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Healthy middle-aged man experiences ‘curtain’ in vision of right eye
A funduscopic exam revealed a serous retinal detachment and choroidal effusions.
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A 54-year-old man was referred to the Lahey Clinic by an outside ophthalmologist for a retinal detachment of the right eye. The patient described a gradual onset of a “black curtain” over the superior part of his visual field in the right eye for 1 month. The vision loss was not preceded by flashes or floaters. He denied pain or redness in the eye and did not have a headache. A complete review of systems was otherwise negative.
The patient’s ocular history was remarkable for recent peripheral laser iridotomies in both eyes for anatomical narrow angles, hyperopia and mild cataracts. His medical history included hypertension controlled on hydrochlorothiazide. There was no family ocular history. He was a nonsmoker and denied alcohol or illicit drug use.
Examination
On examination, the patient’s best corrected visual acuity was 20/400 in the right eye and 20/20 in the left eye. Refractive error at distance was +4.75 × –1.00 @ 090 in the right eye and +3.50 × –0.75 @ 081 in the left eye. The pupil was sluggishly reactive to light in the right eye, but no definite afferent pupillary defect was seen. Confrontational visual fields showed a superior visual field defect in the right eye and full field in the left eye. There was right exotropia on primary gaze, but extraocular movements were full. IOP was 15 mm Hg in both eyes.
Anterior segment exam showed a shallow anterior segment with 1 to 2+ pigment on the anterior capsule of the lens, 2 to 3+ pigment in the anterior vitreous, patent laser iridotomies and 2+ nuclear sclerosis in both eyes. On gonioscopy, the right eye had a steep configuration to the iris, and no angle structures could be seen. In the left eye, the angle was shallow but open. On posterior segment examination of the right eye, there was an inferior, macula-off, bullous retinal detachment with shifting fluid. There were choroidal effusions in the anterior periphery for 360° (Figure 1). No retinal tears could be found. The fundus in the left eye was flat with a large temporal congenital hypertrophy of the retinal pigment epithelium.
B-scan of the right eye further revealed probable scleral thickening but no masses. The retinal detachment and anterior choroidal effusions were visualized (Figure 2). Axial lengths were measured by A-scan and found to be 20.87 mm in the right eye and 21.60 mm in the left eye.
Figure 1. Fundus photograph demonstrating a macula-off inferior retinal detachment with shifting fluid.
Images: Bartolini CE, Blaha GR
Figure 2. B-scan of the right eye showing an inferior retinal detachment and possible scleral thickening.
What is your diagnosis?
Serous detachment
The differential diagnosis for a middle-aged man with a normal IOP, unilateral serous detachment and choroidal effusions includes uveal effusion syndrome, posterior scleritis, uveal melanoma, metastatic tumor, rhegmatogenous retinal detachment with uveal detachment, multifocal choroiditis, severe hypertensive choroidopathy, Vogt-Koyanagi- Harada disease and systemic disease, such as multiple myeloma or myxedema associated with thyroid dysfunction.
Posterior scleritis is usually a manifestation of autoimmune disease; it is painful and more common in women. Neoplasm was excluded with a B-scan negative for any masses. The patient had recently seen his primary care physician, who reported that his blood pressure was under good control, and he was otherwise feeling well, which made a systemic cause unlikely. Vogt-Koyanagi-Harada is typically a bilateral uveitic disease seen in younger, darkly pigmented individuals with possible finding of vitiligo, poliosis, alopecia and dysacousia.
The short axial length of the patient’s eye supported the diagnosis of uveal effusion syndrome, which is often seen in nanophthalmic-like eyes.
Follow-up
The patient was taken to the operating room for scleral windows with scleral cut-down. The scleral flaps were removed and sent to pathology. The diagnosis of uveal effusion syndrome was confirmed by H&E and Alcian blue stain, which showed the characteristic finding of increased mucin between collagen fibers in the scleral stroma.
Three months after surgery, vision was 20/50 in the right eye. On dilated exam, there was only shallow subretinal fluid remaining in the inferior periphery. The choroidal effusions were resolved. The macula was flat.
Discussion
The term uveal effusion, first coined by Schepens and Brockhurst in 1963, refers to a pathoanatomic condition characterized by an abnormal collection of fluid in the suprachoroidal space. There are many causes of uveal effusion, including hypotony, nanophthalmos, trauma, previous ocular surgery, or an underlying inflammatory, infectious, infiltrative or hydrostatic condition. In the absence of a known cause, the term idiopathic uveal effusion syndrome (UES) is used. Idiopathic UES is a rare disease that typically presents in otherwise healthy middle-aged men and is thought to be related to a primary abnormality of the sclera and choroid.
Because it is a rare disease, the true incidence and prevalence of idiopathic UES remain unknown. The pathophysiology of the disease is likely multifactorial. Suggested mechanisms include vortex vein compression, increased choroidal vessel permeability and abnormal accumulation of proteins in the sclera and suprachoroidal space. Interestingly, histologic studies have shown thickened sclera and abnormalities in the scleral composition of these patients, including disorganization of the collagen bundle lamellar arrangement and abnormal deposition of glycosaminoglycans (GAGs) between collagen bundles. This thickened, abnormally composed sclera likely contributes to both compression of the vortex veins and increased resistance to transscleral outflow of protein, leading to accumulation of protein in the suprachoroidal space, which in turn results in reduced movement of fluid from the suprachoroidal space into the choroidal capillaries and causes serous ciliochoroidal effusion. Some authors suggest that aging and hormonal changes may exacerbate the anomalies in protein transport across the sclera.
Idiopathic UES can present in both sexes but is more common in men. It is a bilateral condition in 65% of cases. Unilateral cases tend to present in older men. Patients present with the complaint of decreased vision in one or both eyes. Retinal detachments often result in a central or superior scotoma. Patients often have short axial length and hyperopia. On examination, IOP should be normal. There may be dilated episcleral vessels and anterior chamber flare, but not cell. Gonioscopy can reveal blood in Schlemm’s canal and a narrow anterior chamber angle. Nonpigmented vitreous cells are commonly seen. The retinal detachment may be limited to the macula or involve the inferior retina with shifting fluid when the patient is upright. Choroidal detachments are brown- orange, smooth, dome-shaped elevations located anteriorly and sometimes with an annular arrangement. Unlike retinal detachment, ciliochoroidal detachments do not undulate with ocular movements. The ora serrata is sometimes easily visible secondary to elevation of the pars plana and peripheral choroid. Leopard-spot retinal pigmentary changes can be seen with chronic UES.
Idiopathic UES is a diagnosis of exclusion. Secondary causes of uveal effusion must be ruled out, including ocular hypotony, true nanophthalmos, trauma, surgery, posterior scleritis, a sulfa or carbonic anhydrase inhibitor drug reaction, amyloidosis, Hunter syndrome, arteriovenous fistula, malignant hypertension, metastatic carcinoma, lymphoproliferative choroidal infiltration or uveal melanoma. B-scan ultrasonography can rule out a neoplasm as well as demonstrate the retinal and choroidal detachments and thickening of the choroidal-scleral layer typical of UES. Nanophthalmos is ruled out using A-scan ultrasonography to demonstrate a shortened axial length, typically less than 20 mm. In addition, patients with nanophthalmos have more than 7 D of hyperopia and may have a small corneal diameter, a shallow anterior chamber and a high lens-to-eye volume ratio. Scleral tissue can be sent for pathology, which will show the characteristic disarray of collagen fibers, variation in the diameter of the fibers and increased extracellular matrix consisting of accumulated GAGs.
Unlike inflammatory causes of uveal effusion, patients with idiopathic UES respond poorly to nonsurgical treatment, including corticosteroids or antimetabolites. Idiopathic UES can be successfully treated with quadrantic partial-thickness sclerectomies. This surgical procedure was first described by Gass in 1983 and consists of creating 5 mm × 7 mm, one-half to two-thirds thickness sclerectomies in each quadrant, centered 1 mm to 2 mm anterior to the equator. The long axis of the sclerectomy is oriented circumferentially, and a 2-mm linear sclerostomy is made in the center of each sclerectomy bed and enlarged with a 1 mm to 2 mm scleral punch. Approximately half of patients who require surgery in one eye will eventually require surgery in the other eye. Without treatment, a protracted clinical course may occur, with remissions and recurrences occurring over months to years, and may result in a permanent decrease in vision.
Our patient had a shorter than normal axial length. There is significant clinical and histologic overlap between nanophthalmic eyes and hyperopic short eyes with idiopathic UES. On histology in nanophthalmic eyes, Uyama et al found the same pathologic disorganization of scleral fibers and proteoglycan deposits found in idiopathic UES. Although our patient did not have true nanophthalmos, some authors consider idiopathic UES and nanophthalmos to be in the same spectrum of diseases with a congenital primary scleral abnormality.
Conclusion
In summary, a 54-year-old man presented with a painless superior scotoma in his right eye and on examination was found to have ciliochoroidal effusions and a serous retinal detachment with shifting fluid. B-scan ultrasonography revealed the absence of a mass lesion, ciliochoroidal effusions and retinal detachment associated with thickened sclera. The diagnosis of idiopathic uveal effusion syndrome was supported by an A-scan showing a shorter than normal axial length and was confirmed with pathologic analysis of the sclera, which on Alcian blue staining demonstrated increased mucin between collagen fibers. The patient was treated successfully with scleral windows with scleral cut-down.