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Unilateral Acute

Idiopathic Maculopathy

Lawrence A. Yannuzzi, MD; Lee M. Jampol, MD; Maurice F. Rabb, MD; John A. Sorenson, MD; Charles Beyrer, MD;
\s=b\ This is a

report of nine patients who

experienced sudden,

severe, unilateral
central vision loss following a flulike
illness. Each patient had an exudative
detachment of the macula. All patients
experienced a spontaneous resolution of
the acute macular manifestations with
near-complete recovery of vision. A characteristic "bull's-eye" appearance in the
macula persisted. The acute manifestations of the disorder did not recur in any
of the patients during the period of follow-up. The constellation of findings was
suggestive of an inflammatory disease of
the retinal pigment epithelium, but a specific causative agent could not be identified. The acute clinical and angiographic
features, the natural course, and the residual pigment epithelial derangement
were not consistent with any previously
described disorder.
(Arch Ophthalmol. 1991 ;109:1411-

1416)

Accepted for publication May 13,

1991.
From The LuEsther T. Mertz Retinal Research
Laboratory, Manhattan Eye, Ear and Throat
Hospital (Drs Yannuzzi and Sorenson), The Macula Foundation, Inc (Drs Yannuzzi and Sorenson),
and Columbia University School of Medicine (Drs
Yannuzzi and Sorenson), New York, NY; Department of Ophthalmology, Northwestern University Medical School, Chicago, Ill (Dr Jampol); Department of Ophthalmology and Visual Sciences,
Eye and Ear Infirmary, UIC Eye Center, University of Illinois at Chicago (Dr Rabb); Stony
Brook (NY) University (Dr Beyrer); Southside
Hospital, Bayshore, NY (Dr Beyrer); Concord
(NH) Hospital (Dr Wilcox); Veterans Administration Hospital, Boston, Mass (Dr Wilcox); and
Cornell University Medical College, New York

"1 his is a report of a series of young


adults who experienced sudden, se
vere, unilateral loss of central vision
following a flulike illness. The loss of
vision was secondary to an exudative
maculopathy that was associated with
clinical and angiographie findings that
have not been described in any previ
ously reported disorder, to our knowl
edge. The natural course of this macu

lopathy was a fairly rapid spontaneous

resolution of the clinical manifestations


with improvement of vision. The resid
ual damage to the retinal pigment epi
thelium (RPE) was morphologically
characteristic in this series and also
not typical of any previously described

maculopathies.

PATIENTS AND METHODS


The demographic and clinical features of
the patients in this report are summarized
in the Table. We compiled nine patients:
five females and four males. Eight of the
patients were white, and one was black.
The age of the patients ranged from 15 to 45

years, with a mean age of 32 years. Seven


of the nine patients had a definite history of
a flulike prodromal illness that preceded
their visual symptoms by a period of 2 to 5

days.

Most patients (eight of nine) presented


with a visual acuity of 20/200 or less. In one
patient, the presenting visual acuity was
20/60 (patient 9). Each patient also de
scribed a central scotoma that was noted on
Amsler's grid testing. None of these pa
tients had a history of any other systemic
illness. All patients demonstrated a neuro
sensory detachment of the macula usually
with irregular margins, typically in a
wedged configuration. There was also an
area of white, gray, or yellow subretinal
thickening at the level of the RPE beneath
the neurosensory detachment. Three of the
nine patients were noted to have intrare
tinal hemorrhages in the detached neuro
sensory retina (Figs 1 and 2). Slit-lamp
biomicroscopic examination with a contact
lens revealed that four of the nine patients
also had cells in the posterior vitreous
humor.
The early fluorescein angiogram in these
patients varied, but it generally revealed a
minimal degree of subretinal hypofluores-

Clinical Characteristics
Patient

Age,

y Race Sex

1/15/W/F
2/38/W/M

Flulike
Prodrome

Initial
Visual Acuity*

Final

Vitreous

Visual Acuity

Yes

20/400

20/25

Cells
No

Yes

CF

3/38/W/F_No_CF_20/25_No

4/37/W/M

Yes

5/15/W/F
6/45/B/M

Yes

20/300

20/20

Yes

7/24/W/F_Yes_20/200_20/160_No

(Dr Wilcox).
Reprint requests

to Manhattan Eye, Ear and


Throat Hospital, 210 E 64th St, New York, NY
10021 (Dr Yannuzzi).

Lloyd M. Wilcox, Jr, MD

8/31/W/F

9/38/W/M

*CF

indicates counting fingers.

Yes

CF

20/200

Yes

and hyperfluorescence beneath the


neurosensory detachment (Figs 1, top
right, and 2, top right). The late-stage
angiogram was very consistent, demon
strating two levels of hyperfluorescence
from staining of the subretinal thickening at
the level of the RPE and pooling of the dye
within the subneurosensory retinal space
(Figs 1, center left, 2, bottom center, and
3). There was also a variation in the intensi
ty of the hyperfluorescence in the central
part of the macula. In the area of subretinal
thickening, the hyperfluorescence was
cence

more intense, except for blockage by xanthophyll pigment (Figs 1, center left and 2,
bottom left). The irregular early hyper
fluorescence, the late staining of the subre
tinal thickening, and the complete pooling
into the subneurosensory retinal space cre
ated a clinical-angiographic impression of an

wedge-shaped margins. There


hyperfluorescence in the
perifoveal region that corresponds to the
combined subretinal staining external to the
retinal pigment epithelium and pooling within
the subneurosensory retinal space. Center
right, Three weeks later, there is resolution of
the detachment, perifoveal pigment epithelial
hyperpigmentation, and a trace of subfoveal
fibrous metaplasia. The visual acuity has im
triangular
is

Fig 1.Patient 2. Top left, A 45-year-old man


with a 3-day history of severe loss of vision in
the left eye shows an irregular, exudative de
tachment of the neurosensory retina. Perifoveal thickening at the level of retinal pigment
epithelium is also present. The visual acuity is
20/200. Top right, Early-stage fluorescein an
giogram shows irregular hyperfluorescence
and hypofluorescence at the level of the reti
nal pigment epithelium. Center left, Latestage fluorescein angiogram reveals com
plete filling of the neurosensory retinal
detachment that has at least two irregular

a more

or

intense

proved to 20/30. Bottom left, A fluorescein


angiogram shows hypofluorescence from
blockage by the retinal pigment epithelial hy
perpigmentation, encircled by hyperfluores
cence or "window defect" from surrounding
hypopigmentation of the retinal pigment epi
thelium. Bottom right, Same patient, 1 year
later, shows stabilization of the macular

pathologic condition. There is irregular pig


ment epithelium hyperpigmentation that sur
rounds a central area of presumed subretinal
fibrous metaplasia in the foveal region and a
more broad area of pigment epithelial hypo
pigmentation that forms a "bull's-eye" ap
pearance. The visual acuity is 20/25. Bottom
bottom left, Same patient, 10 years later, had
no change in the macular manifestations or
visual function.

"occult" choroidal neovascularized mem


brane beneath the detachment in most pa
tients. In one case (patient 9), during the
acute phase of the disorder, the fluorescein
angiogram revealed principally hyper
fluorescence in the early stage and only a
trace of staining in the late stage of the
study. These findings were explained on the
basis of turbidity of the subretinal fluid.
The clinical findings and course in this
patient were otherwise identical to that in
the other patients. The clinical biomicrosco
pic examination and fluorescein angiograph
ie findings were completely normal in the
opposite eye of all patients in the series.
The natural course in all patients was
associated with a very rapid, spontaneous
improvement of vision in conjunction with
resolution of the neurosensory retinal de
tachment and development of retinal pig
ment epithelial atrophy and hypopigmenta
tion. In most patients, the improvement
occurred during 3 to 6 weeks (Figs 1
through 5). Gradual progressive improve
ment occurred during an additional 3 to 6
months in most patients. One patient was
treated with systemic corticosteroids (60 to
80 mg of oral prednisone). This patient
(patient 6) reported almost immediate im
provement in his vision coincidental with
this treatment. The final visual acuity im
proved to 20/25 or better in seven of the
nine patients. As the serous detachment of
the neurosensory retina was resolving, the
subretinal thickening became hyperpigmented (Figs 1, 3, and 4). This retinal

pigment epithelial hyperpigmentation oc


curred before the complete resolution of the

neurosensory detachment in the first 2


weeks after the onset of visual symptoms
(Figs 3 and 4). Following resolution of the

detachment, pigment epithelial hyperpig


mentation, with, in some instances, a trace
of probable subretinal fibrous metaplasia all
within a surrounding zone of retinal pig
ment epithelial hypopigmentation, could be
seen in the macula. This morphological ap
pearance in several patients resembled a
unilateral "bull's-eye" maculopathy (Fig 1,
bottom left, and Fig 4, right). The bull'spattern was accentuated on the fluores
angiogram (Figs 1, bottom left, 4,
center, and 5, right). The final pigmentary
eye

cein

disturbance was less prominent in the one


patient who had received systemic cortico
steroids during the acute stages of the

Fig 2.Patient 6. Top left, Stereophotographs of a patient with a 3-day history of acute, unilateral loss of
vision. Note the exudative detachment of the neurosensory retina, which is associated with a cluster of
intraretinal hemorrhages. The detachment is present, overlying an irregular yellowish discoloration and
slight thickening at the level of the retinal pigment epithelium. The visual acuity is 20/400. Top right, Early
venous phase fluorescein angiogram reveals a slight degree of hypofluorescence caused by intraretinal
blood. Bottom left, The late-stage angiogram reveals leakage at the site of pigment epithelial thickening
and in the subneurosensory retinal space. The retinal pigment epithelial staining is more intense than the
pooling beneath the neurosensory retinal detachment. There is blockage from the intraretinal hemor
rhages and a slight degree of hypofluorescence at the fovea from the presence of xanthophyll within the
retina. Bottom center, Same patient, 3 weeks later, reveals resolution of the macular lesion. There is mild
pigment epithelial stippling. Bottom right, The angiogram, corresponding to bottom center, is normal. The
visual acuity improved to 20/25.

Fig 3.Top left, A 37-year-old man with uni


lateral sudden loss of central vision. There is
a neurosensory retinal detachment, overlying
a pigmentary disturbance in the foveal region.
The visual acuity is 20/300. Top right, The
fluorescein angiogram reveals complete
staining of the subneurosensory space.
There is a slightly greater intensity of the late
hyperfluorescence at the level of the retinal
pigment epithelium surrounding the pigmen
tary disturbance that is comparatively hypofluorescent. Bottom left, Same patient, 4 days
later, reveals rapid, spontaneous resolution
of the neurosensory detachment and an in
crease in the subretinal pigment epithelial hy
perpigmentation. There was corresponding
improvement in the vision. Bottom right,
Same patient, 1 year later, reveals complete
resolution of the detachment and marked pig
mentary epithelial mottling surrounded by

hypopigmentation.

disease

(Fig 2,

bottom center). There were


of the acute manifestations
the interval of follow-up in these

no recurrences

(luring
patients.
A comparatively

poor visual outcome oc


curred in two of the nine cases (patients 7
and 8). Secondary choroidal neovasculariza
tion occurred in patient 7 after initial reso
lution of his exudative changes and visual
improvement. The neovascularization de
veloped 1 month after the resolution of the
acute manifestations. This led ultimately to
subfoveal disciform scarring with reduced
central acuity to the level of 20/160. In the
other case with a poor outcome (patient 8),
subfoveal pigment degeneration limited the
ultimate visual recovery potential to the
level of 20/200.
Very few laboratory studies were con
ducted in this series of patients because we
generally believed the case to be idiopathic
choroidal neovascularization and because
the natural course was associated with rap
id resolution of the presenting manifesta
tions. Some patients had tests that variably
included a complete blood cell count, a
sedimentation rate, thyroid function stud
ies, a biochemical profile, serology for influ
enza A (Taiwan) and A (Szechwan) and
(Victoria), herpes types 1 and 2, VDRL,
fluorescent treponema] antibody absorption
test, an angiotensin converting enzyme lev
el, skin testing for tuberculosis, and a chest
x-ray film for sarcoidosis. The results of all
of these tests were within normal limits. A
cytomegalovirus titer was positive in one
patient, but within normal limits for the age
grouping of the individual tested, and a
varicella antibody titer was in the midpositive range (0.47), indicating that the patient
had past exposure to varicella (patient 4).

COMMENT

This reported series represents a


group of healthy, young adults who
experienced sudden, severe, unilateral
loss of central vision following a flulike
upper respiratory tract prodromal ill
ness. There was spontaneous improve
ment of vision and no recurrence of the
acute manifestations. Seven of the nine
patients experienced a short viral or
flulike illness that preceded the sudden
loss of central vision. There was no
obvious sex or racial predilection. A
central scotoma was noted in all pa

tients. The significant findings on clini


cal examination were limited to the
posterior segment where an irregular
neurosensory retinal detachment of
the macula was noted. In some pa
tients, there were cells in the vitreous
humor, and in a few patients, there
were intraretinal hemorrhages in the
region of the neurosensory detach
ment. An irregular white, gray, or
yellow thickening to the RPE beneath
a portion of the neurosensory retinal
detachment was consistent with a sub
retinal infiltrative or neovascularized
process or an acute swelling of the
RPE cells. The discoloration was not

localized to the outer retina, as is seen


with layers of the outer retinitis or
outer retinal ischemie infarction (cho
roidal ischemia). The subretinal thick
ening stained irregularly on fluoresce
in angiography, giving it a vascular or
pseudovascular appearance. A peculiar
irregularity to the neurosensory reti
nal detachment, specifically, wedge or
pseudopodal extensions of the mar
gins, was noted in some of these pa
tients (Figs 1, center left, 2, top left,
and 3, top right). Another characteris
tic finding was the complete filling of
the fluorescein dye beneath the neuro
sensory retinal detachment in the late
stage of the angiogram (Figs 1, center
left, and 3, top right).
The natural course in these patients
was consistent in the series. Rapid
improvement of central visual acuity
from the level of 20/200 or less to 20/25
or better was the rule. Only two pa
tients had a significant decline in the
final visual acuity All patients were
followed up for at least 2V2 years, and
one patient (No. 2) was followed up for
13 years (Fig 1). The improvement of
the vision was associated with a corre
sponding resolution of the clinical find
ings. The vitreous cells, intraretinal
hemorrhages, acute thickening at the
level of the RPE, and the neurosenso
ry retinal detachment all disappeared
at approximately the same time. The
disappearance of these acute manifes
tations was usually preceded by a su
bretinal atrophie and pigmentary dis
turbance (Figs 3 and 4). The residual
manifestations in the fundus varied,
but some patients experienced a uni
lateral bull's-eye maculopathy with
central pigment epithelial hyperpig
mentation surrounded by an irregular
ring of RPE hypopigmentation (Figs 1
and 3 through 5). The transition from
an acute, unilateral exudative detach
ment of the macula to a bull's-eye
appearance is not typical of any known
maculopathy. An exception in the se
ries was patient 6, who was treated
with systemic corticosteroids; he had

only

barely perceptible pigmentary

disturbance after resolution of the


acute manifestations (Fig 2, bottom
center). Since the clinical manifesta
tions and courses in these patients do
not resemble any previously reported
disorder, we suggest the name unilat
eral acute idiopathic maculopathy

(UAIM).

diagnosis includes
degenerative, infiltrative,
inflammatory, and infectious disor
ders, such as idiopathic choroidal neo
The differential

numerous

vascularization, serous detachment of


RPE, central serous chorioretino
pathy, Harada's disease, serpiginous
the

choroidopathy, posterior scleritis,


posterior multifocal placoid pig
epitheliopathy (APMPPE), and
secondary placoid syphilitic retinitis.1
Most examiners initially believed

acute
ment

that the manifestations seen in pa


tients with UAIM were the result of
choroidal neovascularization with an
exudative detachment, followed by
spontaneous, autoinfarction with re
gression of the membrane and resolu
tion of the detachment.2 The clinical
and fluorescein angiographie findings
were consistent in some patients with
an "occult" choroidal neovascularized
membrane. Following resolution of the
exudative changes, there was no evi
dence of antecedent neovascularization
or disciform scarring. Except in the
patient who experienced a choroidal
neovascularization (patient 7), the dis
ciform scarring was thought to be sec
ondary to a disturbance of the RPE; in
short, this was a secondary manifesta
tion rather than a primary feature of
the disorder.
The fluorescein angiographie pattern
with UAIM in some aspects resembled
the late, complete, well-delineated
pooling that is seen beneath a serous
detachment of the RPE. Serous pig
ment

epithelial detachments, however,

also demonstrate early, complete hy


perfluorescence that intensifies in the
late stages of the angiogram. This an
giographie pattern was not seen in the
patients with UAIM.
The vitelliform-like neurosensory
retinal detachment that occurs in asso
ciation with cuticular drusen may also
be associated with complete fluoresce
in filling beneath the neurosensory de
tachment. Unilateral acute idiopathic
maculopathy is distinguished from this
disorder by the absence of cuticular
drusen and the characteristic yellowish
subretinal fluid.
A neurosensory retinal detachment
of the macula may be seen unilaterally
in central serous chorioretinopathy,
but this idiopathic disorder is associ
ated with focal or multifocal leaks at
the level of the RPE. No patient in the
UAIM series demonstrated such an
giographie features.
The presence of inflammatory signs
in the vitreous humor, the intraretinal
hemorrhages, the natural course, and
the final clinical appearance in UAIM
are more consistent with an acute in
flammatory or infectious disease pro
cess that involves the RPE and/or
superficial choroid, resulting in break
down of the posterior blood-retinal
barrier and an exudative neurosensory
detachment. Irregular detachments of
the neurosensory retina may be seen
with Harada's disease or other disor-

Fig 4. Patient 5. Left, A 15-year-old girl with a 2-week history of an acute exudative detachment of the macula with severe
vision loss. An area of retinal pigment epithelial hyperpigmentation has become evident beneath a resolving neurosensory
retinal detachment. The visual acuity is 20/300. Center, Complete resolution of the neurosensory detachment and
progression of the subretinal pigment epithelial hyperpigmentation within a zone of hypopigmentation. Right, Same patient,
1 month later, shows a "bull's-eye" pattern in the macula from pigment epithelial hyperpigmented and hypopigmented
changes. The visual acuity is 20/25.

Fig 5. Patient 6. Left, A 38-year-old man with an exudative detachment of the macula and sudden loss of vision. Center, There
is spontaneous reattachment less than 3 weeks later. Pigment epithelial hyperpigmentation and hypopigmentation are
evident. Right, The fluorescein angiogram reveals central hypofluorescence surrounded by patchy hyperfluorescence from the
retinal pigment epithelial changes that resemble a "bull's-eye" pattern. (Courtesy of Andrew J. Packer, MD.)

ders that may mimic it, such as poste


rior scleritis, sympathetic ophthalmia,
toxemia, malignant hypertension, dis
seminated intravascular coagulopathy,
leukemia, and certain tumefactions of
the choroid. These detachments may
be associated with complete filling of
fluorescein dye beneath the neurosen
sory retinal detachment. However,
none of these conditions has been de
scribed to be associated with the clini
cal and angiographie manifestations
and the natural course of the patients
who were seen in this series.1
It is possible that UAIM represents
a part of the spectrum of APMPPE.
Features that are consistent with
APMPPE include acute, severe visual
loss in young patients following a flu
like illness in association with a plac
oid, grayish discoloration at the level
of the RPE, late-homogeneous fluores
cein staining of the RPE in the acute
stages, and spontaneous recovery of
vision to near-normal levels. However,

there

are

clinical and

angiographie fea

tures of patients with UAIM that ap


pear to be exclusionary with respect to

the APMPPE syndrome. These in


clude a large, solitary, central lesion
that involves only one eye, the pres
ence of subretinal fluid or neurosenso
ry retinal detachment, one or more
intraretinal hemorrhages, the develop
ment of pigment epithelial hyperpig
mentation, and the fluorescein angio
graphie features. Acute posterior
multifocal placoid pigment epithelio
pathy has been reported to occur uni
laterally rarely, but it virtually never
presents with a neurosensory retinal
detachment. One case that was classi
fied as APMPPE was associated with a
neurosensory retinal detachment; how
ever, the fluorescein angiographie
study in that patient revealed multiple
areas of involvement elsewhere in the
fundus, inconsistent with the picture of
UAIM.1 Multiple, developing lesions at
various stages in the fundus, systemic

signs

of

vasculitis, and

provement of vision

APMPPE, but these

a
are

gradual im
typical of

are not seen in


UAIM. A bull's-eye appearance in the
macula following resolution of the
acute manifestations may be seen in
patients with UAIM, but this appear
ance has never been described in
APMPPE, to our knowledge. Above
all, the early-phase fluorescein angio
grams in APMPPE in the acute stages
reveals an intense, homogeneous hypo
fluorescence at the site of the placoid
lesions. None of the patients with
UAIM revealed such a pattern in the
acute stages of the disorder (Fig 2,
left). Serpiginous choroidopathy can be
ruled out in the differential diagnosis
on the same basis.
An infectious disease that may re
semble UAIM is the secondary stage
of syphilis which may present as a
large, solitary, placoid lesion in the
macula of one or both eyes. These
patients generally have other signs of

intraocular inflammation, a character


istic course of gradual resolution, and
eventual pigment epithelial mottling.
The visual presentation and course, as
well as the exudative detachment and
residual morphological findings, differ
entiate patients with UAIM from this
form of secondary ocular syphilis. In
addition, the patients in this series
were all healthy, and in three patients
who were tested for syphilis were

negative.
The prodromal, flulike illness in
UAIM is suggestive of a viral causal
agent, but no serological evidence was
obtained in any patient to support this
presumption. A unilateral inflamma
tory disease in the fundus is known to
occur in disorders, such as multiple
evanescent white-spot syndrome and
retinal pigment epitheliitis. If the
pathogenesis of UAIM is caused by an
infectious agent, it is likely, however,
that bilateral, multifocal, and recur
cases may be identified in the
future. It is also possible that extrafo-

rent

veal lesions may occur without patient


The peculiar UAIM predi
lection for the central part of the macu
la could be explained in part on this
basis, since such lesions could be

recognition.

asymptomatic.

In essence, this report has described


group of patients whose conditions
represented a newly diagnosed disor
der of the macula. It occurs in young
patients who experience severe, uni
lateral visual loss and a solitary, macu
lar lesion that consists of characteristic
features, usually following a flulike ill
ness. A rapid, spontaneous recovery of
central vision occurs, leaving charac
teristic findings in the macula. The
acute process appears to be nonrecur
rent. Because of the disturbance in the
RPE, these patients are at risk of
choroidal neovascularization in the fu
ture. The recognition of this disorder is
important to eliminate the possibility
of unnecessary medical or surgical in
tervention, such as steroids or antimi
crobial drugs and even laser photocoa

agulation treatment of subretinal


tissue that may mimic or masquerade
as choroidal neovascularization. It is
also important for the ophthalmic com
munity to recognize that patients may
present with a solitary lesion in the
macula and with severe visual loss that
may resolve spontaneously with a very
good prognosis. While the findings are
consistent with an inflammatory pro
cess in the vicinity of the RPE, addi
tional case recognition and specific
analysis are needed to identify the
precise pathogenesis.

Dennis Orlock, COP, provided photographic


support; Jay Prensky, MD, Andrew Henrich,
MD, and Andrew Packer, MD, referred patients
5, 3, and 9, respectively; and Barry Lipson, MD,
assisted in

preparing the manuscript.


References

1. Gass JD. Stereoscopic Atlas of Macular DisSt Louis, Mo: Mosby-Year Book; 1987:43\x=req-\
219.
2. Campochiaro PA, Morgan KM, Conway BP,
Stathos J. Spontaneous involution of subfoveal neovascularization. Am J Ophthalmol. 1990;1:668-675.
eases.

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