Spectrum of Retinal Vascular Diseases

Associated With Paracentral Acute Middle

Maculopathy
XUEJING CHEN, EHSAN RAHIMY, ROBERT C. SERGOTT, RENATA P. NUNES, EDUARDO C. SOUZA,
NETAN CHOUDHRY, NATHAN E. CUTLER, SAMUEL K.S. HOUSTON, MARION R. MUNK,
AMANI A. FAWZI, SONIA MEHTA, JEAN-PIERRE HUBSCHMAN, ALLEN C. HO, AND DAVID SARRAF
To evaluate the spectrum of retinal diseases
that can demonstrate paracentral acute middle maculopathy and isolated ischemia of the intermediate and deep
capillary plexus.
! DESIGN: Retrospective, multicenter, observational
case series.
! METHODS: This is a retrospective case series review of 9
patients (10 eyes) from 5 centers with paracentral acute
middle maculopathy lesions and previously unreported
retinal vascular etiologies. Case presentations and multimodal imaging, including color photographs, near-infrared
reflectance, fluorescein angiography, spectral-domain optical coherence tomography (SD OCT), and orbital color
Doppler imaging, are described. Baseline and follow-up
findings are correlated with clinical presentation, demographics, and systemic associations.
! RESULTS: Five men and 4 women, aged 2766 years,
were included. Isolated band-like hyperreflective lesions
in the middle retinal layers, otherwise known as paracentral
acute middle maculopathy, were observed in all patients at
baseline presentation. Follow-up SD OCT analysis of these
paracentral acute middle maculopathy lesions demonstrated subsequent thinning of the inner nuclear layer.
Novel retinal vascular associations leading to retinal
vasculopathy and paracentral acute middle maculopathy
include eye compression injury causing global ocular
ischemia, sickle cell crisis, Purtschers retinopathy, inflammatory occlusive retinal vasculitis, post-H1N1 vaccine,
hypertensive retinopathy, migraine disorder, and post
upper respiratory infection.
! PURPOSE:

See Accompanying Editorial on page 1.

Accepted for publication Apr 2, 2015.
From the Stein Eye Institute, Department of Ophthalmology,
University of California Los Angeles (X.C., J.-P.H., D.S.); and Greater
Los Angeles Veterans Affairs Healthcare Center (X.C., D.S.), Los
Angeles, California; Wills Eye Hospital (E.R., R.C.S., N.E.C., S.K.S.H.,
S.M., A.C.H.), Philadelphia, Pennsylvania; Department of
Ophthalmology, Universidade Federal of Sao Paulo (R.P.N., E.C.S.),
Sao Paulo, Brazil; Herzig Eye Institute (N.C.), Toronto, Canada; and
Department of Ophthalmology, Feinberg School of Medicine,
Northwestern University (M.R.M., A.A.F.), Evanston, Illinois.
Inquiries to David Sarraf, Retina Disorders and Ophthalmic Genetics
Division, Stein Eye Institute, 100 Stein Plaza, Los Angeles, CA 90095;
e-mail: dsarraf@ucla.edu

26

2015 BY

Paracentral acute middle maculopathy

lesions may develop in a wide spectrum of retinal vascular
diseases. They are best identified with SD OCT analysis
and may represent ischemia of the intermediate and
deep capillary plexus. These lesions typically result in
permanent thinning of the inner nuclear layer and are
critical to identify in order to determine the cause of
unexplained vision loss. (Am J Ophthalmol
2015;160(1):2634. ! 2015 by Elsevier Inc. All rights
reserved.)
! CONCLUSION:

HE RETINAL CAPILLARY SYSTEM IN THE CENTRAL

macula is a layered vascular structure composed

of the superficial and deep capillary plexus.1,2
The latter consists of an intermediate and deep
plexus along the inner and outer aspect of the inner
nuclear layer, respectively.1,2 The superficial capillary
plexus is visible with fluorescein angiography and
ischemia of this structure may manifest as a cottonwool spot. However, fluorescein angiography fails to
adequately visualize the intermediate and deep plexuses.3 Before the advent of spectral-domain optical
coherence tomography (SD OCT), abnormalities in
these deeper plexuses, located in the middle retina,
were not readily appreciated.
Recently the term paracentral acute middle maculopathy has been added to our vernacular and refers to the
presence of a hyperreflective parafoveal band at the level
of the inner nuclear layer on SD OCT that co-localizes precisely with the intermediate and deep capillary plexuses.4
The subsequent development of inner nuclear layer thinning corresponding to the original paracentral acute middle maculopathy lesion indicates that the primary
etiology may be ischemia of the intermediate and deep
capillary systems.
Paracentral acute middle maculopathy has recently
been associated with various retinal vascular diseases,
including nonproliferative diabetic retinopathy, central
retinal vein occlusion, and retinal artery occlusion,
further supporting the possible underlying ischemic nature of this SD OCT finding.57 This series will present
an additional 9 cases of paracentral acute middle
maculopathy associated with various disorders not listed

ELSEVIER INC. ALL

RIGHTS RESERVED.

0002-9394/$36.00
http://dx.doi.org/10.1016/j.ajo.2015.04.004

TABLE. Cases 59 of This Case Series Describing Retinal Vascular Diseases Associated With Paracentral Acute Middle Maculopathy

Case No.

Age/
Sex

27/F

Presentation

Systemic Disease

Eye Disease

23 months of vision loss OS Migraines, OCP, Hypertensive

and changing vision OD.
Adderall use,
retinopathy
In-clinic BP was 180/80
HTN

Eye

Baseline
BCVA

OD 20/20

OS CF

50/M

Acute-onset blurry vision

OD. Recent BP ranged
from 112/71 to 195/88
depending on medication
compliance

HTN, CHF,
pulmonary
hypertension,
alcohol abuse

Hypertensive
OD 20/30
retinopathy,
pseudophakia

28/M
53/F

Migraine with
_
visual aura
Hypothyroidism, Pseudophakia
HTN, anxiety

OD 20/20

Acute-onset paracentral
scotoma OD
Acute onset of paracentral
scotomas OD 23 days
after H1N1 vaccine

37/M

Acute onset of scotoma OD

2 weeks after a URI

Recent URI

OD 20/25

OD 20/50

Baseline Fundus

Nasal parafoveal, wedgeshaped, yellowish white

lesion
Superonasal, parafoveal,
wedge-shaped, yellowish
white lesion
Nasal parafoveal wedgeshaped yellowish lesion
(localizes to PAMM) with
an adjacent, more
superficial-appearing
white lesion (localizes
to a CWS)
Normal dilated exam

Follow-up
BCVA

20/20

CF

20/40

20/20

Scattered parafoveal
20/50
whitening (localizes with
PAMM), peripapillary
CWSs
Temporal parafoveal wedge- 20/20
shaped light-yellow lesion

BCVA best-corrected visual acuity; BP blood pressure; CF count fingers; CHF congestive heart failure; CWS cotton-wool spot;
HTN hypertension; OCP oral contraceptive pills; PAMM paracentral acute middle maculopathy; URI upper respiratory infection.

above, including ocular compression causing global

ischemia, sickle cell retinopathy, retinal vasculitis,
Purtschers retinopathy, and other etiologies.

METHODS
INSTITUTIONAL REVIEW BOARD APPROVALS FOR RETRO-

spective chart reviews were obtained commensurate with

the respective institutional requirements prior to the beginning of the study. The research adhered to the tenets of the
Declaration of Helsinki and was conducted in accord with
regulations set forth by the Health Insurance Portability
and Accountability Act. This was a multicenter, retrospective, observational case series review of the clinical and
multimodal imaging findings for 10 eyes (9 patients) with
paracentral acute middle maculopathy. The diagnostic
criteria for paracentral acute middle maculopathy included
band-like hyperreflectivity on SD OCT in the middle macula centered on the inner nuclear layer that evolved into
thinning of that retinal layer. Multimodal imaging included
color fundus photographs (Carl Zeiss Meditec, Dublin,
California, USA; Topcon Medical Systems, Oakland,
New Jersey, USA), near-infrared reflectance (SPECTRALIS; Heidelberg Engineering, Heidelberg, Germany),
VOL. 160, NO. 1

fluorescein angiography (Carl Zeiss Meditec; Topcon Medical Systems), SD OCT (SPECTRALIS; Heidelberg Engineering), and orbital color Doppler imaging (Logiq 700;
General Electric Medical Systems, Milwaukee, Wisconsin,
USA). Detailed clinical evaluation was performed and
associated systemic etiologies were identified. Baseline
and follow-up SD OCT findings were correlated with the
clinical presentation, demographics, systemic associations,
and baseline and final visual acuities, as well as adjunctive
imaging findings.

RESULTS
TEN EYES WITH PARACENTRAL ACUTE MIDDLE MACULOP-

athy in 9 patients (5 men and 4 women) were identified

and collected for this study. Patient ages ranged from 27
to 66 years (mean 39 years). Hyperreflectivity on SD
OCT in the middle retinal layers was observed for all patients in the acute presentation, followed by thinning of
the inner nuclear layer with follow-up SD OCT analysis.
All patients presented with a form of retinal vascular disease. Four cases are described in detail below, and 5 other
cases are summarized in the Table and Figure 1, with the
baseline and follow-up SD OCT images included.

RETINAL DISEASES WITH PARACENTRAL ACUTE MIDDLE MACULOPATHY

27

A 61-year-old African-American man with a

history of hypertension, hyperlipidemia, diabetes mellitus,
and obstructive sleep apnea presented with an acute onset
of multiple paracentral scotomas in the right eye after falling asleep overnight with his head on top of his hands. Review of systems was otherwise negative. Visual acuity was
20/25 in the right eye and 20/20 in the left. Anterior
segment and dilated examinations were unremarkable.
Orbital color Doppler imaging showed an absence of flow
in the right central retinal artery, right nasal and temporal
short posterior ciliary artery, and left nasal short posterior
ciliary artery. Flow in the right ophthalmic artery was
normal. Erythrocyte sedimentation rate, C-reactive protein, and platelets were all within normal limits. He was
started immediately on high-dose steroids because of the
concern for giant cell arteritis. A subsequent stroke evaluation showed <50% bilateral carotid stenosis and an incidental 2.2 mm right internal carotid artery aneurysm.
Subsequent bilateral temporal artery biopsies returned
negative for giant cell arteritis and steroid tapering was
initiated. Repeat orbital color Doppler imaging 2 days after
the initial presentation showed improved flow rates and velocities in the right central retinal artery and right temporal
short posterior ciliary artery. An absence of flow persisted
in the short nasal posterior ciliary artery bilaterally. At
the 2-week follow-up interval, the paracentral scotomas
persisted, with vision remaining 20/25 bilaterally. Fluorescein angiography showed bilateral global delayed perfusion. Bilateral near-infrared reflectance imaging showed
multiple parafoveal dark-gray lesions, and SD OCT
through these lesions revealed multiple paracentral acute
middle maculopathy lesions (Figure 2).
! CASE 1:

A 28-year-old African-American woman with

sickle cell disease presented with bilateral paracentral scotomas in the absence of other signs of occlusive crisis (eg,
bone pain, chest pain, abdominal pain). Visual acuity at presentation was 20/50 in her right eye and 20/200 in her left
eye. Macular examination showed a wedge of deep retinal
whitening nasal to each fovea without any other stigmata
of sickle cell retinopathy, such as retinal hemorrhages, iridescent spots, pigment clumps, or peripheral neovascularization. Fluorescein angiography showed bilateral capillary
pruning and temporal retinal vascular nonperfusion. SD
OCT showed hyperreflective bands at the level of the inner
nuclear layer corresponding to the white macular lesions,
consistent with paracentral acute middle maculopathy,
and inner retinal atrophy of the temporal macula typical
of old branch retinal artery occlusions (Figure 3).

! CASE 2:

! CASE 3: A 36-year-old Brazilian woman presented with a

central scotoma of her right eye and floaters in the left eye
for 1 week. She had a prior history of flu-like illness with
gastrointestinal symptoms and right-sided retro-orbital pain.
Medical history was remarkable for a pregnancy-induced
deep vein thrombosis. Visual acuity was 20/150 in the right

28

FIGURE 1. Spectral-domain optical coherence tomography (SD

OCT) images of paracentral acute middle maculopathy associated
with additional examples of retinal vascular disease in Cases 59.
Cases 7 and 9 show en face images of paracentral acute middle
maculopathy lesions with baseline SD OCT. CWS: cotton-wool
spot; PAMM: paracentral acute middle maculopathy.

eye and 20/25 in the left eye. Anterior examination was

normal. Dilated retinal examination showed papillitis, occlusive vasculitis, and multifocal retinal infiltrates in each eye
and inferotemporal retinal ischemia in the right eye, all of
which was confirmed with fluorescein angiography. SD
OCT through the zone of inferotemporal ischemia showed
paracentral acute middle maculopathy temporal to the fovea
and both superficial and deep capillary ischemia nasal to the
fovea. The patient was started on prednisone 60 mg daily.
Vision later improved to 20/25 in the right eye (Figure 4).
A 29-year-old Hispanic man sustained a motor
vehicle accident with loss of consciousness and presented
with vision loss of the left eye. Visual acuity was count fingers in the left eye with a relative afferent pupillary defect.
Anterior segment examination was notable for left-sided
periorbital ecchymosis and subconjunctival hemorrhage.
Fundus examination showed multiple superficial, polygonal, white lesions in the posterior pole radiating from
the optic nerve and parafoveal white-yellow lesions sparing
! CASE 4:

AMERICAN JOURNAL OF OPHTHALMOLOGY

JULY 2015

FIGURE 2. Case 1. Prolonged globe compression leading to severe compromise in the flow of the central retinal artery and short
posterior ciliary artery, as measured by orbital color Doppler imaging and leading to paracentral acute middle maculopathy seen on
spectral-domain optical coherence tomography (SD OCT) analysis. (Left, first and second from top) Normal-appearing fundus photographs 2 days after presentation. (Right, first and second from top) Near-infrared reflectance shows parafoveal light-gray lesions in
the right eye, and SD OCT shows that the light-gray lesions correspond with paracentral acute middle maculopathy (dotted arrows).
(Right, third from top) Near-infrared reflectance and SD OCT are unremarkable for the left eye. (Bottom) Table shows systolic blood
flow velocities as measured by Doppler for Case 1. At baseline presentation, the flow rates within the central retinal artery, nasal posterior ciliary artery, and temporal posterior ciliary artery are absent or severely reduced. On follow-up there is an increase in flow rate
for each (except for nasal posterior ciliary artery of the right eye) corresponding to slow reperfusion after relief of extended intraorbital pressure. OD: right eye; OS: left eye; CRA: central retinal artery; NPCA: nasal posterior ciliary artery; TPCA: temporal posterior ciliary artery; OA: ophthalmic artery. The top portion of the figure has 2 columns of sub-images (not 3) the left column has
square images, the right column has rectangular images.

the retinal vessels. Fluorescein angiography showed hypoperfusion corresponding to these lesions and vascular
leakage. The patient was diagnosed with Purtschers retinopathy. SD OCT through the white lesions showed
edema extending from the nerve fiber layer to the inner
plexiform layer, indicating ischemia of the superficial, intermediate, and deep capillary plexuses. SD OCT through
the parafoveal white-yellow lesions demonstrated paracentral acute middle maculopathy consistent with ischemia of
the intermediate and deep capillary plexuses and previously
referred to as Purtscher Flecken (Figure 5).
VOL. 160, NO. 1

The Table and Figure 1 provide a summary of

the clinical data for Cases 59.

! CASES 59:

DISCUSSION
IN 2013, SARRAF AND ASSOCIATES DESCRIBED PARACEN-

tral acute middle maculopathy lesions in 5 patients aged

5465 years who presented with paracentral scotomas.1,2,8
Two of these patients had no significant history, while the

RETINAL DISEASES WITH PARACENTRAL ACUTE MIDDLE MACULOPATHY

29

FIGURE 3. Case 2. A patient with sickle cell crisis presenting with vision loss and bilateral paracentral acute middle maculopathy
lesions. (Top, first and second left) Color fundus photography shows nasal parafoveal whitening. (Middle, first and second left) Fluorescein angiography shows old temporal branch retinal artery occlusions (solid arrows) but normal nasal parafoveal regions bilaterally.
(Bottom, first and second left) Near-infrared reflectance shows nasal parafoveal light-gray lesions in both eyes. (Right, top 4 images)
Spectral-domain optical coherence tomography (SD OCT) showing nasal paracentral acute middle maculopathy lesions (dotted arrows)
corresponding to the light-gray lesions in near-infrared reflectance and temporal inner and middle retinal atrophy corresponding to the
old branch retinal artery occlusions. (Right, bottom 2 images) Follow-up SD OCT shows stable temporal inner and middle retinal atrophy, as well as inner nuclear layer atrophy (dotted arrows) corresponding to prior paracentral acute middle maculopathy lesions.

remaining 3 presented after severe hypovolemia, flu-like

illness, and excessive coffee use suggestive of vasoconstriction. Since the original description, a number of other
retinal vascular disorders including diabetic retinopathy,
central retinal vein occlusion, and retinal artery occlusion
have been associated with paracentral acute middle maculopathy lesions. Based on the characteristic SD OCT
appearance and the patient demographics associated with
this clinical finding, paracentral acute middle maculopathy
is currently thought to be a result of ischemia of the intermediate and deep capillary plexuses,3,4,9 and may
theoretically be seen in any patient with retinal vascular
disease or systemic vasculopathic risk factors.47,9
30

Paracentral acute middle maculopathy lesions inevitably

transition from their characteristic band-like hyperreflectivity on SD OCT to isolated atrophy of the inner nuclear
layer on extended follow-up. This paper discusses several
novel entities associated with paracentral acute middle
maculopathy lesions, further supporting an underlying
ischemic pathogenesis.
Case 1 illustrates an at-risk vasculopathic patient who
placed prolonged pressure on his globe, increasing the intraorbital pressure above the intraluminal pressure in the retroorbital arteries, resulting in global ischemia of the central
retinal and ciliary vascular systems. This presumably led to
ischemia of the intermediate and deep capillary plexuses

AMERICAN JOURNAL OF OPHTHALMOLOGY

JULY 2015

FIGURE 4. Case 3. The case of occlusive retinitis with paracentral acute middle maculopathy lesions within a bed of inferotemporal
ischemia in the right eye. (Top row) Color fundus photograph and fluorescein angiography of the right eye showing papillitis, scattered
inflammatory infiltrates, and a large area of inferior retinal ischemia. The left eye shows papillitis and scattered inflammatory infiltrates. (Middle row and Bottom row) Spectral-domain optical coherence tomography through the area of inferotemporal ischemia
shows paracentral acute middle maculopathy (dotted arrow) with ischemia of the intermediate and deep capillary plexuses, and
ischemia of the superficial, intermediate, and deep capillary plexuses nasally (solid arrow).

and the development of paracentral acute middle maculopathy lesions with SD OCT analysis. Orbital color Doppler
imaging, an established means to measure retro-orbital
hemodynamics,57,10 demonstrated hypoperfusion, further
supporting the correlation between paracentral acute
middle maculopathy and global ocular ischemia. Reestablishment of perfusion was achieved with relief of
external compression.11
Sickle cell disease can cause occlusion of the retinal
vasculature owing to sickled erythrocytes that impede
intravascular transit, leading to ischemia and neovascularization. Retinal arterial occlusion often occurs along the
horizontal meridian temporal to the fovea involving terminal arteriolar branches.12,13 We identified paracentral
acute middle maculopathy lesions nasally, suggesting that
an additional level of ischemic insult can occur in these
patients. SD OCT imaging may be especially important
in sickle patients to identify these middle-level infarcts
and explain the development of paracentral scotomas
and/or vision loss not explained by clinical examination
or fluorescein angiography.
VOL. 160, NO. 1

Acute occlusive retinal vasculitis can be complicated by

macular edema, intraretinal hemorrhage, and infarction.14,15 Cotton-wool spots and capillary drop-out seen
on fluorescein angiography are frequently described findings associated with retinal vasculitis and related ischemia.
In addition, retinal vasculitis can cause ischemia at the
middle macular level involving the intermediate and
deep capillary plexuses, as suggested by paracentral acute
middle maculopathy with SD OCT analysis in Case 3.5
Purtschers retinopathy occurs from head trauma, and
Purtschers-like retinopathy has been described to occur
in acute pancreatitis, fat embolic syndrome and long
bone injury, chronic renal failure, childbirth, connective
tissue disorders, amniotic fluid embolization, prostate surgery, and pancreatic cancer.16,17 Multiple theories on the
pathogenesis of Purtschers have been proposed, but
occlusion of the precapillary arterioles with intermediatesized emboli is considered the most likely.16 Purtscher
Flecken refer to the typical vessel-sparing, polygonal, white
retinal lesions that develop parafoveally.18 SD OCT imaging in Case 4 demonstrates that Purtscher Flecken are, in

RETINAL DISEASES WITH PARACENTRAL ACUTE MIDDLE MACULOPATHY

31

FIGURE 5. Case 4. Post-traumatic Purtscher retinopathy in the left eye. (Top, first and second from left) Color fundus photography
showing superficial, white cotton-wool spots radiating from the optic nerve in the left eye. Vessel-sparing, wedge-shaped yellowish
white lesions are seen parafoveally. (Top, right) Red-free image of the left eye. (Second row) Spectral-domain optical coherence tomography (SD OCT) showing that cotton-wool spot (solid arrow) spans the full thickness of the inner retina, illustrating ischemia of
the superficial and intermediate capillary plexuses. (Third row and Fourth row) SD OCT through the parafoveal yellow-white lesions
showing paracentral acute middle maculopathy lesions (dotted arrows) correlating to ischemia of the intermediate and deep capillary
plexuses. The paracentral acute middle maculopathy lesions correspond precisely with previously described Purtscher Flecken.

fact, paracentral acute middle maculopathy lesions. Coady

and associates9 described a case of Purtschers-like retinopathy in a woman with metastatic pancreatic cancer also
with paracentral acute middle maculopathy lesions corresponding to Purtscher Flecken.
As is the case with a cotton-wool spot, our study indicates that the list of diseases associated with the SD
32

OCT clinical finding of paracentral acute middle maculopathy is long and diverse and includes common conditions
such as hypertension and migraine disorder, as well as
rare autoimmune etiologies (eg, post-H1N1 vaccination).
Any of these disorders, along with the more common
retinal vasculopathies such as diabetic retinopathy and
retinal vascular occlusion, can cause ischemia at either

AMERICAN JOURNAL OF OPHTHALMOLOGY

JULY 2015

the superficial or deep capillary level, particularly within

the posterior pole.
The oxygen demand of the macula, especially at the level
of the inner nuclear layer, outer plexiform layer, and photoreceptor inner segments, is higher than any other region in the
retina.19,20 While the choroidal vasculature is denser in the
macula to help meet this greater metabolic demand, oxygen
diffusion from the choroid to the retina is inherently
limited by retinal thickness,21 which is greatest parafoveally.
Furthermore, high visual resolution requires optimal optical
penetrance, thereby limiting the density of the parafoveal
capillary system.20 Together these limitations suggest that
the parafoveal macula, particularly the middle retinal layers,
where a watershed-like region exists (as previously discussed
by Yu and associates7), has a high perfusion demand whose
supply is limited by structure, placing it at greatest risk for
an ischemic insult. The fact that paracentral acute middle
maculopathy lesions are in the middle macula and predominantly parafoveal aligns with an ischemic etiology.
Studies in post mortem eyes show that the parafoveal
capillary plexuses are supplied and drained by a 1 arteriole1 venule unit.22 An average of 8.9 such wedgeshaped units surround the foveal avascular zone in a
radial formation.22 Additionally, each parafoveal arteriole is surrounded by a 50-mm capillary-free zone.16 Paracentral acute middle maculopathy lesions and Purtscher
Flecken are characteristically paracentral, wedgeshaped, with a clear region between the retinal whitening

and adjacent arteriole, corresponding precisely to

ischemia of 1 capillary unit from embolization of its
precapillary arteriole.
Fluorescein angiography is currently the gold standard
for imaging ischemia, but its resolution is insufficient to
identify isolated loss of the deep capillary plexus and is,
in fact, typically normal with paracentral acute middle
maculopathy lesions. Ex vivo human studies of paracentral
acute middle maculopathy are unlikely to be helpful as the
lesions are transient. In fact, the true pathogenesis of paracentral acute middle maculopathy lesions can only be
proven with in vivo imaging that has sufficient resolution
to study fine retinal blood flow or animal models of prearteriolar and capillary occlusion. OCT angiography or
adaptive-optics OCT may be the answer, and studies using
these modalities are already ongoing.23,24 The increasing
number of associated retinal vascular diseases, as
illustrated in this article, continues to support an
underlying ischemic origin of paracentral acute middle
maculopathy. Its affinity to localize to the parafoveal
macula, its typical wedge-shaped appearance, periarteriolar sparing in ischemia owing to pre-capillary arteriolar occlusion upstream, peri-venular pattern of ischemia
owing to increased venous pressure downstream in central
retinal vein occlusion, and its presence within the bed of
arterial occlusions all support the theory that paracentral
acute middle maculopathy lesions are attributable to intermediate and deep capillary plexus ischemia.

ALL AUTHORS HAVE COMPLETED AND SUBMITTED THE ICMJE FORM FOR DISCLOSURE OF POTENTIAL CONFLICTS OF INTEREST.
Financial Disclosures: R.C.S. is a consultant for Biogen Idec (Cambridge, MA), Merck (Kenilworth, NJ), Sanofi-Genzyme (Cambridge, MA), Teva
(Israel), Novartis (New York, NY), Heidelberg Engineering (Germany), Janssen (Horsham, PA), Lundbeck (Deerfield, IL), ThromboGenics (Belgium),
Merck Serono (Rockland, MA), Mallinckrodt (Ireland), United States Department of Defense (Washington, DC), and United States Food and Drug
Administration (Silver Spring, MD). N.C. is a consultant for Bayer (Pittsburgh, PA), Novartis (New York, NY), Alcon (Johns Creek, GA), Optos
(Scotland, United Kingdom), Bausch and Lomb (Bridgewater, NJ), and Allergan (Irvine, CA). A.A.F. is a consultant for Carl Zeiss Meditec (Dublin,
CA). A.C.H. is a consultant for Alcon (Johns Creek, GA), Genentech (San Francisco, CA), Janssen (Horsham, PA), Regeneron (Tarrytown, NY),
and ThromboGenics (Belgium). Funding/Support: D.S. has a research grant from Regeneron (Tarrytown, NY) and Genentech (San Francisco, CA),
as well as an optical coherence tomography machine for research use from Optovue (Fremont, CA). All authors attest that they meet the current ICMJE
requirements to qualify as authors.
The authors would like to acknowledge Wonchon Lin, MD and Mitra Nejad, MD (Stein Eye Institute, University of California Los Angeles, Los
Angeles, California) for their assistance in providing information for Cases 4 and 6.

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AMERICAN JOURNAL OF OPHTHALMOLOGY

JULY 2015

Biosketch
Xuejing Chen is a second year ophthalmology resident at the Stein Eye Institute, University of California Los Angeles. She
received her undergraduate degree in electrical and computer engineering from the California Institute of Technology and
her masters degree in computer science from Carnegie Mellon University. She worked as a program officer at ORBIS
International prior to completing medical school at Yale and internship at Memorial Sloan Kettering Cancer Center.

VOL. 160, NO. 1

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