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Sphenoid meningioma enplaque with proptosis: Surgical excision, reconstruction and outcome
Clinical Neurology and Neurosurgery 167 (2018) 147–156
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Sphenoid meningioma enplaque with proptosis: Surgical excision, reconstruction and outcome
T
⁎
Abdelhakeem A. Essaa,1, , Ali R. Hamdanb,1 a b
Department of Neurosurgery, Faculty of Medicine, Assiut University Hospital, Assiut, Egypt Department of Neurosurgery, Qena Faculty of Medicine, South Valley University, Qena, Egypt
A R T I C L E I N F O
A B S T R A C T
Keywords: Meningioma Enplaque Proptosis Reconstruction Outcome
Objective: To evaluate surgical outcome and reconstruction of bone defects after excision of sphenoid meningioma enplaque. Patient and methods: Between June 2012 and May 2016, a series of 15 patients presented by proptosis attended to neurosurgery departments, Assiut university hospital, Qena university hospital, South Valley University and, These patients were diagnosed with sphenoid meningioma enplaque by fulfilling its criteria by neuroimaging (sheet-like meningioma and hyperostosis). All patients received preoperative imaging investigations including Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) to evaluate the extension of both tumor components – soft tissue and bone involvement. Fifteen patients were operated by pterional approach under general anesthesia. Results: Fifteen patients with meningioma enplaque were treated surgically. The mean age of patients at the time of admission was 46.5 years, age ranged between 35–56 years. 80% of patients were females (female to male ratio was 4:1). Ten (66.7%) patients have left sided lesion representing. Proptosis was the main presented manifestation in all patients. Complete tumor excision was done in 10 patients while incomplete excision was done in the rest of patients. Proptosis was improved post-operatively in patients presented by it: Ten (66.7%) patients had complete improvement while the other five (33.3%) patients improved significantly. No mortality occurred in our study. A mean follow-up period of 2.1 years (range: 4 months to 4 years), three (20%) patients have tumor recurrence. Conclusions: Total excision of Sphenoid meningioma enplaque carries difficulties and high rate of post-operative morbidity. Early detection with experienced neurosurgeons, microsurgical techniques and availability of high speed drill decrease the risk of post-operative morbidity. Reconstruction of dural and bone defect is very important regarding functional and cosmetic aspects.
1. Introduction Meningioma en plaque is defined as meningioma with sheet like pattern and whorls of neoplastic tissue associated with hyperostosis. This term was given by Cushing and Eisenhardt [1]. In meningioma, hyperostosis is a specific character of, and is presented in 4.5% of all meningiomas, but is presented more in meningioma en plaque with percentage of 13%–49% [1]. Meningiomas en plaque account about 2–9% of all meningiomas [2–4]. Mainly in the sphenoid wing, although other skull areas may be sites for enplaque [5]. Proptosis in meningioma enplaque is due to hyperostosis and that affect patients prognosis. The precise mechanisms of hyperostosis and intralesional morphological criteria of the infiltrated bone associated with
⁎
1
meningioma still not clear. Females are more susceptible to have Meningioma enplaque three to six times [6]. The histopathology of sphenoid meningiomas en plaque are the same as any meningiomas with predominance of meningothelial and transitional variants. The vascular supply of the sphenoid meningiomas enplaque usually arises from meningeal vessels but may be avascular [7,6]. Meningiomas enplaque have high rate of recurrence due to difficulties during total resection due to extensive bone component of these tumors and complexity of anatomy at site of origin [7].
Corresponding author. E-mail addresses: [email protected] (A.A. Essa), [email protected] (A.R. Hamdan). All authors contributed equally in this work.
https://doi.org/10.1016/j.clineuro.2018.02.028 Received 10 January 2018; Received in revised form 9 February 2018; Accepted 19 February 2018 Available online 21 February 2018 0303-8467/ © 2018 Elsevier B.V. All rights reserved.
Clinical Neurology and Neurosurgery 167 (2018) 147–156
A.A. Essa, A.R. Hamdan
2. Patients and methods
fulfilling its criteria by neuroimaging (sheet-like meningioma) and hyperostosis which larger than soft tissue. Non hyperostotic sphenoid wing meningioma, clinoidal meningioma, cavernous sinus meningioma with orbital extension and optic sheath meningioma were excluded as they do not met the criteria.
Between June 2012 and May 2016, a series of 15 patients presented by proptosis attended to neurosurgery departments, Assiut university hospital, Qena university hospital, South Valley University and, These patients were diagnosed with sphenoid meningioma enplaque by
Fig. 1. (a): CT axial and coronal cuts of skull with left sphenoid and orbital hyper oestosis. (b): Preoperative axial cuts of skull with left sphenoid and orbital hyperostosis of meningioma enplaque.
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A.A. Essa, A.R. Hamdan
Fig. 1. (continued)
3. Imaging findings
surgical excision of sphenoid wing meningioma enplaque. All patients received preoperative Computed Tomography (CT) and Magnetic Resonance Imaging (M.R.I). The axial and coronal bone window of CT revealed hyperostosis of sphenoid ridge, posterolateral orbital wall and
Assessment of both tumor components-soft tissue (dural/intradural) and hyperostosis bone is very important to do proper planning for 149
Clinical Neurology and Neurosurgery 167 (2018) 147–156
A.A. Essa, A.R. Hamdan
Fig. 2. Preoperative M.R.I brain T1 coronal cuts of with contrast showing left sphenoid soft tissue with sheet like enhancement characteristic of meningioma enplaque.
4. Surgical treatment
superior orbital wall (Fig. 1a and b). M.R.I scans showed T1 hyopintensity of soft tissue component of meningioma, T2 hyperintensity and homogenous enhancement after gadolinium injection with sheetlike at anterior temporal pole (Fig. 2).
Fifteen patients were operated by pterional approach under general anesthesia. All patients were placed in supine position with head tilted 150
Clinical Neurology and Neurosurgery 167 (2018) 147–156
A.A. Essa, A.R. Hamdan
interaoperative, as the high speed drill was broken-down so we used chiseling. Completion of bone removal using rongeurs and kerrison punches. Orbital decompression and superolateral orbitotomy was done in all cases, with protection to periorbital fat. Then decompression of superior orbital fissure and orbital cavity were done which were essential in all cases with proptosis (Fig. 4). After bone removal, the dura was opened and soft tissue component was excised microsurgically. Haemostasis was performed followed by dural graft using galea-pericranial flap in all cases with water tight repair to minimize risk of cerebrospinal fluid leak. Reconstruction of orbital and fronto-temporal bone defects was done using titanium mesh (Fig. 5). Histopathological examination of all tumors were meningiomas grade I World Health Organization (WHO). Meningothelial variant in ten patients and transitional type in other five. 5. Results 5.1. Clinical data
Fig. 3. Intraoperative picture after elevation of galea and temporalis muscle.
All features of fifteen patients included in our study are described in Table 1, including clinical presentation, extent of tumor resection, post operative complications and recurrence. Fifteen patients presented with sphenoid meningioma enplaque were surgically treated. The mean age was 46.5 years, age ranged from 35 to 56 years. Female patients were twelve patients representing (80%) of patients with female to male ratio 4:1. The side of lesion was left sided in ten patients representing (66.7%) (Table 2). All patients were presented with proptosis. Nine patients presented by visual impairment, one patient presented with seizure. All symptoms and signs are listed in Table 3 and Fig. 6. 5.2. Surgical results and follow-up Total tumor resection was done in 10 patients while incomplete resection was done in other 5 patients (Table 4 & Fig. 7). During postoperative and follow up period, Proptosis improved in all patients as follows: complete improvement was observed in 10 (66.7%) patients, while the remaining 5(33.3%) patients showed significant improvement. Post-operatively, a transient visual deficit was observed in one patient which improved partially, cerebrospinal fluid (CSF) leak developed in three (20%) patients and managed conservatively, transient epilepsy developed in one (6.7%) patient and controlled medically as shown in Table 5 and Fig. 8. In our study there was no mortality. After a follow-up period of 2.1 years (range: 4 months–4 years), tumor recurrence was observed in three patients (20%) (Fig. 9) belonged to the uncompleted cases. One of them was reoperated with re-do reconstruction with same titanium mesh while other two patients refused the surgery and were treated conservatively. Postoperative radiological studies were obtained as shown in Figs. 10 and 11.
Fig. 4. Intraoperative picture of left sphenoid meningioma enplaque after craniotomy and drilling of left sphenoid ridge and lateral orbital wall.
6. Discussion Meningioma en plaque is defined as meningioma with sheet like pattern and whorls of neoplastic tissue associated with hyperostosis. Complete cure for Sphenoid wing meningiomas en plaque was difficult to be obtained as shown in different case studies due to their extensive bone, orbital, and neural involvement. The growth pattern of enplaque tumors is along the inner table of skull bone invading larger areas. The hyperostosis is often larger relatively than soft tissue component. The diagnosis is based on growth pattern not histopathological examination [8]. A female to male ratio in meningioma approximately 2:1 [9]. In meningiomas enplaque this ratio is higher, from 4:1 to 7:1 in
Fig. 5. Intraoperative picture after cranioplasty with titanium mesh.
20° to contralateral side. Care was given to avoid injury of temporal branche of fascial nerve (Fig. 3). Fronto-temporal craniotomy was done. Then extradural removal of the hyperostotic bone by high speed drills except for one case,
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Table 1 Comprehensive table of patient features. Pt. no.
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
Age
Sex
35 40 43 50 52 38 41 55 46 50 49 51 39 53 56
F F F F M F F F F M F F F F M
Side
Rt. Lt. Lt. Rt. Lt. Rt. Lt. Lt. Lt. Rt. Lt. Lt. Lt. Lt. Rt.
Clinical presentation
Operation
Proptosis
Visual deficit
Fits
Present Present Present Present Present Present Present Present Present Present Present Present Present Present Present
Present Present Absent Absent Absent Present Absent Absent Absent Present Present Present Present Present Present
Absent Absent Absent Absent Absent Absent Present Absent Absent Absent Absent Absent Absent Absent Absent
Complete Complete Complete Complete Complete Incomplete Complete Incomplete Complete Incomplete Complete Complete Complete Incomplete Incomplete
%
Range Mean ± SD
35–56 46.5 ± 6.7
Sex Male Female
3 12
20.0 80.0
Side Rt. Lt.
5 10
33.3 66.7
Table 3 Clinical presentation. Clinical presentation
No.
%
Proptosis Vision Fits
15 9 1
100.0 60.0 6.7
Fig. 6. Clinical presentation with percentage.
Table 4 Extent of tumor resection. Operation
No.
%
Complete Incomplete
10 5
66.7 33.3
Recurrence
Visual Deterioration
CSF Collection
Fits
Yes No No No No No No No No No No No No No No
Present Present Absent Absent Absent Absent Absent Absent Absent Absent Absent Absent Absent Absent Present
Absent Absent Absent Absent Absent Absent Present Absent Absent Absent Absent Absent Absent Absent Absent
No No Yes No No No No No Yes No No No No No Yes
some studies [4,10]. In our present study the female: male ratio was 4:1. The clinical manifestation of meningioma enplaque is mostly due to bone involvement (hyperostosis). Unilateral slow progressive proptosis is the most common presentation in different series, other manifestations include headache, visual impairment, seizures and affection of ocular motility. In our study proptosis was the most common presenting complaint in all 15 patients (100%). Nine patients (60%) manifested with visual impairment, one patient (6.7%) complained of seizure The clinical presentations of those lesions match with what have been reported in other case series [8,10–14]. As regards neuroimaging techniques, all patients with meningioma enplaque should be exposed to MRI and CT scans before surgery to evaluate both tumor components. MRI is the best for evaluation of soft tissue while C.T is better for bone extension. Some radiological features are suggestive of meningioma enplaque like hyperostosis pattern, surface irregularity, inward bulging of skull lesion and intracranial changes [15] which may help in differential diagnosis from other bony lesions like osteoma, fibrous dysplasia, and Paget’s disease. Thin layer of dural enhancement helps differentiation of meningioma en plaque from primary intraosseous meningioma. During excision of sphenoid meningioma enplaque, extradural resection of all involved bone is a first step. Resection of bone should be as generous as we can and planned according to preoperative CT images to allow decompression of orbital cavity, fissures, foramina of base and improvement of preoperative proptosis. Extradural approach provides more protection of the underlying neural structures while drilling of bone is away. Total removal of sphenoid wing meningiomas en plaque is very difficult to be obtained due to complexity of anatomy and behavior of these tumors to extend through fissures, foramina of skull base, orbit, cavernous sinus and rarely the pterygomaxillary fossa [4]. Ringel et al. [10] reported that 63 patients presented with sphenoorbital meningioma, from which 76% of patients had residuals, although 61% of residuals showed stationary course during follow-up, with no indication for more interference. Trials of total excision carry high risk of neurological morbidity, due to tumor extension to orbital cavity and foramina. Risk of postoperative complications should be taken into consideration during planning for surgical interference, as this is a benign lesions. However, some authors prefer total excision with total resection rates ranging from 60% to up to 80% [4,6]. In our study, total resection of meningioma enplaque with hyperoestotic bone took place in (66.7%) of patients while incomplete
Table 2 Demographic data and side of lesion. No.
Complications
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experience [11,6,3]. In our study, recurrence rates were 20% which is in accordance with preceding series as we excluded patients with cavernous sins involvement. In our study we use classic fronto temporal (pterional) approach due to its familiarity in our hospital and most patients need generous surgical exposure, other surgical strategies and approaches include mini lateral orbitotomy which recommended for patients with sphenoid meningioma enplaque extending in the anterior part of the temporal fossa and in the lateral or superomedial compartment of the orbit and anterior clinoid process [17]. Transorbital endoscopic approach, with endonasal decompression of orbit medially, may be alternative to craniotomy in patients of spheno orbital meningioma with lateral orbital wall hyperostosis mainly, less intracranial tumor extension or merely en plaque disease [18,19]. In our study, we preferred dural repair using galea-pericranial flap in all cases with water tight repair to minimize risk of cerebrospinal fluid leak. Reconstruction of orbital and fronto-temporal bone defects was done using titanium mesh, with no complications related to titanium mesh. Regarding cosmetic issue during postoperative and follow up period, Proptosis improved in all patients as follows: complete improvement was observed in 10 (66.7%) patients, while the remaining 5 (33.3%) patients showed significant improvement. Postoperatively a transient visual deficit was observed in one patient which improved partially, cerebrospinal fluid (CSF) leak occurred in three patients and treated conservatively and transient epilepsy developed in one patient and controlled medically. In our study there was no mortality. Postoperative radiation for patients with sphenoid wng meningioma enplaque is still debatable. some authors recommend adjuvant postoperative radiotherapy for residuals as that provide to control tumor growth and decrease rate of recurrence [17], while other authors do not recommend postoperative radiation routinely for patients with residual tumor, except patients with atypical and malignant meningioma [4].
Fig. 7. Extent of tumor resection and percentage.
Table 5 Post-operative complications. Complications
No.
%
Vision Collection Fits
1 3 1
6.7 20.0 6.7
Fig. 8. Post-operative complications.
7. Conclusion Sphenoid wing meningioma enplaque is a challenging tumor that has special criteria as regards clinical presentation, anatomical complexity and difficulties during attempts of total resection which carries high risk of postoperative neurological complications. Early detection with experienced neurosurgeons, microsurgical techniques and availability of high speed drill decrease the risk of postoperative morbidity. Reconstruction of Dural and bone defect is very important regarding functional and cosmetic aspects. Conflict of interest There is no conflict of interest on this manuscript.
Fig. 9. Tumor recurrence.
Funding excision with residuals occurred in (33.3%) of patients which matches with later series. As complete excision of meningioma en plaque is difficult to be obtained, recurrence rates have been as high as (35–50%) [16,4]. In recent series, results of low rates of recurrence was less than (10%), with early and more extensive surgical excision. The authors believe that the aim of surgery and its limitations should be dependent on clinical presentation of patients, extent of tumor, and surgical team
No available fund. Acknowledgment We would like to express our gratitude to Professor Ahmed Ibrahiem El Gheriany, Department of Deurosurgery, Assiut University for his continuous support and important advices and revisions during this work.
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Fig. 10. Postoperative CT brain axial cuts.
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Fig. 11. Postoperative CT skull bone 3D view with reconstruction by titanium mesh of left spheno orbital defect following excision of left sphenoid meningioma.
References
ridge, Surg. Neurol. 55 (2001) 265–269. [3] R.K. Shrivastava, C. Sen, P.D. Costantino, R. Della Rocca, Sphenoorbital meningiomas: surgical limitations and lessons learned in their long-term management, J. Neurosurg. 103 (2005) 491–496. [4] G. Mirone, S. Chibbaro, L. Schiabello, S. Tola, B. George, En plaque sphenoid wing meningiomas: recurrence factors and surgical strategy in a series of 71 patients,
[1] H. Cushing, L. Eisenhardt, Meningiomas. Their Classification, Regional Behavior, Life History, and Surgical End Results, Charles C. Thomas, Springfield, IL, 1938. [2] O. Jesus, M.M. Toledo, Surgical management of meningioma en plaque of the sphenoid
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Meningiomas involving the sphenoid wing outcome after microsurgical treatment–a clinical review of 73 cases, Cent. Eur. Neurosurg. 71 (4) (2010) 189–198. [14] H. Marcus, C. Schwindack, T. Santarius, R. Mannion, R. Kirollos, Image-guided resection of spheno-orbitalskull-base meningiomas with predominant intraosseous component, Acta Neurochir. (Wien) 155 (6) (2013) 981–988. [15] K.S. Kim, L.F. Rogers, D. Goldblatt, CT features of hyperostosing meningioma en plaque, AJR Am. J. Roentgenol. 149 (1987) 1017–1023. [16] J. Cophignon, J. Lucena, C. Clay, D. Marchac, Limits to radical treatment of sphenoorbital meningiomas, Acta Neurochir. Suppl. (Wien) 28 (1979) 375–380. [17] Amirjamshidi, Kazem Abbasioun, Rouzbeh Shams Amiri, Ali Ardalan, Seyyed Mahmood Ramak Hashemi, Lateral orbitotomy approach for removing hyperostosing en plaque sphenoid wing meningiomas. Description of surgical strategy and analysis of findings in a series of 88 patients with long-term follow up, Surg. Neurol Int. 6 (2015) 79. [18] J.P. Almeida, S.B. Omay, S.R. Shetty, Y.N. Chen, A.S. Ruiz-Treviño, B. Liang, et al., Transorbital endoscopic eyelid approach for resection of sphenoorbital meningiomas with predominant hyperostosis: report of 2 cases, J. Neurosurg. (2017) 1–11. [19] I. Dallan, S. Sellari-Franceschini, M. Turri-Zanoni, M. de Notaris, G. Fiacchini, F. Romana Fiorini, et al., Endoscopic transorbital superior eyelid approach for the management of selected spheno-orbital meningiomas: preliminary experience, Oper. Neurosurg. 14 (3) (2017) 243–251.
Neurosurgery 65 (Suppl. 6) (2009) S100–S108. [5] P.J. Derome, A. Visot, Bony reaction and invasion in meningiomas, in: O. Al-Mefty (Ed.), Meningiomas, Raven Press, New York, 1991p. 169. [6] U. Schick, J. Bleyen, A. Bani, W. Hassler, Management of meningiomas en plaque of the sphenoid wing, J. Neurosurg. 104 (2006) 208–214. [7] S. Gaillard, P. Pellerin, P. Dhellemmes, B. Pertuzon, J.P. Lejeune, J.L. Christiaens, Strategy of craniofacial reconstruction after resection of spheno-orbital en plaque meningiomas, Plast. Recontr. Surg. 100 (1997) 1113–1120. [8] S. Honeybul, G. Neil-Dwyer, D.A. Lang, B.T. Evans, D.W. Ellison, Sphenoid wing meningioma en plaque: a clinical review, Acta Neurochir. (Wien) 143 (2001) 749–758. [9] J. Wiemels, M. Wrensch, E.B. Claus, Epidemiology and etiology of meningioma, J. Neurooncol. 99 (2010) 307–314. [10] F. Ringel, C. Cedzich, J. Schramm, Microsurgical technique and result of a series of 63 spheno-orbital meningiomas, Neurosurgery 60 (Suppl. 2) (2007) S214–S222. [11] K. Bikmaz, R. Mrak, O. Al-Mefty, Management of bone-invasive, hyperostotic sphenoid wing meningiomas, J. Neurosurg. 107 (2007) 905–912. [12] P. Scarone, D. Leclerq, F. Heran, G. Robert, Long-term results with exophthalmos in a surgical series of 30 sphenoorbital meningiomas. Clinical article, J. Neurosurg. 111 (5) (2009) 1069–1077. [13] S. Honig, C. Trantakis, B. Frerich, I. Sterker, R.D. Kortmann, J. Meixensberger,
156
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Sphenoid meningioma enplaque with proptosis: Surgical excision, reconstruction and outcome
T
⁎
Abdelhakeem A. Essaa,1, , Ali R. Hamdanb,1 a b
Department of Neurosurgery, Faculty of Medicine, Assiut University Hospital, Assiut, Egypt Department of Neurosurgery, Qena Faculty of Medicine, South Valley University, Qena, Egypt
A R T I C L E I N F O
A B S T R A C T
Keywords: Meningioma Enplaque Proptosis Reconstruction Outcome
Objective: To evaluate surgical outcome and reconstruction of bone defects after excision of sphenoid meningioma enplaque. Patient and methods: Between June 2012 and May 2016, a series of 15 patients presented by proptosis attended to neurosurgery departments, Assiut university hospital, Qena university hospital, South Valley University and, These patients were diagnosed with sphenoid meningioma enplaque by fulfilling its criteria by neuroimaging (sheet-like meningioma and hyperostosis). All patients received preoperative imaging investigations including Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) to evaluate the extension of both tumor components – soft tissue and bone involvement. Fifteen patients were operated by pterional approach under general anesthesia. Results: Fifteen patients with meningioma enplaque were treated surgically. The mean age of patients at the time of admission was 46.5 years, age ranged between 35–56 years. 80% of patients were females (female to male ratio was 4:1). Ten (66.7%) patients have left sided lesion representing. Proptosis was the main presented manifestation in all patients. Complete tumor excision was done in 10 patients while incomplete excision was done in the rest of patients. Proptosis was improved post-operatively in patients presented by it: Ten (66.7%) patients had complete improvement while the other five (33.3%) patients improved significantly. No mortality occurred in our study. A mean follow-up period of 2.1 years (range: 4 months to 4 years), three (20%) patients have tumor recurrence. Conclusions: Total excision of Sphenoid meningioma enplaque carries difficulties and high rate of post-operative morbidity. Early detection with experienced neurosurgeons, microsurgical techniques and availability of high speed drill decrease the risk of post-operative morbidity. Reconstruction of dural and bone defect is very important regarding functional and cosmetic aspects.
1. Introduction Meningioma en plaque is defined as meningioma with sheet like pattern and whorls of neoplastic tissue associated with hyperostosis. This term was given by Cushing and Eisenhardt [1]. In meningioma, hyperostosis is a specific character of, and is presented in 4.5% of all meningiomas, but is presented more in meningioma en plaque with percentage of 13%–49% [1]. Meningiomas en plaque account about 2–9% of all meningiomas [2–4]. Mainly in the sphenoid wing, although other skull areas may be sites for enplaque [5]. Proptosis in meningioma enplaque is due to hyperostosis and that affect patients prognosis. The precise mechanisms of hyperostosis and intralesional morphological criteria of the infiltrated bone associated with
⁎
1
meningioma still not clear. Females are more susceptible to have Meningioma enplaque three to six times [6]. The histopathology of sphenoid meningiomas en plaque are the same as any meningiomas with predominance of meningothelial and transitional variants. The vascular supply of the sphenoid meningiomas enplaque usually arises from meningeal vessels but may be avascular [7,6]. Meningiomas enplaque have high rate of recurrence due to difficulties during total resection due to extensive bone component of these tumors and complexity of anatomy at site of origin [7].
Corresponding author. E-mail addresses: [email protected] (A.A. Essa), [email protected] (A.R. Hamdan). All authors contributed equally in this work.
https://doi.org/10.1016/j.clineuro.2018.02.028 Received 10 January 2018; Received in revised form 9 February 2018; Accepted 19 February 2018 Available online 21 February 2018 0303-8467/ © 2018 Elsevier B.V. All rights reserved.
Clinical Neurology and Neurosurgery 167 (2018) 147–156
A.A. Essa, A.R. Hamdan
2. Patients and methods
fulfilling its criteria by neuroimaging (sheet-like meningioma) and hyperostosis which larger than soft tissue. Non hyperostotic sphenoid wing meningioma, clinoidal meningioma, cavernous sinus meningioma with orbital extension and optic sheath meningioma were excluded as they do not met the criteria.
Between June 2012 and May 2016, a series of 15 patients presented by proptosis attended to neurosurgery departments, Assiut university hospital, Qena university hospital, South Valley University and, These patients were diagnosed with sphenoid meningioma enplaque by
Fig. 1. (a): CT axial and coronal cuts of skull with left sphenoid and orbital hyper oestosis. (b): Preoperative axial cuts of skull with left sphenoid and orbital hyperostosis of meningioma enplaque.
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Fig. 1. (continued)
3. Imaging findings
surgical excision of sphenoid wing meningioma enplaque. All patients received preoperative Computed Tomography (CT) and Magnetic Resonance Imaging (M.R.I). The axial and coronal bone window of CT revealed hyperostosis of sphenoid ridge, posterolateral orbital wall and
Assessment of both tumor components-soft tissue (dural/intradural) and hyperostosis bone is very important to do proper planning for 149
Clinical Neurology and Neurosurgery 167 (2018) 147–156
A.A. Essa, A.R. Hamdan
Fig. 2. Preoperative M.R.I brain T1 coronal cuts of with contrast showing left sphenoid soft tissue with sheet like enhancement characteristic of meningioma enplaque.
4. Surgical treatment
superior orbital wall (Fig. 1a and b). M.R.I scans showed T1 hyopintensity of soft tissue component of meningioma, T2 hyperintensity and homogenous enhancement after gadolinium injection with sheetlike at anterior temporal pole (Fig. 2).
Fifteen patients were operated by pterional approach under general anesthesia. All patients were placed in supine position with head tilted 150
Clinical Neurology and Neurosurgery 167 (2018) 147–156
A.A. Essa, A.R. Hamdan
interaoperative, as the high speed drill was broken-down so we used chiseling. Completion of bone removal using rongeurs and kerrison punches. Orbital decompression and superolateral orbitotomy was done in all cases, with protection to periorbital fat. Then decompression of superior orbital fissure and orbital cavity were done which were essential in all cases with proptosis (Fig. 4). After bone removal, the dura was opened and soft tissue component was excised microsurgically. Haemostasis was performed followed by dural graft using galea-pericranial flap in all cases with water tight repair to minimize risk of cerebrospinal fluid leak. Reconstruction of orbital and fronto-temporal bone defects was done using titanium mesh (Fig. 5). Histopathological examination of all tumors were meningiomas grade I World Health Organization (WHO). Meningothelial variant in ten patients and transitional type in other five. 5. Results 5.1. Clinical data
Fig. 3. Intraoperative picture after elevation of galea and temporalis muscle.
All features of fifteen patients included in our study are described in Table 1, including clinical presentation, extent of tumor resection, post operative complications and recurrence. Fifteen patients presented with sphenoid meningioma enplaque were surgically treated. The mean age was 46.5 years, age ranged from 35 to 56 years. Female patients were twelve patients representing (80%) of patients with female to male ratio 4:1. The side of lesion was left sided in ten patients representing (66.7%) (Table 2). All patients were presented with proptosis. Nine patients presented by visual impairment, one patient presented with seizure. All symptoms and signs are listed in Table 3 and Fig. 6. 5.2. Surgical results and follow-up Total tumor resection was done in 10 patients while incomplete resection was done in other 5 patients (Table 4 & Fig. 7). During postoperative and follow up period, Proptosis improved in all patients as follows: complete improvement was observed in 10 (66.7%) patients, while the remaining 5(33.3%) patients showed significant improvement. Post-operatively, a transient visual deficit was observed in one patient which improved partially, cerebrospinal fluid (CSF) leak developed in three (20%) patients and managed conservatively, transient epilepsy developed in one (6.7%) patient and controlled medically as shown in Table 5 and Fig. 8. In our study there was no mortality. After a follow-up period of 2.1 years (range: 4 months–4 years), tumor recurrence was observed in three patients (20%) (Fig. 9) belonged to the uncompleted cases. One of them was reoperated with re-do reconstruction with same titanium mesh while other two patients refused the surgery and were treated conservatively. Postoperative radiological studies were obtained as shown in Figs. 10 and 11.
Fig. 4. Intraoperative picture of left sphenoid meningioma enplaque after craniotomy and drilling of left sphenoid ridge and lateral orbital wall.
6. Discussion Meningioma en plaque is defined as meningioma with sheet like pattern and whorls of neoplastic tissue associated with hyperostosis. Complete cure for Sphenoid wing meningiomas en plaque was difficult to be obtained as shown in different case studies due to their extensive bone, orbital, and neural involvement. The growth pattern of enplaque tumors is along the inner table of skull bone invading larger areas. The hyperostosis is often larger relatively than soft tissue component. The diagnosis is based on growth pattern not histopathological examination [8]. A female to male ratio in meningioma approximately 2:1 [9]. In meningiomas enplaque this ratio is higher, from 4:1 to 7:1 in
Fig. 5. Intraoperative picture after cranioplasty with titanium mesh.
20° to contralateral side. Care was given to avoid injury of temporal branche of fascial nerve (Fig. 3). Fronto-temporal craniotomy was done. Then extradural removal of the hyperostotic bone by high speed drills except for one case,
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Table 1 Comprehensive table of patient features. Pt. no.
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
Age
Sex
35 40 43 50 52 38 41 55 46 50 49 51 39 53 56
F F F F M F F F F M F F F F M
Side
Rt. Lt. Lt. Rt. Lt. Rt. Lt. Lt. Lt. Rt. Lt. Lt. Lt. Lt. Rt.
Clinical presentation
Operation
Proptosis
Visual deficit
Fits
Present Present Present Present Present Present Present Present Present Present Present Present Present Present Present
Present Present Absent Absent Absent Present Absent Absent Absent Present Present Present Present Present Present
Absent Absent Absent Absent Absent Absent Present Absent Absent Absent Absent Absent Absent Absent Absent
Complete Complete Complete Complete Complete Incomplete Complete Incomplete Complete Incomplete Complete Complete Complete Incomplete Incomplete
%
Range Mean ± SD
35–56 46.5 ± 6.7
Sex Male Female
3 12
20.0 80.0
Side Rt. Lt.
5 10
33.3 66.7
Table 3 Clinical presentation. Clinical presentation
No.
%
Proptosis Vision Fits
15 9 1
100.0 60.0 6.7
Fig. 6. Clinical presentation with percentage.
Table 4 Extent of tumor resection. Operation
No.
%
Complete Incomplete
10 5
66.7 33.3
Recurrence
Visual Deterioration
CSF Collection
Fits
Yes No No No No No No No No No No No No No No
Present Present Absent Absent Absent Absent Absent Absent Absent Absent Absent Absent Absent Absent Present
Absent Absent Absent Absent Absent Absent Present Absent Absent Absent Absent Absent Absent Absent Absent
No No Yes No No No No No Yes No No No No No Yes
some studies [4,10]. In our present study the female: male ratio was 4:1. The clinical manifestation of meningioma enplaque is mostly due to bone involvement (hyperostosis). Unilateral slow progressive proptosis is the most common presentation in different series, other manifestations include headache, visual impairment, seizures and affection of ocular motility. In our study proptosis was the most common presenting complaint in all 15 patients (100%). Nine patients (60%) manifested with visual impairment, one patient (6.7%) complained of seizure The clinical presentations of those lesions match with what have been reported in other case series [8,10–14]. As regards neuroimaging techniques, all patients with meningioma enplaque should be exposed to MRI and CT scans before surgery to evaluate both tumor components. MRI is the best for evaluation of soft tissue while C.T is better for bone extension. Some radiological features are suggestive of meningioma enplaque like hyperostosis pattern, surface irregularity, inward bulging of skull lesion and intracranial changes [15] which may help in differential diagnosis from other bony lesions like osteoma, fibrous dysplasia, and Paget’s disease. Thin layer of dural enhancement helps differentiation of meningioma en plaque from primary intraosseous meningioma. During excision of sphenoid meningioma enplaque, extradural resection of all involved bone is a first step. Resection of bone should be as generous as we can and planned according to preoperative CT images to allow decompression of orbital cavity, fissures, foramina of base and improvement of preoperative proptosis. Extradural approach provides more protection of the underlying neural structures while drilling of bone is away. Total removal of sphenoid wing meningiomas en plaque is very difficult to be obtained due to complexity of anatomy and behavior of these tumors to extend through fissures, foramina of skull base, orbit, cavernous sinus and rarely the pterygomaxillary fossa [4]. Ringel et al. [10] reported that 63 patients presented with sphenoorbital meningioma, from which 76% of patients had residuals, although 61% of residuals showed stationary course during follow-up, with no indication for more interference. Trials of total excision carry high risk of neurological morbidity, due to tumor extension to orbital cavity and foramina. Risk of postoperative complications should be taken into consideration during planning for surgical interference, as this is a benign lesions. However, some authors prefer total excision with total resection rates ranging from 60% to up to 80% [4,6]. In our study, total resection of meningioma enplaque with hyperoestotic bone took place in (66.7%) of patients while incomplete
Table 2 Demographic data and side of lesion. No.
Complications
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experience [11,6,3]. In our study, recurrence rates were 20% which is in accordance with preceding series as we excluded patients with cavernous sins involvement. In our study we use classic fronto temporal (pterional) approach due to its familiarity in our hospital and most patients need generous surgical exposure, other surgical strategies and approaches include mini lateral orbitotomy which recommended for patients with sphenoid meningioma enplaque extending in the anterior part of the temporal fossa and in the lateral or superomedial compartment of the orbit and anterior clinoid process [17]. Transorbital endoscopic approach, with endonasal decompression of orbit medially, may be alternative to craniotomy in patients of spheno orbital meningioma with lateral orbital wall hyperostosis mainly, less intracranial tumor extension or merely en plaque disease [18,19]. In our study, we preferred dural repair using galea-pericranial flap in all cases with water tight repair to minimize risk of cerebrospinal fluid leak. Reconstruction of orbital and fronto-temporal bone defects was done using titanium mesh, with no complications related to titanium mesh. Regarding cosmetic issue during postoperative and follow up period, Proptosis improved in all patients as follows: complete improvement was observed in 10 (66.7%) patients, while the remaining 5 (33.3%) patients showed significant improvement. Postoperatively a transient visual deficit was observed in one patient which improved partially, cerebrospinal fluid (CSF) leak occurred in three patients and treated conservatively and transient epilepsy developed in one patient and controlled medically. In our study there was no mortality. Postoperative radiation for patients with sphenoid wng meningioma enplaque is still debatable. some authors recommend adjuvant postoperative radiotherapy for residuals as that provide to control tumor growth and decrease rate of recurrence [17], while other authors do not recommend postoperative radiation routinely for patients with residual tumor, except patients with atypical and malignant meningioma [4].
Fig. 7. Extent of tumor resection and percentage.
Table 5 Post-operative complications. Complications
No.
%
Vision Collection Fits
1 3 1
6.7 20.0 6.7
Fig. 8. Post-operative complications.
7. Conclusion Sphenoid wing meningioma enplaque is a challenging tumor that has special criteria as regards clinical presentation, anatomical complexity and difficulties during attempts of total resection which carries high risk of postoperative neurological complications. Early detection with experienced neurosurgeons, microsurgical techniques and availability of high speed drill decrease the risk of postoperative morbidity. Reconstruction of Dural and bone defect is very important regarding functional and cosmetic aspects. Conflict of interest There is no conflict of interest on this manuscript.
Fig. 9. Tumor recurrence.
Funding excision with residuals occurred in (33.3%) of patients which matches with later series. As complete excision of meningioma en plaque is difficult to be obtained, recurrence rates have been as high as (35–50%) [16,4]. In recent series, results of low rates of recurrence was less than (10%), with early and more extensive surgical excision. The authors believe that the aim of surgery and its limitations should be dependent on clinical presentation of patients, extent of tumor, and surgical team
No available fund. Acknowledgment We would like to express our gratitude to Professor Ahmed Ibrahiem El Gheriany, Department of Deurosurgery, Assiut University for his continuous support and important advices and revisions during this work.
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Fig. 10. Postoperative CT brain axial cuts.
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Fig. 11. Postoperative CT skull bone 3D view with reconstruction by titanium mesh of left spheno orbital defect following excision of left sphenoid meningioma.
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