Uveal effusion syndrome (UES) is a diagnosis of exclusion. Patients present with idiopathic choroidal swelling and exudative retinal detachments without clinical findings of hypotony or inflammation. The syndrome is also known to occur in nanophthalmic eyes. UES is believed to be associated with an impedance of transscleral flow of protein and fluid.1,2 The accumulation of submacular fluid is common and is often the main cause of vision loss. Due to the recalcitrant nature of this fluid, surgery is frequently performed to hasten the resolution of swelling and restore vision. Rarely, patients with UES may present with concomitant optic neuropathy. We present a case of nanophthalmic UES and optic neuropathy treated with early sclerectomies.
A 48-year-old man presented with a 2-week history of painless, progressive vision loss in the left eye. His ocular history was significant for cataract surgery in the left eye 5 months prior to presentation. His medical history was noncontributory to his presentation.
On presentation, best corrected visual acuity was 20/20 in the right eye and 20/400 in the left. IOP was 18 mm Hg and 13 mm Hg in the right and left eyes, respectively. A mild relative afferent papillary defect was noted in the left eye. Anterior segment examination revealed shallow anterior chambers in both eyes and a well-positioned posterior-chamber IOL in the left eye. Posterior-segment examination of the right eye was unremarkable; however, the left eye showed a swollen, hyperemic optic nerve, retinal folds within the macula, and 360° of annular choroidal effusions (Figure 1A). B-scan ultrasonography showed large hypoechoic choroidal effusions without evidence of a choroidal mass lesion (Figure 1B). Of note, the axial length was 15.76 mm in the left eye. An enlarged blind spot and peripheral field constriction were evident on Goldmann perimetry (Figure 1C).
(A) Preoperative color fundus photo, (B) B scan (transverse 6:00), and (C) Goldmann visual field of left eye showing 360° of choroidal detachment, optic nerve edema, macular folds, choroidal detachments, and enlarged blind spot and peripheral constriction. (D) Postoperative fundus photo, (E) B scan (transverse 6:00), and (F) Goldmann visual field show resolution of choroidal detachments and improvement in the patient’s visual field defects.
The patient was administered oral prednisone (80 mg/day) for 3 days without improvement in visual acuity or examination. Fifty percent partial-thickness sclerectomies (4 × 4 mm) were created in all four quadrants and 1-mm full-thickness sclerotomies in the center of each inferior window. One month postoperatively, BCVA was 20/50, with flattening of the choroidal effusions (Figure 2A–B), improved Goldmann visual field (Figure 2C) decreased optic nerve edema, and the resolution of the relative afferent papillary defect. At 3 months, the choroidal effusions recurred, optic nerve edema increased, and visual acuity declined to 20/100. Sclerectomies were enlarged to 6 × 10 mm. Three months after the second surgery, the choroidal effusions were noted to have flattened, but visual acuity remained 20/150.
(A) Preoperative macular OCT demonstrating intraretinal and subretinal fluid extending from the optic nerve with foveal detachment. (B) Postoperative OCT with resolution of fluid and improvement in foveal contour.
Uveal effusion syndrome is a diagnosis of exclusion that can be considered after other common causes of choroidal effusion including hypotony, inflammation, malignancy, trauma, and certain medications have been ruled out. Nanophthalmic uveal effusion syndrome is a well-described entity. In a healthy patient, the choroid removes fluid and protein by means of the vortex veins, transscleral hydrostatic diffusion, and via flow through scleral emissary channels (vessels and nerves). Scleral tissue is composed of collagen and elastic fibers with interspersed proteoglycans and is readily permeable to passage of proteins and water. Patients with nanophthalmos and UES frequently have increased scleral thickness, an irregular arrangement of collagen fibers, as well as increased deposition of proteoglycans.1,2 Increased scleral thickness may lead to compression of the vortex veins, thus reducing hydraulic conductivity. Impedance of macromolecule permeability leads to accumulation of suprachoroidal protein, with a subsequent increase in osmotic gradient further favoring fluid accumulation.3,4
Clinical findings in patients with UES frequently include annular choroidal effusions. A remitting and relapsing course is common for UES. The presence of submacular fluid is the leading cause of acute and chronic vision loss and often the indication for surgical intervention. Surgical techniques include vortex vein decompression, although the most common surgical intervention currently is formation of scleral windows to decrease the outflow resistance of choroidal fluid.
Optic neuropathy is a rare complication of UES. Previous case reports have suggested that optic neuropathy in UES is a result of compressive forces around the optic nerve.5 In the case described here, following partial-thickness sclerectomies, there was an improvement in the height of the uveal effusions, a notable decrease in optic nerve edema, a reversal of the relative afferent papillary defect, and a dramatic improvement in visual acuity and visual field. The responsiveness of the nerve edema and visual field defect to surgery are highly suggestive of a compressive optic neuropathy. Compressive optic neuropathies have also been described in patients with thyroid eye disease as a result of crowding in the orbital apex leading to reduced axonal conduction along the optic nerve and congestion of its vascular supply. Urgent decompression of the tissue around the optic nerve may result in remission of the optic neuropathy and associated visual dysfunction.6,7 Compressive optic neuropathy can lead to permanent neuropathy due to the chronic reduction in axoplasmic flow or due to secondary ischemia as a result of mechanical forces on blood vessels supplying the optic nerve.8 In cases of UES with associated optic neuropathy, it appears that prompt surgical management may decrease optic nerve edema and reverse visual field defects. Patients must be closely monitored for recurrences, and final visual prognosis may still be poor due to the relapsing and chronic nature of the disease. Unfortunately, the choroidal effusions in our patient recurred, as is typical in patients with UES. Despite anatomic correction of choroidal effusions after a second surgery, visual acuity and visual field did not improve, suggesting his optic nerve may have sustained permanent damage from recurrent compression and/or secondary ischemia.
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