Download Bitemporal Hemianopia Caused by Retinal Disease

Figure 2. Periorbital edema and severe restriction of extraocular movements in the 9 cardinal positions of gaze.
manifestations of blepharoconjunctivitis. Given that 5
cases of laboratory-acquired general vaccinia infection
were reported to the Centers for Disease Control and
Prevention between 2005 and 2007,4 proper personal
protection and immunization guidelines should also
be emphasized in laboratories using vaccinia.
Justin T. L. Baynham, BS
Steven A. Newman, MD
Correspondence: Mr Baynham, Department of Ophthalmology, University of Virginia Health System, PO Box
800715, Charlottesville, VA 22908 ([email protected]).
Financial Disclosure: None reported.
1. Pepose JS, Esposito JJ. Molluscum contagiosum, orf, and vaccinia. In: Pepose
JS, Holland GN, Wilhelmus KR, eds. Ocular Infection and Immunity. St Louis,
MO: Mosby; 1996:851-855.
2. Saloz C, Frommel E. Strabisme passager et réaction méningée consécutifs à
la vaccination jennérienne. Schweiz Med Wochenschr. 1927;57(20):471-472.
3. Hu G, Wang MJ, Miller MJ, et al. Ocular vaccinia following exposure to a smallpox vaccine. Am J Ophthalmol. 2004;137(3):554-556.
4. Centers for Disease Control and Prevention. Laboratory acquired vaccinia exposures and infections: United States, 2005-2007. MMWR Morb Mortal Wkly
Rep. 2008;57(15):401-404.
Bitemporal Hemianopia Caused
by Retinal Disease
A
bitemporal hemianopia is almost always
caused by damage to the optic chiasm and can
occur from the direct or indirect effects of a
variety of lesions, including tumors,1 aneurysms,2 and,
less frequently, inflammatory and ischemic diseases.
We describe a patient with a nonprogressive bitemporal hemianopia caused not by optic chiasmal dysfunction but by retinal disease that was diagnosed by multifocal electroretinography (mfERG) after results from
neuroimaging studies were repeatedly normal.
Report of a Case. A 67-year-old woman with a history
of previously treated tuberculosis, migraine headaches,
and osteopenia was found to have a bitemporal hemianopic defect during a routine visual field test as part of
an eye examination at an outside institution in 2003. Magnetic resonance imaging results were normal. The patient subsequently was seen by us for another opinion
in 2004. On examination, her visual acuity was 20/20 OU
with normal color vision and normal pupillary responses to light and near stimulation. The fundi appeared normal, including the optic discs. A bitemporal
hemianopic defect was confirmed by static perimetry
(Figure 1A); kinetic perimetry revealed that the defect
was scotomatous. Repeat magnetic resonance imaging results were normal. The results of mfERG were interpreted as normal, although there was a great deal of noise
in the tracings that made them difficult to interpret. Visual evoked potentials were slightly delayed bilaterally.
A multifocal visual evoked potential was attempted but
was unsuccessful because of excessive noise. We elected
to follow up with the patient.
The patient was evaluated at regular intervals during
the next 4 years. At each assessment, the visual field de-
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A
30
30
MD – 10.65 dB, P < .005
PSD 12.99 dB, P < .005
MD – 10.64 dB, P < .005
PSD 12.55 dB, P < .005
B
30
30
MD – 11.05 dB, P < .005
PSD 13.80 dB, P < .005
MD – 13.95 dB, P < .005
PSD 14.05 dB, P < .005
C
OS
OD
Figure 1. Static perimetry (Swedish Interactive Threshold Algorithm standard threshold, 24-2 strategy) showing a bitemporal hemianopia, with initial field defects
in January 2005 (A) and field defects in August 2008 (B). MD indicates mean deviation; PSD, pattern standard deviation. C, Fundus photographs of the left (OS)
and right (OD) eyes.
fects appeared stable (Figure 1B). Repeat magnetic resonance imaging and computed tomographic angiography gave normal results.
In 2008, full-field ERG gave normal responses;
however, repeat mfERG showed abnormal topography
bilaterally with severely reduced activity in the nasal
retinas of both eyes corresponding to the temporal
hemifields ( Figure 2 A). A retinal evaluation now
showed attenuation of the retinal arteries in both eyes
with atrophic peripapillary changes bilaterally, more
noticeable in the right eye, that extended some distance from the optic disc along the arcades
(Figure 1C). Fluorescein angiography showed that the
areas of choroidal and retinal atrophy were associated
with marked hyperfluorescence in the mid to late
stages of the angiogram in both eyes but without any
leakage from choroidal or retinal vessels. Optical
coherence tomography showed marked thinning of
the maculae (Figure 2B). The results of dark adapta-
tion testing were consistent with a deficiency in the
rate of both cone and rod responses. A diagnosis of
acute zonal occult outer retinopathy, a disorder
known to produce diffuse or focal field defects, was
made.3,4
Comment. To our knowledge, this is the first case
report of bilateral temporal hemianopic defects from a
retinal disorder. In this case, the funduscopic examination results were initially normal and the results of
mfERG were also thought to be normal, although in
retrospect the tracings were contaminated by excessive
noise. Subsequently, although repeat mfERG results
were abnormal, full-field ERG showed no abnormalities; this was probably because a sufficient proportion
of the retina in both eyes had not yet been affected.
Full-field ERG assesses overall retinal function. Thus,
the poor responses from the abnormal nasal retina that
was initially affected and therefore produced what
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OS
A
OD
0°
20
0
2
4
6
20
8
OS
10
12
14 mV/degrees2
OD
200 mV
0
60 ms
B
OS
OD
S
N
Signal strength (maximum 10)
7
I
Normal
distribution
percentile
155
207
168
217 191 236
❍ 3.45 mm
❍ 6 mm
90°
T
Signal strength (maximum 10)
7
100%
99%
95%
5%
1%
0%
Normal
distribution
percentile
204
175
Map
diameters
223 174 211
159
1.0 mm
3.00 mm
6.00 mm
6.00 mm
❍ 3.45 mm
❍ 6 mm
0 100 200 300 400 500 µm
100%
99%
95%
5%
1%
0%
Map
diameters
3.00 mm
Micrometers
90°
163
216
1.0 mm
N
I
155
178
216
168
S
T
Micrometers
0 100 200 300 400 500 µm
Figure 2. Results of multifocal electroretinography and optical coherence tomography. A, Topographical mapping of multifocal electroretinographic amplitude
responses in the left (OS) and right (OD) eyes (top) showing bilateral nasal abnormalities that correspond to bilaterally abnormal waveform traces (bottom).
B, Optical coherence tomography of the left (OS) and right (OD) maculae showing marked thinning. N indicates nasal; T, temporal; S, superior; and I, inferior.
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appeared to be bilateral temporal scotomatous field
defects were overshadowed by the normal responses
from the part of the nasal retina that was still normal
as well as the intact temporal retina. This case highlights the potential for retinal disorders to produce
visual field defects that mimic those produced by optic
nerve or chiasmal lesions, the importance of obtaining
appropriate and correctly interpreted electrophysiological tests to assess patients with unexplained
visual field defects, and the need to perform serial
examinations and repeat testing when the cause of
such abnormal visual fields is unclear.
Cristian M. Salgado, MD
Gislin Dagnelie, PhD
Neil R. Miller, MD
Correspondence: Dr Miller, Wilmer Eye Institute, Johns
Hopkins Hospital, Maumenee 127, 600 N Wolfe St, Baltimore, MD 21287 ([email protected]).
Author Contributions: Dr Miller had full access to all
of the data in the study and takes responsibility for the
integrity of the data and the accuracy of the data
analysis.
Financial Disclosure: None reported.
1. Quencer RM. Visual loss and bitemporal hemianopsia. J Clin Neuroophthalmol.
1981;1(3):231-235.
2. Krauss HR, Slamovits TL, Sibony PA, Verbalis JG, Nelson PB. Carotid artery
aneurysm simulating pituitary adenoma. J Clin Neuroophthalmol. 1982;2(3):
169-174.
3. Gass JD. Acute zonal occult outer retinopathy: Donders lecture: the Netherlands Ophthalmological Society, Maastricht, Holland, June 19, 1992. J Clin
Neuroophthalmol. 1993;13(2):79-97.
4. Gass JD, Agarwal A, Scott IU. Acute zonal occult outer retinopathy: a longterm follow-up study. Am J Ophthalmol. 2002;134(3):329-339.
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