Download Introduction: Heather Moss, MD, PhD (Attending)

Illinois Eye and Ear Infirmary
UIC Department of Ophthalmology & Visual Sciences
Neuro-Ophthalmology
Introduction: Heather Moss, MD, PhD (Attending)
T
his Grand Rounds issue presents several interesting cases encountered by the
Neuro-Ophthalmology service.
Edited by Rohan Shah, MD and Elizabeth Grace, MD. Photos by Mark Janowicz (unless otherwise noted). All photos are property of UIC.
© Copyright 2012 by the Department of Ophthalmology, University of Illinois College of Medicine. All rights reserved. Cases presented in this issue are from
UI Ophthalmology Grand Rounds from February 1, 2012.
Compressive Optic Neuropathy: Kevin Patel, MD (Resident)
A 50 year-old Caucasian male referred from
the UIC oculoplastics clinic for further evaluation of diplopia. Symptoms began 1 year prior
to presentation when the patient started having
swelling of both eyes, OD worse than OS. His
diplopia is worst in up, down, and left gaze.
He also complains of blurred vision OD for 1
month which is most pronounced centrally.
The patient has a history of multiple sclerosis
and his ocular symptoms were first attributed
to this. However, an MRI was performed which
showed no active signs of MS. IV steroids were used to treat the swelling and diplopia, which caused some improvement in symptoms. Over
the past summer, the diplopia worsened and patient was started on
oral prednisone. There was no improvement in symptoms, however,
when he stopped the steroids, he had acute worsening of swelling
most prominent in the right infraorbital region. He restarted oral prednisone at a higher dose (50 mg) with some mild improvement. The week
prior in the oculoplastics clinic his visual acuity OD was 20/30 and the
patient had mild disc hyperemia with nasal disc edema OD.
Past medical history is significant for multiple sclerosis and hyperthyroidism. His medications include prednisone, Lipitor, methimazole,
citalopram, lansoprazole, ibuprofen, and betaseron. He uses artificial
tears as needed. Social history is significant for occasional alcohol
consumption and marijuana use.
FIGURE 1
CT scan orbits, axial – demonstrating EOM enlargement with sparing of the
tendons.
On exam, BCVA was 20/40 OD and 20/25 OS. The patient had no
rAPD. His extraocular movements were: -1 restriction in abduction
and adduction OD, -2 restriction in elevation OD; -1 restriction in abduction and elevation OS. Hertel measurements were 28 and 27 at a base
of 98. Anterior exam was significant for periorbital edema OU, but worse OD, with chemosis OU, and PEE OD. Dilated fundus exam showed
optic nerve elevation OU (OD>OS).
CT scan of the orbits showed enlargement of the inferior, medial, and superior recti muscles OD and superior rectus muscle OS with sparing
of the tendons (see Figures 1, 2, 3). HVF showed scattered defects superiorly and inferiorly OD (see Figure 4).
THE ILLINOIS EYE AND EAR INFIRMARY: Treating the most serious and complicated ophthalmology cases for over 150 years
Compressive Optic Neuropathy: (Continued)
The patient was diagnosed with thyroid eye disease with optic neuropathy OD. He was given IV solumedrol for 3 days, then transitioned to oral
prednisone 80 mg daily. On follow-up, patient had much less swelling. BCVA had improved to 20/25-3 OD and 20/25 OS. Motility remained
restricted. Dilated fundus exam showed minimal disc elevation OD and no disc elevation OS. A repeat HVF showed resolution of the scattered
defects OD (see Figure 5).
FIGURE 2
CT scan orbits, coronal – demonstrating prominent EOM enlargement
bilaterally.
FIGURE 3
CT scan orbits – EOM enlargement near apex, starting to encroach upon
the right optic nerve.
FIGURE 4 (left)
24-2 HVF OD – demonstrates
scattered visual field defects
superiorly and inferiorly.
FIGURE 5 (right)
24-2 HVF OD – 2 weeks after
IV steroids, shows resolution of
previous visual field defects.
UIC Department of Ophthalmology and Visual Sciences, 1855 West Taylor Street, MC 648, Chicago, IL 60612
Compressive Optic Neuropathy: (Continued)
DISCUSSION Thyroid eye disease (TED) is an autoimmune disease which is usually self-limited but can be progressive. It is most commonly associated with Graves’ disease and hyperthyroidism, but can occur in patients who are euthyroid or hypothyroid. TED incidence is
four times higher in women than men. Diagnosis is usually made clinically but imaging with CT or MRI can be done. Classically this shows
enlargement of the EOMs with sparing of the tendons. Common clinical characteristics consist of inflammation and congestion of EOMs,
proptosis, eyelid retraction, lid lag, diplopia, lid edema, chemosis, exposure keratopathy, and possible optic neuropathy. Treatment starts
with establishing a euthyroid state. The literature has shown that corticosteroids are the most effective initial treatment. There is no additional
benefit from early surgical decompression. Intraorbital steroid injections are another possible form of treatment which has shown to be effective. Radiation therapy is another effective treatment option, especially in patients who have failed treatment with steroids.
REFERENCES:
Phillips ME, Marzban MM, Kathuria SS. Treatment of thyroid eye disease. Current Treatment Options in Neurology. 2010 Jan;12(1):64-9.
Modjtahedi SP, Modjtahedi BS, Mansury AM, Selva D, Douglas RS, Goldberg RA, Leibovitch I. Pharmacological treatments for thyroid eye
disease. Drugs. 2006;66(13):1685-700.
Alkawas AA, Hussein AM, Shahien EA. Orbital steroid injection versus oral steroid therapy in management of thyroid-related ophthalmopathy. Clinical and Experimental Ophthalmology. 2010 Oct;38(7):692-7.
Abboud M, Arabi A, Salti I, Geara F. Outcome of thyroid associated ophthalmopathy treated by radiation therapy. Radiation Oncology. 2011
May;6:46.
Acute Altitudinal Visual Field Defect with Optic Disc Edema: Sara Huh, MD (Resident)
A 68 year old male presents with the chief complaint of left eye vision loss. His medical history includes shingles affecting V1, V2, and V3 branches of his left face 9 months prior to presentation at UIC. His shingles started with a rash on
his skin, foreign body sensation in the left eye with photophobia. He was treated with oral valacyclovir for 30 days and
Lotemax for the ocular involvement. With the resolution of the rash, he developed severe left sided facial pain and was
treated for post-herpetic neuralgia with improvement of his symptoms. CTA and MRI of the brain were performed at
the time showing non-specific periventricular T2 signaling, likely due to small vessel ischemic disease. 2 weeks prior to
presentation, he noted a grey shade being pulled down over the superior portion of his left eye visual field, without ocular
pain. He went to his local ophthalmologist where his visual acuity was 20/100 and examination showed a swollen left
optic nerve. Visual field testing showed a dense superior altitudinal loss in the left eye (see Figure 1). His ophthalmologist ordered a few laboratory tests, including ESR, CRP and platelets which were normal, presumed to rule out giant cell
arteritis. He was started on Valtrex 1 g daily. 1 week later his vision improved to 20/30 in the left eye.
He has no known drug allergies, is currently on valacyclovir, gabapentin, and Lotemax eye drops to his left eye. He has a 20 pack year smoking
history; however he quit 30 years ago and he consumes alcohol socially.
On presentation to the neuro-ophthalmology clinic, his left eye visual acuity was 20/30 with full color plates. He had a Relative Afferent Pupillary
Defect (rAPD) on the left and superior field defect on confrontational visual field testing. His intraocular pressures were within normal limits.
His anterior segment examination was significant for blepharitis bilaterally, some conjunctival injection in the left eye with dense inferior punctate
staining with fain subepithelial infiltrates. There was no evidence of corneal pseudodendrites.
On dilated fundus examination, the vitreous was clear with small cup to disc ratio bilaterally. The left optic nerve was diffusely hyperemic and
edematous. Otherwise his fundus examination was within normal limits.
The question at this point becomes – was this an idiopathic non-arteritic anterior ischemic optic neuropathy or a zoster related optic neuropathy?
The plan was to rule out potential vasculopathy due to Varicella zoster virus (VZV) as an etiology of ischemic optic nerve injury – MRI of the brain
and orbits were ordered and a lumbar puncture performed for IgG and IgM antibodies and VZV DNA by PCR. He was treated with IV acyclovir for
10 days followed by oral acyclovir and IV solumedrol.
THE ILLINOIS EYE AND EAR INFIRMARY: Treating the most serious and complicated ophthalmology cases for over 150 years
Acute Altitudinal Visual Field Defect with Optic Disc Edema: (Continued)
FIGURE 1
24-2 HVF done 2 weeks prior to presentation at UIC, demonstrates a dense superior altitudinal defect in the left eye.
DISCUSSION The precise mechanism of or location of injury is unknown in NAION, or non-arteritic anterior ischemic optic neuropathy.
Some have favored to calling the nonarteritic form of AION as idiopathic anterior ischemic optic neuropathy. It is presumed to be related to
compromise of optic disc microcirculation in the setting of structural crowding of the disc. Ischemia, edema, and compartment syndrome
due to optic disc structure are all thought to play a role. Signs and symptoms include sudden onset of painless vision loss and or associated visual field defect. A relative afferent pupillary defect is present in the affected eye and there is sectoral or generalized optic disc edema
on examination. If the edema persists for over 8 weeks, an alternative diagnosis should be found. Optic disc filling is usually delayed on
fluorescein angiography. Improvement in vision is reported in around 30% of patients after 2 years, according to the ischemic optic nerve
decompression trial. NAION usually affects Caucasians over the age of 50, with no gender predilection. There is a 15% risk of contralateral
involvement in 5 years. There is no proven treatment for NAION, however there are mixed reports about using aspirin for prophylaxis and
levodopa as treatment in the literature.
Primary VZV infection, which occurs in children, results in chicken pox, after which the virus becomes latent in ganglionic neurons. Years
later, as cell mediated immunity to VZV declines with age or from immunosuppression, VZV can reactivate to cause zoster. Zoster is often
followed by chronic pain as well as vasculopathy, myelopathy, retinal necrosis and cerebellitis. VZV reactivation can cause pain and all other
neurological complications of VZV reactivation without rash. Optic nerve involvement may occur simultaneously with the acute vesicular
rash or without a rash, or as a postherpetic complication.
The earliest description of VZV vasculopathy was described as a noninfectious granulomatous angiitis with a predilection for the nervous
system, characterized by thrombosis in cerebral arteries, consisting predominantly of histiocytes, mononuclear cells and multinucleated
giant cells. Patients can present with stroke like symptoms, headaches, changes in mental status, hemianopia, or monocular vision loss.
37% of cases of VZV vasculopathy develop without rash. Brain imaging reveals abnormalities – ischemic lesions usually at the grey-white
matter junctions much like metastatic or embolic disease. Angiographic changes include segmental constriction with poststenotic diltation.
Abnormalities in the CSF include modest pleocytosis with fewer than 100 cells, predominantely mononuclear. CSF protein is increased and
glucose is normal. Oligoclonal bands are present. Anti VZV IgG antibodies are more sensitive than VZV DNA in the CSF for VZV vasculopathy; however both studies are encouraged for patients suspected of VZV vasculopathy. Treatment recommendations include IV acyclovir for
10 to 14 days with or without concurrent corticosteroids. There is not enough evidence to prefer the use or the avoidance of corticosteroids
in VZV vasculopathy.
Back to our case, his MRI showed no additional pathology aside from small vessel ischemic changes seen previously and his lumbar
puncture was within normal limits without evidence of VZV antibodies or DNA. Thus the diagnosis still is debatable between an idiopathic
anterior ischemic optic neuropathy as opposed to ischemic optic neuropathy secondary to VZV vasculopathy.
UIC Department of Ophthalmology and Visual Sciences, 1855 West Taylor Street, MC 648, Chicago, IL 60612
Acute Altitudinal Visual Field Defect with Optic Disc Edema: (Continued)
REFERENCES:
Arnold AC. Pathogenesis of noarteritic anterior ischemic optic neuropathy. J Neuro-Ophthalmol 2003; 23:157-163.
Buono LM et al. Nonarteritic anterior ischemic optic neuropathy. Curr Opin Ophthalmol 2002, 13: 357-561.
Borruat FX and Herbort, CP. Herpes zoster ophthalmicus: anterior ischemic optic neuropathy and acyclovir. J Clin Neuro-ophthalmology,
1992; 12(1): 37-40.
Gilden DH et al. Two patients with unusual forms of varicella-zoster virus vasculopathy. N Engl J Med; 2002: 347 (19): 1500-3.
Gilden DH et al. Varicella zoster virus vasculopathies: diverse cliinical manifestations, laboratory features pathogenesis, and treatment. Lancet Neurol, 2009; 8:731-40.
Salazar R et al. Varicella zoster virus ischemic optic neuropathy and subclinical temporal artery involvement. Arch Neurol, 2011; 68(4): 517520.
Wenkel H et al. Detection of varicella zoster virus DNA and viral antigen in human eyes after herpes zoster ophthalmicus. Ophthalmology,
1998; 105: 1323-1330.
Complicated Orbital Floor Repair: Mark Krakauer, MD (Resident)
MF is a 41 year old man who was referred to the UIC ED by an outside plastic surgeon for acute vision loss. He had
a history of right orbital fracture without globe injury from blunt trauma from a bottle in 1991, and underwent fracture
repair in 1992. He had subsequent enophthalmos without diplopia and underwent repair of the fracture earlier that day
by the plastic surgeon. 3 titanium plates were placed to repair the floor and medial wall fracture. After awakening from
surgery, the patient reported decreased vision. Ophthalmology was consulted.
CT orbits without contrast showed one medial wall plate curved over the medial rectus and effacing the nerve. The
right optic nerve was smaller in diameter than the left. There was intraorbital edema and an irregular and distorted
medial rectus (see Figures 1 and 2). The patient was taken back to the operating room for emergent repositioning of
the plates. A repeat CT showed no impingement on the nerve although the right nerve still appeared smaller than the
left. The patient’s vision did not improve postoperatively. The patient was started on Solumedrol 250 mg IV every 6 hrs.
The patient was sent to the UIC ED the next day.
On exam in the UIC ED, the patient’s vision in the right eye was light perception with projection and 20/25 in the left eye. On Ishihara testing, he
was 0/11 OD and 11/11 OS. Motility exam showed -3 abduction and -4 in all other directions OD and full OS. Pupils were 5mm, fixed and middilated with a large rAPD OD and 3 to 1 mm OS. Confrontation visual fields were unable OD and full OS. Hertel was 24 mm OD and 22 mm OS
at base 124. He had normal facial sensation.
External exam showed nearly complete ptosis and 0.5 mm lagophthalmos, 2+ periorbital edema and a subciliary right lower lid incision w/sutures.
MRD1 was -4 OD and 5 OS. LF showed 0 OD and 18 OS. PF showed 0.5 OD and 9 OS. Applanation pressure was 20 OD and 18 OS. Anterior
segment exam showed subconjunctival heme inferiorly. Posterior segement exam was normal; the nerve showed no edema or pallor.
THE ILLINOIS EYE AND EAR INFIRMARY: Treating the most serious and complicated ophthalmology cases for over 150 years
Complicated Orbital Floor Repair: (Continued)
FIGURE 1: CT scan orbits, sequential coronal cuts
demonstrating impingement of orbital implants on
right optic nerve.
FIGURE 2: CT scan orbit, axial sequence showing
orbital implant pressing upon the optic nerve.
UIC Department of Ophthalmology and Visual Sciences, 1855 West Taylor Street, MC 648, Chicago, IL 60612
Complicated Orbital Floor Repair: (Continued)
In summary, the patient had right optic neuropathy from compression and/or ischemia, with ophthalmoplegia from postoperative edema vs.
CN3 palsy (lid and pupil involved). The patient was started on IV solumedrol 250 mg IV every 6 hrs for 3 days and cephalexin 500 mg PO QID
for 7 days. On postoperative day 11, the vision decreased to no light perception with the remainder of the exam unchanged. At postoperative
month three, the vision was still no light perception, although motility and lid function improved. There was temporal disc pallor. At postoperative month six, the exam was unchanged.
DISCUSSION Direct traumatic optic neuropathy occurs from an anatomical disruption of the optic nerve, typically resulting in severe and
immediate vision loss with a poor prognosis. Indirect traumatic optic neuropathy, as in this case, is due to transmission of forces to the
optic nerve from a distant site without disruption of normal tissue structures (blunt trauma). Primary injury occurs from mechanical shearing
of axons and vessels because the intracanalicular optic nerve dural sheath is adherent to periosteum. Secondary injury occurs from edema
causing compression and ischemia.
In a case series on intraorbital foreign body (Fulcher 2002), 4 patients had traumatic optic neuropathy; all had vision less than 6/60 at presentation and did not improve. One case report of a patient with thyroid eye disease who underwent bilateral endoscopic orbital decompression had vision loss to hand motion due to a fragment of lamina papyracea in contact with the optic nerve. The fragment was removed,
the orbit further decompressed, and vision improved to 20/20 at postoperative month four (Nazir 2009).
REFERENCES:
Nazir SA, Westfall CT, Chacko JG, Phillips PH, Stack BC Jr. Visual recovery after direct traumatic optic neuropathy. Am J Otolaryngol. 2010
May-Jun;31(3):193-4. Epub 2009 Mar 27.
Fulcher TP, McNab AA, Sullivan TJ. Clinical features and management of intraorbital foreign bodies. Ophthalmology. 2002 Mar;109(3):494500.
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THE ILLINOIS EYE AND EAR INFIRMARY: Treating the most serious and complicated ophthalmology cases for over 150 years