A White woman in her 40s was referred to an outside ophthalmologist after developing nausea and a headache following a dilated eye examination. She was found to have narrow angles in both eyes and a laser peripheral iridotomy (LPI) was performed in her left eye. Two weeks later she developed flickering photopsias and a temporal scotoma progressing to a “brown tint” of her vision in her left eye. Her vision had decreased in her left eye and macular and optic nerve edema were noted and she was subsequently referred to us. In our clinic, her visual acuity was 20/20 OD and 20/50 OS, and she had normal intraocular pressure, a shallow anterior chamber but open angle in both eyes, a patent LPI in the left eye, and clear crystalline lenses in both eyes. The rest of her anterior segment was unremarkable. The posterior examination was notable for shifting subretinal fluid with choroidal detachments without retinal breaks, inflammation, or optic nerve edema and leopard spotting, while her right eye was unremarkable (Figure, A). Fluorescein angiography was unrevealing, while optical coherence tomography showed shallow subretinal fluid and an irregular and thickened choroid (Figure, B). Ultrasonography showed an axial length of 20.0 mm, thickened sclera, anteriorly rotated ciliary body, and 360° of ciliochoroidal detachments in both eyes but worse in the left eye. Results of a systemic laboratory evaluation for infectious and noninfectious disorders were unremarkable. Systemic corticosteroids failed to resolve the subretinal fluid and her vision declined.
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Uveal effusion syndrome
C. Sclerectomy in the left eye
Despite high-dose corticosteroids, vision continued to decline and the choroidal effusions and retinal detachment enlarged in the left eye making a subtle, underlying inflammatory-driven disorder unlikely. With small effusions and shallow anterior chamber in the right eye as well, her presentation was unlikely related to the LPI alone. Prolonged hypotony, malignant hypertension, underlying causes of elevated uveal venous pressure (eg, pulmonary hypertension, arteriovenous fistulas, and Sturge-Weber syndrome), uveal effusion syndrome, and several neoplastic conditions are other underlying etiologies of choroidal effusions. However, this patient’s blood pressure had been within normal limits at multiple measurements, she had no known underlying systemic diseases with an unremarkable echocardiogram and pulmonary function test results, and a thorough review of systems did not raise concern for cancer. Thus, this patient’s presentation was most consistent with idiopathic uveal effusion syndrome. On follow-up, the patient’s intraocular pressure had remained within normal ranges making a repeated LPI in the left eye incorrect as she had not developed angle closure (choice A). Furthermore, her current condition had likely been exacerbated by the original LPI. The response in the left eye also would make choice B potentially dangerous because of concern of instigating a similar set of events in the right eye and not solve the worsening issues within the left eye. While a clear lens exchange (choice D) is an effective solution to narrow angles and a shallow anterior chamber, surgical complications are common in eyes with abnormal sclera.1,2 Further, choice D would only address a by-product of the underlying process, narrow angles, and not the disease itself but incur substantial surgical risk making it incorrect.3 While a recent randomized trial showed that cataract surgery with concurrent sclerostomy reduced perioperative complications in nanophthalmic eyes including the development of choroidal effusions, the surgeries were performed in eyes that did not already have choroidal effusions.3 There are 3 subtypes of uveal effusion syndrome.4 Nanophthalmic eyes with hyperopia (type I) and eyes with histologically abnormal sclera but otherwise normal (type II) respond to sclerectomies; however, otherwise normal eyes (type III) do not respond to sclerectomy.4,5 This patient with type II idiopathic uveal effusion syndrome needed the underlying pathophysiology addressed, her abnormal and thick sclera, with a 4-quadrant nearly full-thickness sclerectomy (choice C).6 The choroidal effusions and serous detachment improved following the procedure but did not fully resolve. Thus, a second 4-quadrant sclerectomy with 2 full-thickness sclerostomies were performed resulting in complete resolution of the choroidal effusions and continued improvement in the serous retinal detachment.
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Christopher D. Conrady, MD, PhD, Department of Ophthalmology, University of Nebraska Medical Center, 3902 Leavenworth St, Omaha, NE 68105 (firstname.lastname@example.org).
Published Online: February 10, 2022. doi:10.1001/jamaophthalmol.2021.4988
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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