Patient referred for long-term blurry but stable vision

Bilateral, symmetric macular choroidal folds were noted on dilated fundus exam.

A 29-year-old white man was referred to the New England Eye Center for evaluation of bilateral macular pathology.

The patient said his vision had been blurry over the past 4 years. He remembered the vision abruptly changing, but it had been somewhat stable since that time. However, the vision had not returned to normal. The patient denied headache, use of steroids, metamorphopsia, photopsias or neurologic symptoms.

The patient’s review of systems was unremarkable. His ocular history was also unremarkable. The patient said he was healthy and was not currently taking any prescription medications or eye drops. He denied smoking and drinking.

Examination

On examination, the patient’s best corrected visual acuity was 20/30 in each eye. Refraction was –0.75 sphere in the right eye and –0.25 sphere in the left eye. IOP was 11 mm Hg in each eye. Extraocular motility, confrontation fields, color, pupils and anterior segment examination were normal in both eyes.

Dilated fundus examination was notable for bilateral, symmetric macular choroidal folds (Figure 1). There were no ciliochoroidal detachments in either eye. There was no disc hemorrhage, cotton wool spots, vascular changes, or disc pallor or swelling.

Optical coherence tomography highlighted the choroidal folds with retinal pigment epithelium undulations in both eyes (Figure 2). Also, the overall thickness of both retinas, primarily in the outer retina, was elevated (Figure 3).

Given these macular findings, fluorescein angiography was ordered. The fluorescein angiography did not reveal any vasculitis or any points of leakage from the choroid or retina. B-scan ultrasonography did not show any “T-sign” or obvious scleral thickening. Measurements for axial length were within normal range, approximately 22 mm in both eyes.

What is your diagnosis?

Macular pathology

The findings of bilateral macular choroidal folds without any ocular inflammation, choroidal leakage or retinal leakage is most consistent with a mild case of idiopathic uveal effusion syndrome. Classically, idiopathic uveal effusion syndrome is characterized by non-rhegmatogenous retina detachments, ciliochoroidal detachments, normal IOP and an absence of ocular inflammation. Other findings include retinal pigment epithelium hypertrophy leading to the “leopard” fundus changes.

The differential diagnosis for a young male patient with bilateral choroidal folds and macular thickening without any ocular inflammation is challenging. Post-surgical or traumatic changes can certainly lead the eye to take on a hypotonus state, leading to choroidal folds. However, this patient had no history of either. Another cause would be any space-occupying lesion within the orbit (eg, orbital hemangioma). These classically push the globe anteriorly, creating a hyperopic shift. However, the patient’s B-scan was normal. These choroidal changes could have also represented burnt-out bilateral scleritis; however, there was no significant systemic or ocular history similar to this.

The most classic cause of uveal effusion syndrome is nanophthalmic eyes. However, our patient displayed axial lengths within normal range.

Discussion

Idiopathic uveal effusion syndrome is a rare but well-recognized condition first formally described in 1963. Patients are typically middle-aged men, although women are also implicated, and the age range can be quite wide. It usually presents unilaterally.

There are two groups of patients with idiopathic uveal effusion syndrome. They both have marked thickening of the sclera. This can be demonstrated on either MRI or at the time of surgery, and the sclera can be thickened up to 2 mm. Type 1 patients have axial lengths less then 19 mm. Type 2 patients have axial lengths within normal limits or slightly small, around 21 mm. Both types have abnormal histology of the sclera and respond well to surgical intervention.

Histochemically, the sclera has thickened fibers, deposition of glycosaminoglycans between the fibers and overall disruption of the normal lamellar arrangement of the sclera. These changes decrease the permeability of protein transscleral outflow and increase the congestion of the vortex veins. The congested vortex veins lead to extravascular leakage from choroidal vessels. The decreased transscleral flow of proteins leads to more proteins in the choroid and subsequent fluid accumulation in the choroid, which is seen clinically as ciliochoroidal detachments. With an overall increase of fluid in the choroid, the retinal pigment epithelium begins to lose its pump ability, leading to subretinal fluid accumulation.

Therapy for idiopathic uveal effusion syndrome focuses on creating an efflux for the congested choroid through partial thickness quadrantic sclerectomies, with or without sclerotomies near the equator of the globe without vortex vein decompression. Given this patient’s well-preserved visual acuity, it was elected to closely observe him.

References:

• Gass JD. Uveal effusion syndrome: a new hypothesis concerning pathogenesis and technique of surgical treatment. Trans Am Ophthalmol Soc. 1983;81:246-260.
• Gass JD, Jallow S. Idiopathic serous detachment of the choroids, ciliary body, and retina (uveal effusion syndrome). Ophthalmology. 1982;89(9):1018-1032.
• Johnson MW, Gass JD. Surgical management of the idiopathic uveal effusion syndrome. Ophthalmology. 1990;97(6):778-785.
• Uyama M, Takahashi K,. Kozaki J, et al. Uveal effusion syndrome: clinical features, surgical treatment, histologic examination of the sclera, and pathophysiology. Ophthalmology. 2000;107(3):441-449.

• Vivek Chaturvedi, MD, and Jay S. Duker, MD, can be reached at Tufts Medical Center, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.

• Edited by Jeffrey Chang, MD, and Vivek Chaturvedi, MD. Drs. Chang and Chaturvedi can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Chang and Chaturvedi have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.