Beruflich Dokumente
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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
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)
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
maculopathies.
days.
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
8/31/W/F
9/38/W/M
*CF
Yes
CF
20/200
Yes
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
a more
or
intense
cein
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.
hypopigmentation.
disease
(Fig 2,
no recurrences
(luring
patients.
A comparatively
COMMENT
only
(UAIM).
diagnosis includes
degenerative, infiltrative,
inflammatory, and infectious disor
ders, such as idiopathic choroidal neo
The differential
numerous
acute
ment
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.)
there
are
clinical and
angiographie fea
signs
of
vasculitis, and
provement of vision
a
are
gradual im
typical of
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
recognition.
asymptomatic.
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.