Anita Pék1, Norbert Pesztenlehrer1, Sándor Czirják2, Zoltán Zsolt Nagy3,János Németh3, Krisztina Knézy3

1Petz Aladár Teaching Hospital, Department of Ophthalmology, Győr, Hungary
2National Institute of Neurosurgery, Budapest, Hungary
3Semmelweis University, Department of Ophthalmology, Budapest, Hungary

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Meningioma is the most common primary intracranialspace-occupying tumor. It can appear at any age butmost commonly at women in their 50s. The appearanceof meningiomas are 60% sporadic, 40% causedby unknown factors. In terms of the location two generaltypes can be identified: primary (ONSM) and secondary.The primary tumor starts to grow intraorbital orintracanalicular, while the secondary type develops intracranialmainly from the os sphenoidale or the sellaturcica regions. The former type covers only 1% of allmeningiomas and is more common at neurofibromatosistype 2. Both types are painless, they mightcause slowly deteriorating vision, slight proptosis andsometimes diplopia. If the tumor compresses the opticnerve directly or causes intracranial pressure increase,it leads usually to visual impairment or occasionally toloss of vision on the affected eye. Generally it is histologicallybenign, but at a few cases it might becomemalignant and emerge as metastasis (mainly asbreast or lung carcinoma). It should be differentiatedfrom schwannoma, neurofibroma, ependymoma, astrocytoma,haemangiopericytoma, metastatic carcinoma.

Case report

The patient visited our clinic for the first time in May2014, complaining about feeling of unstableness andappearance of colourful circles when looking into thelight. Her symptoms were present for 6 months. In Februaryshe noticed narrower right palpebral fissure andlachrymation, since March she perceived blurred-vision.

The patient wearing multifocal glasses for yearssummarized her complaints: „I see differently than earlier.”Moreover she had 1.0 BCVA with hypermetropiccorrection.

Her history did not include any serious eye diseases,did not suffer any blows or trauma. From her generalhistory a tonsillectomy and a right-sided hip replacementsurgery can be highlighted.

During the inspection we observed that the rightpalpebral fissure was minimally narrower compared tothe opposite side (5/7mm) (Fig. 1).

During guided eye movements the upward movementof the right eye was limited (Fig. 2), in the other eightdirections the motor functions were normal, howeverlooking left the patient indicated explicit diplopia.

No side differences were observed with Hertel’s exophthalmometer/16/16 mm/. At that time the direct andindirect pupillary reactions were intact, diameter differencesbetween the two pupils were 0.8 mm. Followinga basic slit lamp investigation, no significantanomalies were discovered: neither at the anterior, norat the posterior segment.

The critical fusion frequency (CFF) – which scans thefunction of the optic nerve –showed slight decreaseon both sides (33/35 Hz). Dysfunction of the right andthe over-activity of the left superior rectus muscle wereobserved with the help of Hess-chart. Goldmann-typeperimeter inspection revealed a dent in the isopter onthe top-right side and a minimal widening of the blindspot on the left (!) side. For the sake of completenesswe also carried out an ultrasound examination whichdid not detect any pathological differences in the orbit.


Since no ophthalmological explanation could befound for the eye-movement disorder, right-sided ptosisor for the functional deviation of the optic nerve,suspicion for an intracranial space-occupation wasraised. A week later a cranial- and orbital MRI confirmeda parasellar meningioma at the patient. It intensivelyaccumulated gadolinium on the right side ofthe medium scala with a size of 18x25x30 mm (Fig. 3).

The carotis syphon was medially dislocated: runningalongside the right optic nerve for a short section,reaching the chiasm.Cranial MRI with gadolinium has a prominent role in diagnosingparasellar meningioma, because this tumor’shomogenous enhancement cannot be confusedwith a malignant tumor. Calcified areas can berevealed through CT inspection without contrastagent.

Treatment options: tumors causing no complaints demandclose observation. There are mixed views on thetreatment opinions. Many publications appeared on 3D-CRT and single-fraction stereotactic radiosurgery:these enable no permanent solution and can lead toside effects such as iritis, dry eyes and orbital pain. Incase of complaints, surgical intervention should beconsidered, even though the extension of the resectionis problematic. In case of sphenoid wing meningiomastotal surgical removal is often not possible.

Conservative resection combined with radiosurgeryhas showed similar results as aggressive excision.Another future option would be drug therapy. Mostmeningiomas are immunohistochemically positive toepithelial membrane antigen and vimentin, while negativeto glial fibrillary antigen. Hormones have an effecton the size of the tumor: increase in size was observedduring pregnancy and menses. Olson and othershave studied in vitro the inhibition of meningiomas’cell receptors. Estradiol 17-beta, progesterone and tamoxifenstimulated growth in some cell cultures. At 5out of 14 inoperable cases Mifepriston (RU 486) resultedin a 10% reduction of the tumor, however thestate of 3 other patients worsened. So given the currentstate of our knowledge the drug therapy does notinfluence tumor formation.

Following neurosurgical consultation the surgical removalof the tumor was suggested. However – due toinherent surgical risks (e.g. potential loss of vision) –the patient is reluctant at the moment and refuses theoperation: besides eye care she is searching for alternativecures.


During the observations the patient’s complaints intensified:her right-sided eye movements are limited in alldirections, right-sided pupil dilatation is visible even forthe naked eye. The repeated Hess-chart examinationshows the progression well. The increased complaintsdue to diplopia could not be treated with prismaticglasses: the patient has to continue living with the disturbingdouble-vision. Over a year we observed clearprogression in her ophthalmological status: the controlMRI confirmed the growth of the tumor.

Introduction: Intracranial space-occupying processes can lead to a diverse set of complaints.Ocular anomalies caused by parasellar meningioma and their treatment options are presentedin this paper through one case.

Case report: A 64 year old woman visited our clinic complaining about blurred-vision in her righteye, feeling of unstableness and appearance of colourful circles when looking into the light -negatively affecting her everyday life. With hypermetropic correction she had 1.0 visual acuity(VA) on both eyes. Following an extensive neuroophthalmological investigation, no significantanomalies were discovered: neither at the anterior, nor at the posterior segment. After a coupleof months, with stable VA partial right-sided oculomotor nerve palsy was developed with an increasingdegree of diplopia. Restricted upward eye-movement with aslight ptosis and mydriasiswere also observed. An explanation to the complaints was provided by a cranial- and orbitalMRI: parasellar meningioma has been diagnosed at the patient. The carotid siphon was mediallydislocated: running alongside the right optic nerve for a short section, almost reaching the chiasm.Following neurosurgical consultation the surgical removal of the tumour was suggested.However – due to inherent surgical risks (e.g. potential loss of vision) – the patient is reluctant atthe moment and refuses the operation trying to continue living with the disturbing diplopia.

Conclusion: Space-occupying processes around the orbital apex or the cavernous sinus oftenbegin with ophthalmological complaints that are difficult to detect. If the cause is discoveredbefore the optic nerve is damaged, there is a better chance to preserve vision following a surgicalintervention.

  1. Albert Jacobiec’s, Daniel M. Albert, Joan W. Miller,Dimitri T. Azar, Barbara A. Blodi. Orbital pathology. In: DivaR. Salomao, Anja C. Roden (ed.): Principles and Practice ofOphthalmology. 3t h ed. Vol.3. Saunders, Toronto, Ontario,Canada, 2008; 3769.
  2. Albert Jacobiec’s, Daniel M. Albert, Joan W. Miller,Dimitri T. Azar, Barbara A. Blodi. Pathology of the opticnerve. In: Patricia Chevez-Barrios, Hidehiro Takei (ed.): Principlesand Practice of Ophthalmology. 3th ed. Vol.3. Saunders,Toronto, Ontario, Canada, 2008; 3798-3800.
  3. Albert Jacobiec’s, Daniel M. Albert, Joan W. Miller,Dimitri T. Azar, Barbara A. Blodi. Chiasmal disorders. In:Misha L. Pless (ed.): Principles and Practice of Ophthalmology3th ed. Vol.3. Saunders, Toronto, Ontario, Canada, 2008;3942.
  4. Albert Jacobiec’s, Daniel M. Albert, Joan W. Miller,Dimitri T. Azar, Barbara A. Blodi. Tumors of the Anterior VisualPathways. In: Robert A. Egan (ed.): Principles and Practiceof Ophthalmology. 3th ed. Vol.3. Saunders, Toronto, Ontario,Canada, 2008; 3896-3902.
  5. Anthony Pane, Michael Burdon, Neil Miller. Blurred visionor field loss. The Neuro-Ophthalmology Survival Guide.1st ed. Elsevier, Philadelphia, Pennsylvania, 2006; 72-74.
  6. Anthony Pane, Michael Burdon, Neil Miller. Double vision.The Neuro-Ophthalmology Survival Guide, 1s t ed. Elsevier,Philadelphia, Pennsylvania, 2006; 215-222.
  8. Brkic H, Hodzic M, Moranjkic M. Intracranial Meningeoma-ten-year evalutaion. Med Glas (Zenica). 2013 Feb; 10(1): 157-60.
  9. Ernst-Stecken A, Lambrecht U, Ganslandt O, MuellerR, Fahlbusch R, Sauer R, Grabenbauer G. Radiosurgery ofsmall skull-base lesions. No advantage for intensity-modulatedstereotactic radiosurgery versus conformal arc technique.Strahlenther Onkol 2005 May; 181 (5): 336-44.
  10. Kerty E. Optic nerve sheats meningeoma—from expectationfrom active treatment. Tidsskr Nor Laegeforen.2005 Feb 17; 125 (4): 429-30.
  11. Sheehan JP, Starke RM, Kano H, Kaufmann AM, MathieuD, Zeiler FA, West M, Chao ST, Varma G, Chiang VL, YuJB, McBride HL, Nakaji P, Youssef E, Honea N, Rush S, KondziolkaD, Lee JY, Bailey RL, Kunwar S, Petti P, Lunsford LD.Gamma Knife radiosurgery for sellar and parasellar meningiomas:a multicenter study. J Neurosurg 2014 Jun;1 20(6):1268-77. doi: 10.3171/2014.2. JNS13139. Epub 2014 Mar28.
  12. Yamvamoto J, Takahashi M, Nakano Y, et al. Preoperativeradiological evaluation and surgical strategy forthe preservation of visual acuity in patients with skull basemeningiomas – -two case reports. J UOEH 2011 Sep 1; 33(3): 217-23.
  13. Sato K, Nomura R, Suzuki I. Cyber Knife treatment forparasellar tumors. Brain Nerve 2011 Mar; 63 (3): 203-8.
  14. Williams BJ, Yen CP, Starke RM, Basina B, Nguyen J,Rainey J, Sherman JH, Schlesinger D, Sheehan JP. GammaKnife surgery for parasellar meningiomas: long-term resultsincluding complications, predictive factors, and progression-free survival. J Neurosurg 2011 Jun; 114 (6): 1571-7. doi:10.3171/2011.1.JNS091939. Epub 2011 Feb 11.
  15. Tzekov C, Spiriev T, Cherninkova S, Bussarsky V, LalevaL, Cekov A, Naydenov E, Minkin K, Marinov M, RomanskyK. Characteristics and prognosis of visual deficitcaused by parasellar meningiomas. Khirurgiia (Sofiia)2010;(2-3):19-23.

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