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METHODS
INSTITUTIONAL REVIEW BOARD APPROVAL WAS OBTAINED
2015 BY
through the Wills Eye Hospital, Philadelphia, Pennsylvania, for a retrospective observational case series. Research
adhered to the tenets of the Declaration of Helsinki and
was conducted in accordance with regulations set forth
by the Health Insurance Portability and Accountability
Act (HIPAA).
RIGHTS RESERVED.
2
TABLE 1. Patient Demographics, Examination Findings, and Imaging in Paracentral Acute Middle Maculopathy
Age
(Years)
Sex
Eye
1
2
3
4
5
41
56
82
65
17
M
F
M
M
M
OS
OD
OD
OS
OD
Vision loss
Vision loss
Field cut
Vision loss
Vision loss
CRVO/CR
CRAO
BRAO
BRAO
BRAO
6
7
73
52
M
F
OS
OS
Field cut
Vision loss
BRAO
CRVO/CR
75
OD
Paracentral scotoma
BRAO
9
10
11
12
13
60
76
93
52
21
F
M
M
M
F
OS
OD
OD
OD
OU
Paracentral scotoma
Vision loss
Vision loss
Vision loss
Vision loss
BRAO
CRVO/CR
CRVO/CR
CRVO
BRAO OU
14
15
34
26
F
F
OS
OS
Vision loss
Paracentral scotoma
BRAO
BRAO
16
21
OU
Vision loss
BRAO
Patient
Symptom
Referring
Diagnosis
Associated Condition
VA Presentation
Beta thalassemia
HSV uveitis
HTN
HTN, chiropractic procedure
Prepapillary vascular loop,
trauma
HTN, carotid occlusion
Transcontinental flight/
dehydration
HTN, post-cardiac
catheterization
HTN, DM
HTN
HTN
Amyloidosis
Sickle cell disease,
dehydration on cruise ship
Cosmetic facial filler injection
Mechanical heart valve on
anticoagulation
Post-viral illness, Purtscherlike
20/20
CF
20/25
20/25
20/40
Normal flow
Capillary dropout
Normal flow
Normal flow
Capillary dropout
Arteriolar
Arteriolar
Arteriolar
Arteriolar
Arteriolar
20/40
20/60
Capillary dropout
Normal flow
Arteriolar
Arteriolar
20/25
Normal flow
Arteriolar
Capillary dropout
Normal flow
Normal flow
Normal flow
Capillary dropout OU
Arteriolar
Arteriolar
Arteriolar/fern-like
Fern-like
Multifocal globular
Paracentral scotoma
N/A
N/A
Paracentral scotoma
N/A
Capillary dropout
Normal flow
Focal globular
Focal globular
N/A
Paracentral scotoma
Normal flow OU
Multifocal globular
Paracentral scotoma
20/40
20/40
20/40
20/25
20/25 OD
20/200 OS
HMa
20/30
CF OU
Microperimetry Findings
N/A
N/A
N/A
N/A
N/A
Altitudinal field cut
Altitudinal field cut
N/A
BRAO branch retinal artery occlusion; CF count fingers; CR cilioretinal artery occlusion; CRAO central retinal artery occlusion; CRVO central retinal vein occlusion; DM diabetes
mellitus; HSV herpes simplex virus; HTN hypertension; N/A not available; OCT optical coherence tomography; OCT-A optical coherence tomography angiography; OD right eye; OS
left eye; OU both eyes; VA (Snellen) visual acuity.
a
Patient with concurrent ischemic optic neuropathy.
--- 2015
FIGURE 1. Optical coherence tomography angiography and en face optical coherence tomography features of paracentral acute middle maculopathy. Patient 10 was a 76-year-old man who presented with an inferior field cut in the setting of central retinal vein occlusion. Spectral-domain optical coherence tomography (OCT) demonstrated superior graying on near-infrared reflectance (Top left)
with middle retinal hyperreflectivity in the corresponding area (Top right). Fluorescein angiography (Bottom left) showed areas of
blockage reflecting preretinal and intraretinal heme and intact perfusion to superior macula. En face OCT (Bottom right, top row)
at the level of the superficial (left), middle (middle), and outer (right) retina demonstrated band-like hyperreflectivity at the level
of the middle retina. OCT angiography at the same levels (Bottom right, bottom row) is notable for absence of deep capillary plexus
dropout at the level of the middle retina.
Clinical records and imaging were reviewed to identify all cases with spectral-domain OCT imaging
showing paracentral acute middle maculopathy lesions
at a single academic private-practice office location
from January 1, 2015 to July 30, 2015. Available en
face OCT and OCT angiogaphy imaging cases were
identified. OCT angiography and en face OCT was
performed using the Avanti RTVue XR (Optovue,
Fremont, California, USA), which obtains volumetric
data from horizontal and vertical acquisitions that can
be automatically segmented to specific depths. Using
these data, side-by-side OCT angiography and en face
OCT images can be generated at the levels of the superficial capillary plexus, the deep capillary plexus, the
normally avascular outer retina, and the choriocapillaris.7,8 Baseline and follow-up demographic, clinical,
laboratory, and imaging data were extracted from
patient charts. High-resolution digital color photography, fluorescein angiography, and red-free photography were included in review, when available, for
each patient. Microperimetric examination using the
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RESULTS
THE PATIENT DEMOGRAPHIC, OCULAR EXAMINATION, IM-
FIGURE 2. Optical coherence tomography angiography and en face optical coherence tomography features of paracentral acute middle maculopathy, arteriolar pattern. Patient 9 was a 60-year-old woman who presented with a paracentral scotoma in the setting of a
branch retinal arterial occlusion proven on fluorescein angiography (Top row). Spectral-domain optical coherence tomography
(OCT; Second row, left) demonstrated nasal middle retinal hyperreflectivity approaching the fovea. Montage OCT angiography at
the level of the superficial plexus (Second row, right) disclosed multiple areas of capillary dropout. Review of en face OCT (Third
row) and OCT angiography (Bottom row) demonstrated a large area of middle retinal hyperreflectivity in an arteriolar distribution
with more extensive capillary dropout at the level of the deep plexus. Microperimetry (Bottom right) confirmed a superior altitudinal
defect in the same distribution.
finger count OU and 20/25 OD and 20/200 OS, respectively. The patient with hand motions visual acuity had
concurrent ischemic optic neuropathy.
All patients demonstrated hyperreflective plaque-like
lesions at the level of the inner nuclear layer on
spectral-domain OCT, consistent with paracentral acute
middle maculopathy (Figure 1 top). Fluorescein angiography did not consistently reveal any correlate to these lesions (Figure 1 bottom left). En face OCT highlighted
confluent areas of middle retina hyperreflectivity corresponding to the lesions (Figure 1 bottom right). OCT angiography demonstrated variable areas of capillary dropout
--- 2015
TABLE 2. Patterns of Middle Retina En Face Optical Coherence Tomography Hyperreflectivity in Paracentral Acute Middle
Maculopathy
Pattern
Appearance
Presumed Mechanism
Arteriolar
Fern-like
Globular
DISCUSSION
ONLY
RECENTLY
RECOGNIZED,
PARACENTRAL
ACUTE
FIGURE 3. Optical coherence tomography angiography and en face optical coherence tomography features of paracentral acute middle maculopathy, globular pattern. Patient 15 was a 26-year-old woman who presented with sudden-onset paracentral scotoma.
Spectral-domain optical coherence tomography (OCT; Top row) disclosed an inferotemporal area of middle retinal hyperreflectivity.
Fundus color and red-free photography (Second row) showed a small patch of retinal whitening in the same area. En face OCT at the
level of the middle retina (Third row, left) demonstrated a globular area of hyperreflectivity. Microperimetry (Third row, right)
showed a corresponding paracentral scotoma. Review of en face OCT (Fourth row) and OCT angiography (Bottom row) demonstrated sparing of other retinal levels with intact superficial and deep capillary flow.
--- 2015
FIGURE 4. Optical coherence tomography angiography and en face optical coherence tomography features of paracentral acute middle maculopathy, fern-like pattern. Patient 12 was a 52-year-old man who presented with a central retinal vein occlusion and foveal
blunting in the right eye (Top row, left). Microperimetry showed paracentral scotomas (Top row, middle). En face optical coherence
tomography (OCT) at the level of the middle retina (Top row, right) showed a fern-like pattern of perivenular hyperreflectivity. Review of en face OCT (Middle row) and OCT angiography (Bottom row) demonstrated sparing of other retinal levels with normal superficial and deep capillary plexus flow.
FIGURE 5. Optical coherence tomography angiography and en face optical coherence tomography features of paracentral acute middle maculopathy, evolution on follow-up. Serial en face optical coherence tomography (OCT; Top panels) and OCT angiography
(Middle panels) at the level of the middle retina (Bottom panels) show the evolution of paracentral middle maculopathy for Patient
10. Initial imaging (left column) demonstrated band-like superior hyperreflectivity on en face OCT with normal capillary flow. By
3 weeks (middle column) hyperreflectivity had disappeared on en face OCT with early patchy capillary dropout. By 8 weeks (right
column) there was hyporeflectivity at the level of the middle retina due to atrophy and there was more pronounced deep capillary
plexus pruning.
--- 2015
FUNDING/SUPPORT: THE AUTHORS HAVE NO FUNDING OR SUPPORT TO DISCLOSE FOR THIS PUBLICATION. A.C.H. HAS
received research grant funding in the past from Alcon, Allergan, Avalanche, Genentech, Iconic, Janssen/Johnson & Johnson, NEI/NIH, Ophthotech,
PanOptica, Regeneron, Second Sight, and Thrombogenics. Financial Disclosures: J.M. is a speaker for Regeneron and Genentech and on the advisory
board for Genentech. A.C.H. is a scientific advisor for Aerpio, Alcon, Allergan, DigiSight, Beaver EndoOptiks, Janssen, Genentech, ONL, Ophthotech,
Optovue, PanOptica, PRN, Regeneron, Second Sight, and Thrombogenics. The other authors have no disclosures, nor do they have any proprietary or
financial interest in any of the work discussed in this manuscript. All authors attest that they meet the current ICMJE requirements to qualify as authors.
REFERENCES
1. Sarraf D, Rahimy E, Fawzi AA, et al. Paracentral acute middle
maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia. JAMA
Ophthalmol 2013;131(10):12751287.
2. Rahimy E, Sarraf D. Paracentral acute middle maculopathy
spectral-domain optical coherence tomography feature of
deep capillary ischemia. Curr Opin Ophthalmol 2014;25(3):
207212.
3. Rahimy E, Sarraf D, Dollin ML, Pitcher JD, Ho AC. Paracentral middle maculopathy in nonischemic central retinal vein
occlusion. Am J Ophthalmol 2014;158(2):372380.
4. Yu S, Pang CE, Gong Y, et al. The spectrum of superficial and
deep capillary ischemia in retinal artery occlusion. Am J
Ophthalmol 2015;159(1):5363.
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Biosketch
Dr Jayanth Sridhar received his undergraduate and medical degrees from the University of Miami, FL. He completed his
residency training at the Bascom Palmer Eye Institute and is currently a senior vitreoretinal surgical fellow and clinical
instructor at Wills Eye Hospital and Thomas Jefferson University in Philadelphia, PA. His research interests include
applications of novel imaging techniques to retinal disease and utilizing new technology to improve the quality of
medical student and resident education.
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Biosketch
Abtin Shahlaee, MD, is a post-doctoral research fellow at Wills Eye Hospital. He received his medical degree from Tehran
University of Medical Sciences and was was a former research assistant at the Department of Ophthalmology at the Medical
University of Vienna. His current research projects focus on retinal imaging and diseases of the retina. His career goals
include training in ophthalmology in an academic setting with a long-term plan of becoming a successful clinician scientist.
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--- 2015