Tumors of the olfactory groove may cause unilateral optic atrophy with contralateral papilledema and anosmia (Foster Kennedy syndrome). We describe a case of a young pregnant woman with Foster Kennedy syndrome due to an olfactory groove meningioma

Douglas Anderson

Federico Rodríguez-Porcel

Ian Hughes

John Lee

José Biller

Frontiers in Neurology

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Foster Kennedy Syndrome Due to Meningioma Growth during Pregnancy

  
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CLINICAL CASE STUDY
published: 11 November 2013
doi: 10.3389/fneur.2013.00183
Foster Kennedy syndrome due to meningioma growth
during pregnancy
Federico Rodríguez-Porcel 1, Ian Hughes2, Douglas Anderson3, John Lee2 and José Biller 1*
1 Department of Neurology, Stritch School of Medicine, Loyola University Chicago, Maywood, IL, USA
2 Department of Pathology, Stritch School of Medicine, Loyola University Chicago, Maywood, IL, USA
3 Department of Neurosurgery, Stritch School of Medicine, Loyola University Chicago, Maywood, IL, USA
Edited by:
Gregory Gruener, Loyola University,
USA
Reviewed by:
Gregory Gruener, Loyola University,
USA
Emmanuel Sanya, University of Ilorin
Teaching Hospital, Nigeria
*Correspondence:
José Biller , Department of Neurology,
Stritch School of Medicine, Loyola
University Chicago, 2160 South, 1st
Avenue, Building 105, Room 2700,
Maywood, IL 60153, USA
e-mail: jbiller/ at/ lumc.edu
Tumors of the olfactory groove may cause unilateral optic atrophy with contralateral
papilledema and anosmia (Foster Kennedy syndrome). We describe a case of a young
pregnant woman with Foster Kennedy syndrome due to an olfactory groove meningioma.
Keywords: Foster Kennedy syndrome, meningioma, pregnancy, optic disk atrophy, papilledema, anosmia
CASE PRESENTATION
A 37-year-old right handed woman from Rwanda in her 37th preg-
nancy week presented to our Emergency Department in labor.
She was admitted to the High Risk Maternal Fetal Medicine
Service. Neurology was consulted for assessment of protracted
headaches of approximately 2 years duration, worsening since the
first trimester of her pregnancy. In addition, visual acuity of her
left eye (OS) had gradually worsened during the same period of
time, resulting in complete monocular visual loss.
She had no history of nausea, vomiting, transient visual obscu-
rations, galactorrhea, rhinorrhea, or seizures. She had no exposure
to ionizing radiation or repeated cranial trauma.
On neurologic examination she had bilateral anosmia, lack
of light perception OS, and visual acuity was 20/20 OD. There
was a left relative afferent pupillary defect (APD). Funduscopy
showed pallor of the left optic disk and blurred nasal margins
of the right optic disk. Complete blood count (CBC), complete
metabolic profile (CMP), thyroid stimulating hormone (TSH),
prolactin, follicular stimulating hormone, luteinizing hormone
(LH), and growth hormone (GH) were within the normal range
expected during pregnancy. MRI of the brain without contrast
showed a 3.7 cm× 3.2 cm× 1.4 cm homogeneously isointense on
T1 suprasellar mass along the dural floor of the cranial fossa with
compression of the left optic apex with moderate edema and no
hydrocephalus. This was a significant increase from her previous
MRI done a year earlier in Rwanda (Figure 1). After the first MRI
she was told to come to the US to seek neurosurgical intervention.
At our institution, she received dexamethasone 4 mg every 6 h
and had an uneventful delivery through a cesarean section (C-
Section). Three days after C-section, a bicoronal craniotomy was
done with subtotal tumor removal. A temporary lumbar drain
was placed. Levetiracetam for seizure prophylaxis was given and
dexamethasone taper was started on postoperative day 5. Surgical
pathology showed meningioma WHO grade I, positive for prog-
esterone receptors. The Ki-67 proliferation index overall was less
than 1% (Figures 2 and 3). Following surgery, she was able to
perceive light and motion with her OS.
DISCUSSION
The clinical presentation of unilateral optic atrophy with con-
tralateral papilledema and anosmia was first fully described by
Kennedy in 1911 (1). Mass lesions accounting for Foster Kennedy
syndrome are frequently located in the olfactory groove, falx
cerebri, sphenoid wing, or subfrontal region (2). Most masses
are neoplastic, and meningiomas are the most prevalent lesions.
Other reported mass lesions causing Foster Kennedy syndrome
include frontal lobe abscesses, craniopharyngiomas, pituitary ade-
nomas, plasmacytomas, nasopharyngeal angiofibromas, neurob-
lastomas, and aneurysms (2). Direct compression of one optic
nerve accounts for ipsilateral optic atrophy. Raised intracranial
pressure (ICP) causes contralateral papilledema that usually pre-
cedes the ipsilateral optic atrophy (3) However, bilateral optic
nerve compression without elevation of ICP, and chronic ele-
vated ICP with compression have been postulated as other pos-
sible mechanisms (4). Headaches, emotional lability, memory
loss, nausea, vomiting, or weakness may also be present (4).
Visual changes may present as transient visual obscurations, cen-
tral scotomata, unilateral or bilateral visual loss, or visual field
defects (4).
Meningiomas account for 15% of all intracranial tumors, and
may present as a solitary mass or as multiple masses, the latter
usually associated with neurofibromatosis type 2. Most common
locations of meningiomas are over the convexities of the parasagit-
tal sphenoid ridge, olfactory groove, suprasellar/parasellar regions,
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Rodríguez-Porcel et al. Foster Kennedy syndrome in pregnancy
FIGURE 1 | MRI of the brain with and without contrast done in
Rwanda 1 year previous to admission to our institution showed
3 cm×2.6 cm×1.2 cm homogeneously enhancing suprasellar mass
along the dural floor of the cranial fossa.
FIGURE 2 |The specimen consists of uniform, benign-appearing,
spindle-shaped cells with whorl formations containing hyalinized
material with some degeneration. In time, with calcification, these whorls
will progress to psammoma bodies classically observed in meningiomas.
tentorium cerebelli, pineal region, optic nerve, and foramen
magnum (5).
Olfactory groove meningiomas (OGM) account for 8–13% of
all intracranial meningiomas (5). These tumors arise in the mid-
line of the cribriform plate and the fronto sphenoidal suture, and
may extend from the crista galli to the tuberculum sellae (6).
Extension may be asymmetric and may compromise the ethmoid
sinuses, the nasal cavity, or even the orbit. (7). OGMs receive
their vascular supply primarily from the anterior and posterior
ethmoidal arteries, branches of the ophthalmic artery.
Risk factors for development of meningiomas include chromo-
some deletion, previous ionizing therapy, and head trauma (5).
The reported 2:1 female/male increases during reproductive years
FIGURE 3 | Progesterone receptor antibody staining, showing the
presence of PR in the specimen.
to 3.15:1 (8). Meningiomas may progress following contraceptive
implants, during the luteal phase of the menstrual cycle, or dur-
ing pregnancy (9, 10). Further studies have shown the presence of
estrogen, progesterone, and androgen receptors, with progesterone
receptors being present in 80% of meningiomas among women,
and only 40% among men (11). However, the potential correla-
tion of presence of hormone receptors and the natural history of
meningiomas has been inconsistent.
Incidence of meningiomas in pregnancy is not different than
in non-pregnant women. However, symptoms may flare up dur-
ing the first two trimesters of pregnancy, with subsequent tumoral
shrinkage post-partum and possible recurrence with subsequent
pregnancies (12, 13). Besides the proposed influence of sexual hor-
mones, this clinical flare-up has been attributed to water retention
and engorged blood vessels (14, 15).
In pregnant patients suspected of having an intracranial mas
lesion, MRI remains the preferred imaging modality because of
its greater resolution, increased sensitivity, and lack of ionizing
radiation. The use of gadolinium in pregnant patients controver-
sial as gadolinium can cross the placenta, but has not been shown
to cause birth defects (16, 17). CT carries the risk of ionizing
radiation to the fetus, but given its greater availability, lower cost,
and utility in assessing of calcifications, its use should be consid-
ered in selected circumstances and always with proper abdominal
shielding (18, 19).
Management of meningiomas during pregnancy requires a
multidisciplinary approach with close surveillance of mother and
fetus, with delivery at term and post-partum tumor resection
(20). Vaginal delivery is contraindicated in the presence of raised
ICP, because cerebrospinal fluid (CSF) pressure increases with
each uterine contractions during the second stage of labor (21).
However, tumor resection might be warranted before delivery in
patients with malignancy, active hydrocephalus requiring shunt-
ing, benign growth associated with signs of impending herniation,
or progressive neurologic deficits (14). Peripartum and periopera-
tively edema leading to ICP can be managed with tapering doses of
Frontiers in Neurology | Neurology Education November 2013 | Volume 4 | Article 183 | 2
  
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Rodríguez-Porcel et al. Foster Kennedy syndrome in pregnancy
dexamethasone. (22, 23). Mannitol should be used with caution,
given that it crosses the placenta and may affect the fetus. Its use
should only be limited to emergency situations (24). If seizures
occur, they should be treated appropriately, as the risk associated
with recurrent seizures is far greater than the risks of properly cho-
sen antiepileptic drugs (14). If tumor margins are not well defined,
or the nature of the meningioma is more aggressive with the poten-
tial for high risk of recurrence, adjuvant external beam radiation
in the early post operative period should be considered (21).
Olfactory groove meningiomas may grow rapidly during the
first two trimesters of pregnancy leading to devastating conse-
quences. Close monitoring with thorough neurologic examination
is recommended as the first step to assess any significant changes in
mass effect (25). The combination of unenhanced and enhanced
MRI is recommended for the detection of residual or recurrent
meningiomas after surgery.
Our patient presented with a symptomatic OGM that grew
during her pregnancy. Following a successful delivery, the menin-
gioma was removed.
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Conflict of Interest Statement: The authors declare that the research was conducted
in the absence of any commercial or financial relationships that could be construed
as a potential conflict of interest.
Received: 13 September 2013; accepted: 26 October 2013; published online: 11
November 2013.
Citation: Rodríguez-Porcel F, Hughes I, Anderson D, Lee J and Biller J (2013) Foster
Kennedy syndrome due to meningioma growth during pregnancy. Front. Neurol. 4:183.
doi: 10.3389/fneur.2013.00183
This article was submitted to Neurology Education, a section of the journal Frontiers
in Neurology.
Copyright © 2013 Rodríguez-Porcel, Hughes, Anderson, Lee and Biller . This is an
open-access article distributed under the terms of the Creative Commons Attribution
License (CC BY). The use, distribution or reproduction in other forums is permitted,
provided the original author(s) or licensor are credited and that the original publica-
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Tumors of the olfactory groove may cause unilateral optic atrophy with contralateral papilledema and anosmia (Foster Kennedy syndrome). We describe a case of a young pregnant woman with Foster Kennedy syndrome due to an olfactory groove meningiom

Jose eBiller

Ian eHughes

Douglas eAnderson

John eLee

Federico eRodriguez-Porcel

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Foster Kennedy Syndrome Due To Meningioma Growth During Pregnancy

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