Paracentral acute middle maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia.
OBJECTIVE
With the advent of more sophisticated imaging systems, such as spectral domain optical coherence tomography (SD-OCT), disruption of the inner segment/outer segment (IS/OS) band, and thinning of the outer nuclear layer (ONL) have been identified in association with acute macular neuroretinopathy (AMN).


OBJECTIVE
To characterize a new SD-OCT presentation of AMN as a paracentral acute middle maculopathy and to describe multimodal imaging findings that implicate an underlying pathogenesis related to retinal capillary ischemia.


METHODS
Retrospective observational case series (January 1, 2012, to January 1, 2013) reviewing clinical and imaging data from 9 patients (11 eyes) with AMN at 6 tertiary referral centers. Lesions were classified as type 1 or 2 in relation to the SD-OCT location of the lesion above (type 1) or below (type 2) the outer plexiform layer (OPL) at 6 tertiary referral centers.


RESULTS
Of the 9 patients, 5 were female and 4 were male (mean age, 47.6 years; range, 21-65 years). All patients presented with an acute paracentral scotoma and demonstrated a classic dark gray paracentral lesion with near-infrared imaging. Visual acuity ranged from 20/15 to 20/30. Six eyes (5 patients) had type 1 SD-OCT lesions, also referred to as paracentral acute middle maculopathy, and 5 eyes (4 patients) had type 2 SD-OCT lesions. Although type 1 lesions lead to inner nuclear layer (INL) thinning, type 2 lesions resulted in ONL thinning. Type 2 lesions were always associated with significant outer macular defects, including disruption of the inner segment/outer segment and outer segment/retinal pigment epithelium bands, whereas type 1 lesions spared the outer macula.


CONCLUSIONS
Paracentral acute middle maculopathy may represent a novel variant of AMN that affects the middle layers of the macula above the OPL as diagnosed with SD-OCT imaging. Two types of AMN lesions may be seen with SD-OCT occurring above and below the OPL. Type 1 refers to hyperreflective bands in the OPL/INL region with subsequent INL thinning. Type 2 is hyperreflective bands in the OPL/ONL region with subsequent ONL thinning. Type 2 lesions may be associated with concomitant defects of the inner segment/outer segment layer. We propose that each of these lesions may be explained by occlusion of either the superficial capillary plexus (type 1) or deep capillary plexus (type 2) located in the innermost and outermost portion of the INL, respectively, immediately adjacent to each corresponding lesion type.
David
D
Sarraf
Ehsan
E
Rahimy
Amani A
AA
Fawzi
Elliott
E
Sohn
Irene
I
Barbazetto
David N
DN
Zacks
Robert A
RA
Mittra
James M
JM
Klancnik
Sarah
S
Mrejen
Naomi R
NR
Goldberg
Robert
R
Beardsley
John A
JA
Sorenson
K Bailey
KB
Freund
D000208:Acute Disease; D000328:Adult; D000368:Aged; D002196:Capillaries; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D007511:Ischemia / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D010781:Photography; D012164:Retinal Diseases / Q000175:diagnosis*; D055351:Retinal Neurons / Q000473:pathology*; D055211:Retinal Photoreceptor Cell Inner Segment / Q000473:pathology; D055214:Retinal Photoreceptor Cell Outer Segment / Q000473:pathology; D055213:Retinal Pigment Epithelium / Q000473:pathology; D012171:Retinal Vessels / Q000473:pathology*; D012189:Retrospective Studies; D012607:Scotoma / Q000175:diagnosis*; D041623:Tomography, Optical Coherence; D014792:Visual Acuity; D055815:Young Adult