Egyptian Journal of Neurosurgery Volume 29 / No. 3 / July - September 2014 17-24

Original Article Surgical Management of Sphenoid Wing

Mohamed Emara, Alaa Farag*, Walid Badawy, Hosam Maaty, Fathy Elnos Department of Neurosurgery, Banha University

ARTICLE INFO ABSTRACT Background: Sphenoidal meningiomas constitute 18% of intracranial masses. Sphenoid wing meningiomas still present a difficult surgical challenge especially when they are Received: large in size and involve neurovascular structures. Objective: The aim of the study is to 5 November 2014 shed light on the management of sphenoid wing , study the outcome of

microsurgical resection and factors affecting its resectability. Patients and Methods: In Accepted: 20 December 2014 this prospective study, thirty cases were included thirteen outer and / or middle sphenoid wing, thirteen clinoidal and four meningiomas en plaque. In each case diagnosis was made clinically and confirmed radiologically and histo-pathologically. Results: Key words: Between March 2011 and June 2014, thirty patients with meningiomas primarily Meningioma, originating from sphenoid ridge were operated, 22 (73.3%) patients were females and Sphenoid wing, 8(26.7%) were males. In seventeen cases total resection was achieved, and subtotal Surgical treatment resection in thirteen cases. In this study mortality was recorded in two case and morbidity in nine cases. Eight cases has excellent outcome fourteen were good and six were fair and two died. Conclusion: The surgical treatment of sphenoid wing meningiomas still represents a challenge for neurosurgeons. Surgery for sphenoid wing meningiomas poses a variety of problems reflecting the complex anatomy of the sphenoid region. © 2014 Egyptian Journal of Neurosurgery. Published by MEDC. All rights reserved

INTRODUCTION encase the internal carotid artery (ICA) and middle 1,2,14 cerebral artery (MCA) as well as . Meningiomas are among the most common primary The clinical presentation of sphenoid wing intracranial tumors. They are slow growing tumors meningiomas is manifestation of increased intracranial originating from the arachnoidal cap cells and may tension. Clinical features unique to this group of occur anywhere that arachnoid cells are found8. meningiomas include: exophtalmous, transient Meningioma account for 14.3-19% of primary due to occlumotor nerve dysfunction, periorbital pain or intracranial neoplasms with a reported incidence 4.4 per numbness in the territory of the V1 branch, progressive 6,13 100000 person-year. Female: male ratio is 2:1. The true visual loss, and seizures . prevelance is likely even greater, as incidental Contrasted-enhansed cranial CT and MR imaging meningiomas have been reported in 2.3% of autopsy are the predominant imaging techniques used in the examinations. Most commonly diagnosed in middle- diagnosis and management of meningiomas; however, aged and eldery patients. Cranial meningiomas are in selected cases, MR spectroscopy, MR angiography 7 roughly 10 fold more common than spinal and Positron emission tomography may be useful . meningiomas9 The most appropriate surgical treatment of lateral Deletion of the neurofibro-matosis type 2(NF2) and middle sphenoid wing meningioma is total removal. gene, ionizing radiation and head truma are associ-ated Complete removal implies total removal of the tumor, with an increase risk, while the role of sex hormones in including the dural attachment and bone that is involved 9 meningioma development is still uncertain8 with the tumor Sphenoid wing meningioma account for about Clinoidal meningiomas consid-ered a major 11.9% of intracranial meningiomas, they are classified neurosurgical challenge due to involvement of into lateral sphenoid wing (or pterional) which are important neuron-vascular structures, cranial base usually similar to convexity meningioma, middle (or techniques can be used for surgical removal of these 10 alar) and medial (or clinoidal). The medial type tends to tumors with good results .

Sterotactic can be used as an *Corresponding Author: alternative treatment to surgery. For incomplety Alaa A Farag resected or recurrent tumors conventional radio-therapy Department of Neurosurgery Banha University, Egypt Email: [email protected], Tel.: +2/01287518299 is beneficial. When the lesion is considered unresectable

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or all other treatments have failed; hormonal therapy or Removal of the tumor was started with internal chemotherapy should be considered15. decompression depending on understanding of the bony Future therapies will include combination of and neurovascular anatomy of the middle cranial fossa, targeted molecular agents, and this will most likely be sellar and parasellar region. accomplished through continued progress in the Parts of the tumor adherent to middle cerebral understanding of the genetics and biological changes artery or internal carotid artery or infilterating the associated with meningioma.15 (CS) were not removed. Infilterated The aim of the study is to shed light on the dura and bone was removed. Hemostasis was done with management of sphenoid wing meningioma, study the the aid of surgicell. Watertight dural closure was done outcome of microsurgical resection and factors affecting using pericranial flap or fascia lata graft. The wound its resectability. was closed in anatomical layers The grading for meningioma resection is PATIENTS AND METHODS conventional Simpson grading. In the resection of sphenoidal meningiomas, sometimes it is difficult for Between March 2011 and June 2014, a prospective the surgeon to ascertain that the entire area of dural study including 30 patients with meningiomas primarily involvement or attachment has been exposed, resected, originating from sphenoid ridge was done. The or coagulated. Therefore, we choose to define "Gross meningioma extended along the anterior and middle total resection" in this series as Simpson grade I and II cranial fossae. All patients were admitted to the and "subtotal" for other grades. Neurosurgery department in Banha University Hospital Cases with histological evidence of malignancy or and Nasser Institute hospital. with residual lesions were sent for conventional In this study, the patients were subjected to radiotherapy or gamma knife. thorough neurological examination. Computerized Follow up of cases was conducted in the tomography as well as Magnetic resonance imaging, Neurosurgical outpatient clinic. Evaluation was done with and without contrast enhancement, were done for both clinically and radiologically by the help of CT and all cases preoperatively. MRA or CT angiographies MRI with contrast, to detect any residual of the excised were also done when the relation of tumor to vessels tumor. need to be delinned. All these patients were submitted to In this study, the outcome was considered to be: surgery. Postoperative CT and MRI were done as a ƒ Excellent: Total tumor removal with improvement routine. of clinical condition with no deficit. Tumors were classified according to size into small ƒ Good: Total tumor removal without improvement (less than 3cm in maximum dimention), large (more than of clinical condition or with development of new 3 and less than 5cm in maximum dimention) and giant treatable deficit or In complete tumor removal done (more than 5cm in maximum dimention). with improvement of the clinical condition. Operative Management: ƒ Fair: Incomplete tumor removal without clinical Surgery was performed with general anaesthesia, improvement or Total removal with development of with the aid of an operating microscope and micro- new untreatable deficit or deterioration of previous surgical instrumentation in all cases. deficit. The choice of surgical approach for these ƒ Poor: Incomplete tumor removal with development meningiomas depended on the location of the tumor and of a new untreatable deficit extent of its dural attachment. The aim of surgery was either total or subtotal excision depending on the RESULTS preoperative imaging study. Pterional and fronto–orbito - zygomatic approaches were used. Thirty patients with meningiomas primarily Intra-operatively, the aim was complete originating from sphenoid ridge were operated. Twenty microsurgical removal of the tumor aiming to cure the two (73.3%) patients were females and eight (26.7%) patients unless the tumor was encasing vessels or were males. The mean age was (49.1+10.1) year. The nervous tissue. The plan of tumor removal included patients were divided into three groups according to the infilterated dura and bone. origin of tumor into A, B and, C: A carefully planned craniotomy was done exposing ƒ Group A: 13 patients har-boring inner sphenoidal the tumor and also any part of the dura invaded by the (clinoidal) meningioma meningioma. The dura was coagulated, to provide early ƒ Group B: 13 patient harboring outer and/or middle control of the tumor blood supply. The dura was incised sphenoidal meningiomas. in circumferential manner. ƒ Group C: 4 cases of meningioma en plaque.

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Headache was the most common presenting symptom (63.3%). The above mentioned symptoms symptoms among the studied cases (83.3%), followed were the most common among each individual group by symptoms of increased intracranial tension (76.6%). (Table 1). Visual disturbance was the third common presenting

Table 1: Presenting symptoms among studied groups Group A Group B Group C Total INNER OUTER & EN-PLAQUE No=30 Symptom NO=13 MIDDLE NO=4 No = 13 NO % NO % NO % NO % 10 76.9 11 84.6 4 100 25 83.3 Visual disturbance 12 92.3 5 38.5 2 50 19 63.3 Increased intracranial tension 9 69.2 10 76,9 4 100 23 76.6 Seizures 5 38.5 5 38.5 1 25 11 36.6 Proptosis 0 0 0 0 3 75 3 10 Diplopia 3 23.1 1 7.7 0 0 4 10.3 Motor weakness 2 15.4 4 31.2 0 0 6 20 Impaired consciousness 2 15.4 1 7.7 0 0 3 10

The most common radiological finding in our study in 40% of cases. Vascular encasement were found in 10 was homogenous enhancement of the lesion which patient of clinoidal meningioma (Table 2). occurred in 90% of cases and hyperosteosis were found

Table 2: Radiological finding among studied groups Group A Group B Group C Total INNER OUTER& ENPLAQUE No =30 Radiological findings No = 13 MIDDLE No = 4 No = 13 NO % NO % NO % NO % Homogenous 12 92.3 12 93.2 3 75 27 90 Heterogeneous 1 7.7 1 7.7 1 25 3 10 Hyperostosis 2 15.4 6 46.2 4 100 12 40 Edema 5 38.5 7 53.8 2 50 14 46.6 Calcification 3 23.1 5 38.5 1 25 9 30 Vessels 10 76.9 1 7.7 0 0 11 36.6

In 17 patient (56.6%), tumor were totally resected In this study, there were two mortalities, one from (Fig. 1a-e and Fig. 2a-h) and tumor were subtotally vasospasm and the other from fits. Postoperative resected in 13 patients (43.3%). Subtotal resection was hemorrhage was occurred in three patients which more predominant in group A, nine patients (69.3%). resolved spontaneously with conservative treatment. Total resection was more predominant in group B, Two patients had vasospasm. Two patients had CSF eleven patients (84.6%). leak and three patients developed fits (Table 3). In this study ten patients (33.3%) received adjuvant therapy in the form of conventional radiotherapy or gamma knife.

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Table 3: Complications among studied groups Group A Group B Group C Total INNER OUTER& MIDDLE ENPLAQUE No=30 Complications NO=13 No=13 NO=4 No % No % No % No % Hemorrhage 1 7.7 1 7.7 1 25 3 10 CSF leak 1 7.7 1 7.7 0 0 2 6.7 Wound infection 0 0 1 7.7 1 25 2 6.7 Infraction 2 15.4 0 0 0 0 2 6.7 Seizure 1 7.7 1 7.7 1 25 3 10 Mortality 2 15.4 0 0 0 0 2 6.7

In this study the outcome was good in 14 patients (47%), excellent in 8 patients (26.7%), fair in 6 patients (20%) and poor in 2 patients (6.7%) (Table 4).

Table 4: Outcome among studied groups Group A Group B Group C Total INNER OUTER & MIDDLE EN-PLAQUE No=30 Outcome No=13 No =13 No = 4 NO % NO % NO % NO % Excellent 1 7.7 7 53.8 0 0 8 26.7 Good 7 54 5 38.5 2 50 14 47 Fair 3 23 1 7.7 2 50 6 20 Poor 2 15.4 0 0 0 0 2 6.7

a b

c d e Fig. 1 a-e: a&b. Pre-operative and c-e. post-operative MRI of 60 years male patient with lateral sphenoid wing meningioma with the tumor totally excised

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a b c d

e f g h Fig. 2 a-h: a-d. Pre-operative and e-h. post-operative CT and MRI brain respectively of a. 64 years male patient with clinoidal meningioma totally excised

DISCUSSION In the outer and/or middle sphenoid wing meningioma (group B patients), headache was found to Sphenoid wing is the most common location for be the primary presenting symptom in 86.9% of cases. skull base meningiomas. Meningiomas involving the This is attributed to the fact that these slowly growing sphenoid wing are mostly histopathologically benign(6). tumors are still not discovered in the early appropriate With the advancements in neuroradiological studies, timing and their diagnosis is delayed until they reach one can better appreciate how difficult these quite a large size causing the manifestations. Seizures meningiomas are to cure. Surgical management of these was found in 38.5% of cases. Other symptoms cases becomes increasingly challenging as the site suggestive of increased intracranial tension such as moves inwards from the pterion to the clinoid8 blurring of vision and vomiting was found in 61.5% of In the medial or clinoidal menengioma (Group A cases. Gradual progressive diminution of vision was patients), progressive visual deterioration was found to noted in 38.5% of cases and was usually affecting the be the primary presenting symptom in 92.3% of cases. same side of the tumor. Motor weakness was present in This visual deterioration was gradual, progressive and 31.2% of cases and altered consciousness in 7.7% of asymmetrical, always affecting first the ipsilateral side. cases. Papilledema was found in 69.2% of cases while Headache also occurred in 76.9% of cases of this group. optic atrophy was found in 15.4% of cases. This headache was gradual progressive and usually In the series of Russel and Benjamin published in radiated frontally or temporally. Other symptoms of 2008 including 60 patients of sphenoidal meningioma, group A included, blurring of vision, vomiting, seizures 25 of them were lateral and middle sphenoidal and motor weakness. Fundus abnormalities, meningiomas, the most common presenting symptoms 17 papilledema was found in 38.4% of cases and optic were headache and seizures . In the series of Roser atrophy was found in 53.% of cases . published in 2008 including 256 patients, 19 of them Those results were similar to those of other series were middle sphenoid wing meningioma seizures and where visual deterioration was the most frequent dizziness were the most common presentation. Thirty of presentation. In the series of Roser published in 2008 them were lateral sphenoid wing meningioma; including 256 patients, 174 of them were medial headache and psychological deficit were the most (16) sphenoid wing meningioma. Visual disturbance was the common presentat-ion . most common presentation account for 66.7% if the In the meningioma en plaque cases (Group C cavernous sinus is infilterated and 60.9% if the patients), proptosis on the same side of the tumor was cavernous sinus is not infilterated. The second most found in 3 out of the 4 patients (75%), headache common presentation was headache16. In the series occurred also in all cases (100%), gradual progressive published in 2009 by Bassiouni et al, including 106 ipsilateral diminution of visual acuity occurred in two patients with clinoidal meningioma, the mean symptoms cases (50%), symptoms of increased intracranial tension were deterioration of vision in 57 (53.8%), headache in with blurring of vision in all cases (100%), and fits 30 (28.3%), optic nerve atrophy in 18 (17%) seizures in occurred in 2 cases (25%). 17 (16.0%), dizziness in 15(14.2%) and papilledema in These results go hand in hand with the natural 10 (9.2%) patients3 . history of these tumors as described by Cushing and

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Eisenhardt in 1938, Jesus and Toledo series published in MR images. In Group A (the clinoidal variety), from 2001 and Roser series published in 2008.(16)(5) the 13 cases studied, the tumor was small in three, large Preoperative and postoperative CT and MRI Brain in eight and giant in two cases. Concerning Group B, was done for all patients. In the preoperative images from the 13 cases harboring an outer or middle or whole dense enhancement was found in 27 patients (90%) and sphenoid masses one was small, 7 were large and the was caused by the lack of blood brain barrier in these remaining 5 masses were giant. In Group C, the en extra-axial tumors. The margins of the enhancing mass plaque variety, 2 cases were large, one was small and are sharply demarcated, with broad base against bone of one was giant. the sphenoid ridge and a creeping dural tail, the latter of In this study, we found a significant negative which is highly suggestive and specific7. In our study correlation between degree of resection and tumour we couldn’t find a direct correlation between the dense location (as degree of resection decreases in more enhancement and the vascularity of the meningioma. medial tumors). Also, there was significant negative Some of the densely enhancing masses were not found correlation between degree of resection and vascular as hypervascular as expected. This support the fact that encasement, as more resection can be achived in less enhancement depend mainly on lack of blood brain vascular encasement of the tumors. barrier. In Group A (the clinoidal variety), 13 cases were Pterional approach was the most common approach operated in the study. Total resection was achieved in 4 used in this study, used in 26 patients representing cases (30.7%) and subtotal resection was done in 9 86,7%. This was due to that this approach offers several patients (69.3%). The prognosis was excellent in 1 case advantages. It permited a direct approach to the dural (7.7%), good in 7 cases (54%), fair in 3 cases (23%) and attachment of the tumor along the sphenoid ridge and poor in 2 cases (15.4%). Two patients died post thereby allows for early devascularization. Wide opening operatively one from vasospasm and the other from post of the sylvian fissure improves surgical access and operative fits. Post operative hemorrhage was recorded optimizes visualization of all critical neurovascular in one patients (7.7%). He was managed conservatively. structures (the MCA, ACA, ICA, and anterior optic Two patients (15.4%) developed infarction (vasospasm) pathways). Intradural or extradural removal of one of them died and the other had hemiplegia. CSF hyperostotic bone of the sphenoid wing including the leak occurred in one patient (7.7%). It stopped with the anterior clinoid process could be performed. Good use of lumbar drain. One patient had fits. He needed relaxation of the brain was achieved by opening the basal mechanical ventilation and died later. cisterns and releasing the CSF. This maneuver avoided The extent of resection of clinoidal meningiomas application of self-retaining spatulas in many cases9. This varies in different series. The rates of total resection was also the opinion of Pamir et al in the series published vary between 43 and 91%. Table (5) shows the different in 2008 and Bassiouni et al in 2009.12,3 reported series of clinoidal meningiomas.9 The size of the tumor was calculated on the maximum diameter of tumor obtained from CT scans or

Table 5: Different reported series of clinoidal meningiomas(9) Series No of Mortality Cs Improvement Extent of Major Mean Recurrence patients -% involvement of vision % resection-% morbidity -% follow up /progression -% Almefty, 1990 24 8 38 8 Gross total 83 8 57 m 4 Puzzilli et al, 1999 33 15 39.4 Gross total 12 53 m 26 54.5 Day,2000 6 0 100 Gross total 0 3 m 0 66.6 Goel et al,2000 60 5 23 Gross total 70 12 26 m 2 Lee et al,2001 16 0 14 75 Gross total 87 0 37 m 0 Tobias et al,2003 26 0 23 71 Gross total 77 11.5 42 m 0 Nakamura et al,2006 108 0 64 Gross total 43 7.4 6.5 y 23 Russel et al,2008 43 0 2 85 Gross total 91 18.6 39 m 9.3 Pamir et al,2008 35 0 31 63 Gross total 69 18 12.8 y 9 Bassiouni et al,2009 106 1.9 29 40 Gross total 59 17 6.9

In this study, lateral and/or middle sphenoidal while in 2 patients (15.4%) subtotal resection was meningioma group, aggressive bony removal was performed. In the latter two cases, total removal was not performed to the hyperostotic bone. At the end of the achieved as the MCA or one of its branches was procedures, the dural attachment was coagulated. Gross encased by the tumor. The outcome was excellent in 7 total resection was achieved in 11patients (84.6%), patients (53.8%), good in 5 patients (38.5%), and fair in

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1 patient (7.7%). Post operative hemorrhage was REFERENCES recorded in one patient (7.7%). It did not need surgical intervention. Post operative fits occured in one patient 1. Al-Mefty O, ed. Meningiomas. New York: Raven (7.7%) and also wound infection occurred in one Press Ltd., Krisht A F. Clinoidal Meningiomas. In: patients (7.7%). CSF leak occurred in one patient DeMonte F, McDermot M W, Al-Mefty O, eds. (7.7%) and stopped with repeated dressing. Brotchi and AL–Meftys, Meningiomas. Second edition. Bonnal in 2006 reported the same results with nine Pheladelphia, Thieme 24, pp. 228-256, 1991. 4 patients . In the series of Morokkoff et al published in 2. Al-Mefty O.Clinoidal meningiomas. Neurosurg 73 2008 including 163 cases, there was no mortality, they (6): 840 – 849, 1990. had complication rate of 9.4% and 1.7% developed new 3. Bassiouni H, Asgari S, Sandalcioglu IE, Seifert V, 11 neurological deficit of their cases . Stolke D and Marquardt G. Anterior clinoidal In this study, four cases with meningioma en plaque meningiomas: functional outcome after were operated. Two of them were totally excised (50%) microsurgical resection in a consecutive series of and two patients were subtotally excised. The surgical 106 patients: clinical article. J Neurosurg 111(5): outcome was fair in 2 cases and good in 2 cases. There 1078-1090, 2009. was no mortality, one patient complicated with 4. Brotchi J and Pirotte B. Sphenoid wing hemorrhage post operatively resolved with serial follow meningiomas. In: Sekhar LN. Fessler RG, eds. up, and another one patient complicated with fits. Atlas of Neurosurgical Techniques: Brain. New Superficial wound infection occurred in one patient. It York, NY: Thieme pp 623-632, 2006. resolved with repeated dressing. Proptosis improved 5. De Jesus O and Toledo M M. Surgical Management partially in all patients. In the series of Roser published of Meningioma en Plaque of the Sphenoid Ridge. in 2008, including 86 patients, gross total resection was Surg Neurology 55: 265–9, 2001. achieved in 38% of cases with recurrence rate 32%. In 6. Delfini R: Management of Tumours of Middle his series 5 patients (7.1%) had hygroma, 5 patients had Fossa, In: Sindou M: Practical Handbook of hydrocephalus, infarction occurred in 2 patients and Neurosurgery 1sted, New York: Springer Wien; hemorrhage occurred in 2 patients. One patient died vol 2, 6:95-111, 2009. 16 from pulmonary embolism. 7. Dillon WP and Uzelac A. Modern Imaging Techniques for Meningiomas. In: De Monte F CONCLUSION McDermot MW,Al-Mefty O,eds. AL-Meftys, Meningiomas. Sec-ond edition. Philadelphia, The surgical decision for these tumors should Thieme 13, 107-118, 2010. depends primarily on the clinical finding of the patient 8. Greenberg MS. Tumour, In: Greenberg M.S.: and radiological finding of the tumor. Location of the Handbook of neurosurgery. 7th ed., New York: meningioma is an important factor determining the Thieme; vol 1, 21, pp 613-620, 2010. degree of tumor resectability. 9. Krisht AF. Clinoidal meningiomas. In: DeMonte F, Encasement of ICA and/or MCA and their branches McDermot MW, Al-Mefty O, eds. AL-Mefty, together with CS invasion and extension into superior sMeningiomas. Second edition Philadelphia, orbital fissure are limiting factors for radical removal. Thieme (24), pp. 228-237, 2010. Tumors of the outer third of the sphenoid ridge and 10. Mariniello G, deDivitiis O, Seneca V, Maiuri F. many small middle wing tumors are essentially Classical pterional compared to the extended skull convexity meningiomas with regard to clinical base approach for the removal of clinoidal presentation and surgical treatment. The objective in meningiomas. Journal of Clinical Neuroscience every procedure, should be total removal of the 19: 1646–1650, 2012. meningioma. 11. Morokoff AP, Zaubermanj, Black PM: Surgery for In the clinoidal variety, clinoidal masses not convexity meningiomas. Neurosurgery 63 (3): 427 attached to the carotid artery branches, total resection is - 433, 2008. recommended. For clinoidal masses attached to the 12. Pamir MN, Belirgen M, Ozduman K, KiliT and carotid or cavernous sinus subtotal resection is Ozek M. Anterior clinoidal meningiomas: analysis recommended. of 43 consecutive surgically treated cases. Acta For en plaque variety, an extensive resection of Neurochir (Wien) 150(7): 625 - 635, 2008. tumor, dura and bone will lead to good tumor control 13. Pirotte B and Brotchi J. Lateral and Middle with minimal morbidity, good cosmetic and functional Sphenoid Wing Meningiomas. In: Sekhar LN. results. Fessler RG, eds. Atlas of Neurosurgical Radiotherapy and radiosurgery play an increasing Techniques: Brain. New York, NY: Thieme: 39, role in controlling residual or progressive disease 623-632, 2006. 14. Rhoton AL. The sellarregion. Neurosurgery 51 [Suppl 1]: 335–374, 2002.

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15. Rockhill J, Mrugala M and Chamberlain M.: 17. Russell SM, Benjamin V. Medial sphenoid ridge Intracranial meningiomas: an overview of diagnosis meningiomas: classification, microsurgical and treatment. Neurosurg Focus 23(4): 1-7, 2007. anatomy, operative nuances, and long-term surgical 16. Roser F. Sphenoid Wing Meningiomas. In: Ramina outcome in 35 consecutive patients. Neurosurgery R, Aguiar PH, Tatagiba M, eds Samii’s Essentials 62 (3, Supp 1): 38-50, 2008. in Neuro-surgery. Verlag Berlin Heidelberg, Springer (11): 99-108, 2008.

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