Abstract
Purpose :
To describe the clinical characteristics and clinical transitions between acute idiopathic blind spot enlargement (AIBSE), acute zonal occult outer retinopathy (AZOOR), and multiple evanescent white dot syndrome (MEWDS) with longitudinal follow up.
Methods :
Patients were identified by searching electronic medical records using diagnostic codes. Testing reviewed included visual fields, ophthalmic imaging and electroretinography. Demographic and clinical characteristics were compared. Charts were reviewed by both a neuro-ophthalmologist and a uveitis/retina specialist. Transition between diseases was defined by new appropriate funduscopic findings or testing that best fit with a different phenotype. Statistical analysis was performed using STATA 17 software.
Results :
A total of 43 patients were identified with a distribution of cases as follows: 14 AIBSE, 26 AZOOR, 1 AIBSE to AZOOR transition, 1 AZOOR to MEWDS transition, and 1 co-occurring AZOOR and MEWDS. AIBSE patients (mean age, 33 years) were younger than AZOOR patients (mean age, 41 years) at diagnosis. Women accounted for 93% of AIBSE patients and 77% of AZOOR patients. All transition patients were women. Time from initial diagnosis to clinical transition was 4 years in AIBSE to AZOOR and 0.75 years in AZOOR to MEWDS. AZOOR patients had longer mean follow up time (AIBSE 28 months, AZOOR 44 months) compared to AIBSE patients. Common misdiagnoses at the time of referral were migraines (42% of AIBSE patients, 32% of AZOOR patients) and optic neuritis (29% of AIBSE patients, 23% of AZOOR patients). AZOOR patients were more likely to present bilaterally at initial visit (Fisher’s exact test p < 0.05). Unilateral to bilateral occurrence (21% AIBSE eyes, 35% AZOOR eyes) and recurrences (14% AIBSE eyes, 38% AZOOR eyes) were more frequently observed in AZOOR patients. By the final visit, 30% of AZOOR eyes experienced worsening scotoma area by 20%, and 5% of AZOOR eyes experienced worsening visual acuity by 2 or more Snellen lines compared to no AIBSE eyes. Treatment was initiated in 8 AZOOR patients (31%), all transition patients and no AIBSE patients.
Conclusions :
A greater percentage of AZOOR patients exhibited worsening visual acuity and scotoma area and received immunosuppressive therapy when compared to AIBSE patients. Patients transitioning between these diseases are uncommon. Specifically, only a single case was observed for AIBSE converted to AZOOR.
This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.