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.