Grand Rounds
Niloofar Piri, MD
Jan 17th 2014
 CC: Blind spots and blurry vision OU for more than
2 years (OS more severely affected)
 HPI: A 74-y Caucasian gentleman presented with
gradually increasing visual field defect and night
blindness OU for more than two years. He reports
episodes of exacerbation which never returned to
normal
 He claims a history of blunt trauma OS in 1960s
after which the vision OS has been low, he has
noticed deterioration of VA OS as well for the past
two years.
 PMH: CAD - Coronary angioplasty x2 (1999,
2006)
 DH: Verapamil, ASA 83 mg/d, Crestor ( Statin)
Ocular examination
 BCVA
OD: 20/40 (-0.50 +0.25x90)
OS: CF 1m
 Anterior segment
OD : NS 1+, CC1+
OS: pc-IOL
 IOP
14 mmHg
18 mmHg
No cells in the anterior chamber or anterior vitreous
Color Photo
Peripapillary atrophy OS>OD and pigment clumping OS>OD
June 2013
OCT OD
Epiretinal membrane, Loss of outer hyper-reflective
bands outside the fovea, central outer layers preserved
OS OCT
Loss of outer retinal layers reflected as loss of outer
hyper-reflective bands , central thinning
Autofluorescence: peripapillary hypo-autofluorescence OS>OD ,
multiple concentric rings of hypo and hyper-autofluorescence
extending from the center to the arcades
Multifocal ERG
First order response density demonstrates significantly
reduced cone functions OU (OS>OD)
Full field ERG demonstrates almost completely abolished
scotopic and photropic and flicker responses OS , as well as
diminished amplitudes OD
 Assessment: A 74-y old Caucasian gentleman
with a history of progressive visual field loss and
nyctalopia with some episodes of exacerbations for
the past 2 years OS>>OD
 Impression:
 AZOOR (Acute Zonal Occult Outer Retinopathy)
(Acute Annular Outer Retinopathy (AAOR)
variant)
 AIR (Autoimmune Retinopathy)(CAR, MAR,
npAIR)
Monson DM, Smith JR. Acute Zonal Occult Outer
Retinopathy (AZOOR) : Major review. Surv of
Ophthalmology 2011
 In 1992, Gass reported a ‘syndrome’ characterized by
the sudden onset of photopsia and acute scotomas
related to loss of sectors of outer retinal function
 Early in the course of the disease, funduscopic
appearance was often normal; however, most patients
developed zones of retinal pigment epithelial atrophy or
pigment clumping
 Changes in the electroretinogram (ERG) and persistent
visual field defects were usually observed. Although the
retinal dysfunction in AZOOR is focal clinically, the ERG
changes suggested a global deficit.
 Acute onset of decreased vision in a zone of the visual
field, usually accompanied by photopsia
 Visual field defect and the photopsia worsened in bright
light
 76% in women , mean age 37 (17-79)
 Starts unilaterally in 62% but progresses to bilateral
disease from several weeks to 228 months
 In the group of 51 patients followed by Gass et al,20
stabilization of visual field loss occurred within 6 months
for 77% of patients.
 In 1995 Gass and Stern described acute annular outer
retinopathy (AAOR) as a variant of AZOOR
 The clinical findings of AAOR were considered to be the
same as AZOOR, except for the absence of photopsia
and the development of an evanescent annular opacity
in the retina.
 No known treatment is proved to be effective.