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Clinical Ophthalmology 2013:7 1447–1450
Clinical Ophthalmology
A patient with acute macular neuroretinopathy
and central retinal vein occlusion
Kiriko Hirooka1
Wataru Saito1,2
Kousuke Noda1,2
Susumu Ishida1,2
1Department of Ophthalmology,
Hokkaido University Graduate
School of Medicine, Sapporo, Japan;
2Department of Ocular Circulation
and Metabolism, Hokkaido University
Graduate School of Medicine,
Sapporo, Japan
Correspondence: Wataru Saito
Department of Ocular Circulation
and Metabolism, Hokkaido University
Graduate School of Medicine, Nishi 7,
Kita 15, Kita-ku, Sapporo 060-8638, Japan
Tel +81 11 706 5944
Fax +81 11 706 5948
Email wsaito@med.hokudai.ac.jp
Purpose: The precise mechanism causing acute macular neuroretinopathy (AMN) is still
unknown. A recent report suggested that choroidal circulation impairment correlates with its
pathogenesis. We report a rare case with simultaneous onset of AMN and central retinal vein
occlusion (CRVO), which is a retinal circulation disorder.
Methods: Case report.
Results: A 44-year-old woman complained of central visual loss of the left eye for the previous 2
weeks. The patient’s visual acuity was 0.5 in the left eye (OS). Fundoscopic examination revealed
a wedge-shaped, dark reddish-brown lesion at the macula, and CRVO-like retinal hemorrhages
OS. Fluorescein angiography revealed retinal vasculitis and hypofluorescence corresponding to
the macular lesion. The patient’s scanning laser ophthalmoscopy infrared imaging result led to a
diagnosis of AMN. Two weeks after corticosteroid pulse therapy, her visual acuity improved to
1.2 OS, with improvement of macular findings and Humphrey perimetry. When the dose of oral
corticosteroid was decreased, the AMN lesion worsened, with recurrence of retinal hemorrhages.
Visual functions improved again after an increased dose of corticosteroid.
Conclusion: These results suggest that circulatory disorders almost simultaneously occurred
in choroidal and retinal vessels, resulting in the onset of both AMN and CRVO.
Keywords: choroidal circulation, optical coherence tomography, retinal circulation, systemic
corticosteroid therapy
Introduction
Acute macular neuroretinopathy (AMN) is a rare disease characterized by a wedge-
shaped, dark reddish-brown lesion at the macula.1 Scanning laser ophthalmoscopy (SLO)
infrared imaging clearly reveals these lesions as hyporeflectivity, which is a characteristic
finding in AMN, and this abnormal finding is present even though AMN lesions are not
biomicroscopically evident.2 The development of spectral-domain optical coherence
tomography (OCT) demonstrated that AMN lesions mainly affect the outer retina and not
the inner retina.2 Recently, it was reported that hyper-reflective lesions at the level of the
outer plexiform and outer nuclear layers on OCT temporally appeared despite an intact
photoreceptor inner/outer segment junction (IS/OS) in patients with early-stage AMN;
thereafter, the IS/OS was affected at the same sites.3 Ischemia in the deep inner retinal
capillary vessels was speculated as the cause of these newly discovered early OCT lesions,3
because retinal circulation provides photoreceptors with 10% of their oxygen supply.
These results suggest the onset of both inner and outer retinal disorders in AMN.
Approximately 10% of AMN patients appear to be complicated by intraretinal
hemorrhages, which are inner retinal disorders.4,5 However, the relationship between
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CASE REPORT
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Clinical Ophthalmology 2013:7
these conditions is unknown. Herein, we describe the case
of a patient who developed AMN and central retinal vein
occlusion (CRVO).
Case report
A 44-year-old woman noticed central visual loss with pho-
topsia in her left eye for the previous 2 weeks. The patient’s
medical and family histories were unremarkable; however,
she took oral contraceptives for menstrual irregularities.
The patient’s best-corrected visual acuity (BCVA) was
1.2 in the right eye (OD) and 0.5 in the left eye (OS). There
were no abnormal ocular findings OD. Slit-lamp examina-
tion revealed mild anterior vitreous cells OS. Fundoscopic
examination revealed a wedge-shaped, dark reddish-brown
lesion at the macula, brush- or blot-like intraretinal hemor-
rhages that spread in a pandirectional manner from the optic
disc, a mildly swollen optic disc, and dilatation and tortuos-
ity of the retinal veins (Figure 1A). SLO infrared imaging
revealed that the dark area corresponded to the dark reddish
lesion (Figure 1B). Fluorescein angiography (FA) revealed
hypofluorescence corresponding to the macular lesion and
retinal hemorrhages in the initial phase (Figure 1C), and reti-
nal phlebitis and the leakages from the optic disc in the late
phase (Figure 1D). Indocyanine green angiography (ICGA)
revealed a normal appearance during the initial phase, and
spotted hypofluorescence at the macular area during the late
phase (Figure 1E). Humphrey threshold 10–2 perimetry
showed a central scotoma (mean deviation [MD] value:
−9.40dB) corresponding to the lesion area (Figure 1F). Gold-
mann perimetry showed a central scotoma with enlargement
of the blind spot. Multifocal electroretinography revealed
decreased amplitude on the lesion area. OCT showed loss of
the photoreceptor IS/OS corresponding to the dark reddish
lesion (Figure 1G). No macular edema was evident, possibly
because the relatively mild occlusion of retinal veins appeared
to be restricted mainly to the superotemporal branch and two
nasal branches. Systemic screening comprising serological
analysis or antibody titres for infectious diseases, including
syphilis; QuantiFERON; serum autoantibodies, including
antiphospholipid antibody; full blood counts; erythrocyte
sedimentation rate; serum lipids; serum and urine glucose;
hemoglobin A1c; and endogenous coagulation function
revealed no abnormalities in the patient. The patient was
diagnosed with AMN and CRVO OS and was followed up
with withdrawal of oral contraceptives and no medication.
Two months after the first visit, the area of the macular
lesion and BCVA remained almost unchanged, although
most of the retinal hemorrhages spontaneously disappeared
Figure 1 Findings in the left eye at the rst visit: (A) the fundus photograph showed
a wedge-shaped, dark reddish lesion at the macula (arrowheads), brush- or blot-
like intraretinal hemorrhages that spread in a pandirectional manner from the optic
disc, a mildly swollen optic disc, and dilated and tortuous retinal veins; (B) scanning
laser ophthalmoscopy infrared imaging showed the dark area corresponding to
the dark reddish lesion (arrowheads); (C and D) uorescein angiography revealed
hypouorescence corresponding to the macular lesion (arrowheads) and retinal
hemorrhages at the initial phase (C) and retinal phlebitis (arrowheads) and leakages
from the optic disc in the late phase (D); (E) on indocyanine green angiography,
the dark reddish lesion showed spotted hypouorescence during the late phase
(arrowheads); (F) Humphrey threshold 10-2 perimetry revealed a central scotoma
(mean deviation value: −9.40dB); (G) optical coherence tomography showed the loss
of the photoreceptor inner/outer segment junction corresponding to the macular
lesion (arrows).
(Figure 2A), together with the resolution of retinal phlebi-
tis and the leakages from the optic disc on late-phase FA
(Figure 2B). After informed consent was obtained, corti-
costeroids were initially administered as per the following
schedule: intravenous methyl-prednisolone (mPSL) at
1,000 mg/day for 3 days, oral PSL at 30 mg/day for 7 days,
and intravenous mPSL at 1,000 mg/day for 3 days. Oral
PSL (30 mg/day) was then administrated and tapered by
10 mg/day every 1 month. The patient’s BCVA improved
to 1.2 OS 2 weeks after treatment. The dark reddish-brown
lesion had shrunk (Figure 2C), and the SLO dark area was
reduced (Figure 2D). Humphrey perimetry showed that the
central scotoma had shrunk, with marked improvement of
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Hirooka et al
Clinical Ophthalmology 2013:7
reddish lesion was faintly observed without a dark area on
SLO infrared imaging (Figure 3A and B). Late-phase FA
and ICGA showed resolution of the hypofluorescent lesions
at the macula (Figure 3C and D). OCT revealed complete
recovery of the IS/OS, with partial discontinuity of the cone
outer segment tip line (Figure 3E, arrows).
Discussion
In this study, we describe an AMN patient with nonischemic
CRVO. The lesions and visual functions improved at an early
stage after systemic corticosteroid therapy. To our knowl-
edge, this is the first reported AMN case with CRVO.
AMN with intraretinal hemorrhages was previously
reported in six patients;4,5 however, the extent of retinal hem-
orrhage was mild in those cases. Retinal capillary disorders
following the incidental complication of systemic hyperten-
sion was inferred as the cause of intraretinal hemorrhages
associated with AMN.4,5 However, our patient had no history
of arteriosclerotic diseases, including hypertension.
AMN lesions can show hypofluorescence on FA and
ICGA,6 as observed in the present case. We recently used
laser speckle flowgraphy to demonstrate reduced choroidal
blood flow velocity at the lesion site during the acute phase
Figure 2 Photographs of the left eye 2 months after the rst visit (A and B) and
2 weeks after corticosteroid pulse therapy (C–F): (A) fundus photograph showing the
spontaneous absorption of the retinal hemorrhages, although the area of the macular
lesion remained unchanged (arrowheads); (B) on late-phase uorescein angiography,
retinal phlebitis and the leakages from the optic disc resolved; (C) the dark reddish
lesion improved; (D) the dark area on scanning laser ophthalmoscopy at the lesion
site was reduced; (E) on perimetry, the central scotoma had shrunk (mean deviation
value: −3.58dB); (F) on optical coherence tomography, the discontinuity of the
photoreceptor inner/outer segment junction (arrows) had improved at the fovea.
MD value (−3.58dB) (Figure 2E), and that the discontinuity
of the IS/OS at the macula had improved (Figure 2F).
Three months after treatment (during oral PSL adminis-
tration at 15 mg/day), her BCVA was reduced to 0.6 OS, the
macular lesion and MD value (−7.42dB) worsened, and the
intraretinal hemorrhages recurred. The patient received an
increased oral PSL dose (30 mg/day) and a posterior sub-
Tenon injection of triamcinolone acetonide (40 mg) OS. The
AMN lesion again improved, with the disappearance of the
retinal hemorrhages, and BCVA and MD values increased
to 0.9 and −6.08dB, respectively, 6 months after the initial
treatment. Thereafter, PSL was tapered by 5 mg/day every
2–3 months and was continued for 18 months.
Sixty months after the first visit, the patient’s BCVA and
MD values were 0.8 and –3.88dB, respectively. The dark
Figure 3 Findings in the left eye 60 months after the rst visit: (A and B) the
dark reddish lesion (A) was faintly observed without a dark area on scanning laser
ophthalmoscopy infrared imaging (B); (C and D) on late-phase uorescein angiography
(C) and indocyanine green angiography (D), hypouorescent lesions corresponding
to the acute macular neuroretinopathy lesion almost resolved; (E) optical coherence
tomography revealed complete recovery of the inner/outer segment junction with
partial discontinuity of the cone outer segment tip line (arrows).
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A patient with AMN and CRVO
Clinical Ophthalmology
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Clinical Ophthalmology 2013:7
of AMN.6 In the present case, we could not evaluate choroidal
circulation impairment using this technique, because of the
patient’s poor fixation. Nevertheless, the hypofluorescent
areas observed on FA and ICGA at the lesion site suggest
choroidal hypoperfusion in this case, because there were no
obvious findings on OCT responsible for blocked fluores-
cence within the AMN lesion. These observations suggest
that choroidal circulation impairment is related to the patho-
genesis of AMN. On the other hand, CRVO is caused by cir-
culation disturbances in retinal veins following the stenosis or
occlusion of the central retinal vein near the lamina cribrosa
from unknown causes. CRVO is associated with diseases
causing arteriosclerosis in the elderly, whereas it is thought
to be associated with vasculitis in young adults.7,8 When an
AMN lesion recurred, retinal hemorrhages also appeared
again. Therefore, we infer that the onset of both AMN and
CRVO in the present case was due to circulatory disturbances
that occurred almost simultaneously in both choroidal and
retinal vessels. Inflammatory mechanisms are speculated
because the patient had CRVO with retinal vasculitis and
because the functional and morphological abnormalities
improved from the early stage after systemic corticosteroid
administration, similar to findings in our recently reported
case.6 However, thrombosis following oral contraceptive
use9,10 or other causes cannot be excluded, because in a large
case series of 588 patients with retinal vein occlusion, six of
nine patients aged younger than 35 years were taking oral
contraceptive pills.10 Further studies regarding retinal and
choroidal blood flow are needed in AMN patients.
In the literature review, AMN lesions showed no sponta-
neous improvement in 65% of patients.11 At present, thera-
peutic strategies for AMN remain to be established. In this
case, the IS/OS improved from the early stage after systemic
corticosteroid therapy, and finally completely recovered, as
in our recently reported case.6 Thus, these results suggest
the potential efficacy of systemic corticosteroid therapy for
some AMN patients, and warrant future prospective studies
to examine this intervention.
Conclusion
We reported a case of AMN with CRVO. The results suggest
that circulatory disorders in the retina and/or choroid relate
to the etiology of AMN.
Disclosure
The authors report no conflicts of interest in relation
to this work.
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