Retinal Detachment in Uveitis

Retinal detachments (RD) may be tractional (TRD),^1,2 rhegmatogenous (RRD),^3–9 or exudative—also referred to as serous retinal detachments (SRD).^10–12 Although well-recognized in patients with uveitis, each form of RD affects fewer than 5% of eyes with active inflammation seen at tertiary referral centers, with higher rates observed in some settings and subpopulations. While TRD is seen most typically in eyes with chronic non-infectious vitreous inflammation in the setting of vitreomacular interface abnormalities^13,14 and RRD occurs most often in eyes with infectious retinitis,^15–17 one or more SRD may develop in the setting of both infectious and non-infectious uveitis, by far the most frequent of which is VKH disease.^10–12 Of note, chronic RRD itself can present with ocular inflammation that may mimic intermediate or panuveitis, a presentation often associated with acutely elevated intraocular pressure^18–20 that has come to be known as Schwartz-Matsuo syndrome.^21,22 This issue of Ocular Immunology and Inflammation contains three reports^23–25 related to the occurrence of RD in eyes with uveitis.

Lee and Kim²³ described a 48-year-old Asian woman from South Korea who presented with SRDs, headache, and tinnitus, all suggestive of VKH disease. The presence of myalgias and high fever at the onset of symptoms lead the authors to perform computerized tomography (CT) of the abdomen and pelvis, which revealed a liver mass associated with lymphadenopathy, directed biopsy of which confirmed the diagnosis of systemic diffuse large B-cell lymphoma (DLBCL) with secondary choroidal involvement. Systemic chemotherapy resulted in complete resolution of the SRDs with return of good vision in each eye. The authors summarized the few previously reported cases of choroidal lymphoma mimicking VKH disease and reminded us that while rare, such mimics should be considered when patients present with signs or symptoms atypical for VKH disease, such as fever and myalgias, or when there is an incomplete or temporary response to what should be a therapeutic dose of systemic corticosteroids. Detailed multimodal imaging (MMI) may also reveal atypical features, such as the sharply demarcated choroidal lesions observed on indocyanine green angiography in this patient²³ and reported by others.²⁶

Deb et al²⁴ described a 43-year-old Asian Indian woman from South India who presented with a unilateral SRD in the setting of diffuse unilateral subacute neuroretinitis (DUSN) associated with multifocal choroiditis. Vision was dramatically decreased at 20/1200, or counting fingers. Optical coherence tomography confirmed the presence of a macular SRD with thickening of the underlying choroid and fluorescein angiography revealed multiple leaks at the level of the retinal pigment epithelium (RPE). While a diagnosis of VKH disease was considered at presentation, follow-up examination revealed a live, mobile, subretinal nematode, confirming the diagnosis of DUSN. The patient improved after laser photocoagulation of the worm followed by 1 month treatment with oral corticosteroids and albendazole. As with the DLBCL case above, clinical features atypical for VKH disease were noted, including unilateral involvement—rare in VKH disease,²⁷ and vision loss greater than clinical findings might support—a characteristic feature of DUSN,^28,29 which was once referred to as “unilateral wipe-out syndrome.”³⁰ In fact, early mis-diagnosis and delayed confirmation of DUSN are common, both because the characteristic deep retinochoroidal lesions may be transient and since physically locating the worm on clinical examination can be a tedious and time-consuming task requiring many visits.³¹

Ventakesh et al²⁵ reported two patients from South India, each of whom presented with unilateral acute idiopathic maculopathy (UAIM) associated with an overlying, localized bacillary layer detachment (BLD),^32,33 wherein rapid sheering of the photoreceptor layer between myoid and ellipsoid components allows the outer-most portion of the photoreceptors to remain attached to RPE at the base or floor of an acute SRD. The authors noted the growing list of infectious and inflammatory conditions now reported to be associated with BLD.

Together, these findings expand our understanding of the causes and characteristics of SRDs in eyes with uveitis. They also support the taylored or targeted search for causes beyond the most common infectious and inflammatory conditions, particularly when the clinical presentation and/or fundus features are atypical. A detailed review of systems, careful and complete eye examination, MMI, and selected use of systemic testing and imaging can help avoid delayed or mis-diagnosis.