Rapid resolution of severe exudation in uveal effusion syndrome with anti-vascular endothelial growth factor alone in a case of bilateral nanophthalmos: a case report

Abstract

Background Uveal effusion syndrome is a rare disease characterized by exudative detachments of the choroid, ciliary body, and retina. Various surgical procedures and nonsurgical strategies have been described to treat uveal effusion syndrome with limited success. The treatment for uveal effusion syndrome remains a serious challenge for clinicians. To the best of our knowledge, no previous report has described a severe uveal effusion syndrome patient with nanophthalmos treated by using an anti-vascular endothelial growth factor agent alone. We report here one such case with unexpected positive results. Case presentation A 30-year-old Chinese male patient presented with painless vision loss in both eyes that had persisted for 2 months. Examination of the right eye revealed a best corrected visual acuity of 0.03; the best corrected visual acuity of the left eye was finger count/20 cm. The intraocular pressure was normal on both eyes. A-scan revealed an right eye axial length of 15.88 mm and a left eye axial length of 16.21 mm. In the right eye, half of the peripheral choroid and nearly three-fourths of the retina were detached. The left fundus was not visible because of the total retinal detachment located just behind the lens, which could be clearly observed directly with a slit lamp. Considering all the possibilities and available treatments as well as the patient’s intentions after discussion, we first administered an intravitreal injection of ranibizumab 0.5 ml into both eyes. The patient’s visual perception improved 3 days after the injection. One month later, most of the effusion under the choroid and retina was absorbed. Visual acuity improved from finger count to 0.05 in both eyes, and vision quality was remarkably improved. Encouraged by this good result, the patient opted to undergo a second injection 1 month later. Choroidal and retinal detachment completely vanished 30 days after the second injection. Conclusions Using an anti-vascular endothelial growth factor agent alone may be a potentially effective and safe method for managing some types of uveal effusion syndrome, such as in nanophthalmos. The injection may be administered before considering more aggressive procedures in some uveal effusion syndrome patients.

Clinical Characteristics, Treatment Modalities, and Their Association with Long-term Visual Outcomes in Uveal Effusion Syndrome

“…14,25 In addition, cases of successful UES treatment have been reported after administration of intravitreal anti-VEGF injections. 15,17 This study has several limitations that should be acknowledged. First, it was retrospective in nature, with inherent biases and limitations associated with data collection and analysis.…”

“…4,9,10 Medical treatment, including systemic or periocular corticosteroids, 11 oral carbonic anhydrase inhibitors 12 or nonsteroidal anti-inflammatory drugs, 13 and topical prostaglandin analogues, 14 has yielded variable results, demonstrating significant efficacy in some cases, while resulting in equivocal outcomes in others. Recently, the use of intravitreal antivascular endothelial growth factor (VEGF) agents alone 15 or in combination with surgical intervention 16,17 has been reported as a treatment option in a limited number of UES cases. Nonetheless, owing to the rarity of UES, few studies have comprehensively reported its long-term prognosis and associated factors.…”

“…14,25 In addition, cases of successful UES treatment have been reported after administration of intravitreal anti-VEGF injections. 15,17…”

Surgical Approaches to Serous Retinal Detachment With Retina–Lens Touch in Eyes With Nanophthalmos

“…There are anecdotal reports of the use of medical therapy for exudative RD in nanophthalmos, such as corticosteroids, 29 nonsteroidal anti-inflammatory drugs, 6,30 and intravitreal vascular endothelial growth factor. 31 Medical therapy with oral corticosteroids in uveal effusion syndrome is well established but was deemed ineffective in nanophthalmos. 10,11 Elgin et al 9 described a patient with nanophthalmos and mild exudative RD that resolved rapidly after administration of the topical corticosteroid alone.…”

Medical Therapy and Scleral Windows for Uveal Effusion Syndrome: A Case Series and Literature Review

“…The molecular target is vascular endothelial growth factor, which upregulates new blood vessel growth, and pathological vasculature formation. While the exact therapeutic mechanism of anti-VEGF agents in UES remains unclear, Guo et al reported resolution of three intractable cases of UES, and Song et al reported success in one case of nanophthalmic UES [ 71 , 72 ]. One hypothesis is that the increased expression of interleukin (IL)-6, IL-8, and VEGF in UES affects vessel permeability and choroidal congestion.…”