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Man complains of chronic distorted vision in both eyes
Choroidal folds were seen in both maculae, and an exudative retinal detachment was seen in the right eye.
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A 30-year-old man presented with the complaint of gradually increasing bilateral distorted vision, worse in the right eye. He had a history of intermittent blurry vision; the first episode occurred 5 years ago when he was in the Army, with spontaneous resolution. He never saw an eye doctor during the episodes. He denied other systemic complaints.
He had no significant medical history and was not taking any medications. His family history was noncontributory. He used occasional alcohol but denied smoking or illicit drug use.
Examination
On examination, the patient’s best corrected visual acuity was 20/60 in the right eye, with myopic correction to 20/30, and 20/30 in the left eye. The pupillary response was normal in both eyes, without an afferent pupillary defect. IOP by applanation was 11 mm Hg in both eyes. Confrontation visual field testing was within normal limits, and extraocular motions were full bilaterally.
Anterior segment exam of both eyes was unremarkable. Posterior segment exam revealed a clear media and normal-appearing optic nerves. Choroidal folds were seen in both maculae. There were no signs of intraocular inflammation or masses. Mild retinal pigment epithelium changes were observed peripherally in both eyes. A small exudative retinal detachment with shifting fluid was found inferiorly in the right eye. No retinal detachment was observed in the left eye (Figure 1).
Figure 1. Fundus photography at presentation. Right eye (a) and left eye (b).
Images: Ho J, Duker JS
What is your diagnosis?
Fundus findings
The differential diagnosis for the fundus findings includes rhegmatogenous retinal detachment, intraocular mass with secondary retinal detachment, central serous chorioretinopathy, uveal effusion syndrome, posterior scleritis, hypotony maculopathy and nanophthalmos.
Differential diagnosis
Uveal effusion syndrome is a rare disorder that can cause a relapsing-remitting course of vision loss. It is classified into a nanophthalmic type, which is present in individuals who have an axial length smaller than 21 mm and high hyperopia greater than 8 D, and an idiopathic type, which is present in patients with non-nanophthalmic axial lengths. Examination reveals choroidal detachments and exudative retinal detachments in the setting of a normal IOP and an absence of intraocular inflammation. Chronic subretinal fluid leads to retinal pigment epithelium (RPE) changes described as a “leopard spot” fundus. Fluorescein angiography (FA) may show multiple areas of early choroidal hyperfluorescence corresponding to the leopard spots, with some later coalescence of the hyperfluorescence, in the absence of leakage of fluorescein from the choroid into the subretinal space. Optical coherence tomography imaging demonstrates choroidal folds and, in some instances, subretinal fluid if it is close to the macula.
Central serous chorioretinopathy (CSCR) is a condition associated with serous detachments over single or multiple leaks through the RPE. Often times the leak could be observed occurring close to pigment epithelial detachments. It occurs predominantly in men, with an average age of onset between 30 years and 50 years. FA generally shows pooling of the fluorescein into the subretinal space. While the prognosis of the condition is usually good, a small percentage of patients go on to develop chronic CSCR, with multiple RPE detachments, widespread pigmentary disturbances and inferior serous detachments. On OCT, in addition to the presence of subretinal fluid, the choroid often shows increased thickness on enhanced depth imaging.
Posterior scleritis is defined as uveoscleral inflammation centered behind the equator. Symptoms may include blurry vision, ocular pain and photophobia. Rarely, it presents without symptoms as an isolated mass. In one series by McCluskey et al, 29% of the cases were associated with autoimmune disease, lymphoma or systemic vasculitis. Examination typically reveals exudative retinal detachment, choroidal detachments, optic disc swelling, subretinal exudates or hemorrhage. FA classically shows areas of subretinal leakage that gradually increases in intensity. B-scan ultrasonography often shows a classic “T sign” with a dark area behind Tenon’s capsule, representing fluid.
Hypotony maculopathy may be the result of trauma, ocular inflammation or a postoperative state, especially after glaucoma filtration surgery. IOP is low, usually less than 6.5 mm Hg. Fundus findings may include chorioretinal folds, optic nerve edema and tortuosity of the retinal vasculature. FA shows only mildly increased choroidal hyperfluorescence at the crest of choroidal folds from RPE thinning and hypofluorescence at the troughs.
Diagnosis and management
Red-free photography of the posterior segment showed linear radiations outward from the fovea corresponding to choroidal folds in the right eye (Figure 2a). FA in the right eye showed early patchy hypofluorescence (Figure 2b), followed by spots of parafoveal hyperfluorescence (Figure 2c), which coalesced and decreased in intensity without evidence of dye leakage or pooling throughout the angiogram (Figure 2d). Cirrus HD-OCT (Carl Zeiss Meditec) raster scan of the right eye demonstrated retinal wrinkling from chorioretinal folding, with a small amount of subretinal fluid nasally (Figure 3). B-scan ultrasound showed diffuse uniform choroidal thickening with an axial length of 22.5 mm without a “T sign” in either eye.
Figure 2. Red-free photography (a). Early-phase fluorescein angiography (b). Mid-phase fluorescein angiography (c). Late-phase fluorescein angiography (d).
Figure 3. Spectral-domain OCT raster scan of the right eye. OCT en face reconstruction using the internal limiting membrane slab (a). Horizontal raster scan through the fovea (b).
The presence of uveal effusions and serous retinal detachment, in the setting of normal IOP, absence of intraocular inflammation and absence of leakage on FA in a non-nanophthalmic patient is consistent with the diagnosis of idiopathic uveal effusion syndrome. Chronic CSCR is a suggestive diagnosis, given the patient’s presentation and age. However, it is less likely given the absence of fluid leakage on FA. Posterior scleritis is less likely, given the absence of ocular inflammation, eye discomfort and associated FA findings. There was no evidence, such as trauma or glaucoma surgery, to suggest hypotony maculopathy. There were no retinal tears or breaks found to suggest rhegmatogenous retinal detachment. Lastly, there were no intraocular masses on exam to suggest secondary exudative retinal detachment.
The options of observation, a short trial of oral prednisone and scleral windows were discussed with the patient. The patient wanted to first proceed with oral prednisone (80 mg daily). On follow-up 1 month later, there was no significant reduction in the amount of serous fluid. Subsequently, scleral windows consisting of two 6 mm × 2 mm partial thickness sclerectomies in the inferonasal and inferotemporal quadrants at the equator were performed. Histopathologic studies of the removed sclera showed irregularity in arrangement and widths of collagen bundles and marked deposition of glycosaminoglycan between bundles on alcian blue staining (Figure 4).
Figure 4. Histopathology in uveal effusion syndrome.
Figure 5. Color fundus mosaic after scleral window creation in the right eye.
Initially, the serous retinal detachment in the right eye improved and decreased down to 50%. However, 4 months later, the detachment increased, along with the presence of shallow subfoveal subretinal fluid. A second operation, consisting of two additional scleral windows with partial thickness sclerectomies in the superior nasal and temporal quadrants at the equator, followed by 2-mm radial extension of the existing scleral windows and dissection down to 80% to 90% of the scleral depth, was performed in the right eye. The serous detachment resolved completely following the second procedure and has not recurred during a 3-year follow-up interval. Moreover, visual acuity remained at 20/20. Fundus exam showed marked central and peripheral RPE changes (Figure 5).
The left eye developed an inferior serous retinal detachment 2 years later and underwent a scleral window procedure in all four quadrants, similar to the technique described above. After an initial period of complete resolution of the subretinal fluid lasting 14 months, it recurred. Repeat surgical intervention is currently being considered in the left eye.
Discussion
Uveal effusion syndrome was first described by Schepens and Brockhurst in 1963 as a condition that causes a relapsing-remitting course of vision loss associated with exudative retinal and choroidal detachments. Bilateral involvement is found in two-thirds of the cases. There is a male predominance with typical age of onset occurring during middle age. The underlying pathogenesis of the condition is hypothesized to be secondary to an abnormally thickened sclera leading to decreased transscleral outflow, in addition to increased congestion of the vortex vein. The increases in osmotic and hydrostatic forces lead to an accumulation of fluid in the choroid, resulting in choroidal detachments and subsequently pump dysfunction in the RPE with accumulation of subretinal fluid.
The current standard treatment for uveal effusion syndrome is surgical intervention including the creation of scleral windows. Gass et al described a technique utilizing 5 mm × 7 mm partial thickness sclerectomies without a flap made at 1 mm to 2 mm anterior to the equator in all four quadrants. Then 2-mm linear sclerotomies were made at the center of sclerectomies. Uyama et al performed scleral windows using subscleral sclerectomies. They created two-thirds thickness scleral flaps measuring 4 mm × 5 mm at the equator in the inferonasal and temporal quadrants. Sclerectomies were then performed down to the choroid. Finally, the wound edges were cauterized and the flaps were loosely sutured.
The prognosis after surgery, defined as the resolution of subretinal fluid, ranges from 50% to 85% after one procedure. In a case series by Johnson et al, almost a quarter of eyes developed recurrent disease after formation of scleral windows. Involvement in the fellow eye occurred in about two-thirds of patients. Fifty-six percent of patients experienced a two line or more improvement in visual acuity, while 35% demonstrated stable visual acuity, and 9% had reduced visual acuity at 6 months.
In conclusion, uveal effusion syndrome is a rare disorder that presents with a relapsing-remitting course of choroidal and retinal detachments. It serves as a challenge both diagnostically and therapeutically.