ACUTE MACULAR NEURORETINOPATHY

IRA. A. P R I L U C K , M.D.,

HELMUT BUETTNER,
AND
D E N N I S M. R O B E R T S O N , M.D.
Rochester,
1

In 1975, Bos and Deutman described a

newly recognized macular disorder,
called acute macular neuroretinopathy. It
is characterized by mild visual impairment, paracentral scotomata corresponding to wedge-shaped parafoveal retinal
lesions, and normal fluorescein angiography. A single case report of this disorder
occurring in the United States was recently published by Rush. 2 We describe herein another case of acute macular neuroretinopathy.
CASE REPORT

Minnesota
ment epithelium, and nerve fiber layer were normal
in the areas of these lesions.
Fluorescein angiography revealed subtle areas of
relative hypofluorescence corresponding to the lesions of the deep sensory retina (Fig. 4). Electrore-

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A 17-year-old boy came to the Ophthalmology

Department of the Mayo Clinic with a two-day
history of bilateral central "bluish-green shadows."
During the previous week, he complained of flu-like
symptoms, including a sore throat and fever. His
past medical history was otherwise noncontributory.
He had not taken any systemic medications.
Ophthalmic examination revealed visual acuity of
6/6 (20/20) in both eyes. The patient was able to
reproduce his bilateral and relative scotomata on the
Amsler grid (Fig. 1). External examination, intraocular pressures, and slit-lamp biomicroscopy of the
anterior segments and vitreous were normal. Kinetic
perimetry confirmed the paracentral defects and
showed intact peripheral visual fields (Fig. 2, top).
Static perimetry through two of the defects demonstrated dense scotomata (Fig. 2, bottom).
Ophthalmoscopic examination using contact lens
biomicroscopy of the right fundus showed an irregular dark appearing disturbance of the deep sensory
retina just nasal to the fovea. The left fundus
showed similar and more numerous lesions located
in the deep sensory retina. These disturbances were
best seen by using red-free light (Fig. 3). Except for
a small intraretinal hemorrhage located inferior to
the left macula, the retinal vasculature, retinal pigFrom the Department of Ophthalmology, Mayo
Clinic, and the Mayo Medical School, Rochester,
Minnesota. This study was supported in part by a
Fight for Sight Research Fellowship (Dr. Priluck),
Fight for Sight, Inc., New York, New York.
Reprint requests to Ira. A. Priluck, M.D.,
Creighton University School of Medicine, 601 N.
30th St., Omaha, NE 68131.

M.D.,

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Fig. 1 (Priluck, Buettner, and Robertson). Amsler

grid demonstrating parafixational scotomata of right
eye (top) and left eye (bottom).

AMERICAN JOURNAL O F OPHTHALMOLOGY 86:775-778, 1978

775

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AMERICAN JOURNAL OF OPHTHALMOLOGY

DECEMBER, 1978

tinography, eleetro-oculography, dark adaptometry,

and Farnsworth-Munsell color vision testing were
all normal.
A follow-up examination three months later revealed persistent paracentral scotomata, greater in
the left eye than the right eye. Visual acuity was
6/6 (20/20), both eyes, and defects on Amsler grid
testing remained essentially unchanged. Ophthalmoscopie examination revealed the same subtle
disturbances of the deep sensory retina, left eye
greater than right eye. The previously noted parafoveal intraretinal hemorrhage of the left macula
had resolved. Repeat fluorescein angiography again
demonstrated the minimal parafoveal areas of hypofluorescence.
DISCUSSION

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Fig. 2 (Friluck, Buettner, and Robertson) Tangent

screen visual field testing (top) and static Goldmann
perimetry (bottom) along the 105-degree meridian
(left eye) and the 75-degree meridian (right eye)
demonstrating dense paracentral scotomata. The
dotted line represents the normal static perimetry
through the respective meridians.

Our case exhibits all the characteristic

ocular findings of acute macular neuroretinopathy as previously described by Bos
and Deutman, 1 and Rush. 2 Our patient
had a recent history of an upper respiratory infection, presumably of viral origin,
and complained of minor visual impairment. Significant ocular findings were
limited to wedge-shaped parafoveal lesions radiating peripherally from the
macular regions, which corresponded to
reproducible paracentral scotomata on
the Amsler grid. Both static and kinetic

Fig. 3 (Priluck, Buettner, and Robertson). Left eye, Stereoscopic photographs taken with red-free light.
Posterior pole shows dark cuneate parafoveal lesions located in the deep sensory retina (viewed best with
+ 7.00 diopter lenses).

VOL. 86, NO. 6

ACUTE MACULAR NEURORETINOPATHY

777

nite but subtle findings of hypofluorescence probably represent a blockage of

the underlying choroidal fluorescence
by the visible lesions located at the level
of the deep sensory retina. We are not
certain whether the small intraretinal
hemorrhage observed in the left eye was a
coincidental finding or indicative of small
vessel disease. Bos and Deutman 1 reported somewhat dilated perimacular capillaries in two of their cases. It is not certain
that an abnormality of the retinal capillaries is of significance in this disorder.
The retinal lesions were best seen by
using red-free light and contact lens biomicroscopy. We believe the deep sensory
retina, and specifically the photoreceptor
cell layer, to be the principal site of
involvement in this disease. Functional
impairment as evidenced by dense paracentral scotomata is consistent with the
interpretation that the photoreceptor cell
layer is of pathophysiologic significance.
Contact lens biomicroscopy demonstrating the defects to be at the level of the
sensory retina also supports this supposition.

Fig. 4 (Priluck, Buettner, and Robertson). Top,

Arterial venous phase of fluorescein angiogram of
left eye demonstrates subtle areas of hypofluorescence, which correspond to the disturbance of the
deep sensory retina. Bottom, Late venous phase of
angiography shows persistence of these hypofluorescent areas (arrows).

perimetry confirmed the presence of these

scotomata. Retinal function studies were
otherwise normal.
The fluorescein angiographic findings
are unlike those seen with acute disorders
of the choriocapillaris or retinal pigment
epithelium, such as acute posterior multifocal placoid pigment epitheliopathy 3 or
acute retinal pigment epitheliitis. Defi-

All previously reported cases 1 ' 2 have

been associated with a recent infectious
process. Our case further supports this
observation. Bos and Deutman 1 reported
six cases, five of which were in females,
and of these, four were taking oral contraceptives. Additionally, the patient described by Rush 2 was also using oral
contraceptives. However, our patient and
the one male described by Bos and
Deutman 1 make it difficult to implicate
sex predilection or oral contraceptives
with acute macular neuroretinopathy.
SUMMARY

A 17-year-old boy had minor visual

impairment, paracentral scotomata, and
parafoveal retinal lesions following a presumed viral infection. Contact lens biomicroscopy demonstrated the retinal de-

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AMERICAN JOURNAL OF OPHTHALMOLOGY

fects to be located at the level of the deep

sensory retina. Fluorescein angiography
revealed subtle areas of hypofluorescence
which corresponded to the visible disturbances of the sensory retina. Follow-up
examination three months later revealed
persistent complaints of paracentral scotomata and persistent, but less recognizable retinal lesions. We believe the primary site of involvement to be the deep
sensory retina.

DECEMBER, 1978

REFERENCES
1. Bos, P. J. M. and Deutman, A. F.: Acute macular neuroretinopathy. Am. J. Ophthalmol. 80:573,
1975.
2. Rush, J. A.: Acute macular neuroretinopathy.
Am. J. Ophthalmol. 83:490, 1977.
3. Gass, J. D. M.: Acute posterior multifocal placoid pigment epitheliopathy. Arch. Ophthalmol. 80:
177, 1968.
4. Krill, A. E., and Deutman, A. F.: Acute retinal
pigment epitheliitis. Am. J. Ophthalmol. 74:193,
1972.

O P H T H A L M I C MINIATURE

The little children were in a pitiable conditionthey all had sore

eyes, and were otherwise afflicted in various ways. They say that
hardly a native child in all the East is free from sore eyes, and that
thousands of them go blind of one eye or both every year. I think
this must be so, for I see plenty of blind people every day, and I do
not remember seeing any children that hadn't sore eyes. And, would
you suppose that an American mother could sit for an hour, with her
child in her arms, and let a hundred flies roost upon its eyes all that
time undisturbed? I see that every day. It makes my flesh creep.
Yesterday we met a woman riding on a little jackass, and she had a
little child in her arms; honestly, I thought the child had goggles on
as we approached, and I wondered how its mother could afford so
much style. But when we drew near, we saw that the goggles were
nothing but a camp-meeting of flies assembled around each of the
child's eyes, and at the same time there was a detachment prospecting its nose. The flies were happy, the child was contented, and so
the mother did not interfere.
Mark Twain, Innocents Abroad
New York, Harper and Harper, 1911