Disease of the Year: Differential Diagnosis of Uveitic Macular Edema

Abstract

Uveitic cystoid macular edema (UME) is an important cause of visual morbidity among patients with both infectious and non-infectious uveitis. UME may be associated in more than 30% cases of active uveitis. However, even patients with minimal features of intraocular inflammation may develop recurrent or chronic UME. Therefore, the evaluation and management of UME in patients with uveitis may be challenging. A number of vitreoretinal pathologies may result in UME and accumulation of fluid in the intra-or subretinal space. These need to be carefully distinguished from each other so that appropriate management can be initiated. All types of uveitis, including anterior uveitis (where the primary site of inflammation is not in the posterior segment) can present with UME. Other conditions such as diabetes, and surgery, can present with macular edema. This index review highlights various differential diagnoses of UME and provides illustrative case examples with multimodal imaging evaluation.

Should all macular edema be treated with intravitreal injection? Importance of fundus fluorescein angiography in macular edema

“…[7] OCT has advantages to evaluate the vitreomacular interphase, while FFA has the advantage to evaluate the macular perfusion and involvement of central and peripheral retinal vasculature and optic disc. [5] In both of our cases we present, the misdiagnosis was caused by the lack of proper use of appropriate multimodal imaging techniques as both patients had no FFA images taken before administration to our clinic. ME is an entity that can occur as a result of a wide range of uveitic and non-uveitic diseases leading to breakdown of the bloodretinal barrier, increased inflammatory mediators, vascular compromise/hyperpermeability, or dysfunction of the retinal pigment epithelial pump mechanism.…”

“…Uveitic cystoid ME is a common cause of visual morbidity and can lead to substantial sight-threatening visual loss in more than 30% of uveitis patients. [3][4][5] It is thought to be caused by fluid leakage over the blood-retinal barrier and build-up in the macular area, with a distinctive distribution in the outer plexiform layer and subretinal area. This results in macular thickening and central visual disturbance.…”

“…[2] Chronic and persistent uveitic ME can result in permanent visual impairment by causing photoreceptor and retina pigment epithelial cell damage and macular ischemia. [3,5,6] ME can be detected easily by macular OCT imaging. However, OCT alone is not enough to determine the underlying cause.…”

“…ME is an entity that can occur as a result of a wide range of uveitic and non-uveitic diseases leading to breakdown of the bloodretinal barrier, increased inflammatory mediators, vascular compromise/hyperpermeability, or dysfunction of the retinal pigment epithelial pump mechanism. [5] Uveitic ME is most commonly associated with noninfectious causes of posterior, intermediate, and panuveitis including HLA B27-positive anterior uveitis, juvenile idiopathic arthritis, sarcoidosis, multiple sclerosis, and pars-planitis, Behcet's disease, HLA-A29 positive Birdshot retinochoroidopathy, sympathic ophthalmia, and also infectious retinitis. [8] In uveitic ME, the main mechanism is the breakdown of blood-retina barrier due to inflammatory cytokines.…”

Involvement of the systemic microcirculation in pediatric uveitis

“…The following ophthalmological ndings were reported: presence of cystoid macular edema (CME), papillitis, retinal vasculitis and/or retinal capillary leakage on uorescein angiography (FA). CME was de ned as the presence of macular thickening with cyst formation visible on macular OCT and/or FA [19][20][21]. Papillitis was de ned as the presence of optic disc hyper uorescence and/or leakage on FA, in ammatory optic disc swelling, and/or retinal nerve ber layer thickness of > 130 µm on OCT while excluding all other nonin ammatory causes of optic disc swelling [22].…”