Spectrum of Retinal Vascular Diseases Associated With Paracentral Acute Middle Maculopathy


To evaluate the spectrum of retinal diseases that can demonstrate paracentral acute middle maculopathy and isolated ischemia of the intermediate and deep capillary plexus.


Retrospective, multicenter, observational case series.


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.


Five men and 4 women, aged 27–66 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, Purtscher’s retinopathy, inflammatory occlusive retinal vasculitis, post-H1N1 vaccine, hypertensive retinopathy, migraine disorder, and post–upper respiratory infection.


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.

The retinal capillary system in the central macula is a layered vascular structure composed of the superficial and deep capillary plexus. The latter consists of an intermediate and deep plexus along the inner and outer aspect of the inner nuclear layer, respectively. The superficial capillary plexus is visible with fluorescein angiography and ischemia of this structure may manifest as a cotton-wool spot. However, fluorescein angiography fails to adequately visualize the intermediate and deep plexuses. 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. 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. This series will present an additional 9 cases of paracentral acute middle maculopathy associated with various disorders not listed above, including ocular compression causing global ischemia, sickle cell retinopathy, retinal vasculitis, Purtscher’s retinopathy, and other etiologies.


Institutional review board approvals for retrospective 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), 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.


Ten eyes with paracentral acute middle maculopathy 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.


Cases 5–9 of This Case Series Describing Retinal Vascular Diseases Associated With Paracentral Acute Middle Maculopathy

Case No. Age/Sex Presentation Systemic Disease Eye Disease Eye Baseline
Baseline Fundus Follow-up BCVA
5 27/F 2–3 months of vision loss OS and “changing vision” OD. In-clinic BP was 180/80 Migraines, OCP, Adderall use, HTN Hypertensive retinopathy OD 20/20 Nasal parafoveal, wedge-shaped, yellowish white lesion 20/20
OS CF Superonasal, parafoveal, wedge-shaped, yellowish white lesion CF
6 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 retinopathy, pseudophakia OD 20/30 Nasal parafoveal wedge-shaped yellowish lesion (localizes to PAMM) with an adjacent, more superficial-appearing white lesion (localizes to a CWS) 20/40
7 28/M Acute-onset paracentral scotoma OD Migraine with visual aura _ OD 20/20 Normal dilated exam 20/20
8 53/F Acute onset of paracentral scotomas OD 2–3 days after H1N1 vaccine Hypothyroidism, HTN, anxiety Pseudophakia OD 20/50 Scattered parafoveal whitening (localizes with PAMM), peripapillary CWSs 20/50
9 37/M Acute onset of scotoma OD 2 weeks after a URI Recent URI _ OD 20/25 Temporal parafoveal wedge-shaped light-yellow lesion 20/20

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.

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 5–9. 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.

Case 1

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 ).

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.

Case 2

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 ).

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.

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 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 ).

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).

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Jan 7, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Spectrum of Retinal Vascular Diseases Associated With Paracentral Acute Middle Maculopathy

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