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Journal Français d'Ophtalmologie
Volume 40, n° 9
pages e361-e366 (novembre 2017)
Doi : 10.1016/j.jfo.2017.07.001
Posters de la SFO

Spheno-orbital meningioma during pregnancy: Case report of a 37-year-old woman
Méningiome sphéno-orbitaire de la femme enceinte : cas d’une femme de 37 ans

R.K. Khanna a, , S. Arsene a, S. Velut b, I. Zemmoura b, K. Gaillot c, P.-J. Pisella a, M.-L. Le Lez a
a Service ophthalmology, CHRU de Tours, 2, boulevard Tonnelé, 37044 Tours, France 
b Service neurosurgery, CHRU de Tours, 2, boulevard Tonnelé, 37044 Tours, France 
c Service radiology, CHRU de Tours, 2, boulevard Tonnelé, 37044 Tours, France 

Corresponding author. 14, rue de l’Elysée, 37000 Tours, France.

Spheno-orbital meningiomas are benign tumors, which arise from the orbital dura. They extend to the orbit through the superior orbital fissure or the optic canal or else through bone invasion. In most cases, orbital meningiomas are in the greater wing of the sphenoid bone. Symptoms are caused by reactive orbital hyperostosis leading to mass effect: proptosis, compressive optic neuropathy, oculomotor disorders. Classically, these symptoms occur lately because of slow tumor growth [1]. Some of these tumors are characterized by hormonal dependency to progesterone or estrogen hormones making them more common in females [2, 3]. Thus, the natural evolution of orbital meningioma is slow but can be accelerated during specific periods of life like pregnancy or menopause. We report the case of a pregnant woman with proptosis and impaired visual function leading to the diagnosis of fast-growing spheno-orbital meningioma.

Case report

A 37-year-old pregnant woman at 26 weeks of amenorrhea (WA) came to our ophthalmology department for decreased visual acuity and proptosis of her right eye. At first examination, right eye proptosis measurement using Hertel's exophthalmometer was 20 millimeters (16 millimeters on the left eye). Visual acuity was 20/20 Parinaud 2 on both eyes. Right fundus examination revealed paleness on the temporal side of the optic disk (Figure 1). Goldmann visual field exam unveiled unilateral relative cecocentral scotoma (Figure 2). Visual early-evoked potentials examination showed delayed P100 response only with flash stimulus. We suspected right compressive optic neuropathy. Neuroimaging (computed tomograph and magnetic resonance imaging) confirmed grade 2 proptosis and disclosed a spheno-orbital meningioma extending from greater wing of the right sphenoid bone. Mass effect on lateral rectus and optic nerve was identified with optic canal and superior orbital fissure stenosis (Figure 3). According to the patient's will and to our multidisciplinary care team composed by a neurosurgeon, an obstetrician, an anesthesiologist and an ophthalmologist, risk-benefit ratio of a surgical procedure was estimated to be high. Surgery's main risks were aortocaval compression and fetal distress. Therefore, we established active surveillance every two weeks.

Figure 1

Figure 1. 

Fundus photography. Right optic disk temporal palor.


Figure 2

Figure 2. 

Right eye goldmann visual field perimetry at 26 weeks of amenorrhea. Relative cecocentral scotoma. Constriction of the I3 isopter.


Figure 3

Figure 3. 

a and b: bone window head computed tomography. Spheno-orbital meningioma in greater wing of the sphenoid bone (green arrow). Optic canal (red arrow) and superior ophthalmic fissure (orange arrow) stenosis; c: head magnetic resonance imaging: gadolinium-enhanced T1; d: head magnetic resonance imaging: T2. Right eye lateral rectus and optic nerve deviation. Dural attachment of the meningioma near the temporal lobe.


At 32 WA, visual acuity decreased to 20/100 with occurrence of an inferior relative scotoma. Mass effect secondary to peritumoral optic nerve edema was suspected. Clinical improvement was obtained under systemic corticosteroid treatment. However, therapy induced steroid diabetes, which leaded to hospitalization for insulin therapy and close monitoring of blood sugar level.

At 37 WA, visual acuity decreased to 20/30 Parinaud 10 because of a severe absolute central scotoma on visual field exam (Figure 4). Vaginal delivery was induced leading to smooth birth without any incident. At day two following delivery, visual acuity remained decreased. MRI showed mass effect increase on the intracanalicular portion of the optic nerve. Intravenous bolus of 1 gram a day of methylprednisolone was administrated to the patient over 3 days without significant visual acuity recovery. Thus, the patient underwent surgery. Meningioma resection was partial and extent of surgery was Simpson grade 3 because of remaining intracavernous sinus dural attachment [4]. Histological analysis of bone and dural biopsies confirmed spheno-orbital meningioma. Ki67 proliferation index was low but the tumor presented high concentration of receptors to progesterone. These results explained strong tumor growth during pregnancy. One month after surgery, visual acuity improved to 20/20 Parinaud 2. Visual field examination revealed sequelar superonasal quadrantanopia (Figure 5). Esthetic result was satisfactory despite persistent proptosis (Figure 6). Neuroimaging showed optic canal and superior orbital fissure opening and mass effect decrease (Figure 7).

Figure 4

Figure 4. 

Right eye Goldmann visual field perimetry at 37 weeks of amenorrhea. Central absolute scotoma.


Figure 5

Figure 5. 

Right eye Goldmann visual field perimetry one month after surgery. Sequelar superonasal quadrantanopia.


Figure 6

Figure 6. 

Photography. Right eye proptosis and superior eye-brow swelling.


Figure 7

Figure 7. 

Bone window head computed tomography after surgery (a and b) compared to before surgery (c and d). Subtotal resection of the spheno-orbital meningioma. Opening of the optic canal (red arrow) and the superior ophthalmic fissure (orange arrow).



In the case of meningioma during pregnancy, compressive optic neuropathy can threaten the mother's visual function but there are also other hazards involving the baby's health, prematurity, aortocaval compression and fetal distress. Considering vital prognosis for the child and functional prognosis for the mother, risk-benefit ratio evaluation requires a multidisciplinary care team including an ophthalmologist, a neurosurgeon, an anesthesiologist and an obstetrician.

Corticosteroid therapy can be helpful in case of visual loss secondary to an inflammatory process. Computed tomography can be realized during pregnancy and is indicated for evaluating the bone boundaries of the orbital meningioma. MRI is also indicated for assessing optic canal, superior orbital fissure narrowing and optic nerve deviation, which are correlated to the visual prognosis [1, 5].

Simpson grading system evaluates the extent of the resection, which is an important predictive factor of recurrence [6, 7].

Hormonal dependency to progesterone contraindicates oral contraceptives with progesterone and hormone replacement therapy. The patient has to be informed on recurrence risk in case of another pregnancy. Long-term follow-up has to schedule for several years especially in case of incomplete resection.

Disclosure of interest

The authors declare that they have no competing interest.

 This letter to the editor has been presented as an e-poster at the 123rd annual congress of the French Society of Ophthalmology in 2017.


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