosis, sex, background of neurofibromatosis type 2 (NF2) or meningiomatosis, location of meningioma, quality of tumor resection and whether the patient received early postoperative radiotherapy. These prognosis factors were studied using statistical tests. RESULTS: We included 167 patients (94 women, 73 men, sex ratio = 1.28); the mean age at diagnosis was 53.8 years. Twenty-seven patients received early adjuvant radiotherapy after surgery. Patients who received early postoperative radiotherapy had a significantly longer progression-free survival (8.2 years) than patients without radiotherapy (5.7 years, p = 0.04). In multivariate analysis, quality of tumor resection and early postoperative radiotherapy decrease the risk of recurrence of meningioma (p < 0.05). CONCLUSION: Adjuvant radiotherapy is an important therapeutic tool in the treatment of patients with grade 2 meningioma. It delays tumor progression and reduces the need for further surgery. Adjuvant radiotherapy must be considered as a treatment option in oncological multidisciplinary meetings, regardless of the quality of surgical resection.

Prognostic value and management of spinal tumors in neurofibromatosis type 2 patients.

PURPOSE: Neurofibromatosis type 2 (NF2) is a well-studied disease. Although spinal tumors are frequent, many issues concerning their prognosis and management still have to be clarified. The authors studied the clinical impact and radiological progression of spinal tumors in patients with NF2 to determine their prognostic value. METHODS: A total of 80 NF2 patients were followed in the Lille University Hospital between 1987 and 2011. Clinical, radiological and genetic data were retrospectively recorded and analyzed. Patients underwent annual cranial and spinal MRI. Both the location and size of each tumor were reported. The diagnosis of NF2 was confirmed either because the patient met the Manchester criteria or by the presence of genetic mutation. RESULTS: The mean follow-up period was 8.8 years (range 1 to 24 years; SD: ±0.8), and the mean age at diagnosis was 27.2 years (range 6 to 73 years; SD: ±1.7). Among all patients, 48 harbored spinal tumors. Twenty of them were symptomatic, and 21 were operated on. Patients with spinal tumors had a lower age at diagnosis (p = 0.02), a higher number of intracranial meningiomas (p = 0.028) and schwannomas (p = 0.03), and more nonsense and frameshift mutations (p = 0.04). CONCLUSION: Spinal tumors are common in NF2, and all patients should be regularly monitored by spinal MRI. The presence of spinal tumors seems to be a factor indicating poor prognosis. Clinical and radiological monitoring of spinal tumors could lead to early treatment both when clinical symptoms are present and in case of proven radiological evolution, maintaining a favorable functional prognosis as long as possible.

Intracranial meningiomas and neurofibromatosis type 2.

PURPOSE: In spite of the few clinical studies regarding the occurrence of intracranial meningiomas, their prognosis in neurofibromatosis type 2 (NF2) has not been accurately assessed and their management remains controversial. This study aims to compare NF2 patients with intracranial meningiomas to those without, and consequently to identify prognostic factors in attempt to improve the management of these tumors. METHODS: This retrospective study includes a total of 80 NF2 patients followed at Lille Hospital Center between 1987 and 2011. The diagnosis of NF2 was confirmed either because the patient met the Manchester criteria or by the presence of genetic mutation. Clinical, radiological and genetic data were retrospectively recorded and analyzed. Patients underwent annual cranial and spinal MRI. Both location and size of each tumor were reported. RESULTS: The mean follow-up period was 8.8 years (range 1-24 years; SD: ±0.8) and the mean age at diagnosis was 27.2 years (range 6-73 years; SD: ±1.7). Among all patients, 34 harbored intracranial meningiomas. Patients with intracranial meningiomas had a higher number of intracranial schwannomas, spinal tumors and cutaneous tumors (p < 0.05). They underwent more surgical procedures (p < 0.012). Twenty five intracranial meningiomas were surgically removed in 17 patients. The decision to perform surgery was taken in 10 cases for symptomatic tumors and in 15 cases for growing asymptomatic tumors determined by radiology. The histological analysis found a high rate of fibroblastic, transitional or grade 2 meningiomas preferentially located at the cerebri falx. CONCLUSION: Intracranial meningiomas are common in NF2. They are associated with poor prognosis factors. Clinical and radiological monitoring could lead to early treatment of these tumors both when clinical symptoms are present and in case of proven radiological evolution, and thus trying to maintain a favorable functional prognosis for as long as possible.

Selective clipping of giant anterior communicating artery aneurysms remains a reliable therapeutic option.

BACKGROUND: Giant anterior communicating artery (AcomA) aneurysm represent a significant surgical challenge. Our study aimed to discuss the therapeutic strategy in patients with a giant AcomA aneurysm treated by selective neck clipping through a pterional approach. METHODS: Among all operated patients from an intracranial aneurysm between January 2015 and January 2022 (n = 726) in our institution, three patients with a giant AcomA aneurysm treated by neck clipping were included. Early (<7days) outcome was noted. Early postoperative CT scan was performed in all patients to detect any complications. Early DSA was also performed to confirm giant AcomA aneurysm exclusion. The mRS score was recorded 3 months after treatment. The mRS≤ 2 was considered as a good functional outcome. Control DSA was performed one year after treatment. RESULTS: In the three patients, after a large frontopterional approach, a selective exclusion of their giant AcomA aneurysm was obtained after a partial pars orbitalis of the inferior frontal gyrus resection. Ischemic lesion was noted in 1 patient and chronic hydrocephalus in 2 patients with ruptured aneurysm. The mRS score after 3 months was good in 2 patients. Long term complete occlusion of the aneurysm were noted in the three patients. CONCLUSION: Selective clipping of a giant AcomA aneurysm is a reliable therapeutic option after a careful evaluation of local vascular anatomy. An adequate surgical exposure is frequently obtained through an enlarged pterional approach with an anterior basifrontal lobe resection, especially in an emergency situation and/or in case of high position of anterior communicating artery.

Unruptured cerebral arteriovenous malformation in children: Outcome in treated and untreated patients.

BACKGROUND: The management of unruptured cerebral arteriovenous malformation (URCAVM) is highly controversial; however, data regarding URCAVM in children are scarce. MATERIAL AND METHODS: We retrospectively reviewed consecutive children followed for URCAVM in our department between 2001 and 2021. RESULTS: Out of 36 patients, 12 were initially managed by observation, and 24 underwent first-line treatment: 8 by microsurgery, 10 by radiosurgery, 2 by embolization, and 4 by combined treatment. Mean follow-up of the whole group was 63months. Complete cure of the malformation was obtained in 14 patients (58%) in the treatment group: 8/8 in the microsurgery group, 5/10 in the radiosurgery group, 1/4 in the combined treatment group, and none in the embolization group. Two of the initially non-treated patients presented cerebral hemorrhage, with significant neurological consequences. In the treatment group, 5 patients presented new neurological deficits, only 1 of which, however, was functionally significant. Headache improved in 11 cases, mostly in the treatment group. Overall, 6 patients in the treatment group became asymptomatic, versus none in the observation group. CONCLUSIONS: The treatment of URCAVM is a reasonable option in many pediatric cases, considering the cumulative risk of cerebral hemorrhage during the child's lifetime, as well as the symptoms specific to URCAVM. Microsurgery, when feasible, offers the best functional results and control of the AVM; however, the risk-benefit ratio should be weighed on a case-by-case basis. More studies will be needed to inform treatment decisions in pediatric URCAVM.