Overview
- Definition:
Rare, idiopathic, self-limited inflammatory condition of the outer retina and retinal pigment epithelium of unknown etiology
Also known as Krill’s disease
- Symptoms:
- Painless loss of vision
Acuity 20/20 to 20/100
Central scotoma, seen on Amsler grid testing
Metamorphopsia
- Laterality
Unilateral or bilateral
- Course
Acute onset of symptoms (days) with resolution over the next few months
- Age of onset
20s to 50s
- Gender/race
No gender or racial predilection.
2/3rds of case reports described in male patients.
- Systemic association:
Flu-like prodrome 1–2 weeks prior
No other systemic association
Exam: Ocular
Anterior Segment
No signs of inflammation
Posterior Segment
Fine hyperpigmented stippling of the RPE within the macula surrounded by a hypopigmented halo
Exam: Systemic
Flu-like prodrome
Imaging
OCT : Dome-shaped hyperreflective lesion of the photoreceptors and outer retina with disruption of ellipsoid (EZ) and interdigitation (IZ) zones; upward displacement of the external limiting membrane (ELM); lesions normalize with disease resolution starting with ELM, then EZ, then IZ
FAF: slight increase in hyperfluorescence within the fovea and the areas corresponding to whitish dots (acute lesions) seen on examination
FA: transmission window defects within lesion without leakage with central blocking lesions corresponding to hyperpigmentation
ICG: mid-to-late phase hyperfluorescence of the lesion
VF: decreased threshold sensitivity within central field
mfERG: depressed central amplitudes
Laboratory and Radiographic Testing
No contributory testing
Differential Diagnosis
Multiple evanescent white dot syndrome (MEWDS)
Acute macular neuroretinopathy (AMN)
Unilateral acute idiopathic maculopathy (UAIM)
Solar retinopathy
Treatment
No treatment necessary, self-limited
Visual acuity returns to baseline within 2 months in 89%
Notably, oral steroids have been shown to slow recovery
Referral/Comanagement
None