Optical Coherence Tomography Angiography and En Face Optical Coherence Tomography Features of Paracentral Acute Middle Maculopathy




Purpose


To characterize the optical coherence tomography (OCT) angiography, en face OCT, and microperimetry features of paracentral acute middle maculopathy in both the acute phase and after resolution, and to propose a classification of distinct subtypes of this entity.


Design


Retrospective observational case series.


Methods


Clinical histories, high-resolution digital color imaging, spectral-domain OCT images, fluorescein angiography, OCT angiography images, and en face OCT images of 16 patients with paracentral acute middle maculopathy were evaluated. Microperimetry was available in 6 patients.


Results


The most common referring diagnoses were isolated branch retinal arterial occlusion (5/16), combined central retinal vein and cilioretinal artery occlusion (4/16), and isolated central retinal vein occlusion (4/16). All patients demonstrated hyperreflective plaque-like lesions at the level of the inner nuclear layer on spectral-domain OCT, with no fluorescein angiographic correlate. OCT angiography demonstrated variable areas of capillary dropout within the superficial and deep retinal capillary plexi in these areas. En face OCT highlighted confluent areas of middle retina hyperreflectivity corresponding to these lesions. Three distinct en face OCT patterns were observed: arteriolar, fern-like, and globular. Microperimetry demonstrated relative scotomas mapping to the area of middle retinal hyperreflectivity seen on en face OCT.


Conclusions


Paracentral acute middle maculopathy may be best evaluated with the use of en face OCT imaging, which corresponds to subjective and objective visual field defects. En face OCT appearance may be used to classify paracentral acute maculopathy into distinct subtypes.


Paracentral acute middle maculopathy is a recently characterized presentation of deep retinal capillary ischemia, manifesting as hyperreflective bands within the middle retina on spectral-domain optical coherence tomography (OCT) imaging. While the initial description detailed lesions occurring in isolation, subsequent reports have observed this finding in conjunction with other retinovascular conditions, including branch (BRAO) and central (CRAO) retinal arterial occlusion, central retinal vein occlusion (CRVO), diabetic retinopathy, sickle cell retinopathy, and Purtscher retinopathy. Most recently, Christenbury and associates described the first case of paracentral acute middle maculopathy imaged with OCT angiography, demonstrating attenuation of the deep capillary plexus in the area of involvement. Taken together, these observations further support a vaso-occlusive origin for these lesions.


The purpose of the current study was to describe the natural evolution of paracentral acute middle maculopathy in 16 additional cases evaluated with OCT angiography, and to better elucidate the involvement of the adjacent retinal capillary plexuses, which to date have been unable to be adequately assessed with traditional fluorescein angiography. Furthermore, these findings were correlated to en face OCT imaging, which has not been previously reported for this condition, and microperimetry, when available. A new classification of distinct subtypes of paracentral acute middle maculopathy is then proposed based on en face OCT appearances.


Methods


Institutional review board approval was obtained 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).


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. 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 Macular Integrity Assessment (MAIA; CenterVue, Padova, Italy) was available and reviewed for 6 patients.




Results


The patient demographic, ocular examination, imaging, and microperimetry findings (when available) are summarized in Table 1 . The mean age at presentation was 53 years, and 9 of the 16 patients (56%) were male. Ten individuals (63%) presented with acute-onset nonspecific visual loss, 3 (19%) reported a paracentral scotoma, and 3 (19%) described an altitudinal field cut. Of the 10 patients with sudden vision loss, 2 (20%) experienced a partial resolution of symptoms within 30 minutes of onset.



Table 1

Patient Demographics, Examination Findings, and Imaging in Paracentral Acute Middle Maculopathy
















































































































































































































Patient Age (Years) Sex Eye Symptom Referring Diagnosis Associated Condition VA Presentation Deep Capillary Plexus OCT-A Findings Middle Retina En Face OCT Pattern Microperimetry Findings
1 41 M OS Vision loss CRVO/CR Beta thalassemia 20/20 Normal flow Arteriolar N/A
2 56 F OD Vision loss CRAO HSV uveitis CF Capillary dropout Arteriolar N/A
3 82 M OD Field cut BRAO HTN 20/25 Normal flow Arteriolar N/A
4 65 M OS Vision loss BRAO HTN, chiropractic procedure 20/25 Normal flow Arteriolar N/A
5 17 M OD Vision loss BRAO Prepapillary vascular loop, trauma 20/40 Capillary dropout Arteriolar N/A
6 73 M OS Field cut BRAO HTN, carotid occlusion 20/40 Capillary dropout Arteriolar Altitudinal field cut
7 52 F OS Vision loss CRVO/CR Transcontinental flight/dehydration 20/60 Normal flow Arteriolar Altitudinal field cut
8 75 M OD Paracentral scotoma BRAO HTN, post-cardiac catheterization 20/25 Normal flow Arteriolar N/A
9 60 F OS Paracentral scotoma BRAO HTN, DM 20/40 Capillary dropout Arteriolar Paracentral scotoma
10 76 M OD Vision loss CRVO/CR HTN 20/40 Normal flow Arteriolar N/A
11 93 M OD Vision loss CRVO/CR HTN 20/40 Normal flow Arteriolar/fern-like N/A
12 52 M OD Vision loss CRVO Amyloidosis 20/25 Normal flow Fern-like Paracentral scotoma
13 21 F OU Vision loss BRAO OU Sickle cell disease, dehydration on cruise ship 20/25 OD
20/200 OS 		Capillary dropout OU Multifocal globular N/A
14 34 F OS Vision loss BRAO Cosmetic facial filler injection HM a Capillary dropout Focal globular N/A
15 26 F OS Paracentral scotoma BRAO Mechanical heart valve on anticoagulation 20/30 Normal flow Focal globular Paracentral scotoma
16 21 F OU Vision loss BRAO Post-viral illness, Purtscher-like CF OU Normal flow OU Multifocal globular Paracentral scotoma

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.



The most common referring diagnoses were isolated BRAO (5/16, 31%), combined CRVO with cilioretinal artery occlusion (4/16, 25%), and isolated CRVO (4/16, 25%). Seven patients had known histories of hypertension; 1 of these patients also had diabetes mellitus, 1 patient presented after cardiac catheterization, and another lost vision 1 hour after a chiropractic procedure. Associated conditions in the remaining 9 patients included recent cosmetic facial filler injection, post-viral illness, amyloidosis, beta thalassemia, presumed herpes simplex uveitis, prepapillary vascular loop with trauma, sickle cell anemia with dehydration on a cruise ship, mechanical heart valve on anticoagulation, and transcontinental flight with dehydration.


Snellen best-corrected visual acuity (BCVA) on presentation ranged from 20/20 to hand motions. Two individuals (13%) had bilateral vision loss; vision in these cases was 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 within the superficial and deep retinal capillary plexi in the area corresponding to the paracentral acute middle maculopathy lesions ( Figure 1 , bottom right, bottom row). Microperimetry disclosed relative scotomas mapping to the area of middle retinal hyperreflectivity as seen on en face OCT ( Figure 2 ).




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.



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.


Three distinct patterns of these areas on en face OCT were observed: arteriolar, globular, and fern-like ( Table 2 ). The arteriolar pattern was seen in 11 patients (63%) and showed a characteristic band-like middle retina hyperreflectivity mirroring the distribution of a large retinal arteriole ( Figure 2 ). The globular pattern was noted in 4 patients (25%) with either a focal ovoid patch or multifocal ovoid patches of middle retina hyperreflectivity ( Figure 3 ). The fern-like pattern was observed in 2 patients (13%) in the setting of CRVO with multilobulated parafoveal middle retina hyperreflectivity ( Figure 4 ). Of note, 1 patient with a combined CRVO and cilioretinal artery occlusion had both the arteriolar and fern-like pattern.



Table 2

Patterns of Middle Retina En Face Optical Coherence Tomography Hyperreflectivity in Paracentral Acute Middle Maculopathy




















Pattern Appearance Presumed Mechanism
Arteriolar Band-like areas in distribution of major arteriole Transient or true arterial occlusion
Fern-like Multilobulated central area tracking along veins Perivenular capillary ischemia
Globular Ovoid focal or multifocal areas Distal precapillary or capillary ischemia



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.



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.


On follow-up en face OCT, areas of middle retina hyperreflectivity showed resolution, with variable areas of hyporeflectivity representing middle retinal atrophy ( Figure 5 ). OCT angiography of the deep capillary plexus showed capillary dropout in the same areas.




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.




Results


The patient demographic, ocular examination, imaging, and microperimetry findings (when available) are summarized in Table 1 . The mean age at presentation was 53 years, and 9 of the 16 patients (56%) were male. Ten individuals (63%) presented with acute-onset nonspecific visual loss, 3 (19%) reported a paracentral scotoma, and 3 (19%) described an altitudinal field cut. Of the 10 patients with sudden vision loss, 2 (20%) experienced a partial resolution of symptoms within 30 minutes of onset.



Table 1

Patient Demographics, Examination Findings, and Imaging in Paracentral Acute Middle Maculopathy
















































































































































































































Patient Age (Years) Sex Eye Symptom Referring Diagnosis Associated Condition VA Presentation Deep Capillary Plexus OCT-A Findings Middle Retina En Face OCT Pattern Microperimetry Findings
1 41 M OS Vision loss CRVO/CR Beta thalassemia 20/20 Normal flow Arteriolar N/A
2 56 F OD Vision loss CRAO HSV uveitis CF Capillary dropout Arteriolar N/A
3 82 M OD Field cut BRAO HTN 20/25 Normal flow Arteriolar N/A
4 65 M OS Vision loss BRAO HTN, chiropractic procedure 20/25 Normal flow Arteriolar N/A
5 17 M OD Vision loss BRAO Prepapillary vascular loop, trauma 20/40 Capillary dropout Arteriolar N/A
6 73 M OS Field cut BRAO HTN, carotid occlusion 20/40 Capillary dropout Arteriolar Altitudinal field cut
7 52 F OS Vision loss CRVO/CR Transcontinental flight/dehydration 20/60 Normal flow Arteriolar Altitudinal field cut
8 75 M OD Paracentral scotoma BRAO HTN, post-cardiac catheterization 20/25 Normal flow Arteriolar N/A
9 60 F OS Paracentral scotoma BRAO HTN, DM 20/40 Capillary dropout Arteriolar Paracentral scotoma
10 76 M OD Vision loss CRVO/CR HTN 20/40 Normal flow Arteriolar N/A
11 93 M OD Vision loss CRVO/CR HTN 20/40 Normal flow Arteriolar/fern-like N/A
12 52 M OD Vision loss CRVO Amyloidosis 20/25 Normal flow Fern-like Paracentral scotoma
13 21 F OU Vision loss BRAO OU Sickle cell disease, dehydration on cruise ship 20/25 OD
20/200 OS 		Capillary dropout OU Multifocal globular N/A
14 34 F OS Vision loss BRAO Cosmetic facial filler injection HM a Capillary dropout Focal globular N/A
15 26 F OS Paracentral scotoma BRAO Mechanical heart valve on anticoagulation 20/30 Normal flow Focal globular Paracentral scotoma
16 21 F OU Vision loss BRAO Post-viral illness, Purtscher-like CF OU Normal flow OU Multifocal globular Paracentral scotoma

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Jan 6, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Optical Coherence Tomography Angiography and En Face Optical Coherence Tomography Features of Paracentral Acute Middle Maculopathy

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