Grand Rounds Shivani V. Reddy, M.D. University of Louisville Department of Ophthalmology and Visual...
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Transcript of Grand Rounds Shivani V. Reddy, M.D. University of Louisville Department of Ophthalmology and Visual...
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
HISTORYPOHx: 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 TP P
20/20-2
20/20
19
18 no RAPD
EOM OD: -3 upgaze restriction, -2 leftgaze restriction OS: full
Hertel 15mm - (90mm)- 14mm
Marginal Reflex Distance 2mm OD, 3mm OS
4→3
4→3
Disc Photos OD OS
Pink disc with sharp margins, normal vasculature
2+ disc edema with blurred disc margins
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 metso 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/uo BCVA 20/30 , Red saturation 60% of
normal
HVF 24-2 1 year later OD: re-demonstration 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
o Observationo 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
Treatment
RADIATION TOXICITYEYELIDS 20- 26 GyCONJUNCTIVA
30 Gy (conjunctivitis)
LACRIMAL SYSTEM
30-45 Gy – toxicity within 5-10 years >65 Gy – toxicity within 9-10 months
CORNEA 30 – 50 Gy – punctate epithelial erosions40 – 50Gy – corneal edema >60 Gy - corneal perforation
IRIS >70 Gy – iritis, neovascularization
LENS 6.5 – 11.5 Gy – radiation to cataract time 4 years in 66%
RETINA 30 – 50 Gy – radiation retinopathy , 6 mo - 3 years after
OPTIC NERVE
>55 Gy , Fraction > 1.9 - radiation neuropathy
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
Clinical Neurology and Neurosurgery 115 (2013) 2426–243
o Results o Tumor resection achieved in 58.3% of patients o No patient developed any deterioration of contralateral
vision during follow-up periodo 5/5 patients with pre-op proptosis had significant
improvement post-opo 67% of patients had no tumor recurrence
o Of the 4 patients with recurrence, 2/4 had only undergone partial resection
o Conclusiono Pre-chiasmatic transection of the optic nerve can help
achieve better tumor control and preserve vision of the contralateral eye
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
References