Grand Rounds
Shivani V. Reddy, M.D.
University of Louisville
Department of Ophthalmology and Visual Sciences
HISTORY
o CC: “I see double”
o HPI: 38 y/o WF presents with around 2 years
of progressive vertical diplopia and right upper
lid drooping. She also c/o decreased right
peripheral vision.Denies visual acuity changes,
eye pain, flashes and scotomas
HISTORY
POHx: Refractive Error
PMHx: Mitral Valve Prolapse, hypothyroidism, irritable bowel
syndrome, meniere’s disease, migraine headaches
FAMHx: Father: glaucoma, strabismus
ROS: sinusitis
MEDS: synthroid, claritin, tylenol
ALLERGIES: PCN
Exam
VA
20/20-2
TP
20/20
19
18
P
4→3
no RAPD
4→3
EOM OD: -3 upgaze restriction, -2 leftgaze restriction
OS: full
Hertel 15mm - (90mm)- 14mm
Marginal Reflex Distance 2mm OD, 3mm OS
Exam
OD
OS
LIDS/LASHES
WNL
WNL
CONJ
WNL
WNL
CORNEA
WNL
WNL
IRIS
WNL
WNL
LENS
WNL
WNL
Disc Photos
OD
OS
2+ disc edema with blurred disc
margins
Pink disc with sharp margins, normal
vasculature
Visual Fields
HVF 30-2 OD displaying enlargement of blind spot. OS WNL
CT ORBITS
CT ORBITS W/O CONTRAST: Demonstrating right sided lesion surrounding the optic
nerve with preservation of nerve architecture
MRI ORBITS
TI – Weighted Image: Right sided homogenous
enhancement surrounding but not involving
lucent optic nerve
T2 – Weighted Image:concentric thickening of
optic nerve diameter with nonenhancing center
Summary
o 38 y/o WF presents with 2 years of progressive vertical diplopia
and subjectively decreased peripheral vision OD. Fundus exam with
disc edema OD. MRI demonstrates an intraconal homogenous
lesion surround the optic nerve extending to the orbital apex. VA
20/20 OU, HVF with enlargement of blind spot OD
o Differential
o Optic Nerve Sheath Meningioma
o Optic Nerve Glioma
o Orbital mets
o Orbital lymphoma
o Sarcoidosis
Treatment
o Plan: Close Observation
o 1 YEAR LATER
o BCVA 20/30-1
o + RAPD OD, OD red saturation 60% of
normal
o Plan: Radiation therapy
o Patient underwent intensely modulated radiation
therapy of 50.4 Gy divided into 1.8 Gy fractions
o Post Radiation f/u
o BCVA 20/30 , Red saturation 60% of normal
HVF 24-2 1 year later OD: redemonstration of enlarged blind
spot
MRI ORBITS RECENT
TI – Weighted Image with Fat suppression: Right
sided homogenous enhancement surrounding but
not involving non-enhancing optic nerve
T1– Weighted Image with Fat Suppression:
:concentric thickening of optic nerve diameter
with non-enhancing center
Treatment
o Recent exam: 2014
o BCVA 20/70
o Ishihara Plates 4/15
o + OD RAPD
o Radiation Oncology not
pursuing treatment at this time
due to concerns for toxicity
HVF 30-2 OD: stable enlarged blind
spot OD
Optic Nerve Sheath Meningioma
o Arises from meningoepithelial cells (arachnoid
cap cells) lining the sheath of the intraorbital
and intracanalicular portions of the optic nerve
Optic Nerve Sheath Meningioma
o 1% - 2% of all meningiomas but 1/3 of all optic nerve
tumors
o 2nd most common primary optic nerve tumor after
optic nerve glioma
o Epidemiology
o Adults, age 40-50
o 3 times more common in women
o 4-7% occur in children
Optic Nerve Sheath Meningioma
o Classic Diagnostic Triad
o Painless, slow, monocular vision loss , Optic atrophy and
Optociliary shunt vessels
o Disc edema seen with anterior extension of the tumor
o RAPD, VF defects (enlarged blind spot, constriction)
o Mild proptosis in 54% at presentation
o motility defects , especially upgaze restriction
Imaging
o MRI is gold standard.
o Intense homogeneous enhancement with gadolinium
o Best displayed on T1- weighted fat suppression
o Concentric thickening tram track sign on axial
sequences, doughnut sign on coronal
o CT especially useful for evaluating the sphenoid bone and
the optic nerve canal
o Differs from optic nerve glioma by usual lack of
calcification, and that enlargement occurs outside the nerve
Treatment
o Observation
o Patients with high functioning vision and
negligible decline in visual field
o When tumor is situated around the orbital apex
o Close f/u of 3-6 month intervals with repeat
MRI every 6-12 months suggested
o VA of <20/40 and constriction of fields
suggested as a marker for treatment initiation
Treatment
o Surgery
o Only indicated in patients with aggressive tumors
with intracranial extension to prevent spread
o Typically a blinding procedure due to shared
blood supply- pial vessels are usually
incorporated into the tumor
o Biopsy usually undertaken in atypical
presentations and when there is documented
intracranial progression to the chiasm
Treatment
o Radiation Therapy
o
First documented as an effective therapy in 1981
o
Well defined borders of the tumor make it
amenable to highly conformal therapy – delivers
high dose of radiation while avoiding adjacent
normal tissues
o
Stereotactic Fractionated Radiation Therapy
(SRFT) is the most widely reported technique ,
usually 50 – 54 Gy given in 1.8Gy fractions
Treatment
o Radiation Therapy
o
Stereotactic Radiation Surgery (SRS) with Gamma
Knife has more recently come into favor
o
SRFT, SRS and Proton beam therapy have all shown
comparable rates of tumor regression
o
Current literature shows that SRS has a higher risk of
visual loss but provides excellent control of long term
toxicity –related complications. Great treatment for
patients with poor visual recovery potential
RADIATION TOXICITY
EYELIDS
20- 26 Gy
CONJUNCTIVA
LACRIMAL
SYSTEM
CORNEA
30 Gy (conjunctivitis)
30-45 Gy – toxicity within 5-10 years
>65 Gy – toxicity within 9-10 months
30 – 50 Gy – punctate epithelial erosions
40 – 50Gy – corneal edema
>60 Gy - corneal perforation
>70 Gy – iritis, neovascularization
IRIS
LENS
RETINA
OPTIC NERVE
6.5 – 11.5 Gy – radiation to cataract time 4 years
in 66%
30 – 50 Gy – radiation retinopathy , 6 mo - 3
years after
>55 Gy , Fraction > 1.9 - radiation neuropathy
Clinical Neurology and Neurosurgery 115 (2013) 2426–243
o
Retrospective Study
o
12 patients with monocular blindness from ONSM with tumor growth from
the orbit to optic canal with extension in the intracranial space without chiasm
involvement
o
Mean age of 43+/- 17.5 years, 9 females, 3 males
o
Patients underwent tumor resection and pre-chiasmatic transection of the
optic nerve
o
Follow-up ranged over 50.6 +/- 25.7 months
o Results
o
Tumor resection achieved in 58.3% of patients
o
No patient developed any deterioration of contralateral vision during
follow-up period
o
5/5 patients with pre-op proptosis had significant improvement post-op
o
67% of patients had no tumor recurrence
o
Of the 4 patients with recurrence, 2/4 had only undergone partial resection
o Conclusion
o
Pre-chiasmatic transection of the optic nerve can help achieve better
tumor control and preserve vision of the contralateral eye
References
1.
BSCS Book 5, Neuro-ophthalmology
2.
U. Schick, U. Dott, W. Hassler. Surgical management of meningiomas involving the
optic nerve sheath. J Neurosurg, 101 (2004), pp. 951–959
3.
J. Shapey, H.V. Danesh-Meyer, A.H. Kaye. Diagnosis and management of optic nerve
glioma. J Clin Neurosci, 18 (2011), pp. 1585–1591
4.
J.Shapey, H.I. Sabin, H.V. Danesh-Meyer, A.H. Kaye. Diagnosis and management of
optic nerve sheath meningiomas. J Clin Neurosci, 20 (2013), pp. 1045 – 1056.
5.
B. Jeremic, S. Pitz. Primary optic nerve sheath meningioma: stereotactic fractionated
radiation therapy as an emerging treatment of choice. Cancer, 110 (2007), pp. 714–722
6.
T. Eng, N. Albright, G. Kuwahara, et al..Precision radiation therapy for optic nerve
sheath meningiomas. Int J Radiat Oncol Biol Phys, 22 (1992), pp. 1093–1098
7.
P.D. Moyer, K.C. Golnik, J. Breneman.Treatment of optic nerve sheath meningioma
with three-dimensional conformal radiation. Am J Ophthlmol, 129 (2000), pp. 694–696
8.
R.L. Lesser, J.P.S. Knisely, S.L. Wang, et al. Long-term response to fractionated
radiotherapy of presumed optic nerve sheath meningioma. Br J Opthalmol, 94 (2010),
pp. 559–563
9.
Edward C. Halperin, David E. Wazer, Carlos A. Perez, and Luther W. Brady. Perez and
Brady’s Principles and Practice of Radiation Oncology, 6th edition
Treatment
o Patient underwent right orbitotomy with bone flap to
biopsy lesion (fresh section)
WHO GRADE 1 OPTIC NERVE SHEATH MENINGIOMA
o PLAN: Close Observation